HEART AND LUNG SOUNDS

Reading for IVMS Heart and Lung Auscultation Page

Compiled by Marc Imhotep Cray, M.D.

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Contents
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Articles
Heart sounds Heart murmur Aortic valve stenosis Mitral regurgitation Pulmonary valve stenosis Tricuspid insufficiency Atrial septal defect Functional murmur Aortic insufficiency Mitral stenosis Tricuspid valve stenosis Pulmonary valve insufficiency Patent ductus arteriosus Wheeze Stridor Rhonchi Crackles Pertussis 1 7 12 22 30 32 35 44 46 53 59 61 63 68 70 72 73 75

References
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Heart sounds

Heart sounds
Normal heart sounds Normal heart sounds as heard with a stethoscope

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Heart sounds are the noises generated by the beating heart and the resultant flow of blood through it. Specifically, the sounds reflect the turbulence created when the heart valves snap shut. In cardiac auscultation, an examiner may use a stethoscope to listen for these unique and distinct sounds that provide important auditory data regarding the condition of the heart. In healthy adults, there are two normal heart sounds often described as a lub and a dub (or dup), that occur in sequence with each heartbeat. These are the first heart sound (S1) and second heart sound (S2), produced by the closing of the AV valves and semilunar valves, respectively. In addition to these normal sounds, a variety of other sounds may be present including heart murmurs, adventitious sounds, and gallop rhythms S3 and S4.

Heart murmurs are generated by turbulent flow of blood, which may occur inside or outside the heart. Murmurs may be physiological (benign) or pathological (abnormal). Abnormal murmurs can be caused by stenosis restricting the opening of a heart valve, resulting in turbulence as blood flows through it. Abnormal murmurs may also occur with valvular insufficiency (regurgitation), which allows backflow of blood when the incompetent valve closes with only partial effectiveness. Different murmurs are audible in different parts of the cardiac cycle, depending on the cause of the murmur.

Front of thorax, showing surface relations of bones, lungs (purple), pleura (blue), and heart (red outline). The location of best auscultation for each heart valve are labeled with "M", "T", "A", and "P". First heart sound: caused by atrioventricular valves - Mitral (M) and Tricuspid (T). Second heart sound caused by semilunar valves -- Aortic (A) and Pulmonary/Pulmonic (P).

Heart sounds

Primary heart sounds

Normal heart sounds are associated with heart valves closing, causing changes in blood flow.

S1
The first heart tone, or S1, forms the "lub" of "lub-dub" and is composed of components M1 and T1. Normally M1 precedes T1 slightly. It is caused by the sudden block of reverse blood flow due to closure of the atrioventricular valves, i.e. tricuspid and mitral (bicuspid), at the beginning of ventricular contraction, or systole. When the ventricles begin to contract, so do the papillary muscles in each ventricle. The papillary muscles are attached to the tricuspid and mitral valves via chordae tendineae, which bring the cusps or leaflets of the valve closed; the chordae tendineae also prevent the valves from blowing into the atria as ventricular pressure rises due to contraction. The closing of the inlet valves prevents regurgitation of blood from the ventricles back into the atria. The S1 sound results from reverberation within the blood associated with the sudden block of flow reversal by the valves.[1] If M1 occurs slightly after T1, then the patient likely has a dysfunction of conduction of the left side of the heart such as a left bundle branch block.

Diagram showing relations of opened heart to front of thoracic wall. Ant. Anterior segment of tricuspid valve. A O. Aorta. A.P. Anterior papillary muscle. In. Innominate artery. L.C.C. Left common carotid artery. L.S. Left subclavian artery. L.V. Left ventricle. P.A. Pulmonary artery. R.A. Right atrium. R.V. Right ventricle. V.S. Ventricular septum.

S2
The second heart tone, or S2, forms the "dub" of "lub-dub" and is composed of components A2 and P2. Normally A2 precedes P2 especially during inspiration when a split of S2 can be heard. It is caused by the sudden block of reversing blood flow due to closure of the semilunar valves (the aortic valve and pulmonary valve) at the end of ventricular systole and the beginning of ventricular diastole. As the left ventricle empties, its pressure falls below the pressure in the aorta. Aortic blood flow quickly reverses back toward the left ventricle, catching the pocket-like cusps of the aortic valve, and is stopped by aortic valve closure. Similarly, as the pressure in the right ventricle falls below the pressure in the pulmonary artery, the pulmonary valve closes. The S2 sound results from reverberation within the blood associated with the sudden block of flow reversal. Splitting of S2, also known as physiological split, normally occurs during inspiration because the decrease in intrathoracic pressure increases the time needed for pulmonary pressure to exceed that of the right ventricular pressure. A widely split S2 can be associated with several different cardiovascular conditions, including right bundle branch block, pulmonary stenosis, and atrial septal defect.

Heart sounds

Extra heart sounds

The rarer extra heart sounds form gallop rhythms and are heard in both normal and abnormal situations.

S3
Rarely, there may be a third heart sound also called a protodiastolic gallop, ventricular gallop, or informally the "Kentucky" gallop as an onomatopoeic reference to the rhythm and stress of S1 followed by S2 and S3 together (S1=Ken; S2=tuck; S3=y). "lub-dub-ta" or "slosh-ing-in" If new, indicates heart failure or volume overload. It occurs at the beginning of diastole after S2 and is lower in pitch than S1 or S2 as it is not of valvular origin. The third heart sound is benign in youth, some trained athletes, and sometimes in pregnancy but if it re-emerges later in life it may signal cardiac problems, such as a failing left ventricle as in dilated congestive heart failure (CHF). S3 is thought to be caused by the oscillation of blood back and forth between the walls of the ventricles initiated by blood rushing in from the atria. The reason the third heart sound does not occur until the middle third of diastole is probably that during the early part of diastole, the ventricles are not filled sufficiently to create enough tension for reverberation. It may also be a result of tensing of the chordae tendineae during rapid filling and expansion of the ventricle. In other words, an S3 heart sound indicates increased volume of blood within the ventricle. An S3 heart sound is best heard with the bell-side of the stethoscope (used for lower frequency sounds). A left-sided S3 is best heard in the left lateral decubitus position and at the apex of the heart, which is normally located in the 5th left intercostal space at the midclavicular line. A right-sided S3 is best heard at the lower-left sternal border. The way to distinguish between a left and right-sided S3 is to observe whether it increases in intensity with inspiration or expiration. A right-sided S3 will increase on inspiration, while a left-sided S3 will increase on expiration.

S4
S4 when audible in an adult is called a presystolic gallop or atrial gallop. This gallop is produced by the sound of blood being forced into a stiff or hypertrophic ventricle. "ta-lub-dub" or "a-stiff-wall" It is a sign of a pathologic state, usually a failing or hypertrophic left ventricle, as in systemic hypertension, severe valvular aortic stenosis, and hypertrophic cardiomyopathy. The sound occurs just after atrial contraction at the end of diastole and immediately before S1, producing a rhythm sometimes referred to as the "Tennessee" gallop where S4 represents the "Ten-" syllable. It is best heard at the cardiac apex with the patient in the left lateral decubitus position and holding his breath. The combined presence of S3 and S4 is a quadruple gallop, also known as the "Hello-Goodbye" gallop. At rapid heart rates, S3 and S4 may merge to produce a summation gallop, sometimes referred to as S7. Atrial contraction must be present for production of an S4. It is absent in atrial fibrillation and in other rhythms in which atrial contraction does not precede ventricular contraction.

Heart sounds

Murmurs
Heart murmurs are produced as a result of turbulent flow of blood strong enough to produce audible noise. They are usually heard as a whooshing sound. The term murmur only refers to a sound believed to originate within blood flow through or near the heart; rapid blood velocity is necessary to produce a murmur. It should be noted that most heart problems do not produce any murmur and most valve problems also do not produce an audible murmur. Murmurs can be heard in many situations in adults without major congenital heart abnormalities: Regurgitation through the mitral valve is by far the most commonly heard murmur, producing a pansystolic/holosystolic murmur which is sometimes fairly loud to a practiced ear, even though the volume of regurgitant blood flow may be quite small. Yet, though obvious using echocardiography visualization, probably about 20% of cases of mitral regurgitation do not produce an audible murmur. Stenosis of the aortic valve is typically the next most common heart murmur, a systolic ejection murmur. This is more common in older adults or in those individuals having a two, not a three leaflet aortic valve. Regurgitation through the aortic valve, if marked, is sometimes audible to a practiced ear with a high quality, especially electronically amplified, stethoscope. Generally, this is a very rarely heard murmur, even though aortic valve regurgitation is not so rare. Aortic regurgitation, though obvious using echocardiography visualization, usually does not produce an audible murmur. Stenosis of the mitral valve, if severe, also rarely produces an audible, low frequency soft rumbling murmur, best recognized by a practiced ear using a high quality, especially electronically amplified, stethoscope. Other audible murmurs are associated with abnormal openings between the left ventricle and right heart or from the aortic or pulmonary arteries back into a lower pressure heart chamber.
Gradations of Murmurs[1] Grade Grade 1 (Defined based on use of an acoustic, not a high-fidelity amplified electronic stethoscope)

Description Very faint, heard only after listener has "tuned in"; may not be heard in all positions. Only heard if the patient "bears down" or performs the Valsalva maneuver. Quiet, but heard immediately after placing the stethoscope on the chest. Moderately loud. Loud, with palpable thrill (a tremor or vibration felt on palpation) [2]

Grade 2 Grade 3 Grade 4 Grade 5 Grade 6

Very loud, with thrill. May be heard when stethoscope is partly off the chest. Very loud, with thrill. May be heard with stethoscope entirely off the chest.

Though several different cardiac conditions can cause heart murmurs, the murmurs can change markedly with the severity of the cardiac disease. An astute physician can sometimes diagnose cardiac conditions with some accuracy based largely on the murmur, related physical examination, and experience with the relative frequency of different heart conditions. However, with the advent of better quality and wider availability of echocardiography and other techniques, heart status can be recognized and quantified much more accurately than formerly possible with only a stethoscope, examination, and experience.

Effects of inhalation/expiration
Inhalation pressure causes an increase in the venous blood return to the right side of the heart by increasing intrathoracic negative pressure making it more negative (pulling blood into the right side of the heart via a vacuum-like effect). Therefore, right-sided murmurs generally increase in intensity with inspiration. The increased (more negative) intrathoracic pressure has an opposite effect on the left side of the heart, making it harder for the blood to exit into circulation. Therefore, left-sided murmurs generally decrease in intensity during inspiration.

Heart sounds With expiration, the opposite haemodynamic changes occur: left-sided murmurs generally increase in intensity with expiration. If a patient lies supine with his legs up at a 45-degree angle, venous return to the right side of the heart produces effects similar to inhalation-increased blood flow.

Interventions that change murmurs

There are a number of interventions that can be performed that alter the intensity and characteristics of abnormal heart sounds. These interventions can differentiate the different heart sounds to more effectively obtain a diagnosis of the cardiac anomaly that causes the heart sound.

Other abnormal sounds

Clicks Heart clicks are short, high-pitched sounds that can be appreciated with modern non-invasive imaging techniques. Rubs The pericardial friction rub can be heard in pericarditis, an inflammation of the pericardium, the sac surrounding the heart. This is a characteristic scratching, creaking, high-pitched sound emanating from the rubbing of both layers of inflamed pericardium. It is the loudest in systole, but can often be heard at the beginning and at the end of diastole. It is very dependent on body position and breathing, and changes from hour to hour.

Surface anatomy
The aortic area, pulmonic area, tricuspid area and mitral area are areas on the surface of the chest where the heart is auscultated. Heart sounds result from reverberation within the blood associated with the sudden block of flow reversal by the valves closing. Because of this, auscultation to determine function of a valve is usually not performed at the position of the valve, but at the position to where the sound waves reverberate.
Pulmonary valve (to pulmonary trunk) left second intercostal space Aortic valve (to aorta) Erb's point Mitral valve (to left ventricle) Tricuspid valve (to right ventricle) left upper sternal border

right second intercostal space right upper sternal border Left third intercostal space left fifth intercostal space left fifth intercostal space medial left sternal border medial to left midclavicular line lower left sternal border

Recording heart sounds

Using electronic stethoscopes, it is possible to record heart sounds via direct output to an external recording device, such as a laptop or MP3 recorder. The same connection can be used to listen to the previously recorded auscultation through the stethoscope headphones, allowing for more detailed study of murmurs and other heart sounds, for general research as well as evaluation of a particular patient's condition.

Notes and references

[1] http:/ / en. wikipedia. org/ wiki/ Heart_sounds#endnote_Abnormal_sounds [2] "thrill". (http:/ / www2. merriam-webster. com/ cgi-bin/ mwmednlm?book=Medical& va=thrill) Medline Plus Medical Dictionary.

Heart sounds

External links
University of Michigan Heart Sound and Murmur Library. (http://www.med.umich.edu/lrc/psb/heartsounds/ index.htm) Heart Sounds & Murmurs. (http://www.dundee.ac.uk/medther/Cardiology/hsmur.html) University of Dundee. Auscultation Assistant. (http://www.med.ucla.edu/wilkes/intro.html) UCLA Heart Sounds - Heart Murmurs. (http://www.practicalclinicalskills.com/heart-sounds-murmurs.aspx) practicalclinicalskills.com

Heart murmur

Heart murmur
Cardiac murmurs and other cardiac sounds
Auscultogram from normal and abnormal heart sounds ICD-10 ICD-9 DiseasesDB MedlinePlus MeSH R01 [1] [2] -785.3 [3]

785.2 29151

[4] [5] [6]

003266

D006337

Murmurs are pathologic heart sounds that are produced as a result of turbulent blood flow that is sufficient to produce audible noise. Most murmurs can only be heard with the assistance of a stethoscope ("or auscultation"). A functional murmur or "physiologic murmur" is a heart murmur that is primarily due to physiologic conditions outside the heart, as opposed to structural defects in the heart itself. Functional murmurs are benign (an "innocent murmur"). Murmurs may also be the result of various problems, such as narrowing or leaking of valves, or the presence of abnormal passages through which blood flows in or near the heart. Such murmurs, known as pathologic murmurs, should be evaluated by an expert. Heart murmurs are most frequently categorized by timing, into systolic heart murmurs and diastolic heart murmurs. However, continuous murmurs cannot be directly placed into either category.

Classification
Murmurs can be classified by seven different characteristics: timing, shape, location, radiation, intensity, pitch and quality. Timing refers to whether the murmur is a systolic or diastolic murmur. Shape refers to the intensity over time; murmurs can be crescendo [7], decrescendo [8] or crescendo-decrescendo. Location refers to where the heart murmur is usually auscultated best. There are four places on the anterior chest wall to listen for heart murmurs; each of the locations roughly corresponds to a specific part of the heart and should be auscultated with the patient lying supine. The four locations are: Aortic region - the 2nd right intercostal space. Pulmonic region - the 2nd left intercostal spaces. Tricuspid region - the 5th left intercostal space. Mitral region - the 5th left mid-clavicular intercostal space. Additional maneuvers can be performed for additional auscultation: Left lateral decubitis. With the patient sitting upright. With the patient leaning forward and exhaling. Radiation refers to where the sound of the murmur radiates. The general rule of thumb is that the sound radiates in the direction of the blood flow. Intensity refers to the loudness of the murmur, and is graded according to the Levine scale, from 1 to 6: 1. The murmur is only audible on listening carefully for some time.

Heart murmur The murmur is faint but immediately audible on placing the stethoscope on the chest. A loud murmur readily audible but with no palpable thrill. A loud murmur with a palpable thrill. A loud murmur with a palpable thrill. The murmur is so loud that it is audible with only the rim of the stethoscope touching the chest. 6. A loud murmur with a palpable thrill. The murmur is audible with the stethoscope not touching the chest but lifted just off it. Pitch may be low, medium or high and is determined by whether it can be auscultated best with the bell or diaphragm of a stethoscope. Quality refers to unusual characteristics of a murmur, such as blowing, harsh, rumbling or musical. A mnemonic to remember what characteristics to look for when listening to murmurs is SCRIPT: Site, Configuration (shape), Radiation, Intensity, Pitch and quality, and Timing in the cardiac cycle. The use of two simple mnemonics may help differentiate systolic and diastolic murmurs; PASS and PAID. Pulmonary and aortic stenoses are systolic while pulmonary and aortic insufficiencies (regurgitation) are diastolic. Mitral and tricuspid defects are opposite. 2. 3. 4. 5.

Interventions that change murmur sounds

Inhalation leads to an increase in intrathoracic negative pressure, which increases the capacity of pulmonary circulation, thereby prolonging ejection time. This will affect the closure of the pulmonary valve. This finding, also called Carvallo's maneuver, has been found by studies to have a sensitivity of 100% and a specificity of 80% to 88% in detecting murmurs originating in the right heart. specifically positive Carvallo's sign describes the increase in intensity of a tricuspid regurgitation murmur with inspiration.[9] abrupt standing Squatting, by increasing afterload and increasing preload. Handgrip maneuver, by increasing afterload Valsalva maneuver. One study found the Valsalva maneuver to have a sensitivity of 65%, specificity of 96% in detecting hypertrophic obstructive cardiomyopathy (HOCM). Both standing and Valsalva maneuver will decrease venous return and subsequently decrease left ventricular filling, resulting in an increase in the loudness of the murmur of hypertrophic cardiomyopathy, since outflow obstruction is increased by decreasing preload. Alternatively, squatting increases systemic vascular resistance, increasing afterload and helping to hold the obstruction in a more open configuration, decreasing the murmur. Maximum handgrip exercise also results in a decrease in the loudness of the murmur.[10] post ectopic potentiation Inhaled amyl nitrite is a vasodilator that diminishes systolic murmurs in left-to-right shunts in ventricular septal defects, and reveals right-to left shunts in the setting of a pulmonic stenosis and a ventricular septal defect. methoxamine positioning of the patient. That is, putting patients in the left lateral position will allow a murmur in the mitral valve area to be more pronounced.

Heart murmur

Anatomic sources of murmur

Systolic Aortic valve stenosis typically is a crescendo/decrescendo systolic murmur best heard at the right upper sternal border sometimes with radiation to the carotid arteries. In mild aortic stenosis, the crescendo-decrescendo is early peaking whereas in severe aortic stenosis, the crescendo is late-peaking, and the S2 heart sound may be obliterated. Stenosis of Bicuspid aortic valve is similar to the aortic valve stenosis heart murmur, but a systolic ejection click may be heard after S1 in calcified bicuspid aortic valves. Symptoms tend to present between 40 and 70 years of age. Mitral regurgitation typically is a holosystolic murmur heard best at the apex, and may radiate to the axilla or precordium. A systolic click may be heard if there is associated mitral valve prolapse. Valsalva maneuver in mitral regurgitation associated with mitral valve prolapse will increase left ventricular preload and move the murmur onset closer to S1, and isometric handgrip, which increases left ventricular afterload, will increase murmur intensity. In acute severe mitral regurgitation, a holosystolic murmur may not be heard. Pulmonary valve stenosis typically is a crescendo-decrescendo murmur heard best at the left upper sternal border, associated with a systolic ejection click that diminishes with inspiration and sometimes radiates to the left clavicle. Tricuspid valve regurgitation presents as a holosystolic murmur at the left lower sternal border with radiation to the left upper sternal border. Prominent v and c waves may be seen in the jugular venous pulse. The murmur will increase with inspiration. Hypertrophic obstructive cardiomyopathy (or hypertrophic subaortic stenosis) will be a systolic crescendo-decrescendo murmur best heard at the left lower sternal border. Valsalva maneuver will increase the intensity of the murmur, as will changing positions from squatting to standing. Atrial septal defect will present with a systolic crescendo-decrescendo murmur best heard at the right upper sternal border due to increased volume going through the pulmonary valve, and is associated with a fixed, split S2 and a right ventricular heave. Ventricular septal defect (VSD) will present as a holosystolic murmur at the left lower sternal border, associated with a palpable thrill, and increases with isometric handgrip. A right to left shunt (Eisenmenger syndrome) may develop with uncorrected VSDs due to worsening pulmonary hypertension, which will increase the murmur intensity and be associated with cyanosis. Flow murmur may be heard at the right upper sternal border in certain conditions, such as anemia, hyperthyroidism, fever, and pregnancy. Diastolic Aortic valve regurgitation will present as a diastolic decrescendo murmur heard at the left lower sternal border or right lower sternal border (when associated with a dilated aorta). This may be associated with bounding carotid and peripheral pulses (Corrigan's pulse, Waterhammer pulse), and a widened pulse pressure. Mitral stenosis typically presents as a diastolic low-pitched decrescendo murmur best heard at the cardiac apex in the left lateral decubitus position. It may be associated with an opening snap. Increasing severity will shorten the time between S2 and the opening snap. Tricuspid valve stenosis presents as a diastolic decrescendo murmur at the left lower sternal border, and signs of right heart failure may be seen on exam. Pulmonary valve regurgitation presents as a diastolic decrescendo murmur at the left lower sternal border. A palpable S2 in the second left intercostal space correlates with pulmonary hypertension due to mitral stenosis. Continuous and Combined Systolic/Diastolic Patent ductus arteriosus may present as a continuous murmur radiating to the back.

Heart murmur Severe coarctation of the aorta can present with a continuous murmur: a systolic component at the left infraclavicular region and the back due to the stenosis, and a diastolic component over the chest wall due to blood flow through collateral vessels. Acute severe aortic regurgitation is associated with a three phase murmur, specifically a midsystolic murmur followed by S2, followed by a parasternal early diastolic and mid-diastolic murmur (Austin Flint murmur). Although the exact cause of an Austin Flint murmur is unknown, it is hypothesized that the mechanism of murmur is from the severe aortic regurgitation jet vibrating the anterior mitral valve leaflet, colliding with the mitral inflow during diastole, with increased mitral inflow velocity from the narrowed mitral valve orifice leading to the jet impinging on the myocardial wall. Another not that common cause of a continuous murmur is a ruptured sinus of valsalva. Usually the murmur is well heard in the aortic area and along the left sternal border.

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Murmur Types and Disease Associations

Continuous Machinery Murmur, at the left upper sternal border Classic for a patent ductus arteriosus, and in serious cases associated with poor feeding, failure to thrive and respiratory distress. Other examination findings may include widened pulse pressures and bounding pulses. Systolic Murmur loudest below the left scapula Classic for a coarctation of the aorta which is often seen in Turner's Syndrome, (gonadal dysgenesis), an X-linked disorder with a part missing of the X-chromosome. Other findings of this murmur is radio-femoral delay, and different blood pressures in the upper and lower extremities. Harsh holosystolic murmur at the left lower sternal border Classic for a ventricular septal defect. It is in these children that the delayed-onset cyanotic heart disease occurs known as Eisenmenger syndrome, which is a reversal of the left-to-right heart shunt as the right ventricle hypertrophies, causing a right-to-left shunt and resulting cyanosis. Widely split fixed S2 and systolic ejection murmur at the left upper sternal border Classically due to a patent foramen ovale or atrial septal defect, which is lack of closure of the foramen ovale. This produces a left-to-right shunt initially, thus does not produce cyanosis, but causes pulmonary hypertension. Longstanding uncorrected atrial septal defects can also result in Eisenmenger's syndrome with resultant cyanosis.

Cooing dove murmur

The cooing dove murmur is a cardiac murmur with a musical quality (high pitched - hence the name) and is associated with aortic valve regurgitation (or mitral regurgiation before rupture of chordae). It is a diastolic murmur which can be heard over the mid-precordium.[11]

References
[1] [2] [3] [4] [5] [6] [7] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ R01 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=785. 2 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=785. 3 http:/ / www. diseasesdatabase. com/ ddb29151. htm http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 003266. htm http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2009/ MB_cgi?field=uid& term=D006337 http:/ / en. wiktionary. org/ wiki/ crescendo

[8] http:/ / en. wiktionary. org/ wiki/ decrescendo [9] Harrison's Internal Medicine 17th, chapter 5, "Disorders of the cardiovascular system," question 32, self assessment and board review [10] Harrison's Internal Medicine 17th, chapter 5, "Disorders of the cardiovascular system," question 86-87, self assessment and board review

Heart murmur
[11] https:/ / www. jstage. jst. go. jp/ article/ ihj1960/ 22/ 5/ 22_5_861/ _pdf

11

External links
Information on heart murmurs in children (http://heartcenter.seattlechildrens.org/conditions_treated/ heart_murmurs.asp) from Seattle Children's Heart Center Heart Murmurs in Pediatric Patients (http://www.aafp.org/afp/990800ap/558.html) Lehrer, Steven. Understanding Pediatric Heart Sounds. Elsevier 2002. Hanifin, Christopher. Heart Sounds: A Cardiac Auscultation Primer. CreateSpace, 2010 Texas Heart Institute (http://www.texasheartinstitute.org/education/cme/explore/events/eventdetail_5469. cfm) Scroll down to listen to heart murmurs. The Auscultation Assistant (http://www.med.ucla.edu/wilkes/intro.html) Provides recordings of heart murmurs. Heart murmurs in children (http://www.gosh.nhs.uk/medical-conditions/search-for-medical-conditions/ heart-murmurs-innocent/heart-murmurs-innocent-information/) information for parents.

Aortic valve stenosis

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Aortic valve stenosis

Aortic valve stenosis
Classification and external resources

In the center is an aortic valve with severe stenosis due to rheumatic heart disease. The valve is surrounded by the aorta. The pulmonary trunk is at the lower right. The right coronary artery, cut lengthwise, is at the lower left. The left main coronary artery, also cut lengthwise, is on the right. ICD-10 ICD-9 DiseasesDB MedlinePlus eMedicine I35.0 395.0 844 [1] , I06.0 [2] , Q23.0 , 746.3 [3] [6]

[4]

, 396.0

[5]

[7] [8] [9]

000178

med/157

Aortic valve stenosis (AS) is a disease of the heart valves in which the opening of the aortic valve is narrowed.[10] The aortic valve is the valve located between the left ventricle of the heart and the aorta, the largest artery in the body which carries the entire output of blood to the systemic circulation. Aortic stenosis is now the most common valvular heart disease in the Western World.

Signs and symptoms

Symptoms related to aortic stenosis depend on the degree of valve stenosis. Most people with mild to moderate aortic stenosis are asymptomatic. Symptoms usually present in individuals with severe aortic stenosis, though they may occur in those with mild to moderate aortic stenosis as well. The three cardinal symptoms of aortic stenosis are syncope, anginal chest pain and dyspnea or other symptoms of heart failure such as orthopnea, exertional dyspnea, paroxysmal nocturnal dyspnea, or pedal edema.

Angina
Angina in the setting of heart failure also increases the risk of death. In patients with angina, the 5 year mortality rate is 50%, if the aortic valve is not replaced. Angina in the setting of AS is secondary to the left ventricular hypertrophy (LVH) that is caused by the constant production of increased pressure required to overcome the pressure gradient caused by the AS. While the myocardium (i.e., heart muscle) of the LV gets thicker, the arteries that supply the muscle do not get significantly longer or bigger, so the muscle may become ischemic (i.e., does not receive an adequate blood supply). The ischemia may first be evident during exercise, when the heart muscle requires increased blood supply to compensate for the increased workload. The individual may complain of exertional angina. At this stage, a stress test with imaging may

Aortic valve stenosis be suggestive of ischemia. Eventually, however, the cardiac muscle will require more blood supply at rest than can be supplied by the coronary artery branches. At this point there may be signs of ventricular strain pattern (ST segment depression and T wave inversion) on the EKG, suggesting subendocardial ischemia. The subendocardium is the region that becomes ischemic because it is the most distant from the epicardial coronary arteries.

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Syncope
Syncope (fainting spells) from aortic valve stenosis is usually exertional.[11] In the setting of heart failure it increases the risk of death. In patients with syncope, the 3 year mortality rate is 50%, if the aortic valve is not replaced.[citation
needed]

It is unclear why aortic stenosis causes syncope. One popular theory is that severe AS produces a nearly fixed cardiac output.[citation needed] When the patient exercises, their peripheral vascular resistance will decrease as the blood vessels of the skeletal muscles dilate to allow the muscles to receive more blood to allow them to do more work. This decrease in peripheral vascular resistance is normally compensated for by an increase in the cardiac output. Since patients with severe AS cannot increase their cardiac output, the blood pressure falls and the patient will syncopize due to decreased blood perfusion to the brain. A second theory as to why syncope may occur in AS is that during exercise, the high pressures generated in the hypertrophied LV cause a vasodepressor response, which causes a secondary peripheral vasodilation that, in turn, causes decreased blood flow to the brain resulting in loss of consciousness. Indeed, in aortic stenosis, because of the fixed obstruction to bloodflow out from the heart, it may be impossible for the heart to increase its output to offset peripheral vasodilation. A third mechanism may sometimes be operative. Due to the hypertrophy of the left ventricle in aortic stenosis, including the consequent inability of the coronary arteries to adequately supply blood to the myocardium (see "Angina" below), arrhythmias may develop. These can lead to syncope. Finally, in calcific aortic stenosis[12] at least, the calcification in and around the aortic valve can progress and extend to involve the electrical conduction system of the heart. If that occurs, the result may be heart block - a potentially lethal condition of which syncope may be a symptom.

Congestive heart failure

Congestive heart failure (CHF) carries a grave prognosis in patients with AS. Patients with CHF attributable to AS have a 2 year mortality rate of 50% if the aortic valve is not replaced.[citation needed] CHF in the setting of AS is due to a combination of left ventricular hypertrophy with fibrosis, systolic dysfunction (a decrease in the ejection fraction) and diastolic dysfunction (elevated filling pressure of the LV).

Associated symptoms

In Heyde's syndrome, aortic stenosis is associated with gastrointestinal bleeding due to angiodysplasia of the colon. Recent research has shown that the stenosis causes a form of von Willebrand disease by breaking down its associated coagulation factor (factor VIII-associated antigen, also called von Willebrand factor), due to increased turbulence around the stenosed valve.

Density-Dependent Colour Scanning Electron Micrograph SEM (DDC-SEM) of cardiovascular calcification, showing in orange calcium phosphate spherical particles (denser material) and, in green, the extracellular matrix (less dense [] material).

Aortic valve stenosis

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Complications
Notwithstanding the foregoing, the American Heart Association has recently changed its recommendations regarding antibiotic prophylaxis for endocarditis. Specifically, as of 2007, it is recommended that such prophylaxis be limited only to those with prosthetic heart valves, those with previous episode(s) of endocarditis, and those with certain types of congenital heart disease.[citation needed] Since the stenosed aortic valve may limit the heart's output, people with aortic stenosis are at risk of syncope and dangerously low blood pressure should they use any of a number of medications for cardiovascular diseases that often coexist with aortic stenosis. Examples include nitroglycerin, nitrates, ACE inhibitors, terazosin (Hytrin), and hydralazine. Note that all of these substances lead to peripheral vasodilation. Under normal circumstances, in the absence of aortic stenosis, the heart is able to increase its output and thereby offset the effect of the dilated blood vessels. In some cases of aortic stenosis, however, due to the obstruction of blood flow out of the heart caused by the stenosed aortic valve, cardiac output cannot be increased. Low blood pressure or syncope may ensue.

Etiology
Aortic stenosis is most commonly caused by age-related progressive calcification of a normal (three-leafed) aortic valve (>50% of cases) with a mean age of 65 to 70 years old. Other causes of aortic stenosis include calcification of a congenital bicuspid aortic valve[13] (30-40% of cases) and acute rheumatic fever post-inflammatory (less than 10% of cases).[14] Rare causes of aortic stenosis include Fabry disease, systemic lupus erythematosus, Paget disease, hyperuricemia, and infection. Normal aortic valves have three leaves (tricuspid), but some individuals are born with an aortic valve that has two leaves (bicuspid). Typically, aortic stenosis due to calcification of a bicuspid valve appears earlier, in the 40s or 50s, whereas aortic stenosis due to calcification of a normal tricuspid aortic valve appears later, usually in the 70s and 80s.
Illustration depicting aortic stenosis

Aortic valve stenosis

15

Pathophysiology
The human aortic valve normally consists of three leaflets (trileaflets) and has an orifice of 3.0-4.0 square centimeters. When the left ventricle (LV) contracts, it forces blood through the valve into the aorta and subsequently to the rest of the body. When the LV expands again, the aortic valve closes and prevents the blood in the aorta from flowing backward into the left ventricle. In aortic stenosis, the opening of the aortic valve becomes narrowed or constricted (stenotic) (i.e., due to calcification). Degenerative aortic stenosis, the most common variety, and bicuspid aortic stenosis both begin with damage to endothelial cells from increased mechanical stress. Inflammation is thought to be involved in the earlier stages of the pathogenesis of AS and its associated risk factors are known to promote the deposition of LDL cholesterol and a highly damaging substance known as Lipoprotein(a) into the aortic valve resulting in significant damage and stenosis over time. As a consequence of this stenosis, the left ventricle must generate a higher pressure with each contraction to effectively move blood forward into the aorta. Initially, the LV generates this increased pressure by thickening its muscular walls (myocardial hypertrophy). The type of hypertrophy most commonly seen in AS is known as concentric hypertrophy, in which the walls of the LV are (approximately) equally thickened.

Simultaneous left ventricular and aortic pressure tracings demonstrate a pressure gradient between the left ventricle and aorta, suggesting aortic stenosis. The left ventricle generates higher pressures than what is transmitted to the aorta. The pressure gradient, caused by aortic stenosis, is represented by the green shaded area. (AO = ascending aorta; LV = left ventricle; ECG = electrocardiogram.)

In the later stages, the left ventricle dilates, the wall thins, and the systolic function deteriorates (resulting in impaired ability to pump blood forward). Morris and Innasimuthu et al. showed that different coronary anatomy is associated with different valve diseases. Research is ongoing to see if different coronary anatomy might lead to turbulent flow at the level of valves leading to inflammation and degeneration.[15][16][17]

Aortic valve stenosis

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Risk factors
Risk factors known to influence disease progression of AS include lifestyle habits similar to those of coronary artery disease such as hypertension, advanced age, being male, hyperlipidemia, diabetes mellitus, cigarette smoking, metabolic syndrome, and end-stage renal disease.

Diagnosis
Aortic stenosis is most often diagnosed when it is asymptomatic and can sometimes be detected during routine examination of the heart and circulatory system. Good evidence exists to demonstrate that certain characteristics of the peripheral pulse can rule in the diagnosis. In particular, there may be a slow and/or sustained upstroke of the arterial pulse, and the pulse may be of low volume. This is sometimes referred to as pulsus parvus et tardus. There may also be a noticeable delay between the first heart sound (on auscultation) and the corresponding pulse in the carotid artery (so-called 'apical-carotid delay'). In similar manner, there may be a delay between the appearance of each pulse in the brachial artery (in the arm) and the radial artery (in the wrist). The first heart sound may be followed by a sharp ejection sound ("ejection click") best heard at the lower left sternal border and the apex, and, thus, appear to be "split". The ejection sound, caused by the impact of left ventricular outflow against the partially fused aortic Phonocardiograms from normal and abnormal valve leaflets, is more commonly associated with a mobile bicuspid heart sounds aortic valve than an immobile calcified aortic valve. The intensity of this sound does not vary with respiration, which helps distinguish it from the ejection click produced by a stenotic pulmonary valve, which will diminish slightly in intensity during inspiration. An easily heard systolic, crescendo-decrescendo (i.e., 'ejection') murmur is heard loudest at the upper right sternal border, at the 2nd right intercostal space, and radiates to the carotid arteries bilaterally. The murmur increases with squatting, decreases with standing and isometric muscular contraction, which helps distinguish it from hypertrophic obstructive cardiomyopathy (HOCM). The murmur is louder during expiration, but is also easily heard during inspiration. The more severe the degree of the stenosis, the later the peak occurs in the crescendo-decrescendo of the murmur. The second heart sound (A2) tends to become decreased and softer as the aortic stenosis becomes more severe. This is a result of the increasing calcification of the valve preventing it from "snapping" shut and producing a sharp, loud sound. Due to increases in left ventricular pressure from the stenotic aortic valve, over time the ventricle may hypertrophy, resulting in a diastolic dysfunction. As a result, one may hear a fourth heart sound due to the stiff ventricle. With continued increases in ventricular pressure, dilatation of the ventricle will occur, and a third heart sound may be manifest. Finally, aortic stenosis often co-exists with some degree of aortic insufficiency (aortic regurgitation). Hence, the physical exam in aortic stenosis may also reveal signs of the latter, for example an early diastolic decrescendo murmur. Indeed, when both valve abnormalities are present, the expected findings of either may be modified or may not even be present. Rather, new signs that reflect the presence of simultaneous aortic stenosis and insufficiency, e.g., pulsus bisferiens, emerge. According to a meta analysis, the most useful findings for ruling in aortic stenosis in the clinical setting were slow rate of rise of the carotid pulse (positive likelihood ratio ranged 2.8130 across studies), mid to late peak intensity of

Aortic valve stenosis the murmur (positive likelihood ratio, 8.0101), and decreased intensity of the second heart sound (positive likelihood ratio, 3.150). Other peripheral signs include: sustained, heaving apex beat, which is not displaced unless systolic dysfunction of the left ventricle has developed A precordial thrill narrowed pulse pressure

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Electrocardiogram
Although aortic stenosis does not lead to any specific findings on the electrocardiogram (ECG), it still often leads to a number of electrocardiographic abnormalities. ECG manifestations of left ventricular hypertrophy (LVH) are common in aortic stenosis and arise as a result of the stenosis having placed a chronically high pressure load on the left ventricle (with LVH being the expected response to chronic pressure loads on the left ventricle no matter what the cause). As noted above, the calcification process that occurs in aortic stenosis can progress to extend beyond the aortic valve and into the electrical conduction system of the heart. Evidence of this phenomenon may rarely include ECG patterns characteristic of certain types of heart block such as Left bundle branch block.

Heart catheterization
Cardiac chamber catheterization provides a definitive diagnosis, indicating severe stenosis in valve area of <1.0cm2 (normally about 3cm2).[18] It can directly measure the pressure on both sides of the aortic valve. The pressure gradient may be used as a decision point for treatment. It is useful in symptomatic patients before surgery. However, cardiac catheterization is not recommended to assess the severity of aortic stenosis when noninvasive testing is sufficient to make the diagnosis.

Echocardiogram
Severity of aortic stenosis Degree of aortic stenosis Mean gradient (mmHg) <25 25 - 40 >40 >70 Aortic valve area (cm2) >1.5 1.0 - 1.5 < 1.0 < 0.6

Mild aortic stenosis Moderate aortic stenosis Severe aortic stenosis Critical aortic stenosis

Echocardiogram (heart ultrasound) is the best non-invasive tool / test to evaluate the aortic valve anatomy and function. The aortic valve area can be calculated non-invasively using echocardiographic flow velocities. Using the velocity of the blood through the valve, the pressure gradient across the valve can be calculated by the continuity equation or using the modified Bernoulli's equation: Gradient = 4(velocity) mmHg A normal aortic valve has a gradient of only a few mmHg. A decreased valvular area causes increased pressure gradient, and these parameters are used to classify and grade the aortic stenosis as mild, moderate or severe. The pressure gradient can be abnormally low in the presence of mitral stenosis, heart failure, co-existent aortic regurgitation and also ischaemic heart disease (disease related to decreased blood supply and oxygen causing

Aortic valve stenosis ischaemia). Echocardiogram may also show left ventricular hyperthrophy, thickened and immobile aortic valve and dilated aortic root. However, it may appear deceptively normal in acute cases.

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Chest X-ray
Chest X-ray can also assist in the diagnosis, showing calcific aortic valve, and, in longstanding disease, enlarged left ventricle and atrium.

Management
Treatment is generally not necessary in people without symptoms. In moderate cases, echocardiography is performed every 12 years to monitor the progression, possibly complemented with a cardiac stress test. In severe cases, echocardiography is performed every 36 months. In both moderate and mild cases, the patient should immediately make a revisit or be admitted for inpatient care if any new related symptoms appear.

Medical Management
The effect of statins on the progression of AS is still unclear. The latest trials do not show any benefit in slowing AS progression, but did demonstrate a decrease in ischemic cardiovascular events. Angiotensin-converting enzyme (ACE) and angiotensin II receptors have been found in stenotic aortic valves. This leads to the hypothesis that the renin-angiotensin system may play a role in the progression of the disease. To date, there is no randomized trial examining the impact of ACE inhibitors in AS. Innasimuthu et al. showed that patients on bisphosphonates have less progression of aortic stenosis and some regressed.[19][20][21] This finding led to multiple trials which is ongoing. Subsequent research has failed to confirm the initial positive result. In general, medical therapy has relatively poor efficacy in treating aortic stenosis. However, it may be useful to manage commonly coexisting conditions that correlate with aortic stenosis: Any angina is generally treated with beta-blockers and/or calcium blockers. Nitrates are contraindicated due to their potential to cause profound hypotension in aortic stenosis.[22] Any hypertension is treated aggressively, but caution must be taken in administering beta-blockers Any heart failure is generally treated with digoxin and diuretics, and, if not contraindicated, cautious inpatient administration of ACE inhibitors. As for angina, nitrates are contraindicated. Since calcific aortic stenosis shares many pathological features and risk factors with atherosclerosis, and since atherosclerosis may be prevented and/or reversed by cholesterol lowering, there has been interest in attempting to modify the course of calcific aortic stenosis by lowering cholesterol levels with statin drugs. Although a number of small, observational studies demonstrated an association between lowered cholesterol and decreased progression, and even regression, of calcific aortic stenosis, a recent, large randomized clinical trial, published in 2005, failed to find any predictable effect of cholesterol lowering on calcific aortic stenosis. A 2007 study did demonstrate a slowing of aortic stenosis with the statin rosuvastatin. However, a large randomized controlled trial published in the New England Journal of Medicine in 2008 failed to find any beneficial effect of intensive cholesterol lowering on the course of aortic stenosis.

Aortic valve stenosis

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Aortic valve replacement

In adults, symptomatic severe aortic stenosis usually requires aortic valve replacement (AVR). While AVR has been the standard of care for aortic stenosis for several decades, currently aortic valve replacement approaches include open heart surgery, minimally invasive cardiac surgery (MICS) and minimally invasive catheter-based (percutaneous) aortic valve replacement.

Apicoaortic conduit
Apicoaortic Conduit (AAC), or Aortic Valve Bypass (AVB), has been shown to be an effective treatment for aortic stenosis.[23] There is long-term stability of the left ventricular hemodynamics after AVB, with no further biologic progression of native aortic valve stenosis. Once the pressure gradient across the native valve is substantially reduced, the narrowing and calcification of the native valve halts.

Surgical Valve Replacement

A diseased aortic valve is most commonly replaced using a surgical procedure with either a mechanical or a tissue valve. The procedure is done either in an open-heart surgical procedure or, in a smaller but growing number of cases, a minimally invasive cardiac surgery (MICS) procedure.

Percutaneous (Transcatheter)Aortic Valve Replacement

Globally more than 40,000 patients have received transcatheter aortic valve replacement. For patients who are not candidates for surgical valve replacement, transcatheter valve replacement may be a suitable alternative. When selecting the optimal therapy for individual patients, the percutaneous (transcatheter) approach must be carefully weighed against the excellent results achieved with conventional surgery.

Balloon valvuloplasty
For infants and children, balloon valvuloplasty, where a balloon is inflated to stretch the valve and allow greater flow, may also be effective. In adults, however, it is generally ineffective, as the valve tends to return to a stenosed state. The surgeon will make a small incision at the top of the patient's leg and proceed to insert the balloon into the artery and then inflate it to get a better flow of blood around the patient's body.[24]

Prognosis
If untreated, severe symptomatic aortic stenosis carries a poor prognosis with a 2-year mortality rate of 50-60% and a 3-year survival rate of less than 30%.

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Epidemiology
Approximately 2% of people over the age of 65, 3% of people over age 75, and 4% percent of people over age 85 have aortic valve stenosis.[25] The prevalence is increasing with the aging population in North America and Europe.[26]

History
Aortic stenosis was first described by French physician Lazare Riviere in 1663.

References
[1] [2] [3] [4] [5] [6] [7] [8] [9] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I35. 0 http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I06. 0 http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ Q23. 0 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=395. 0 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=396. 0 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=746. 3 http:/ / www. diseasesdatabase. com/ ddb844. htm http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 000178. htm http:/ / www. emedicine. com/ med/ topic157. htm

[10] Aortic stenosis (http:/ / www. mountsinai. org/ Other/ Diseases/ Aortic stenosis) at Mount Sinai Hospital [11] Chapter 1: Diseases of the Cardiovascular system > Section: Valvular Heart Disease in: [12] Miller, J. D. Cardiovascular calcification: Orbicular origins.Nature Materials12, 476-478 (2013). [13] Ricardo Zalaquett, Cristbal Campl, et al. (2005). "Ciruga reparadora de la vlvula artica bicspide insuficiente". Rev Md Chile, 133(3): pp. 279-86. (http:/ / www. scielo. cl/ scielo. php?script=sci_arttext& pid=S0034-98872005000300002& lng=es& nrm=iso) ISSN 0034-9887. [14] VOC=VITIUM ORGANICUM CORDIS, a compendium of the Department of Cardiology at Uppsala Academic Hospital. By Per Kvidal September 1999, with revision by Erik Bjrklund May 2008 [15] G. Morris, Innasimuthu A L, J.P. Fox, R.A. Perry; The association of heart valve diseases with a dominant left coronary circulation European Heart Journal, 2009; 30:682 [16] GM Morris, A L Innasimuthu, JP. Fox, RA. Perry, The Association of Heart Valve Diseases with Coronary Artery Dominance The Journal of Heart Valve Disease 2010;19:389-393 [17] Innasimuthu A L, Morris G, Rao G K, Perry R A; Left Dominant Coronary arterial system and Aortic stenosis: an association, cause or effect Heart 2007; 93 (Suppl 1): A39. [18] Yale atlas of echocardiography (http:/ / www. yale. edu/ imaging/ echo_atlas/ entities/ aortic_stenosis_senile. html) [19] Innasimuthu A L, Katz W E; Effect of Bisphosphonates in Progression of Aortic Stenosis Echocardiography 2010; Jan;28(1):1-7. [20] Nathaniel S, Saligram S, Innasimuthu AL, Aortic stenosis: an update World Journal of Cardiology 2010; 2(6): 135-139 [21] Innasimuthu A L, Katz W E; Effect of Bisphosphonates in Progression of Aortic Stenosis Journal of the American College of Cardiology 2009; 53:A413 [22] 1 Rutherford SD, Braunwald E. Chronic ischaemic heart disease. In: Braunwald E, ed. Heart disease: A textbook of cardiovascular medicine. 4th ed. Philadelphia: WB Saunders, 1992:1292-1364. [23] Vliek CJ, Balaras E, Li S, Lin JY, Young CA, DeFillippi CR, Griffith BP, Gammie JS, Early and Midterm Hemodynamics After Aortic Valve Bypass (Apicoaortic Conduit) Surgery, Ann Thorac Surg 2010;90:13643. [24] Mayo Clinic > Aortic valve stenosis > Treatments and drugs (http:/ / www. mayoclinic. com/ health/ aortic-valve-stenosis/ DS00418/ DSECTION=8) Retrieved September 2010 [25] Stewart BF, Siscovick D, Lind BK, Gardin JM, Gottdiener JS, Smith VE. Clinical factors associated with calcific aortic valve disease. Cardiovascular Health Study. J Am Coll Cardiol. 1997; 29: 630-634. [26] clinical Anesthesiology by Edward Morgan

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External links
Aortic valve stenosis (http://www.dmoz.org/Health/Conditions_and_Diseases/Cardiovascular_Disorders/ Heart_Disease/Valvular/Aortic_Valve//) at the Open Directory Project Aortic Stenosis (http://www.gosh.nhs.uk/medical-conditions/search-for-medical-conditions/ aortic-valve-stenosis/aortic-valve-stenosis-information/) information for parents. Aortic Stenosis Infographic (http://www.heart-valve-surgery.com/heart-surgery-blog/2013/04/01/ aortic-stenosis-infographic/) for patients, their family members and friends.

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Mitral regurgitation
Mitral regurgitation
Classification and external resources

Mitral regurgitation (schematic drawing) During systole, contraction of the left ventricle causes abnormal backflow (arrow) into the left atrium. 1 Mitral valve 2 Left Ventricle 3 Left Atrium 4 Aorta ICD-10 ICD-9 DiseasesDB MedlinePlus eMedicine MeSH I05.1 394.1 8275 [1] , I34.0 [2] , Q23.3 , 746.6 [3] [6]

[4] [7]

, 424.0

[5]

000176

[8] [9]

emerg/314 D008944

[10]

Mitral regurgitation (MR), mitral insufficiency or mitral incompetence is a disorder of the heart in which the mitral valve does not close properly when the heart pumps out blood. It is the abnormal leaking of blood from the left ventricle, through the mitral valve, and into the left atrium, when the left ventricle contracts, i.e. there is regurgitation of blood back into the left atrium.[11] MR is the most common form of valvular heart disease.

Mitral regurgitation

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Symptoms and signs

The symptoms associated with mitral regurgitation are dependent on which phase of the disease process the individual is in. Individuals with acute mitral regurgitation will have the signs and symptoms of decompensated congestive heart failure (i.e. shortness of breath, pulmonary edema, orthopnea, and paroxysmal nocturnal dyspnea), as well as symptoms suggestive of a low cardiac output state (i.e. decreased exercise tolerance). Palpitations are also common. Cardiovascular collapse with shock (cardiogenic shock) may be seen in individuals with acute mitral regurgitation due to papillary muscle rupture or rupture of a chorda tendinea. Individuals with chronic compensated mitral regurgitation may be asymptomatic, with a normal exercise tolerance and no evidence of heart failure. These individuals may be sensitive to small shifts in their intravascular volume status, and are prone to develop volume overload (congestive heart failure). Findings on clinical examination depend on the severity and duration heart sounds of mitral regurgitation. The mitral component of the first heart sound is usually soft and with a laterally displaced apex beat, often with heave. The first heart sound is followed by a high-pitched holosystolic murmur at the apex, radiating to the back or clavicular area. Its duration is, as the name suggests, the whole of systole. The loudness of the murmur does not correlate well with the severity of regurgitation. It may be followed by a loud, palpable P2, heard best when lying on the left side. A third heart sound is commonly heard. Commonly, atrial fibrillation is found. In acute cases, the murmur and tachycardia may be only distinctive signs. Patients with mitral valve prolapse often have a mid-to-late systolic click and a late systolic murmur.
Phonocardiograms from normal and abnormal

Cause
The mitral valve comprises two valve leaflets: the mitral valve annulus which forms a ring around the valve leaflets, and the papillary muscles which tether the valve leaflets to the left ventricle and prevent them from prolapsing into the left atrium. The chordae tendineae are also present and connect the valve leaflets to the papillary muscles. A dysfunction of any of these portions of the mitral valve apparatus can cause mitral regurgitation. The most common cause of mitral regurgitation is mitral valve prolapse (MVP). Mitral valve prolapse is in turn is caused by myxomatous degeneration, and is the most common cause of primary mitral regurgitation in the United States, causing about 50% of primary mitral regurgitation. Myxomatous degeneration of the mitral valve is more common in women as well as with advancing age which causes a stretching of the leaflets of the valve and the chordae tendineae. Such elongation prevent the valve leaflets from fully coming together when the valve closes, causing the valve leaflets to prolapse into the left atrium, thereby causing mitral regurgitation. Ischemic heart disease causes mitral regurgitation by the combination of ischemic dysfunction of the papillary muscles, and the dilatation of the left ventricle. This can lead to the subsequent displacement of the papillary muscles and the dilatation of the mitral valve annulus. Rheumatic fever and Marfan's syndrome are other typical causes of mitral regurgitation. Mitral regurgitation and mitral valve prolapse are also common in Ehlers Danlos Syndrome. [12]

Mitral regurgitation Secondary mitral regurgitation is due to the dilatation of the left ventricle that causes stretching of the mitral valve annulus and displacement of the papillary muscles. This dilatation of the left ventricle can be due to any cause of dilated cardiomyopathy including aortic insufficiency, nonischemic dilated cardiomyopathy, and Noncompaction Cardiomyopathy. Because the papillary muscles, chordae, and valve leaflets are usually normal in such conditions, it is also called functional mitral regurgitation.[13] Acute mitral regurgitation is most often caused by endocarditis, mainly S. aureus.[11] Rupture or dysfunction of the papillary muscle are also common causes in acute cases, dysfunction which can include mitral valve prolapse.[14]

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Pathophysiology
The pathophysiology of mitral regurgitation can be broken into three phases of the disease process: the acute phase, the chronic compensated phase, and the chronic decompensated phase.

Acute phase
Acute mitral regurgitation (as may occur due to the sudden rupture of a chorda tendinae or papillary muscle) causes a sudden volume overload of both the left atrium and the left ventricle. The left ventricle develops volume overload because with every contraction it now has to pump out not only the volume of blood that goes into the aorta (the forward cardiac output or forward stroke volume), but also the blood that regurgitates into the left atrium (the regurgitant volume). The combination of the forward stroke volume and the regurgitant volume is known as the total stroke volume of the left ventricle. In the acute setting, the stroke volume of the left ventricle is increased (increased ejection fraction), this happens because of more complete emptying of heart. However, as it progresses the LV volume increases and the contractile function deteriorates and thus leading to dysfunctional LV and a decrease in ejection fraction.[14] The increase in stroke volume is explained by the FrankStarling mechanism, in which increased ventricular pre-load stretches the myocardium such that contractions are more forceful. The regurgitant volume causes a volume overload and a pressure overload of the left atrium. The increased pressures in the left atrium inhibit drainage of blood from the lungs via the pulmonary veins. This causes pulmonary congestion.

Chronic phase
Compensated If the mitral regurgitation develops slowly over months to years or if the acute phase cannot be managed with medical therapy, the individual will enter the chronic compensated phase of the disease. In this phase, the left ventricle develops eccentric hypertrophy in order to better manage the larger than normal stroke volume. The eccentric hypertrophy and the increased diastolic volume combine to increase the stroke volume (to levels well above normal) so that the forward stroke volume (forward cardiac output) approaches the normal levels. In the left atrium, the volume overload causes enlargement of the chamber of the left atrium, allowing the filling pressure in the left atrium to decrease. This improves the drainage from the pulmonary veins, and signs and symptoms of pulmonary congestion will decrease. These changes in the left ventricle and left atrium improve the low forward cardiac output state and the pulmonary congestion that occur in the acute phase of the disease. Individuals in the chronic compensated phase may be asymptomatic and have normal exercise tolerances.

Mitral regurgitation Decompensated An individual may be in the compensated phase of mitral regurgitation for years, but will eventually develop left ventricular dysfunction, the hallmark for the chronic decompensated phase of mitral regurgitation. It is currently unclear what causes an individual to enter the decompensated phase of this disease. However, the decompensated phase is characterized by calcium overload within the cardiac myocytes. In this phase, the ventricular myocardium is no longer able to contract adequately to compensate for the volume overload of mitral regurgitation, and the stroke volume of the left ventricle will decrease. The decreased stroke volume causes a decreased forward cardiac output and an increase in the end-systolic volume. The increased end-systolic volume translates to increased filling pressures of the left ventricle and increased pulmonary venous congestion. The individual may again have symptoms of congestive heart failure. The left ventricle begins to dilate during this phase. This causes a dilatation of the mitral valve annulus, which may worsen the degree of mitral regurgitation. The dilated left ventricle causes an increase in the wall stress of the cardiac chamber as well. While the ejection fraction is less in the chronic decompensated phase than in the acute phase or the chronic compensated phase of mitral regurgitation, it may still be in the normal range (i.e.: > 50 percent), and may not decrease until late in the disease course. A decreased ejection fraction in an individual with mitral regurgitation and no other cardiac abnormality should alert the physician that the disease may be in its decompensated phase.

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Diagnosis
There are many diagnostic tests that have abnormal results in the presence of mitral regurgitation. These tests suggest the diagnosis of mitral regurgitation and may indicate to the physician that further testing is warranted. For instance, the electrocardiogram (ECG) in long standing mitral regurgitation may show evidence of left atrial enlargement and left ventricular hypertrophy. Atrial fibrillation may also be noted on the ECG in individuals with chronic mitral regurgitation. The ECG may not show any of these finding in the setting of acute mitral regurgitation.

Comparison of acute and chronic phases of mitral regurgitation

Acute Electrocardiogram Heart size Systolic murmur Apical thrill Normal Normal Chronic P mitrale, Atrial fibrillation, left ventricular hypertrophy Cardiomegaly, left atrial enlargement

Heard at the base, radiates to the neck, spine, or top of head Heard at the apex, radiates to the axilla May be absent Present Absent

Jugular venous distension Present

The quantification of mitral regurgitation usually employs imaging studies such as echocardiography or magnetic resonance angiography of the heart.

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Chest x-ray
The chest x-ray in individuals with chronic mitral regurgitation is characterized by enlargement of the left atrium and the left ventricle. The pulmonary vascular markings are typically normal, since pulmonary venous pressures are usually not significantly elevated.

Echocardiography
The echocardiogram is commonly used to confirm the diagnosis of mitral regurgitation. Color doppler flow on the transthoracic echocardiogram (TTE) will reveal a jet of blood flowing from the left ventricle into the left atrium during ventricular systole. Also, it may detect a dilated left atrium and ventricle and decreased left ventricular function.

transesophageal echocardiogram of mitral valve prolapse

Because of the inability in getting accurate images of the left atrium and the pulmonary veins on the transthoracic echocardiogram, a transesophageal echocardiogram may be necessary to determine the severity of the mitral regurgitation in some cases. Factors that suggest severe mitral regurgitation on echocardiography include systolic reversal of flow in the pulmonary veins and filling of the entire left atrial cavity by the regurgitant jet of MR.

Electrocardiography
P mitrale is broad, notched P waves in several or many leads with a prominent late negative component to the P wave in lead V1, and may be seen in mitral regurgitation, but also in mitral stenosis, and, potentially, any cause of overload of the left atrium.[15] Thus, P-sinistrocardiale may be a more appropriate term.

Quantification of mitral regurgitation

The degree of severity of mitral regurgitation can be quantified by the regurgitant fraction, which is the percentage of the left ventricular stroke volume that regurgitates into the left atrium. regurgitant fraction = where Vmitral and Vaortic are respectively the volumes of blood that flow forward through the mitral valve and aortic valve during a cardiac cycle. Methods that have been used to assess the regurgitant fraction in mitral regurgitation include echocardiography, cardiac catheterization, fast CT scan, and cardiac MRI. The echocardiographic technique to measure the regurgitant fraction is to determine the forward flow through the mitral valve (from the left atrium to the left ventricle) during ventricular diastole, and comparing it with the flow out of the left ventricle through the aortic valve in ventricular systole. This method assumes that the aortic valve does not suffer from aortic insufficiency. Another way to quantify the degree of mitral regurgitation is to determine the area of the regurgitant flow at the level of the valve. This is known as the regurgitant orifice area, and correlates with the size of the defect in the mitral valve. One particular echocardiographic technique used to measure the orifice area is measurement of the proximal isovelocity surface area (PISA). The flaw of using PISA to determine the mitral valve regurgitant orifice area is that it measures the flow at one moment in time in the cardiac cycle, which may not reflect the average performance of the regurgitant jet.

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Determination of the degree of mitral regurgitation

Degree of mitral regurgitation Mild mitral regurgitation Moderate mitral regurgitation Regurgitant fraction Regurgitant Orifice area < 20 percent 20 - 40 percent

Moderate to severe mitral regurgitation 40 - 60 percent Severe mitral regurgitation > 60 percent > 0.4cm2

Treatment
The treatment of mitral regurgitation depends on the acuteness of the disease and whether there are associated signs of hemodynamic compromise. In acute mitral regurgitation secondary to a mechanical defect in the heart (i.e. rupture of a papillary muscle or chordae tendineae), the treatment of choice is mitral valve surgery. If the patient is hypotensive prior to the surgical procedure, an intra-aortic balloon pump may be placed in order to improve perfusion of the organs and to decrease the degree of mitral regurgitation. If the individual with acute mitral regurgitation is normotensive, vasodilators may be of use to decrease the afterload seen by the left ventricle and thereby decrease the regurgitant fraction. The vasodilator most commonly used is nitroprusside. Individuals with chronic mitral regurgitation can be treated with vasodilators as well to decrease afterload. In the chronic state, the most commonly used agents are ACE inhibitors and hydralazine. Studies have shown that the use of ACE inhibitors and hydralazine can delay surgical treatment of mitral regurgitation. The current guidelines for treatment of mitral regurgitation limit the use of vasodilators to individuals with hypertension, however. Any hypertension is treated aggressively, e.g. by diuretics and a low sodium diet. In both hypertensive and normotensive cases, digoxin and antiarrhythmics are also indicated. Also, chronic anticoagulation is given where there is concomitant mitral valve prolapse or atrial fibrillation. Medical therapy is non-curative and is generally used for mild-to-moderate regurgitation or in patients who cannot tolerate surgery. Surgery is curative of mitral valve regurgitation. There are two surgical options for the treatment of mitral regurgitation: mitral valve replacement and mitral valve repair. Mitral valve repair is preferred to mitral valve replacement where a repair is feasible as bioprosthetic replacement valves have a limited lifespan of 10 to 15 years, whereas synthetic replacement valves require ongoing use of blood thinners to reduce the risk of stroke. There are two general categories of approaches to mitral valve repair: Resection of the prolapsed valvular segment (sometimes referred to as the 'Carpentier' approach), and installation of artificial chordae to "anchor" the prolapsed segment to the papillary muscle (sometimes referred to as the 'David' approach). With the resection approach, any prolapsing tissue is resected, in effect removing the hole through which the blood is leaking. In the artificial chordae approach, ePTFE (expanded polytetrafluoroethylene, or Gore-Tex sutures are used to replace the broken or stretched chordae tendonae, bringing the natural tissue back into the physiological position, thus restoring the natural anatomy of the valve. With both techniques, an annuloplasty ring is typically secured to the annulus, or opening of the mitral valve, to provide additional structural support. In some cases, the "double orifice" (or 'Alfieri') technique for mitral valve repair, the opening of the mitral valve is sewn closed in the middle, leaving the two ends still able to open. This ensures that the mitral valve closes when the left ventricle pumps blood, yet allows the mitral valve to open at the two ends to fill the left ventricle with blood before it pumps. Mitral valve surgery generally requires "open-heart" surgery in which the heart is arrested and the patient is placed on a heart-lung machine (cardiopulmonary bypass). This allows the complex surgery to proceed in a still environment.

Mitral regurgitation Due to the physiological stress associated with open-heart surgery, elderly and very sick patients may be subject to increased risk, and may not be candidates for this type of surgery. As a consequence, there are attempts to identify means of correcting mitral regurgitation on a beating heart. The Alfieri technique for instance, has been replicated using a percutaneous catheter technique which installs a clip to hold the middle of the mitral valve closed.

28

Surgery
Indications for surgery for chronic mitral regurgitation include signs of left ventricular dysfunction with ejection fraction less than 60%, severe pulmonary hypertension with pulmonary artery systolic pressure greater than 50mmHg at rest or 60mmHg during activity, and new onset atrial fibrillation.

Indications for surgery for chronic mitral regurgitation

Symptoms NYHA II NYHA III-IV Asymptomatic Asymptomatic with pulmonary hypertension > 30 percent < 30 percent 30 - 60 percent LV EF < 55mm > 55mm 40mm LVESD

LV EF > 60 percent and pulmonary artery systolic pressure >50-60mmHg < 40mm

Asymptomatic and chance for a repair without residual MR is >90% > 60 percent

Epidemiology
It has a prevalence of approximately 2% of the population, affecting males and females equally.[16] It is one of the two most common valvular heart disease in the elderly.[17]

References
[1] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I05. 1 [2] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I34. 0 [3] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ Q23. 3 [4] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=394. 1 [5] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=424. 0 [6] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=746. 6 [7] http:/ / www. diseasesdatabase. com/ ddb8275. htm [8] http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 000176. htm [9] http:/ / www. emedicine. com/ emerg/ topic314. htm [10] http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2013/ MB_cgi?field=uid& term=D008944 [11] Mitral valve regurgitation (http:/ / www. mountsinai. org/ Other/ Diseases/ Mitral valve regurgitation) at Mount Sinai Hospital [12] (http:/ / www. ncbi. nlm. nih. gov/ pubmedhealth/ PMH0002439) NIH US National Library of Medicine, A.D.A.M. Medical Encyclopedia, Ehlers Danlos Syndrome- PMH0002439 [13] Functional mitral regurgitation (http:/ / www. uptodate. com/ patients/ content/ topic. do?topicKey=~Ux3kbGWsXmiaqiH) By William H Gaasch, MD. Retrieved on Jul 8, 2010 [14] Harrison's Internal Medicine 17th edition [15] medilexicon.com < P mitrale (http:/ / www. medilexicon. com/ medicaldictionary. php?t=70229) Citing. Stedman's Medical Dictionary. Copyright 2006 [16] The Cleveland Clinic Center for Continuing Education > Mitral Valve Disease: Stenosis and Regurgitation (http:/ / www. clevelandclinicmeded. com/ medicalpubs/ diseasemanagement/ cardiology/ mitral-valve-disease/ ) Authors: Ronan J. Curtin and Brian P. Griffin. Retrieved September 2010 [17] Valvular heart disease in elderly adults (http:/ / www. uptodate. com/ patients/ content/ topic. do?topicKey=~PxxZxAzdJkaEgaZ) Authors: Dania Mohty, Maurice Enriquez-Sarano. Section Editors:Catherine M Otto, Kenneth E Schmader. Deputy Editor: Susan B Yeon. This topic last updated: April 20, 2007. Last literature review version 18.2: May 2010

Mitral regurgitation

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External links
MitraClip Film (http://www.realites-cardiologiques.com/film-mitraclip) Echocardiographic features of mitral regurgitation at Wikiecho (http://www.wikiecho.com/wiki/index. php?title=Mitral_regurgitation) Mitral Regurgitation information (http://heartcenter.seattlechildrens.org/conditions_treated/ mitral_valve_abnormalities.asp) from Seattle Children's Hospital Heart Center

Pulmonary valve stenosis

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Pulmonary valve stenosis

Pulmonary valve stenosis
Classification and external resources

Pulmonary valve stenosis ICD-10 ICD-9 OMIM DiseasesDB MedlinePlus eMedicine I37.0 424.3 [1] , I37.2 [2] , Q22.1 [3]

[4]

, 746.02

[5]

265500 11025

[6]

[7] [8] [9]

001096

emerg/491

Pulmonary valve stenosis is a heart valve disorder in which outflow of blood from the right ventricle of the heart is obstructed at the level of the pulmonic valve. This results in the reduction of flow of blood to the lungs. Valvular pulmonic stenosis accounts for 80% of right ventricular outflow tract obstruction. While the most common cause of pulmonary valve stenosis is congenital heart disease, it may also be due to rheumatic heart disease or a malignant carcinoid tumor. Both stenosis of the pulmonary artery and pulmonary valve stenosis are causes of pulmonic stenosis.

Symptoms and Signs

Symptoms include jugular vein distension, cyanosis (usually visible in the nailbeds), right ventricular hypertrophy, and general symptoms of lowered oxygenation of the blood. When the stenosis is mild, it can go unnoticed for many years and have no negative symptoms. If stenosis is severe, sudden fainting or dizziness many occur when exercising. An enlarged liver (hepatomegaly) and swelling in the legs (edema) may also be apparent.

Evaluation
The initial evaluation of pulmonary valve stenosis involves echocardiography. The degree of stenosis is typically determined by the peak pressure gradient across the valve. Pulmonary stenosis is mild if the valve area is larger than 1.0 cm2 per square meter and the trans-valvular gradient is 30-50 mmHg, or the peak RV systolic pressure is less than 75 mmHg. The stenosis is moderate if valve area is 0.5-1.0 cm2 per square meter, trans-valvular gradient is 50-75 mmHg, or right ventricle systolic pressure is 75-100 mmHg. It is severe when the valve area is less than 0.5 cm2, and the gradient is more than 75 mmHg.[10]

Pulmonary valve stenosis

31

Treatment
Valve replacement or surgical repair (depending upon whether the stenosis is in the valve or vessel) may be indicated. If the valve stenosis is of congenital origin, balloon valvuloplasty is another option, depending on the case.

References
[1] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I37. 0 [2] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I37. 2 [3] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ Q22. 1 [4] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=424. 3 [5] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=746. 02 [6] http:/ / omim. org/ entry/ 265500 [7] http:/ / www. diseasesdatabase. com/ ddb11025. htm [8] http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 001096. htm [9] http:/ / www. emedicine. com/ emerg/ topic491. htm [10] http:/ / www. rjmatthewsmd. com/ Definitions/ pulmonary_stenosis. htm

External links
Overview (http://www.americanheart.org/presenter.jhtml?identifier=11070) at American Heart Association Pulmonary Stenosis information (http://heartcenter.seattlechildrens.org/conditions_treated/ pulmonary_stenosis.asp) from Seattle Children's Hospital Heart Center Animation of pulmonary stenosis (http://www.aboutkidshealth.ca/En/ResourceCentres/ CongenitalHeartConditions/UnderstandingDiagnosis/DiagnosisofCongenitalHeartDefects/Pages/ Pulmonary-Stenosis.aspx) from AboutKidsHealth.ca Echocardiographic assessment of Pulmonary valve stenosis (http://www.wikiecho.com/wiki/index. php?title=Pulmonary_stenosis) from WikiEcho Xray and CT of Pulmonic Stenosis (http://rad.usuhs.edu/medpix/cow_image.html?pt_id=14213& imageid=58264&quiz=no) Radiologic Diagnosis

Tricuspid insufficiency

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Tricuspid insufficiency
Tricuspid insufficiency
Classification and external resources ICD-10 ICD-9 DiseasesDB MedlinePlus eMedicine MeSH I07.1 397.0 13348 [1] , I36.1 [2]

[3] [4] [5] [6]

000169

med/2314 D014262

[7]

Tricuspid insufficiency (TI), a valvular heart disease also called tricuspid regurgitation (TR), refers to the failure of the heart's tricuspid valve to close properly during systole. As a result, with each heart beat some blood passes from the right ventricle to the right atrium, the opposite of the normal direction. Tricuspid regurgitation occurs in roughly less than 1% of people and is usually asymptomatic.

Causes
Although congenital causes of tricuspid insufficiency exist, most cases are due to dilation of the right ventricle.[8] Such dilation leads to derangement of the normal anatomy and mechanics of the tricuspid valve and the muscles governing its proper function. The result is incompetence of the tricuspid valve. Left ventricular failure is, in turn, the most common cause of right ventricular dilation. Other common causes of right ventricular dilation include right ventricular infarction, inferior myocardial infarction and cor pulmonale. Other diseases can directly affect the tricuspid valve. The most common of these is rheumatic fever, which is a complication of untreated strep throat infections. It is usually accompanied by mitral and aortic valvular disease. Another condition directly harming the valve is tricuspid endocarditis. It may be found in those with a type of congenital heart disease called Ebstein's anomaly. Other infrequent causes of tricuspid regurgitation include: Carcinoid tumors, which release a hormone which damages the valve Connective tissue diseases such as Marfan syndrome Systemic lupus erythematosus Myxomatous degeneration Injury Rheumatoid arthritis Radiation therapy

Another important risk factor for tricuspid regurgitation is use of the diet medications called "Fen-Phen" (phentermine and fenfluramine) or dexfenfluramine

Tricuspid insufficiency

33

Symptoms and Signs

Symptoms Tricuspid insufficiency may be asymptomatic, especially if right ventricular function is well preserved. Symptoms are generally those of right-sided heart failure, such as ascites, hepatomegaly, edema and jugular venous distension Vague upper abdominal discomfort (from a congested liver), and fatigue (due to diminished cardiac output) can all be present to some degree. Signs On examination, the jugular venous pressure is usually elevated, and 'CV' waves can be seen. It features prominent V waves and rapid y descents in jugular venous pressure The liver may be enlarged and is often pulsatile (the latter finding being virtually diagnostic of tricuspid insufficiency). Peripheral edema is often found. In severe cases, there may be ascites and even cirrhosis (so-called 'cardiac cirrhosis'). Tricuspid insufficiency may lead to the presence of a pansystolic heart murmur. Such a murmur is usually of low frequency and best heard low on the lower left sternal border. It tends to increase with inspiration, and decrease with expiration and Valsalva maneuver. However, the murmur may be inaudible reflecting the relatively low pressures in the right side of the heart. A third heart sound may also be present, also heard best with inspiration at the left lower sternal border. Parasternal heave may be felt along the left lower sternal border as well. Atrial fibrillation is usually present.

Diagnosis
Diagnosis is usually made by echocardiography identifying tricuspid prolapse or flail. The finding of a pulsatile liver and/or the presence of prominent CV waves in the jugular pulse is also essentially diagnostic. Electrocardiography assists in the diagnosis, indicating enlargement of right ventricle and atrium.

Therapy
The main therapy is treatment of underlying cause. In most cases, surgery is not indicated since the root problem lies with a dilated or damaged right ventricle. Medical therapy with diuretics is the mainstay of treatment. Unfortunately, this can lead to volume depletion and decreased cardiac output. Indeed, one must often accept a certain degree of symptomatic tricuspid insufficiency in order to prevent a decrease in cardiac output. Treatment with medicines to reduce cardiac afterload may also be of benefit but a similar risk of depressed cardiac output applies. Where surgery has to be performed, the following alternatives are available: Tricuspid valvular repair Valvuloplasty Valve replacement (rarely performed)

Tricuspid insufficiency

34

References
[1] [2] [3] [4] [5] [6] [7] [8] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I07. 1 http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I36. 1 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=397. 0 http:/ / www. diseasesdatabase. com/ ddb13348. htm http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 000169. htm http:/ / www. emedicine. com/ med/ topic2314. htm http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2013/ MB_cgi?field=uid& term=D014262 Chapter 1: Diseases of the Cardiovascular system > Section: Valvular Heart Disease in:

External links
http://www.merck.com/mmpe/sec07/ch076/ch076i.html http://emedicine.medscape.com/article/158484-overview http://www.nlm.nih.gov/medlineplus/ency/article/000169.htm

Atrial septal defect

35

Atrial septal defect

Atrial septal defect
Classification and external resources

Illustration of an atrial septal defect. ICD-10 ICD-9 OMIM DiseasesDB Q21.1 745.5 [1] [2] -745.6 [3]

108800 1089

[4]

[5]

MedlinePlus 000157 [6] eMedicine MeSH med/3519 [7] article/894813 [9] [8]

C14.240.400.560.375

Atrial septal defect (ASD) is a form of congenital heart defect that enables blood flow between two compartments of the heart called the left and right atria. Normally, the right and left atria are separated by a septum called the interatrial septum. If this septum is defective or absent, then oxygen-rich blood can flow directly from the left side of the heart to mix with the oxygen-poor blood in the right side of the heart, or vice versa.[10] This can lead to lower-than-normal oxygen levels in the arterial blood that supplies the brain, organs, and tissues. However, an ASD may not produce noticeable signs or symptoms, especially if the defect is small. A "shunt" is the presence of a net flow of blood through the defect, either from left to right or right to left. The amount of shunting present, if any, determines the hemodynamic significance of the ASD (see Pathophysiology below). A "right-to-left-shunt" typically poses the more dangerous scenario. (see Pathophysiology below.) During development of the fetus, the interatrial septum develops to separate the left and right atria. However, a hole in the septum called the foramen ovale /fremnHelp:IPA for English#Keyovli/, allows blood from the right atrium to enter the left atrium during fetal development. This opening allows blood to bypass the nonfunctional fetal lungs while the fetus obtains its oxygen from the placenta. A layer of tissue called the septum primum acts as a valve over the foramen ovale during fetal development. After birth, the pressure in the right side of the heart drops as the lungs open and begin working, causing the foramen ovale to close entirely. In approximately 25% of adults, the foramen ovale does not entirely seal. In these cases, any elevation of the pressure in the pulmonary circulatory system (due to pulmonary hypertension, temporarily while coughing, etc.) can cause the foramen ovale to remain open. This is known as a patent foramen ovale (PFO), which is a type of atrial septal defect.

Atrial septal defect

36

Pathophysiology
In unaffected individuals, the chambers of the left side of the heart are under higher pressure than the chambers of the right side of the heart. This is because the left ventricle has to produce enough pressure to pump blood throughout the entire body, while the right ventricle needs only to produce enough pressure to pump blood to the lungs. In the case of a large ASD (>9mm), which may result in a clinically remarkable left-to-right shunt, blood will shunt from the left atrium to the right atrium. This extra blood from the left atrium may cause a volume overload of both the right atrium and the right ventricle. If untreated, this condition can result in enlargement of the right side of the heart and ultimately heart failure. Any process that increases the pressure in the left ventricle can cause worsening of the left-to-right shunt. This includes hypertension, which increases the pressure that the left ventricle has to generate in order to open the aortic valve during ventricular systole, and coronary artery disease which increases the stiffness of the left ventricle, thereby increasing the filling pressure of the left ventricle during ventricular diastole. The left-to-right shunt increases the filling pressure of the right heart (preload) and forces the right ventricle to pump out more blood than the left ventricle. This constant overloading of the right side of the heart will cause an overload of the entire pulmonary vasculature. Eventually, pulmonary hypertension may develop. The pulmonary hypertension will cause the right ventricle to face increased afterload. The right ventricle will be forced to generate higher pressures to try to overcome the pulmonary hypertension. This may lead to right ventricular failure (dilatation and decreased systolic function of the right ventricle). When the pressure in the right atrium rises to equal the pressure in the left atrium, there is no longer a pressure gradient between these heart chambers, and the left-to-right shunt will diminish or cease. In other words, there is no longer a net flow of blood across the ASD. If the ASD is left uncorrected, the pulmonary hypertension progresses and the pressure in the right side of the heart will become greater than the left side of the heart. This reversal of the pressure gradient across the ASD causes the shunt to reverse; a right-to-left shunt will exist. This phenomenon is known as Eisenmenger's syndrome. Once right-to-left shunting occurs, a portion of the oxygen-poor blood will get shunted to the left side of the heart and ejected to the peripheral vascular system. This will cause signs of cyanosis.

Heart of human embryo of about thirty-five days

Atrial septal defect with left-to-right shunt

Illustration depicting atrial septal defect.

Atrial septal defect

37

Epidemiology
As a group, atrial septal defects are detected in 1 child per 1500 live births. PFO are quite common (appearing in 1020% of adults) but asymptomatic and therefore undiagnosed. ASDs make up 30 to 40% of all congenital heart diseases that are seen in adults. The ostium secundum atrial septal defect accounts for 7% of all congenital heart lesions. This lesion shows a female preponderance, with a male:female ratio of 1:2.

Genetic research
In May 2013 team of researchers led by Professor Bernard Keavney of British Heart Foundation (BHF) discovered a new gene associated with the disease. According to them a common genetic variation near a gene called MSX1 is strongly associated with the risk of an ASD and this discovery of the particular gene is an important step forward as it will lead to better understanding of why some patients are born with ASD.

Types of atrial septal defects

There are many types of atrial septal defects. They are differentiated from each other by whether they involve other structures of the heart and how they are formed during the developmental process during early fetal development.

Ostium secundum atrial septal defect

The ostium secundum atrial septal defect is the most common type of atrial septal defect, and comprises 610% of all congenital heart diseases. The secundum atrial septal defect usually arises from an enlarged foramen ovale, inadequate growth of the septum secundum, or excessive absorption of the septum primum. Ten to twenty percent of individuals with ostium secundum ASDs also have mitral valve prolapse. If the ostium secundum ASD is accompanied by an acquired mitral valve stenosis, that is called Lutembacher's syndrome. Natural history Most individuals with an uncorrected secundum ASD do not have significant symptoms through early adulthood. More than 70 percent develop symptoms by about 40 years of age. Symptoms are typically decreased exercise tolerance, easy fatigueability, palpitations, and syncope. Complications of an uncorrected secundum ASD include pulmonary hypertension, right-sided heart failure, atrial fibrillation or flutter, stroke, and Eisenmenger's syndrome. While pulmonary hypertension is unusual before 20 years of age, it is seen in 50 percent of individuals above the age of 40. Progression to Eisenmenger's syndrome occurs in 5 to 10 percent of individuals late in the disease process. Patent foramen ovale A patent foramen ovale (PFO) is a small channel that has some hemodynamic consequence; it is a remnant of the fetal foramen ovale. Clinically it is linked to decompression sickness, paradoxical embolism and migraine. On echocardiography, there may not be any shunting of blood noted except when the patient coughs. There is debate within the neurology and cardiology communities about the role of a PFO in cryptogenic (i.e. of unknown cause) neurologic events such as strokes and transient ischemia attacks (TIAs) without any other potential cause. Some data suggested that PFOs may be involved in the pathogenesis of some migraine headaches. [citation needed] Wikipedia:Disputed statement Several clinical trials are currently underway to investigate the role of PFO in these clinical situations. [citation needed]

Atrial septal defect

38

Ostium primum atrial septal defect

A defect in the ostium primum is occasionally classified as an atrial septal defect, but it is more commonly classified as an atrioventricular septal defect. Ostium primum defects are less common than ostium secundum defects.

Sinus venosus atrial septal defect

A sinus venosus ASD is a type of atrial septum defect in which the defect in the septum involves the venous inflow of either the superior vena cava or the inferior vena cava. A sinus venosus ASD that involves the superior vena cava makes up 2 to 3% of all interatrial communication. It is located at the junction of the superior vena cava and the right atrium. It is frequently associated with anomalous drainage of the right-sided pulmonary veins into the right atrium (instead of the normal drainage of the pulmonary veins into the left atrium).

Common or single atrium

Common (or single) atrium is a failure of development of the embryologic components that contribute to the atrial septal complex. It is frequently associated with heterotaxy syndrome.

Mixed atrial septal defect

The inter atrial septum can be divided into 5 septal zones. If the defect involves 2 or more of the 5 septal zones, then the defect is termed a mixed atrial septal defect.

Ultrasound picture of the heart, seen in a subcostal view. The apex towards the right, atria to the left. ASD secundum seen as a discontinuation of the white band of the atrial septum. Enlarged right atrium below. Enlarged pulmonary veins seen entering left atrium above.

Atrial septal defect

39

Diagnosis
Diagnosis in children
Most individuals with a significant ASD are diagnosed in utero or in early childhood with the use of ultrasonography or auscultation of the heart sounds during physical examination.

Diagnosis in adults
Some individuals with an ASD will have undergone surgical correction of their ASD during childhood. The development of signs and symptoms due to an ASD are related to the size of the intracardiac shunt. Individuals with a larger shunt tend to present with symptoms at a younger age. Adults with an uncorrected ASD will present with symptoms of Abnormal chest X-ray as seen in a patient of dyspnea on exertion (shortness of breath with minimal exercise), Atrial septal defect congestive heart failure, or cerebrovascular accident (stroke). They may be noted on routine testing to have an abnormal chest x-ray or an abnormal ECG and may have atrial fibrillation. If the ASD causes a left-to-right shunt, the pulmonary vasculature in both lungs may appear dilated on chest x-ray, due to the increase in pulmonary blood flow. Physical exam auscultation of the heart The physical findings in an adult with an ASD include those related directly to the intracardiac shunt, and those that are secondary to the right heart failure that may be present in these individuals. Upon auscultation of the heart sounds, there may be a systolic ejection murmur that is attributed to the pulmonic valve. This is due to the increased flow of blood through the pulmonic valve rather than any structural abnormality of the valve leaflets. In unaffected individuals, there are respiratory variations in the splitting of the second heart sound (S2). During respiratory inspiration, the negative intrathoracic pressure causes increased blood return into the right side of the heart. The increased blood volume in the right ventricle causes the pulmonic valve to stay open longer during ventricular systole. This causes a normal delay in the P2 component of S2. During expiration, the positive intrathoracic pressure causes decreased blood return to the right side of the heart. The reduced volume in the right ventricle allows the pulmonic valve to close earlier at the end of ventricular systole, causing P2 to occur earlier. In individuals with an ASD, there is a fixed splitting of S2. The reason that there is a fixed splitting of the second heart sound is that the extra blood return during inspiration gets equalized between the left and right atrium due to the communication that exists between the atria in individuals with ASD. The right ventricle can be thought of as continuously overloaded because of the left to right shunt, producing a widely split S2. Because the atria are linked via the atrial septal defect, inspiration produces no net pressure change between them, and has no effect on the splitting of S2. Thus, S2 is split to the same degree during inspiration as expiration, and is said to be fixed.

Atrial septal defect Echocardiography In transthoracic echocardiography, an atrial septal defect may be seen on color flow imaging as a jet of blood from the left atrium to the right atrium. If agitated saline is injected into a peripheral vein during echocardiography, small air bubbles can be seen on echocardiographic imaging. It may be possible to see bubbles travel across an ASD either at rest or during a cough. (Bubbles will only flow from right atrium to left atrium if the RA pressure is greater than LA). Because better visualization of the atria is achieved with transesophageal echocardiography, this test may be performed in individuals with a suspected ASD which is not visualized on transthoracic imaging. Newer techniques to visualize these defects involve intracardiac imaging with special catheters that are typically placed in the venous system and advanced to the level of the heart. This type of imaging is becoming more common and involves only mild sedation for the patient typically. If the individual has adequate echocardiographic windows, it is possible to use the echocardiogram to measure the cardiac output of the left ventricle and the right ventricle independently. In this way, it is possible to estimate the shunt fraction using echocardiograpy. Transcranial Doppler (TCD) bubble study A less invasive method for detecting a PFO or other ASDs than transesophagal ultrasound is Transcranial Doppler with bubble contrast. This method reveals the cerebral impact of the ASD or PFO. Electrocardiogram The ECG findings in atrial septal defect vary with the type of defect the individual has. Individuals with atrial septal defects may have a prolonged PR interval (a first degree heart block). The prolongation of the PR interval is probably due to the enlargement of the atria that is common in ASDs and the increased distance due to the defect itself. Both of these can cause an increased distance of internodal conduction from the SA node to the AV node. In addition to the PR prolongation, individuals with a primum ASD have a left axis deviation of the QRS complex while those with a secundum ASD have a right axis deviation of the QRS complex. Individuals with a sinus venosus ASD exhibit a left axis deviation of the P wave (not the QRS complex). A common finding in the ECG is the presence of incomplete right bundle branch block. The presence of a right bundle branch block is so characteristic that if it is absent, the diagnosis of ASD should be reconsidered.

40

Treatment
Once someone is found to have an atrial septal defect, a determination of whether it should be corrected has to be made. Surgical mortality due to closure of an ASD is lowest when the procedure is performed prior to the development of significant pulmonary hypertension. The lowest mortality rates are achieved in individuals with a pulmonary artery systolic pressure of less than 40 mmHg. If Eisenmenger's syndrome has occurred, there is significant risk of mortality regardless of the method of closure of the ASD. In individuals who have developed Eisenmenger's syndrome, the pressure in the right ventricle has raised high enough to reverse the shunt in the atria. If the ASD is then closed, the afterload that the right ventricle has to act against has suddenly increased. This may cause immediate right ventricular failure, since it may not be able to pump the blood against the pulmonary hypertension. Closure of an ASD in individuals under age 25 has been shown to have a low risk of complications, and individuals have a normal lifespan (comparable to a healthy age-matched population). Closure of an ASD in individuals between the ages of 25 and 40 who are asymptomatic but have a clinically significant shunt is controversial. Those that perform the procedure believe that they are preventing long-term deterioration in cardiac function and preventing the

Atrial septal defect progression of pulmonary hypertension. Methods of closure of an ASD include surgical closure and percutaneous closure.

41

Evaluation prior to correction

Prior to correction of an ASD, an evaluation is made of the severity of the individual's pulmonary hypertension (If present at all) and whether it is reversible (Closure of an ASD may be recommended for prevention purposes, to avoid such a complication in the first place. Pulmonary hypertension is not always present in adults that are diagnosed with an ASD in adulthood). If pulmonary hypertension is present, the evaluation may include a right heart catheterization. This involves placing a catheter in the venous system of the heart and measuring pressures and oxygen saturations in the SVC, IVC, right atrium, right ventricle, pulmonary artery, and in the wedge position. Individuals with a pulmonary vascular resistance (PVR) of less than 7 wood units show regression of symptoms (including NYHA functional class). On the other hand, individuals with a PVR of greater than 15 wood units have increased mortality associated with closure of the ASD. If the pulmonary arterial pressure is more than 2/3 the systemic systolic pressure, there should be a net left-to-right shunt of at least 1.5:1 or evidence of reversibility of the shunt when given pulmonary artery vasodilators prior to surgery. (If Eisenmenger's physiology has set in, it must be proven that the right-to-left shunt is reversible with pulmonary artery vasodilators prior to surgery.)

Catheter procedure
Until the early 1990s, surgery was the usual method for closing all ASDs. Now, thanks to medical advances, doctors can use catheter procedures to close secundum ASDs, the most common type of ASD. For this procedure, the patient is given medicine so he or she will sleep through it and not feel any pain. During the procedure, the doctor inserts a catheter (a thin, flexible tube) into a vein in the groin (upper thigh) and threads it to the heart's septum. The catheter has a tiny umbrella-like device folded up inside it. When the catheter reaches the septum, the device is pushed out of the catheter and positioned so that it plugs the hole between the atria. The device is secured in place and the catheter is withdrawn from the body. Within 6 months, normal tissue grows in and over the device. There is no need to replace the closure device throughout the patient's life. Doctors often use echocardiography (echo) or transesophageal echo (TEE) as well as angiography to guide them in threading the catheter to the heart and closing the defect. TEE is a special type of echo that takes pictures of the heart through the esophagus (the passage leading from the mouth to the stomach). Catheter procedures are much easier on patients than surgery because they involve only a needle puncture in the skin where the catheter is inserted. This means that recovery is faster and easier. The outlook for patients having this procedure is excellent. Closures are successful in more than 9 out of 10 patients, with no significant leakage. Rarely, a defect is too large for catheter closure and surgery is needed.

Atrial septal defect

42

Surgical ASD closure

Surgical closure of an ASD involves opening up at least one atrium and closing the defect with a patch under direct visualization.

Percutaneous ASD closure

Percutaneous closure of an ASD is currently only indicated for the closure of secundum ASDs with a sufficient rim of tissue around the septal defect so that the closure device does not impinge upon the SVC, IVC, or the tricuspid or mitral valves. The Amplatzer Septal Occluder (ASO) is commonly used to close ASDs. The ASO consists of two self-expandable round discs connected to each other with a 4mm waist, made up of 0.0040.005 Nitinol wire mesh filled with Dacron fabric. Implantation of the device is relatively easy. The prevalence of residual defect is low. The disadvantages are a thick profile of the device and concern related to a large amount of nitinol (a nickel-titanium compound) in the device and consequent potential for nickel toxicity. Percutaneous closure is the method of choice in most centres.

Illustration depicting surgical patch closure of ASD

Complications
Due to the communication between the atria that occurs in ASDs, disease entities or complications from the condition, are possible. Patients with an uncorrected atrial septal defects may be at increased risk for developing a cardiac arrhythmia, as well as more frequent respiratory infections.

Decompression sickness
ASDs, and particularly PFOs, are a predisposing risk factor for decompression sickness in divers because a proportion of venous blood carrying inert gases, such as helium or nitrogen does not pass through the lungs. The only way to release the excess inert gases from the body is to pass the blood carrying the inert gases through the lungs to be exhaled. If some of the inert gas-laden blood passes through the PFO, it avoids the lungs and the inert gas is more likely to form large bubbles in the arterial blood stream causing decompression sickness.

Eisenmenger's syndrome
If a net flow of blood exists from the left atrium to the right atrium, called a left-to-right shunt, then there is an increase in the blood flow through the lungs. Initially, this increased blood flow is asymptomatic, but if it persists, the pulmonary blood vessels may stiffen, causing pulmonary hypertension. The pulmonary hypertension increases the pressures in the right side of the heart, leading to the reversal of the shunt into a right-to-left shunt. Once the reversal of the shunt occurs, and the blood begins flowing in the opposite direction through the ASD, that is called Eisenmenger's syndrome. The syndrome is a rare and late complication of an ASD.

Atrial septal defect

43

Paradoxical embolus
Venous thrombi (clots in the veins) are quite common. Embolizations (dislodgement of thrombi) normally go to the lung and cause pulmonary emboli. In an individual with ASD, these emboli can potentially enter the arterial system. This can cause any phenomenon that is attributed to acute loss of blood to a portion of the body, including cerebrovascular accident (stroke), infarction of the spleen or intestines, or even a distal extremity (i.e., finger or toe). This is known as a paradoxical embolus because the clot material paradoxically enters the arterial system instead of going to the lungs.

Migraine
Some recent research has suggested that a proportion of cases of migraine may be caused by patent foramen ovale. While the exact mechanism remains unclear, closure of a PFO can reduce symptoms in certain cases. This remains controversial. 20% of the general population have a PFO, which for the most part, is asymptomatic. 20% of the female population have migraines. And, the placebo effect in migraine typically averages around 40%. The high frequency of these facts makes statistically significant relationships between PFO and migraine difficult (i.e., the relationship may just be chance or coincidence). In a large randomized controlled trial the higher prevalence of patent foramen ovale in migraine patients was confirmed, but migraine headache cessation was not more prevalent in the group of migraine patients that underwent closure of their patent foramen ovale.

Associated conditions
Down Syndrome - patients with Down Syndrome have higher rates of ASDs, especially a particular type that involves the ventricular wall. As many as one half of Down Syndrome patients have some type of septal defect. Ebstein's anomaly Fetal alcohol syndrome - about one in four patients with fetal alcohol syndrome has either an ASD or a ventricular septal defect. HoltOram syndrome - Both the osteium secundum and osteum primum types of ASD are associated with HoltOram syndrome Lutembacher's syndrome - the presence of a congenital ASD along with acquired mitral stenosis.

References
[1] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ Q21. 1 [2] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=745. 5 [3] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=745. 6 [4] http:/ / omim. org/ entry/ 108800 [5] http:/ / www. diseasesdatabase. com/ ddb1089. htm [6] http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 000157. htm [7] http:/ / www. emedicine. com/ med/ topic3519. htm [8] http:/ / emedicine. medscape. com/ article/ 894813-overview [9] http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2013/ MB_cgi?mode=& term=Atrial+ Septal+ Defects& field=entry#TreeC14. 240. 400. 560. 375 [10] Atrial septal defect (http:/ / www. mountsinai. org/ patient-care/ health-library/ diseases-and-conditions/ atrial-septal-defect) at Mount Sinai Hospital

This article incorporatespublic domain material from the United States Department of Health and Human Services document "National Heart, Lung, and Blood Institute" (http:/ / www. nhlbi. nih. gov/ health/ dci/ Diseases/ holes/holes_treatments.html).

Atrial septal defect

44

Additional references
Goldman, Lee (2011). Goldman's Cecil Medicine (24th ed ed.). Philadelphia: Elsevier Saunders. pp.270, 400401. ISBN1437727883.

External links
Pediatric Heart Surgery (http://www.pediatricheartsurgery.com) The Congenital Heart Surgery Video Project (http://www.youtube.com/user/Redmond111) Pediatric Cardiac Surgery: Atrial Septal Defect Repair (http://www.youtube.com/watch?v=PbQhiv6OB0E) Atrial septal defect (http://www.gosh.nhs.uk/medical-conditions/search-for-medical-conditions/ atrioventricular-septal-defect/) information for parents.

Functional murmur
A functional murmur (innocent murmur, physiologic murmur) is a heart murmur that is primarily due to physiologic conditions outside the heart, as opposed to structural defects in the heart itself. Serious conditions can arise even in the absence of a primary heart defect, and it is possible for peripheral conditions to generate abnormalities in the heart. Therefore, caution should be applied to use of the terms "innocent" or "benign" in this context. Use of the term dates to the mid 19th century.

Benign pediatric heart murmur

Functional murmurs are an important consideration in the precordial examination of an infant or child.

Characteristics
Soft, less than 3/6 in intensity (although note that even when structural heart disease is present, intensity does not predict severity.) Often position-dependent. Murmurs heard while supine and may disappear when upright or sitting.[1] Otherwise healthy individual, no concerns about growth, no symptoms of heart failure such as dyspnea on exertion. (In infants, ask if the baby tires during feeding, becomes diaphoretic, or develops a rapid respiratory rate. In older children, this can be elucidated by asking whether or not the child can keep up with peers during play.) Occurs during systole or continuously during both systole and diastole. (Murmurs occurring only during diastole are always pathologic.) Physiologic splitting of S2 (A2 and P2 components should only be resolvable during inspiration and should merge during expiration.) No palpable thrill (A thrill is a vibration caused by turbulent blood flow.)

Functional murmur

45

Prognosis
Innocent murmurs are inconsequential and usually disappear as the child grows. ECG and Chest XRAY are normal.

Types, description and DDx Benign Paediatric Heart Murmurs

Name Still's murmur Location inferior aspect of LLSB (lower left sternal border), systolic ejection sound, vibratory/musical quality superior aspect of LLSB, ejection sound DDx subaortic stenosis, small VSD Pulmonary stenosis, atrial septal defect PDA

Pulmonary ejection

Venous hum

Infraclavicular throughout the cardiac cycle (right side > left side), diminishes with jugular vein palpation or neck turning

Supraclavicular arterial bruit (Systemic Above clavicles Flow Murmur) Peripheral pulmonary stenosis (Pulmonary flow murmur) High-pitch with radiation to back and armpit

aortic stenosis, bicuspid aortic valve PDA, pulmonary stenosis

In the adult, hyperdynamic circulation of the blood may also produce a functional murmur, such in anemia or thyrotoxicosis.

References
[1] Thomas Biancaniello. Innocent Murmurs (http:/ / circ. ahajournals. org/ content/ 111/ 3/ e20. full) Circulation. 2005; 111: e20-e22.

5. Circulation 2005: Innocent Murmurs http://circ.ahajournals.org/cgi/content/full/111/3/e20

External links
Heart Murmurs in Pediatric Patients: When Do You Refer? (http://www.aafp.org/afp/990800ap/558.html) AAFP

Aortic insufficiency

46

Aortic insufficiency
Aortic insufficiency
Classification and external resources

Micrograph of myxomatous degeneration a cause of aortic insufficiency. ICD-10 ICD-9 DiseasesDB MedlinePlus eMedicine MeSH I06 [1] , I35 [2] , Q23.1 [5] [3]

395.1 829

[4]

, 746.4

[6] [7] [8] emerg/39 [9] ped/2487 [10]

000179

med/156 D001022

[11]

Aortic insufficiency (AI), also known as aortic regurgitation (AR), is the leaking of the aortic valve of the heart that causes blood to flow in the reverse direction during ventricular diastole, from the aorta into the left ventricle.[12] Aortic insufficiency can be due to abnormalities of either the aortic valve or the aortic root (the beginning of the aorta).

Etiology
About half of the cases of aortic insufficiency are due to the aortic root dilatation (annuloaortic ectasia), which is idiopathic in over 80% of cases, but otherwise may result from aging, syphilitic aortitis, osteogenesis imperfecta, aortic dissection, Behet's disease, reactive arthritis and systemic hypertension.[13] In about 15% the cause is innate bicuspidal aortic valve, while another 15% cases are due to retraction of the cusps as part of postinflammatory processes of endocarditis in rheumatic fever and various collagen vascular diseases. Additionally, aortic insufficiency has been linked to the use of some medications, specifically medications containing fenfluramine or dexfenfluramine isomers and dopamine agonists. Other potential causes that affects the valve directly include: Marfan's syndrome, EhlersDanlos syndrome, ankylosing spondylitis and systemic lupus erythematosus. In acute cases of aortic insufficiency, the main causes are infective endocarditis,[14] aortic dissection or trauma.

Aortic insufficiency

47

Physiology
In individuals with a normally functioning aortic valve, the valve is only open when the pressure in the left ventricle is higher than the pressure in the aorta. This allows the blood to be ejected from the left ventricle into the aorta during ventricular systole. The amount of blood that is ejected by the heart is known as the stroke volume. Under normal conditions, 5070% of the blood in a filled left ventricle is ejected into the aorta to be used by the body (called the 'ejection fraction'). After ventricular systole, the pressure in the left ventricle decreases as it relaxes and begins to fill up with blood from the left atrium. This relaxation of the left ventricle (early ventricular diastole) causes a fall in its pressure. When the pressure in the left ventricle falls below the pressure in the aorta, the aortic valve will close, preventing blood in the aorta from going back into the left ventricle.

Pathophysiology
In aortic insufficiency (AI), when the pressure in the left ventricle falls below the pressure in the aorta, the aortic valve is not able to completely close. This causes a leaking of blood from the aorta into the left ventricle. This means that some of the blood that was already ejected from the heart is regurgitating back into the heart. The percentage of blood that regurgitates back through the aortic valve due to AI is known as the regurgitant fraction. For instance, if an individual with AI has a stroke volume of 100 ml and during ventricular diastole 25 ml regurgitates back through the aortic valve, the regurgitant fraction is 25%. This regurgitant flow causes a decrease in the diastolic blood pressure in the aorta, and therefore an increase in the pulse pressure (systolic pressure diastolic pressure). Thus, physical examination will reveal a bounding pulse, especially in the radial artery. Since some of the blood that is ejected during systole regurgitates back into the left ventricle during diastole, there is decreased effective forward flow in AI.
Illustration depicting aortic regurgitation

Note that while diastolic blood pressure is diminished and the pulse pressure widens, systolic blood pressure generally remains normal or can even be slightly elevated. This is because sympathetic nervous system and the renin-angiotensin-aldosterone axis of the kidneys compensate for the decreased cardiac output. Catecholamines will increase the heart rate and increase the strength of ventricular contraction, directly increasing cardiac output. Catecholamines will also cause peripheral vasoconstriction, which causes increased systemic vascular resistance and ensures that core organs are adequately perfused. Renin, a proteolytic enzyme, cleaves angiotensinogen to angiotensin I, which is converted to angiotensin II, which is also a potent vasoconstrictor. In the case of chronic aortic insufficiency with resultant cardiac remodeling, heart failure will develop, and it is possible to see systolic pressures diminish. Aortic insufficiency causes both volume overload (elevated preload) and pressure overload (elevated afterload due to increased stroke volume) of the heart. The volume overload (due to elevated pulse pressure and the systemic effects of neuroendocrine hormones) causes left ventricular hypertrophy (LVH). There is both concentric hypertrophy and eccentric hypertrophy in AI. The concentric hypertrophy is due to the increased left ventricular pressure overload associated with AI, while the eccentric hypertrophy is due to volume overload caused by the regurgitant fraction.

Aortic insufficiency

48

Hemodynamics
The hemodynamic sequelae of AI are dependent on the rate of onset of AI. Acute AI and chronic AI will have different hemodynamics and individuals will have different signs and symptoms.

Acute aortic insufficiency

In acute AI, as may be seen with acute perforation of the aortic valve due to endocarditis, there will be a sudden increase in the volume of blood in the left ventricle. The ventricle is unable to deal with the sudden change in volume. In terms of the Frank-Starling curve, the end-diastolic volume will be very high, such that further increases in volume result in less and less efficient contraction. The filling pressure of the left ventricle will increase. This causes pressure in the left atrium to rise, and the individual will develop pulmonary edema. Severe acute aortic insufficiency is considered a medical emergency. There is a high mortality rate if the individual does not undergo immediate surgery for aortic valve replacement. If the acute AI is due to aortic valve endocarditis, there is a risk that the new valve may become seeded with bacteria. However, this risk is small. Acute AI usually presents as florid congestive heart failure, and will not have any of the signs associated with chronic AI since the left ventricle had not yet developed the eccentric hypertrophy and dilatation that allow an increased stroke volume, which in turn cause bounding peripheral pulses. On auscultation, there may be a short diastolic murmur and a soft S1. S1 is soft because the elevated filling pressures close the mitral valve in diastole (rather than the mitral valve being closed at the beginning of systole).

Chronic aortic insufficiency

If the individual survives the initial hemodynamic derailment that acute AI presents as, the left ventricle adapts by eccentric hypertrophy and dilatation of the left ventricle, and the volume overload is compensated for. The left ventricular filling pressures will revert to normal and the individual will no longer have overt heart failure. In this compensated phase, the individual may be totally asymptomatic and may have normal exercise tolerance. Eventually (typically after a latency period) the left ventricle will become decompensated, and filling pressures will increase. While most individuals would complain of symptoms of congestive heart failure to their physicians, some enter this decompensated phase asymptomatically. Proper treatment for AI involves aortic valve replacement prior to this decompensation phase.

Aortic insufficiency

49

Symptoms
Symptoms of aortic insufficiency are similar to those of heart failure and include dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea. Palpitations and angina pectoris may also be felt. In acute cases there may be cyanosis and circulatory shock.

Physical examination
The physical examination of an individual with aortic insufficiency involves auscultation of the heart to listen for the murmur of aortic insufficiency and the S3 heart sound (S3 gallop correlates with development of LV dysfunction). The murmur of chronic aortic insufficiency is typically described as early diastolic and decrescendo, which is best heard in the third left intercostal space and may radiate along the left sternal border. If there is increased stroke volume of the left ventricle due to volume overload, an ejection systolic 'flow' murmur may also be present when auscultating the same aortic area. Unless there is concomitant aortic valve stenosis, the murmur should not start with an ejection click. There may also be an Austin Flint murmur, a soft mid-diastolic rumble heard at the apical area. It appears when regurgitant jet from the severe aortic insufficiency renders partial closure of the anterior mitral leaflet. Peripheral physical signs of aortic insufficiency are related to the high Phonocardiograms from normal and abnormal pulse pressure and the rapid decrease in blood pressure during diastole heart sounds due to blood returning to the heart (the wrong way) from the aorta through the incompetent aortic valve, although the usefulness of some of the eponymous signs has been questioned: large-volume, 'collapsing' pulse also known as: Watson's water hammer pulse Corrigan's pulse (rapid upstroke and collapse of the carotid artery pulse) low diastolic and increased pulse pressure de Musset's sign (head nodding in time with the heart beat) Quincke's sign (pulsation of the capillary bed in the nail; named for Heinrich Quincke) Traube's sign (a 'pistol shot' systolic sound heard over the femoral artery; named for Ludwig Traube) Duroziez's sign (systolic and diastolic murmurs heard over the femoral artery when it is gradually compressed with the stethoscope)

Also, these are usually less detectable in acute cases. Less used signs include:[15] Lighthouse sign (blanching & flushing of forehead) Landolfi's sign (alternating constriction & dilatation of pupil) Becker's sign (pulsations of retinal vessels) Mller's sign (pulsations of uvula) Mayne's sign (diastolic drop of BP>15mm Hg with arm raised) Rosenbach's sign (pulsatile liver) Gerhardt's sign (enlarged spleen)

Hill's sign - a 20 mmHg difference in popliteal and brachial systolic cuff pressures, seen in chronic severe AI. Considered to be an artefact of sphygmomanometric lower limb pressure measurement.

Aortic insufficiency Lincoln sign (pulsatile popliteal) Sherman sign (dorsalis pedis pulse is quickly located & unexpectedly prominent in age>75 yr) Ashrafian sign (Pulsatile pseudo-proptosis) Unfortunately, none of the above putative signs of aortic insufficiency is of utility in making the diagnosis, but they may help as pointers. What is of value is hearing a diastolic murmur itself, whether or not the above signs are present.

50

Diagnostic evaluation
The most common test used for the evaluation of the severity of aortic insufficiency is transthoracic echocardiography, which can provide two-dimensional views of the regurgitant jet, allow measurement of velocity using Doppler, and estimate jet volume. The findings in severe aortic regurgitation, based on the 2006 American College of Cardiology/American Heart Association guidelines include: An AI color jet width > 65 percent of the left ventricular outflow tract (LVOT) diameter (may not be true if the jet is eccentric) Doppler vena contracta width > 0.6cm The pressure half-time of the regurgitant jet is < 250 msec Early termination of the mitral inflow (due to increase in LV pressure due to the AI.) Holodiastolic flow reversal in the descending aorta. Regurgitant volume > 60 ml Regurgitant fraction > 50 percent Regurgitant orifice area > 0.3cm2 Increased left ventricular size

In acute aortic regurgitation, echocardiography may show early closure of the mitral valve. Chest X-ray can assist in making the diagnosis, showing left ventricular hypertrophy and dilated aorta. ECG typically indicates left ventricular hypertrophy. Cardiac chamber catheterization assists in assessing the severity of regurgitation and any left ventricular dysfunction.

Prognosis
The risk of death in individuals with aortic insufficiency, dilated ventricle, normal ejection fraction who are asymptomatic is about 0.2 percent[citation needed] per year. Risk increases if the ejection fraction decreases or if the individual develops symptoms.

Treatment

Aortic insufficiency

51

Indications for surgery for chronic severe aortic insufficiency

Symptoms Present (NYHA II-IV) Absent Any Ejection fraction Additional Findings

> 50%

Abnormal exercise test, severe LV dilatation (systolic ventricular diameter >55mm)

Absent

<=50 %

Cardiac surgery for other cause (i.e.: CAD, other valvular disease, ascending aortic aneurysm)

Aortic insufficiency can be treated either medically or surgically, depending on the acuteness of presentation, the symptoms and signs associated with the disease process, and the degree of left ventricular dysfunction. Surgical treatment is controversial in asymptomatic patients, however has been recommended if the ejection fraction falls to 50% or below, in the face of progressive and severe left ventricular dilatation, or with symptoms or abnormal response to exercise testing. For both groups of patients, surgery before the development of worsening ejection fraction/LV dilatation, is expected to reduce the risk of sudden death, and is associated with lower peri-operative mortality. Also, surgery is optimally performed immediately in acute cases.

Medical treatment
Medical therapy of chronic aortic insufficiency that is stable and asymptomatic involves the use of vasodilators. Small trials have shown a short term benefit in the use of ACE inhibitors or angiotensin II receptor antagonists, nifedipine, and hydralazine in improving left ventricular wall stress, ejection fraction, and mass. The use of these vasodilators is only indicated in individuals who suffer from hypertension in addition to AI. The goal in using these pharmacologic agents is to decrease the afterload so that the left ventricle is somewhat spared. The regurgitant fraction may not change significantly, since the gradient between the aortic and left ventricular pressures is usually fairly low at the initiation of treatment. Other rather conservative medical treatments for stable and asymptomatic cases include low sodium diet, diuretics, digoxin, calcium blockers and avoiding very strenuous activity. As of 2007, the American Heart Association no longer recommends antibiotics for endocarditis prophylaxis before dental procedures in patients with aortic insufficiency. Antibiotic prophylaxis to prevent endocarditis before gastrointestinal or genitourinary procedures is no longer recommended for any patient with valvular disease. In mild to moderate cases, echocardiography and cardiac stress test should be followed up every 12 years. In severe moderate/severe cases, echocardiography with cardiac stress test and/or isotope perfusion imaging should be performed every 36 months.

Surgical treatment
The surgical treatment of choice at this time is an aortic valve replacement. This is currently an open-heart procedure, requiring the individual to be placed on cardiopulmonary bypass. In the case of severe acute aortic insufficiency, all individuals should undergo surgery if there are no absolute contraindications for surgery. Individuals with bacteremia with aortic valve endocarditis should not wait for treatment with antibiotics to take effect, given the high mortality associated with the acute AI. Instead, replacement with an aortic valve homograft should be performed if feasible. A percutaneous approach to aortic valve replacement is now feasible, but the main experience has been in the treatment of aortic stenosis.

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52

References
[1] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I06 [2] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I35 [3] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ Q23. 1 [4] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=395. 1 [5] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=746. 4 [6] http:/ / www. diseasesdatabase. com/ ddb829. htm [7] http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 000179. htm [8] http:/ / www. emedicine. com/ med/ topic156. htm [9] http:/ / www. emedicine. com/ emerg/ topic39. htm# [10] http:/ / www. emedicine. com/ ped/ topic2487. htm# [11] http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2013/ MB_cgi?field=uid& term=D001022 [12] Aortic regurgitation (http:/ / www. mountsinai. org/ Other/ Diseases/ Aortic regurgitation) at Mount Sinai Hospital [13] Chapter 1: Diseases of the Cardiovascular system > Section: Valvular Heart Disease in: [14] VOC=VITIUM ORGANICUM CORDIS, a compendium of the Department of Cardiology at Uppsala Academic Hospital. By Per Kvidal September 1999, with revision by Erik Bjrklund May 2008 [15] Ashrafian H. Pulsatile pseudo-proptosis, aortic regurgitation and 31 eponyms. Int J Cardiol. 2006 Mar 8;107(3):421-3.

External links
Heart Disease and Life After (http://www.protraineronline.com/past/JunJul06/article3.cfm) by Richard Hinkle Additional resource on the Aortic Valve (http://www.thekeyholeheartclinic.com/procedures/ aortic-valve-surgery/)

Mitral stenosis

53

Mitral stenosis
Mitral stenosis
Classification and external resources

Mitral stenosis with marked thickening of the leaflets and left atrial hypertrophy. Superior view. Autopsy preparation. ICD-10 ICD-9 DiseasesDB MedlinePlus eMedicine MeSH I05.0 394.0 8288 [1] , I34.2 [2] , Q23.2 , 746.5 [3] [5]

[4] [6]

, 396.0

[5]

000175

[7] [8] ped/2517 [9]

emerg/315 D008946

[10]

Mitral stenosis is a valvular heart disease characterized by the narrowing of the orifice of the mitral valve of the heart.

Signs and symptoms

Symptoms of mitral stenosis include: Heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea (PND) Palpitations Chest pain Hemoptysis Thromboembolism in later stages when the left atrial volume is increased (i.e. dilation). The latter leads to increase risk of atrial fibrillation of which increases the risk of blood stasis (motionless). This increases the risk of coagulation.
Illustration of mitral stenosis, with close-up on mitral valve

Ascites and edema and hepatomegaly (if right-sided heart failure develops) Symptoms increase with exercise and pregnancy Fatigue wasting (weakness)

Mitral stenosis

54

Cause
Almost all cases of mitral stenosis are due to disease in the heart secondary to rheumatic fever and the consequent rheumatic heart disease.[13] Uncommon causes of mitral stenosis are [11][12] calcification of the mitral valve leaflets, and as a form of congenital heart disease. However, there are primary causes of mitral stenosis that emanate from a cleft mitral valve.[citation needed]

Rheumatic heart disease at autopsy with characteristic findings (thickened mitral valve, thickened chordae tendineae, hypertrophied left ventricular myocardium).

It is the most common valvular heart disease in pregnancy Other causes include infective endocarditis where the vegetations may favor increase risk of stenosis.

Pathophysiology
The normal area of the mitral valve orifice is about 4 to 6cm2. In normal cardiac physiology, the mitral valve opens during left ventricular diastole, to allow blood to flow from the left atrium to the left ventricle. A normal mitral valve will not impede the flow of blood from the left atrium to the left ventricle during (ventricular) diastole, and the pressures in the left atrium and the left ventricle during ventricular diastole will be equal. The result is that the left ventricle gets filled with blood during early ventricular diastole, with only a small portion of extra blood contributed by contraction of the left atrium (the "atrial kick") during late ventricular diastole.[citation needed] When the mitral valve area goes below 2cm2, the valve causes an impediment to the flow of blood into the left ventricle, creating a Intracardiac pressure measurements in an pressure gradient across the mitral valve. This gradient may be individual with severe mitral stenosis. Pressure increased by increases in the heart rate or cardiac output. As the tracings in the left atrium (LA) and the left ventricle (LV) in an individual with severe mitral gradient across the mitral valve increases, the amount of time stenosis. Blue areas represent the diastolic necessary to fill the left ventricle with blood increases. Eventually, the pressure gradient due to the stenotic valve. left ventricle requires the atrial kick to fill with blood. As the heart rate increases, the amount of time that the ventricle is in diastole and can fill up with blood (called the diastolic filling period) decreases. When the heart rate goes above a certain point, the diastolic filling period is insufficient to fill the ventricle with blood and pressure builds up in the left atrium, leading to pulmonary congestion.[citation needed] When the mitral valve area goes less than 1cm2, there will be an increase in the left atrial pressures (required to push blood through the stenotic valve). Since the normal left ventricular diastolic pressures is about 5 mmHg, a pressure gradient across the mitral valve of 20 mmHg due to severe mitral stenosis will cause a left atrial pressure of about 25 mmHg. This left atrial pressure is transmitted to the pulmonary vasculature and causes pulmonary hypertension. Pulmonary capillary pressures in this level cause an imbalance between the hydrostatic pressure and the oncotic

Mitral stenosis pressure, leading to extravasation of fluid from the vascular tree and pooling of fluid in the lungs (congestive heart failure causing pulmonary edema).[citation needed] The constant pressure overload of the left atrium will cause the left atrium to increase in size. As the left atrium increases in size, it becomes more prone to develop atrial fibrillation. When atrial fibrillation develops, the atrial kick is lost (since it is due to the normal atrial contraction).[citation needed] In individuals with severe mitral stenosis, the left ventricular filling is dependent on the atrial kick. The loss of the atrial kick due to atrial fibrillation can cause a precipitous decrease in cardiac output and sudden congestive heart failure.[citation needed] Patients with mitral stenosis prompts a series of hemodynamic changes that frequently cause deterioration of the patient's clinical status. A reduction in cardiac output, associated with acceleration of heart rate and shortening of the diastolic time, frequently leads to congestive heart failure. In addition, when AF sets in, systemic embolization becomes a real danger.[13] Mitral stenosis typically progresses slowly (over decades) from the initial signs of mitral stenosis to NYHA functional class II symptoms to the development of atrial fibrillation to the development of NYHA functional class III or IV symptoms. Once an individual develops NYHA class III or IV symptoms, the progression of the disease accelerates and the patient's condition deteriorates.[citation needed]

55

Diagnosis
Physical examination
Upon auscultation of an individual with mitral stenosis, the first heart sound is usually loud and may be palpable (tapping apex beat) because of increased force in closing the mitral valve. The first heart sound is made by the mitral and tricuspid heart valves closing. These are normally synchronous, and the sounds are termed M1 and T1 respectively. M1 becomes louder in mitral stenosis. It may be the most prominent sign. If pulmonary hypertension secondary to mitral stenosis is severe, the P2 (pulmonic) component of the second heart sound (S2) will become loud.[citation needed] An opening snap which is a high pitched additional sound may be heard after the A2 (aortic) component of the second heart sound (S2), which correlates to the forceful opening of the mitral valve. The mitral valve opens when the pressure in the left atrium is greater than the pressure in the left ventricle. This happens in ventricular diastole (after Phonocardiograms from normal and abnormal closure of the aortic valve), when the pressure in the ventricle heart sounds precipitously drops. In individuals with mitral stenosis, the pressure in the left atrium correlates with the severity of the mitral stenosis. As the severity of the mitral stenosis increases, the pressure in the left atrium increases, and the mitral valve opens earlier in ventricular diastole. A mid-diastolic rumbling murmur with presystolic accentuation will be heard after the opening snap. The murmur is best heard at the apical region and is not radiated. Since it is low-pitched it is heard best with the bell of the stethoscope. Its duration increases with worsening disease. Rolling the patient towards left, as well as isometric exercise will accentuate the murmur. A thrill might be present when palpating at the apical region of the precordium.[citation needed]

Mitral stenosis Advanced disease may present with signs of right-sided heart failure such as parasternal heave, jugular venous distension, hepatomegaly, ascites and/or pulmonary hypertension, the latter often presenting with a loud P2. Almost all signs increase with exercise and pregnancy. Other peripheral signs include: Malar flush - due to back pressure and build up of carbon dioxide (CO2). CO2 is a natural vasodilator. Atrial fibrillation - irregular pulse and loss of 'a' wave in jugular venous pressure Left parasternal heave - presence of right ventricular hypertrophy due to pulmonary hypertension Tapping apex beat which is not displaced

56

Medical signs of atrial fibrillation include:[citation needed] Heart rate is about 100-150/min. Irregularly irregular pulse with a pulse deficit>10. varying first heart sound intensity. Opening snap is not heard sometimes. Absent a waves in the neck veins. Presystolic accentuation of diastolic murmur disappears. Embolic manifestations may appear.

Echocardiography Severity of mitral stenosis

Degree of mitral stenosis Mean gradient Mitral valve area Mild mitral stenosis Moderate mitral stenosis Severe mitral stenosis <5 mmHg 5 - 10 mmHg > 10 mmHg >1.5cm2 1.0 - 1.5cm2 < 1.0cm2

In most cases, the diagnosis of mitral stenosis is most easily made by echocardiography, which shows left atrial enlargement, thick and calcified mitral valve with narrow and "fish-mouth"-shaped orifice and signs of right ventricular failure in advanced disease. It can also show decreased opening of the mitral valve leaflets, and increased blood flow velocity during diastole. The trans-mitral gradient as measured by Doppler echocardiography is the gold standard in the evaluation of the severity of mitral stenosis.[citation needed]

Cardiac chamber catheterization

Another method of measuring the severity of mitral stenosis is the simultaneous left and right heart chamber catheterization. The right heart catheterization (commonly known as Swan-Ganz catheterization) gives the physician the mean pulmonary capillary wedge pressure, which is a reflection of the left atrial pressure. The left heart catheterization, on the other hand, gives the pressure in the left ventricle. By simultaneously taking these pressures, it is possible to determine the gradient between the left atrium and left ventricle during ventricular diastole, which is a marker for the severity of mitral stenosis. This method of evaluating mitral stenosis tends to overestimate the degree of mitral stenosis, however, because of the time lag in the pressure tracings seen on the right heart catheterization and the slow Y descent seen on the wedge tracings. If a trans-septal puncture is made during right heart catheterization, however, the pressure gradient can accurately quantify the severity of mitral stenosis.[citation needed]

Mitral stenosis

57

Other assisting diagnostic techniques

Chest X-ray may also assist in diagnosis, showing left atrial enlargement. Electrocardiography may show P mitrale, that is, broad, notched P waves in several or many leads with a prominent late negative component to the P wave in lead V1, and may also be seen in mitral regurgitation, and, potentially, any cause of overload of the left atrium.[14] Thus, P-sinistrocardiale may be a more appropriate term.

Natural history
The natural history of mitral stenosis secondary to rheumatic fever (the most common cause) is an asymptomatic latent phase following the initial episode of rheumatic fever. This latent period lasts an average of 16.3 5.2 years. Once symptoms of mitral stenosis begin to develop, progression to severe disability takes 9.2 4.3 years.[citation
needed]

In individuals who were offered mitral valve surgery but refused, survival with medical therapy alone was 44 6% at 5 years, and 32 8% at 10 years after they were offered correction.[citation needed]

Treatment
Treatment is not necessary in asymptomatic patients. The treatment options for mitral stenosis include medical management, mitral valve replacement by surgery, and percutaneous mitral valvuloplasty by balloon catheter.[citation needed] The indication for invasive treatment with either a mitral valve replacement or valvuloplasty is NYHA functional class III or IV symptoms.[citation needed] Another option is balloon dilatation. To determine which patients would benefit from percutaneous balloon mitral valvuloplasty, a scoring system has been developed. Scoring is based on 4 echocardiographic criteria: leaflet mobility, leaflet thickening, subvalvar thickening, and calcification. Individuals with a score of 8 tended to have suboptimal results. Superb results with valvotomy are seen in individuals with a crisp opening snap, score < 8, and no calcium in the commissures. Treatment also focuses on concomitant conditions often seen in mitral stenosis: Any angina is treated with short-acting nitrovasodilators, beta-blockers and/or calcium blockers[14] Any hypertension is treated aggressively, but caution must be taken in administering beta-blockers Any heart failure is treated with digoxin, diuretics, nitrovasodilators and, if not contraindicated, cautious inpatient administration of ACE inhibitors

Mitral valvuloplasty
Mitral valvuloplasty is a minimally invasive therapeutic procedure to correct an uncomplicated mitral stenosis by dilating the valve using a balloon. Under local anaesthetic, a catheter with a special balloon is passed from the right femoral vein, up the inferior vena cava and into the right atrium. The interatrial septum is punctured and the catheter passed into the left atrium using a "trans-septal technique." The balloon is sub-divided into 3 segments and is dilated in 3 stages. 1st the distal portion (lying in the left ventricle) is inflated and pulled against the valve cusps. Second the proximal portion is dilated, in order to fix the centre segment at the valve orifice. Finally, the central section is Illustration of mitral valvuloplasty inflated, this should take no longer than 30 seconds since full inflation obstructs the valve and causes congestion, leading to circulatory arrest and flash pulmonary edema.[citation needed]

Mitral stenosis With careful patient pre-selection, percutaneous balloon mitral valvuloplasty (PBMV) is associated with good success rates and a low rate of complications. By far the most serious adverse event is the occurrence of acute severe mitral regurgitation. Severe mitral regurgitation usually results from a tear in one of the valve leaflets or the subvalvular apparatus. It can lead to pulmonary oedema and hemodynamic compromise, necessitating urgent surgical mitral valve replacement.[citation needed] Other serious complications with PBMV usually relate to the technique of trans-septal puncture (TSP). The ideal site for TSP is the region of the fossa ovalis in the inter-atrial septum. Occasionally, however, the sharp needle used for TSP may inadvertently traumatize other cardiac structures, leading to cardiac tamponade or serious blood loss.[citation
needed]

58

Although the immediate results of PBMV are often quite gratifying, the procedure does not provide permanent relief from mitral stenosis. Regular follow-up is mandatory, to detect restenosis. Long-term follow up data from patients undergoing PBMV indicates that up to 70-75% individuals can be free of restenosis 10 years following the procedure. The number falls to about 40% 15 years post-PBMV.

References
[1] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I05. 0 [2] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I34. 2 [3] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ Q23. 2 [4] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=394. 0 [5] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=746. 5 [6] http:/ / www. diseasesdatabase. com/ ddb8288. htm [7] http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 000175. htm [8] http:/ / www. emedicine. com/ emerg/ topic315. htm [9] http:/ / www. emedicine. com/ ped/ topic2517. htm# [10] http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2013/ MB_cgi?field=uid& term=D008946 [11] Bertazzo, S.et al. Nano-analytical electron microscopy reveals fundamental insights into human cardiovascular tissue calcification.Nature Materials12, 576-583 (2013). [12] Miller, J. D. Cardiovascular calcification: Orbicular origins.Nature Materials12, 476-478 (2013). [13] American Heart Journal [14] medilexicon.com < P mitrale (http:/ / www. medilexicon. com/ medicaldictionary. php?t=70229) Citing. Stedman's Medical Dictionary. Copyright 2006

External links
Echocardiography in Mitral stenosis at Wikiecho (http://www.wikiecho.com/wiki/index. php?title=Mitral_Stenosis) Echocardiographic features of Mitral Stenosis (http://www.echocardiology.org/mitralstenosis.htm) Mitral Valve Repair at The Mount Sinai Hospital (http://www.mitralvalverepair.org)

Tricuspid valve stenosis

59

Tricuspid valve stenosis

Tricuspid valve stenosis
Classification and external resources ICD-10 ICD-9 I07.0 397.0 [1] , I36.0 [2] , Q22.4 [3]

[3]

, 746.9

[4]

DiseasesDB 13353 [5] eMedicine MeSH med/2315 D014264 [6]

[7]

Tricuspid valve stenosis is a valvular heart disease which results in the narrowing of the orifice of the tricuspid valve of the heart. It is a relatively rare condition that causes stenosis- increased resistance to blood flow through the valve.

Causes
It is almost always caused by rheumatic fever and is generally accompanied by mitral stenosis. Rare other causes include carcinoid syndrome, endocarditis, endomyocardial fibrosis, lupus erythematosus, right atrial myxoma and congenital tricuspid atresia.

Diagnosis
A mid diastolic murmur can be heard during auscultation caused by the blood flow through the stenotic valve. It is best heard over the left sternal border with rumbling character and tricuspid opening snap with wide splitting S2. May increase in intensity with inspiration (Carvallo's sign). The diagnosis and the severity can be assessed by echocardiography.

Treatment
Tricuspid valve stenosis itself usually doesn't require treatment. However, if there is damage to other valves in the heart as well, then surgical repair or replacement must be considered. The treatment is usually by surgery (tricuspid valve replacement) or percutaneous balloon valvuloplasty. The resultant tricuspid regurgitation from percutaneous treatment is better tolerated than insufficiency occurring during mitral valvuloplasty

Tricuspid valve stenosis

60

References
[1] [2] [3] [4] [5] [6] [7] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I07. 0 http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I36. 0 http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ Q22. 4 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=746. 9 http:/ / www. diseasesdatabase. com/ ddb13353. htm http:/ / www. emedicine. com/ med/ topic2315. htm http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2013/ MB_cgi?field=uid& term=D014264

External links
Echocardiographic features of tricuspid stenosis (http://www.wikiecho.org/wiki/Tricuspid_stenosis)

Pulmonary valve insufficiency

61

Pulmonary valve insufficiency

Pulmonary valve insufficiency
Classification and external resources

ICD-10 ICD-9 DiseasesDB eMedicine MeSH

I37.1 424.3

[1]

, Q22.2

[2] [3]

[4]

, 746.09

11014

[4] [5]

med/1964 D011665

[6]

Pulmonary valve insufficiency (or incompetence, or regurgitation) is a condition where the pulmonary valve is not strong enough to prevent backflow to the right ventricle. If it is secondary to pulmonary hypertension it is referred to as a Graham Steell murmur.The three primary pathological mechanisms causing Pulmonary Valve insufficiency are dilatation of the pulmonic valve ring, acquired alteration of pulmonic valve leaflet morphology, or congenital absence or malformation of the valve.

External links
http://www.uwhealth.org/page.asp?contentid=11094 http://www.cvphysiology.com/Heart%20Disease/HD005.htm Congenital Heart Surgery [7] The Congenital Heart Surgery Video Project [8] Adult Congenital Surgery: Pulmonary Valve Replacement [9] http://emedicine.medscape.com/article/157639-overview#a0104

Pulmonary valve insufficiency

62

References
[1] [2] [3] [4] [5] [6] [7] [8] [9] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ I37. 1 http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ Q22. 2 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=746. 09 http:/ / www. diseasesdatabase. com/ ddb11014. htm http:/ / www. emedicine. com/ med/ topic1964. htm http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2013/ MB_cgi?field=uid& term=D011665 http:/ / www. pediatricheartsurgery. com http:/ / www. youtube. com/ user/ Redmond111 http:/ / www. youtube. com/ watch?v=i9jrNh2wFzE

Patent ductus arteriosus

63

Patent ductus arteriosus

Patent ductus arteriosus
Classification and external resources

Heart cross-section with PDA ICD-10 ICD-9 OMIM DiseasesDB MedlinePlus eMedicine MeSH Q25.0 747.0 [1] [2] [3]

607411 9706

[4] [5] [6] [7]

001560

emerg/358

C14.240.400.340

Patent ductus arteriosus (PDA) is a congenital disorder in the heart wherein a neonate's ductus arteriosus fails to close after birth. Early symptoms are uncommon, but in the first year of life include increased work of breathing and poor weight gain. With age, the PDA may lead to congestive heart failure if left uncorrected. The ductus arteriosus is a normal fetal blood vessel that closes soon after birth. In a patent ductus arteriosus (PDA) the vessel does not close and remains "patent" (open) resulting in irregular transmission of blood between two of the most important arteries close to the heart, the aorta and the pulmonary artery. PDA is common in neonates with persistent respiratory problems such as hypoxia, and has a high occurrence in premature children. In hypoxic newborns, too little oxygen reaches the lungs to produce sufficient levels of bradykinin and subsequent closing of the DA. Premature children are more likely to be hypoxic and thus have PDA because of their underdeveloped heart and lungs. A patent ductus arteriosus allows a portion of the oxygenated blood from the left heart to flow back to the lungs by flowing from the aorta (which has higher pressure) to the pulmonary artery. If this shunt is substantial, the neonate becomes short of breath: the additional fluid returning to the lungs increases lung pressure to the point that the neonate has greater difficulty inflating the lungs. This uses more calories than normal and often interferes with feeding in infancy. This condition, as a constellation of findings, is called congestive heart failure. In some cases, such as in transposition of the great vessels (the pulmonary artery and the aorta), a PDA may need to remain open. In this cardiovascular condition, the PDA is the only way that oxygenated blood can mix with deoxygenated blood. In these cases, prostaglandins are used to keep the patent ductus arteriosus open.

Patent ductus arteriosus

64

Signs and symptoms

While some cases of PDA are asymptomatic, common symptoms include: tachycardia respiratory problems dyspnea - shortness of breath continuous machine-like heart murmur cardiomegaly - enlarged heart left subclavicular thrill bounding pulse widened pulse pressure patients typically present in good health, with normal respirations and heart rate. If the ductus is moderate or large, widened pulse pressure and bounding peripheral pulses are frequently present, reflecting increased left ventricular stroke volume and diastolic runoff of blood into the initially lower-resistant pulmonary vascular bed. Prominent suprasternal and carotid pulsations may be noted secondary to increased left ventricular stroke volume. poor growth[8] differential cyanosis, i.e. cyanosis of the lower extremities but not of the upper body.

Diagnosis
PDA is usually diagnosed using non-invasive techniques. Echocardiography, in which sound waves are used to capture the motion of the heart, and associated Doppler studies are the primary methods of detecting PDA. Electrocardiography (ECG), in which electrodes are used to record the electrical activity of the heart, is not particularly helpful as there are no specific rhythms or ECG patterns which can be used to detect PDA.[citation needed] A chest X-ray may be taken, which reveals the overall size of neonate's heart (as a reflection of the combined mass of the cardiac chambers) and the appearance of the blood flow to the lungs. A small PDA most often shows a normal sized heart and normal blood flow to the lungs. A large PDA generally shows an enlarged cardiac silhouette and increased blood flow to the lungs.

Normal anatomy
Phonocardiograms from normal and abnormal heart sounds In the developing fetus, the ductus arteriosus (DA) is the vascular connection between the pulmonary artery and the aortic arch that allows most of the blood from the right ventricle to bypass the fetus' fluid-filled compressed lungs. During fetal development, this shunt protects the right ventricle from pumping against the high resistance in the lungs, which can lead to right ventricular failure if the DA closes in-utero.

When the newborn takes its first breath, the lungs open and pulmonary vascular resistance decreases. After birth, the lungs release bradykinin to constrict the smooth muscle wall of the DA and reduce bloodflow through the DA as it narrows and completely closes, usually within the first few weeks of life. In most newborns with a patent ductus arteriosus the blood flow is reversed from that of in utero flow, i.e. the blood flow is from the higher pressure aorta to the now lower pressure pulmonary arteries.

Patent ductus arteriosus In normal newborns, the DA is substantially closed within 1224 hours after birth, and is completely sealed after three weeks. The primary stimulus for the closure of the ductus is the increase in neonatal blood oxygen content. Withdrawal from maternal circulating prostaglandins also contributes to ductal closure. The residual scar tissue from the fibrotic remnants of DA, called the ligamentum arteriosum, remains in the normal adult heart.

65

Treatment
Neonates without adverse symptoms may simply be monitored as outpatients, while symptomatic PDA can be treated with both surgical and non-surgical methods.[9] Surgically, the DA may be closed by ligation (though support in premature infants is mixed), wherein the DA is manually tied shut, or with intravascular coils or plugs that leads to formation of a thrombus in the DA. This was first performed in humans by Robert E. Gross[citation needed]. Because Prostaglandin E1 is responsible for keeping the ductus patent, NSAIDS (inhibitors of prostaglandin synthesis) such as indomethacin or a special form of ibuprofen have been used to help close a PDA.[10] This is an especially viable alternative for premature infants.[citation needed] In certain cases it may be beneficial to the neonate to prevent closure of the ductus arteriosus[citation needed]. For example, in transposition of the great vessels, a PDA may prolong the newborn's life until surgical correction is possible. The ductus arteriosus can be induced to remain open by administering prostaglandin analogs such as alprostadil or misoprostol (prostaglandin E1 analogs)[citation needed]. More recently, PDAs can be closed by percutaneous interventional method[citation needed]. Via the femoral vein or femoral artery, a platinum coil can be deployed via a catheter, which induces thrombosis (coil embolization). Alternatively, a PDA occluder device (AGA Medical)[citation needed], composed of nitinol mesh, is deployed from the pulmonary artery through the PDA. The larger skirt of the device sits on the aortic side while the ampulla of the device hugs the walls of the PDA, with care taken to avoid occlusion of the pulmonary arterial lumen by the device[citation needed]. These methods permit closure without open heart surgery.

Prevention
There is evidence to suggest that giving indomethacin on the first day of life, prophylactically to all preterm infants (less than 37 weeks gestation) reduces the risk of developing a PDA (relative risk 0.29 95% CI 0.22, 0.38) and the complications associated with PDA. There is also a decreased need for surgical intervention to treat PDA in premature infants prophylactically treated with indomethacin (relative risk of 0.51 95%CI 0.37, 0.71). Complications of PDA include intraventricular hemorrhage which can lead to severe brain damage. There is not evidence however to show that giving indomethacin prophylactically improves survival for these infants (RR 0.96; 95% CI 0.81, 1.12). There is also no significant evidence to suggest prophylactic indomethacin decreases long term disability (cerebral palsy, visual impairment, hearing impairment, decreased cognitive performance) rates.

Etiology
A patent ductus arteriosus can be idiopathic (i.e. without an identifiable cause), or secondary to another condition. Some common contributing factors in humans include: Preterm birth Congenital rubella syndrome Chromosomal abnormalities such as Down syndrome

Patent ductus arteriosus

66

Prognosis
Without treatments, the disease may progress from left-to-right (noncyanotic heart) shunt to right-to-left shunt (cyanotic heart) called Eisenmenger's syndrome. Also, a long-term effect would be pulmonary hypertension, which can lead to needing a heart and/or lung transplant.

History
Robert E. Gross, MD performed the first successful ligation of a patent ductus arteriosus on an eight year old girl at Children's Hospital Boston in 1938.

Additional images

patent ductus arteriosus

An echocardiogram of a stented persisting ductus arteriosus. One can see the aortic arch and the stent leaving. Pulmonary artery not seen.

An echocardiogram of a coiled persisting ductus arteriosus. One can see the aortic arch,the pulmonary artery and the coil between them.

References
[1] [2] [3] [4] [5] [6] [7] [8] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ Q25. 0 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=747. 0 http:/ / omim. org/ entry/ 607411 http:/ / www. diseasesdatabase. com/ ddb9706. htm http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 001560. htm http:/ / www. emedicine. com/ emerg/ topic358. htm http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2013/ MB_cgi?mode=& term=Ductus+ Arteriosus,+ Patent& field=entry#TreeC14. 240. 400. 340 MedlinePlus > Patent ductus arteriosus (http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 001560. htm) Update Date: 21 December 2009 [9] Zahaka, KG and Patel, CR. "Congenital defects'". Fanaroff, AA and Martin, RJ (eds.). Neonatal-perinatal medicine: Diseases of the fetus and infant. 7th ed. (2002):1120-1139. St. Louis: Mosby. [10] MayoClinic > Patent ductus arteriosus (PDA) (http:/ / www. mayoclinic. com/ health/ patent-ductus-arteriosus/ DS00631/ DSECTION=treatments-and-drugs) Dec. 22, 2009

^ Long-Term Neurological Outcomes Following Neonatal Heart Surgery: Implications for Cerebral Protections in Adults. Sandra Bellezza MD. March 2, 2011

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67

External links
Patent Ductus Arteriosus information (http://heartcenter.seattlechildrens.org/conditions_treated/ patent_ductus_arteriosus.asp) from Seattle Children's Hospital Heart Center Patent Ductus Arteriosus Causes from US Department of Health and Human Services (http://www.nhlbi.nih. gov/health/dci/Diseases/pda/pda_causes.html) Patent Ductus Arteriosus from Merck (http://www.merck.com/mmhe/sec23/ch265/ch265b. html#sec23-ch265-ch265b-293) Fetal Circulation at berkeley.edu (http://mcb.berkeley.edu/courses/mcb135e/fetal.html) Information about PDA (http://www.aboutkidshealth.ca/En/ResourceCentres/PrematureBabies/ AboutPrematureBabies/HeartConditions/Pages/Patent-Ductus-Arteriosus-PDA.aspx) - The Hospital for Sick Children Down's Heart Group (http://www.dhg.org.uk/information/persistentductusarteriosus.aspx) Easy to understand diagram and explanation of PDA. PDA Occluder (http://www.amplatzer.com/USProducts/PDADevice/tabid/193/Default.aspx) Amplatzer PDA occluder device used for percutaneous closure of PDAs. Children's Hospital Boston Archives (http://www.childrenshospital.org/archives) Patent ductus arteriosus (http://www.gosh.nhs.uk/medical-conditions/search-for-medical-conditions/ patent-ductus-arteriosus/patent-ductus-arteriosus-information/) information for parents.

Wheeze

68

Wheeze
Wheezing
ICD-10 ICD-9 R06.2 [1] [2]

786.07

MedlinePlus 003070 [3]

Wheezing The sound of wheezing as heard with a stethoscope.

Problems playing this file? See media help.

A wheeze (formally called "sibilant rhonchi" in medical terminology) is a continuous, coarse, whistling sound produced in the respiratory airways during breathing. For wheezes to occur, some part of the respiratory tree must be narrowed or obstructed, or airflow velocity within the respiratory tree must be heightened. Wheezing is commonly experienced by persons with a lung disease; the most common cause of recurrent wheezing is asthma attacks, though it can also be a symptom of lung cancer. The differential diagnosis of wheezing is wide, and the cause of wheezing in a given patient is determined by considering the characteristics of the wheezes and the historical and clinical findings made by the examining physician.

Characteristics
Wheezes occupy different portions of the respiratory cycle depending on the site of airway obstruction and its nature. The fraction of the respiratory cycle during which a wheeze is produced roughly corresponds to the degree of airway obstruction. Bronchiolar disease usually causes wheezing that occurs in the expiratory phase of respiration. The presence of expiratory phase wheezing signifies that the patient's peak expiratory flow rate is less than 50% of normal. Wheezing heard in the inspiratory phase on the other hand is often a sign of a stiff stenosis, usually caused by tumors, foreign bodies or scarring. This is especially true if the wheeze is monotonal, occurs throughout the inspiratory phase (i.e. is "holoinspiratory"), and is heard more proximally, in the trachea. Inspiratory wheezing also occurs in hypersensitivity pneumonitis. Wheezes heard at the end of both expiratory and inspiratory phases usually signify the periodic opening of deflated alveoli, as occurs in some diseases that lead to collapse of parts of the lungs. The location of the wheeze can also be an important clue to the diagnosis. Diffuse processes that affect most parts of the lungs are more likely to produce wheezing that may be heard throughout the chest via a stethoscope. Localized processes, such as the occlusion of a portion of the respiratory tree, are more likely to produce wheezing at that location, hence the sound will be loudest and radiate outwardly. The pitch of a wheeze does not reliably predict the degree of narrowing in the affected airway. A special type of wheeze is stridor. Stridor the word is from the Latin, strdor[4] is a harsh, high-pitched, vibrating sound that is heard in respiratory tract obstruction. Stridor heard solely in the expiratory phase of respiration usually indicates an upper respiratory tract obstruction, "as with aspiration of a foreign body (such as the fabled pediatric peanut)." Stridor in the inspiratory phase is usually heard with obstruction in the upper airways, such as the trachea, epiglottis, or larynx; because a block here means that no air may reach either lung, this condition is a

Wheeze medical emergency. Biphasic stridor (occurring during both the inspiratory and expiratory phases) indicates narrowing at the level of the glottis or subglottis, the point between the upper and lower airways.

69

Diagnosis
1 out of 3 preschool children and 2 out of 3 school children with recurrent wheezing/coughing are allergic.[citation needed] . The reaction creates an inflammation which, in turn, can lead to a variety of symptoms such as wheezing. Over the last decade allergy has increased by 18% in the United States. Today one child in four is allergic.[citation needed] Early diagnosis of allergy is important for the development of the child later in life.[citation needed] There are many patients with symptoms suggesting eczema, rhinitis, hay fever, asthma or wheezing.

References
[1] [2] [3] [4] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ R06. 2 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=786. 07 http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 003070. htm Simpson JA, Weiner ESC (eds). "stridor, n. 2." Oxford English Dictionary 2nd ed. Oxford: Clarendon Press, 1989. OED Online Oxford University Press. Accessed September 10, 2005. http:/ / dictionary. oed. com.

Further reading
Godfrey S, Uwyyed K, Springer C, Avital A (Mar 2004). "Is clinical wheezing reliable as the endpoint for bronchial challenges in preschool children?". Pediatr Pulmonol. 37 (3): 193200. doi: 10.1002/ppul.10434 (http:/ /dx.doi.org/10.1002/ppul.10434). PMID 14966812 (http://www.ncbi.nlm.nih.gov/pubmed/14966812).

Stridor

70

Stridor
Stridor
ICD-10 ICD-9 R06.1 786.1 [1] [2]

MedlinePlus 003074 [3]

Stridor Inspiratory and expiratory stridor in a 13 month child with croup.

Problems playing this file? See media help.

Stridor (Latin for "creaking or grating noise") is a high-pitched musical sound resulting from turbulent air flow in the upper airway. Stridor is a physical sign which is caused by a narrowed or obstructed airway. It can be inspiratory, expiratory or biphasic, although it is usually heard during inspiration. Inspiratory stridor often occurs in children with "croup." It may be indicative of serious airway obstruction from severe conditions such as epiglottitis, a foreign body lodged in the airway, or a laryngeal tumor. Stridor should always command attention to establish its cause. Visualization of the airway by medical experts equipped to control the airway may be needed.

Treatments
The first issue of clinical concern in the setting of stridor is whether or not tracheal intubation or tracheostomy is immediately necessary. A reduction in oxygen saturation is considered a late sign of airway obstruction, particularly in a child with healthy lungs and normal gas exchange. Some patients will need immediate tracheal intubation. If intubation can be delayed for a period, a number of other potential options can be considered, depending on the severity of the situation and other clinical details. These include: Expectant management with full monitoring, oxygen by face mask, and positioning the head on the bed for optimum conditions (e.g., 45 - 90 degrees). Use of nebulized racemic adrenaline epinephrine (0.5 to 0.75 ml of 2.25% racemic epinephrine added to 2.5 to 3 ml of normal saline) in cases where airway edema may be the cause of the stridor. (Nebulized cocaine in a dose not exceeding 3mg/kg may also be used, but not together with racemic adrenaline [because of the risk of ventricular arrhythmias].) Use of dexamethasone (Decadron) 48mg IV q 8 - 12 h in cases where airway edema may be the cause of the stridor; note that some time (in the range of hours) may be needed for dexamethasone to work fully. Use of inhaled Heliox (70% helium, 30% oxygen); the effect is almost instantaneous. Helium, being a less dense gas than nitrogen, reduces turbulent flow through the airways. Always ensure an open airway. In obese patients elevation of the panniculis has shown to relieve symptoms by 80%.

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Causes
Stridor may occur as a result of: Foreign bodies (e.g., aspirated peanut, aspirated food bolus); Tumor (e.g., laryngeal papillomatosis, squamous cell carcinoma of larynx, trachea or esophagus); ALL (T-cell ALL can present with mediastinal mass that compresses the trachea and causes inspiratory stridor); Infections (e.g., epiglottitis, retropharyngeal abscess, croup); Subglottic stenosis (e.g., following prolonged intubation or congenital); Airway edema (e.g., following instrumentation of the airway, tracheal intubation, drug side effect, allergic reaction); Subglottic hemangioma (rare); Vascular rings compressing the trachea; Many thyroiditis such as Riedel's thyroiditis; Vocal cord palsy; Tracheomalacia or Tracheobronchomalacia (e.g., collapsed trachea). Congenital anomalies of the airway are present in 87% of all cases of stridor in infants and children. Vasculitis. Upper-Airway Resistance Syndrome (UARS) in sleep. A milder form of Obstructive Sleep Apnea.

Diagnosis
Stridor is usually diagnosed on the basis of history and physical examination, with a view to revealing the underlying problem or condition. Chest and neck x-rays, bronchoscopy, CT-scans, and/or MRIs may reveal structural pathology. Flexible fiberoptic bronchoscopy can also be very helpful, especially in assessing vocal cord function or in looking for signs of compression or infection.

References
[1] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ R06. 1 [2] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=786. 1 [3] http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 003074. htm

External links
Stridor (http://www.emedicine.com/ped/topic2159.htm#) at eMedicine Congenital stridor (http://www.emedicine.com/ped/topic2624.htm#) at eMedicine MedlinePlus Encyclopedia Breathing sounds - abnormal (stridor) (http://www.nlm.nih.gov/medlineplus/ ency/article/003074.htm) DDB 27190 (http://www.diseasesdatabase.com/ddb27190.htm) Stridor sounds at R.A.L.E. Lung Sounds (http://www.rale.ca/Stridor.htm)

Rhonchi

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Rhonchi
Rhonchi
ICD-10 R09.8 ICD-9 786.7 [1]

Rhonchi is the coarse rattling sound somewhat like snoring, usually caused by secretion in bronchial airways. Rhonchi is the plural form of the singular word rhonchus. Since the mid-1990s, it has no longer been considered appropriate terminology in auscultation of the thorax as there has been much confusion in the published literature which confuses this between crepitations and wheezes, so the exact nature of this term is unclear (see Fleischner Society for standardized terminology)[citation needed]. Similarly rales are no longer used in much of Europe, North America and Australia with regard to description of auscultatory findings. More appropriate terms for auscultation of the thorax are breath sounds, and adventitious sounds (wheezes, crackles, pleural rubs).

Description
These have variably been associated with low-pitched, continuous sounds that are similar to wheezes and / or crackles.

Related techniques
Other terminology for sounds that may be auscultated in the thorax include crackles, wheezes, pleural rubs, pulmonary murmurs, egophony, whispering pectoriloquy and vocal fremitus. Also, percussion is often used to assess diseases of the chest.

References
[1] http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=786. 7

External links
Adventitious Sounds of Respiratory System (http://www.mediscuss.org/ respiratory-auscultation-tips-audio-mp3-examples-71-page3.html)

Crackles

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Crackles
Crackles
ICD-10 R09.8 [1] ICD-9 786.7 [1]

Crackles Crackles heard in the lungs of a person with pneumonia using a stethoscope.

Problems playing this file? See media help.

Crackles, crepitations, or rales ( i/rls/ RAHLS or /rls/ RALS) are the clicking, rattling, or crackling noises that may be made by one or both lungs of a human with a respiratory disease during inhalation. They are often heard only with a stethoscope ("on auscultation"). Bilateral crackles refers to the presence of crackles in both lungs. Basal or basilar crackles (not to be confused with the basilar artery of the brain) are crackles apparently originating in or near the base of the lung. Bibasal or bibasilar crackles refer to crackles at the bases of both the left and right lungs. Bilateral basal crackles also refers to the presence of basal crackles in both lungs. Crackles are caused by the "popping open" of small airways and alveoli collapsed by fluid, exudate, or lack of aeration during expiration. The word "rales" derives from the French word rle meaning "rattle". Crackles can be heard in patients with pneumonia, atelectasis, pulmonary fibrosis, acute bronchitis, bronchiectasis, or post thoracotomy or metastasis ablation. Pulmonary edema secondary to left-sided congestive heart failure can also cause rales.

Terminology
Ren Laennec developed the term 'rles' ('rattles' in French) to describe the added breath sounds which are now referred to as 'crackles'. He described them using unusual daily examples, such as 'whistling of little birds', 'crackling of salt on a heated dish', 'cooing of the woodpidgeon' etc. but soon realised that he was unable to use this term in front of his patients because it conjured the association of 'le rle de la mort', which translates to 'death rattle' i.e. the noise that people who are about to die make when they can no longer clear secretions. Therefore at the bedside, he used the Latin equivalent, 'rhonchus'. This was not clearly understood by his translator, John Forbes, and the terminology became very confusing after the publication of De L'Auscultation Mediate. Therefore in 1977, a standardization was established by the American Thoracic Society and American College of Chest Physicians. As a result of this, the term 'rles' was abandoned, and 'crackles' became its official substitute. Therefore these sounds should be correctly described as crackles.

The sound of crackles

Crackles are caused by explosive opening of small airways and are discontinuous, nonmusical, and brief. Crackles are much more common during the inspiratory than the expiratory phase of breathing, but they may be heard during the expiratory phase. Crackles are often associated with inflammation or infection of the small bronchi, bronchioles, and alveoli. Crackles that do not clear after a cough may indicate pulmonary edema or fluid in the alveoli due to heart failure, pulmonary fibrosis, or acute respiratory distress syndrome. Crackles that partially clear or change after

Crackles coughing may indicate bronchiectasis. Crackles are often described as fine, medium, and coarse. They can also be characterized as to their timing: fine crackles are usually late-inspiratory, whereas coarse crackles are early inspiratory. Fine crackles are soft, high-pitched, and very brief. This sound can be simulated by rolling a strand of hair between one's fingers near the ears, or by moistening one's thumb and index finger and separating them near the ears. Their presence usually indicates an interstitial process, such as pulmonary fibrosis or congestive heart failure. The sounds from interstitial pulmonary fibrosis been described as sounding like opening a Velcro fastener. Coarse crackles are somewhat louder, lower in pitch, and last longer than fine crackles. Their presence usually indicates an airway disease, such as bronchiectasis. They can also be described as unilateral or bilateral, as well as dry or moist/wet.[2] Crackles can be heard over the lower lobe of the lungs. Pulmonary edema makes it much more audible.

74

References
[1] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ R09. 8 [2] Wrongdiagnosis.com > Crackles (http:/ / www. wrongdiagnosis. com/ symptoms/ bilateral_crackles/ book-causes-13a. htm) Book Source Details: * Book Title: Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series * Author(s): Springhouse

* Year of Publication: 2007

Pertussis

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Pertussis
Pertussis
Classification and external resources

A young boy coughing due to pertussis. ICD-10 ICD-9 DiseasesDB MedlinePlus eMedicine MeSH A37 033 [1] [2] [3] [4] [5] ped/1778 [6]

1523

001561

emerg/394 D01491

[7]

Pertussis commonly called whooping cough (/hupHelp:IPA for English#Keykf/ or /hwupHelp:IPA for English#Keykf/) is a highly contagious bacterial disease caused by Bordetella pertussis. In some countries, this disease is called the 100 days' cough or cough of 100 days. Symptoms are initially mild, and then develop into severe coughing fits, which produce the namesake high-pitched "whoop" sound in infected babies and children when they inhale air after coughing.[8] The coughing stage lasts approximately six weeks before subsiding. Prevention by vaccination is of primary importance given the seriousness of the disease in children. Although treatment is of little direct benefit to the person infected, antibiotics are recommended because they shorten the duration of infectiousness. It is currently estimated that the disease annually affects 48.5million people worldwide, resulting in nearly 295,000deaths.

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Signs and symptoms

The classic symptoms of pertussis are a paroxysmal cough, inspiratory whoop, and fainting and/or vomiting after coughing. The cough from pertussis has been documented to cause subconjunctival hemorrhages, rib fractures, urinary incontinence, hernias, post-cough fainting, and vertebral artery dissection. Violent coughing can cause the pleura to rupture, leading to a pneumothorax. If there is vomiting after a coughing spell or an inspiratory whooping sound on coughing, the likelihood almost doubles that the illness is pertussis. On the other hand, the absence of a paroxysmal cough or posttussive emesis makes it almost half as likely. The incubation period is typically seven to ten days with a range of four to 21 days and rarely may be as long as 42 days,[9] after which there are usually mild respiratory symptoms, mild coughing, sneezing, or runny nose. This is known as the catarrhal stage. After one to two weeks, the coughing classically develops into uncontrollable fits, each with five to ten forceful coughs, followed by a high-pitched "whoop" sound in younger children, or a gasping sound in older children, as the patient struggles to breathe in afterwards (paroxysmal stage). Fits can occur on their own or can be triggered by yawning, stretching, laughing, eating or yelling; they usually occur in groups, with multiple episodes every hour around the clock. This stage usually lasts two to eight weeks, or sometimes longer. A gradual transition then occurs to the convalescent stage, which usually lasts one to two weeks. This stage is marked by a decrease in paroxysms of coughing, both in frequency and severity, and a cessation of vomiting. A tendency to produce the "whooping" sound after coughing may remain for a considerable period after the disease itself has cleared up.

Diagnosis
Methods used in laboratory diagnosis include culturing of nasopharyngeal swabs on Bordet-Gengou medium, polymerase chain reaction (PCR), direct immunofluorescence (DFA), and serological methods. The bacteria can be recovered from the patient only during the first three weeks of illness, rendering culturing and DFA useless after this period, although PCR may have some limited usefulness for an additional three weeks. For most adults and adolescents, who often do not seek medical care until several weeks into their illness, serology may be used to determine whether antibody against pertussis toxin or another component of B. pertussis is present at high levels in the blood of the Gram stain of the bacteria Bordetella pertussis patient. By this stage they have been contagious for some weeks and may have spread the infection to many people. Because of this, adults, who are not in great danger from pertussis, are increasingly being encouraged to be vaccinated. A similar, milder disease is caused by B. parapertussis.

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Prevention
The primary method of prevention for pertussis is vaccination. There is insufficient evidence to determine the effectiveness of antibiotics in those who have been exposed but are without symptoms. Prophylactic antibiotics, however, are still frequently used in those who have been exposed and are at high risk of severe disease (such as infants).

Vaccine
Pertussis vaccines are effective, routinely recommended by the World Health Organization and the Center for Disease Control and Prevention, and saved over half a million lives in 2002. The multi-component acellular pertussis vaccine, for example, is between 71-85% effective with greater effectiveness for more severe disease. Despite widespread use of the vaccine however, pertussis has persisted in vaccinated populations and is today one of the most prevalent vaccine-preventable diseases in Western countries. Recent resurgences in pertussis infections are attributed to a combination of waning immunity and new mutations in the bacteria that existing vaccines are unable to effectively control. Immunization against pertussis does not confer lifelong immunity, a 2011 study by the CDC indicated that the duration of protection may only last three to six years. This covers childhood, which is the time of greatest exposure and greatest risk of death from pertussis. For children, the immunizations are commonly given in combination with immunizations against tetanus, diphtheria, polio and haemophilus influenzae type B at ages two, four, six, and 1518 months. A single later booster is given at four to six years of age. (US schedule). In the UK, pertussis vaccinations are given at 2, 3 and 4 months, with a pre-school booster at 3 years 4 months. Dr. Paul Offit, chief of the Director of the Vaccine Education Center at the Children's Hospital of Philadelphia, comments that the last pertussis vaccination people receive may be their booster at age 11 or 12 years old. However, he states that it is important for adults to have immunity as well to prevent transmission of the disease to infants. While adults rarely die if they contract pertussis after the effects of their childhood vaccinations have worn off, they may transmit the disease to people at much higher risk of injury or death. To reduce morbidity and spread of the disease, Canada, France, the U.S. and Germany have approved pertussis vaccine booster shots. In 2012, a federal advisory panel recommended that all U.S. adults receive vaccination.[10] Later that year, health officials in the UK recommended the vaccination of pregnant women (between 28 38 weeks of pregnancy) in order to protect their unborn children. Designed to protect babies from birth until their first standard vaccination at eight weeks of age, this vaccine was introduced in response to the ongoing outbreak of pertussis in the UK, the worst in over a decade. The pertussis booster for adults is combined with a tetanus vaccine and diphtheria vaccine booster; this combination is abbreviated "Tdap" (Tetanus, diphtheria, acellular pertussis). It is similar to the childhood vaccine called "DTaP" (Diphtheria, Tetanus, acellular Pertussis), with the main difference that the adult version contains smaller amounts of the diphtheria and pertussis components this is indicated in the name by the use of lower-case "d" and "p" for the adult vaccine. The lower-case "a" in each vaccine indicates that the pertussis component is acellular, or cell-free, which improves safety by dramatically reducing the incidence of side effects. Adults should request the Tdap instead of just a tetanus vaccination in order to receive the multi-vaccine. The pertussis component of the original DPT vaccine accounted for most of the minor local and systemic side effects in many vaccinated infants (such as mild fever or soreness at the injection site). The newer acellular vaccine, known as DTaP, has greatly reduced the incidence of adverse effects compared to the earlier "whole-cell" pertussis vaccine, however the efficacy of the acellular vaccine declines faster that the whole-cell vaccine. Infection with pertussis induces incomplete natural immunity that wanes over time.[11] Natural immunity lasts longer than vaccine-induced immunity, with one study reporting maximum effectiveness as long as 20 years in the former and 12 in the latter.

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Management
People with pertussis are infectious from the beginning of the catarrhal stage (runny nose, sneezing, low-grade fever, symptoms of the common cold) through the third week after the onset of paroxysms (multiple, rapid coughs) or until 5 days after the start of effective antimicrobial treatment. A reasonable guideline is to treat people age >1 year within 3 weeks of cough onset and infants age <1 year and pregnant women (especially near-term) within 6 weeks of cough onset. If the patient is diagnosed late, antibiotics will not alter the course of the illness and, even without antibiotics, the patient should no longer be spreading pertussis. Antibiotics decrease the duration of infectiousness and thus prevent spread. The antibiotic erythromycin or azithromycin is a front-line treatment Newer macrolides are frequently recommended due to lower rates of side effects. Trimethoprim-sulfamethoxazole (TMP-SMZ) may be used in those with allergies to first-line agents or in infants who have a risk of pyloric stenosis from macrolides. Effective treatments of the cough associated with this condition have not been developed. Erythromycin, clarithromycin, and azithromycin are preferred for the treatment of pertussis in persons 1 month of age.

Prognosis
Common complications of the disease include pneumonia, encephalopathy, earache, or seizures. Most healthy older children and adults will have a full recovery from pertussis, however those with comorbid conditions can have a higher risk of morbidity and mortality. Infection in newborns is particularly severe. Pertussis is fatal in an estimated 1.6% of hospitalized infants who are under one year of age. Infants under one are also more likely to develop complications e.g., pneumonia (20%), encephalopathy(0.3%), seizures (1%), failure to thrive, and death (1%) -perhaps due to the ability of the bacterium to suppress immune response against it. Pertussis can cause severe paroxysm-induced cerebral hypoxia and 50% of infants admitted to hospital will suffer apneas. Reported fatalities from pertussis in infants have increased substantially over the past 20 years.

Epidemiology
Worldwide, whooping cough affects 48.5 million people yearly. As of 2010 it caused about 81,000 deaths, down from 167,000 in 1990. This is despite generally high coverage with the DTP and DTaP Disability-adjusted life year for pertussis per 100,000inhabitants.no data vaccines. Pertussis is one of the 2525-5050-7575-100100-150150-200200-300300-400400-500500-600600-700 700 leading causes of vaccine-preventable deaths world-wide. 90% of all cases occur in developing countries. Before vaccines, an average of 178,171 cases were reported in the U.S., with peaks reported every two to five years; more than 93% of reported cases occurred in children under 10 years of age. The actual incidence was likely much higher. After vaccinations were introduced in the 1940s, incidence fell dramatically to less than 1,000 by 1976. Incidence rates have increased since 1980. In 2012, rates in the United States reached a high of 41,880 people; this is the highest it has been since 1955 when numbers reached 62,786.[12] Pertussis is the only vaccine-preventable disease that is associated with increasing deaths in the U.S. The number of deaths increased from four in 1996 to 17 in 2001, almost all of which were infants under one year. In Canada, the

Pertussis number of pertussis infections has varied between 2,000 and 10,000 reported cases each year over the last ten years.[13] Australia reports an average of 10,000 cases a year, but the number of cases has increased in recent years. In the U.S. pertussis in adults has increased significantly since about 2004.[14]

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Outbreaks in USA
2010 In 2010 ten infants in California died and health authorities declared an epidemic with 9,120 cases. Investigating the cases of the ten infant fatalities, it found that doctors had been misdiagnosing the infants' condition despite having seen infants on multiple visits. Statistical analysis identified significant overlap in communities with cluster of nonmedical exemptions for children and cases of whooping cough. The study found the number of exemption varies widely among communities but tends to be highly clustered, in some schools more than of parents filed for exemptions not to vaccinate their children. The data suggest vaccine refusal based on nonmedical reason and personal belief may have been one of several factors, in addition to less long lasting effect of the current vaccine and most vaccinated adults and older children have not received a booster shot, contributed to the California outbreak in 2010. 2012 In April and May 2012, pertussis was declared to be at epidemic levels in the US state of Washington with 3,308 cases by December. In December 2012, the state of Vermont declared a pertussis epidemic with 522 cases. The state of Wisconsin has the highest incidence rate, with 3,877 cases of whooping cough, however it did not release an official epidemic declaration.

History
B. pertussis was isolated in pure culture in 1906 by Jules Bordet and Octave Gengou, who also developed the first serology and vaccine. Efforts to develop an inactivated whole-cell pertussis vaccine began soon after B. pertussis was grown in pure culture in 1906. In the 1920s, Dr. Louis W. Sauer developed a vaccine for whooping cough at Evanston Hospital (Evanston, IL). In 1925, the Danish physician Thorvald Madsen was the first to test a whole-cell pertussis vaccine on a wide scale. He used the vaccine to control outbreaks in the Faroe Islands in the North Sea. In 1942, the American scientist Pearl Kendrick combined the whole-cell pertussis vaccine with diphtheria and tetanus toxoids to generate the first DTP combination vaccine. To minimize the frequent side effects caused by the pertussis component of the vaccine, the Japanese scientist Yuji Sato developed an acellular pertussis vaccine consisting of purified haemagglutinins (HAs: filamentous strep throat and leucocytosis-promoting-factor HA), which are secreted by B. pertussis into the culture medium. Sato's acellular pertussis vaccine was used in Japan since 1981. Later versions of the acellular pertussis vaccine used in other countries consisted of additional defined components of B. pertussis and were often part of the DTaP combination vaccine. The complete B. pertussis genome of 4,086,186 base pairs was sequenced in 2004.

Society and culture

Much of the controversy surrounding the DPT vaccine in the 1970s and 1980s related to the question of whether the whole-cell pertussis component caused permanent brain injury in rare cases, called pertussis vaccine encephalopathy. Despite this possibility, doctors recommended the vaccine due to the overwhelming public health benefit, because the claimed rate was very low (one case per 310,000 immunizations, or about 50 cases out of the 15 million immunizations each year in the United States), and the risk of death from the disease was high (pertussis killed thousands of Americans each year before the vaccine was introduced).

Pertussis No studies showed a causal connection, and later studies showed no connection of any type between administration of the DPT vaccine and permanent brain injury. The alleged vaccine-induced brain damage proved to be an unrelated condition, infantile epilepsy. Eventually evidence against the hypothesized existence of pertussis vaccine encephalopathy mounted to the point that in 1990, the Journal of American Medical Association called it a "myth" and "nonsense". However, before that point, criticism of the studies showing no connection and a few well-publicized anecdotal reports of permanent disability that were blamed on the DPT vaccine gave rise to anti-DPT movements in the 1970s. The negative publicity and fear-mongering caused the immunization rate to fall in several countries, including Great Britain, Sweden, and Japan. In many cases, a dramatic increase in the incidence of pertussis followed. Unscientific claims about the vaccine pushed suppliers of the vaccines out of the market. In the United States, low profit margins and an increase in vaccine-related lawsuits led many manufacturers to stop producing the DPT vaccine by the early 1980s. In 1982, the television documentary "DPT: Vaccine Roulette" depicted the lives of children whose severe disabilities were inaccurately blamed on the DPT vaccine by reporter Lea Thompson. The negative publicity generated by the documentary led to a tremendous increase in the number of lawsuits filed against vaccine manufacturers. By 1985, manufacturers of vaccines had difficulty obtaining liability insurance. The price of the DPT vaccine skyrocketed, leading to shortages around the country. Only one manufacturer of the DPT vaccine remained in the U.S. by the end of 1985. To avert a vaccine crisis, Congress in 1986 passed the National Childhood Vaccine Injury Act (NCVIA), which established a federal no-fault system to compensate victims of injury caused by mandated vaccines. The majority of claims that have been filed through the NCVIA have been related to injuries allegedly caused by the whole-cell DPT vaccine. The concerns about side effects led Yuji Sato to introduce an even safer acellular version of the pertussis vaccine for Japan in 1981. The acellular pertussis vaccine was approved in the United States in 1992 for use in the combination DTaP vaccine. Research has shown that the acellular vaccine has a rate of adverse events similar to that of a Td vaccine (a tetanus-diphtheria vaccine containing no pertussis vaccine).

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References
[1] [2] [3] [4] [5] [6] [7] [8] [9] http:/ / apps. who. int/ classifications/ icd10/ browse/ 2010/ en#/ A37 http:/ / www. icd9data. com/ getICD9Code. ashx?icd9=033 http:/ / www. diseasesdatabase. com/ ddb1523. htm http:/ / www. nlm. nih. gov/ medlineplus/ ency/ article/ 001561. htm http:/ / www. emedicine. com/ emerg/ topic394. htm http:/ / www. emedicine. com/ ped/ topic1778. htm# http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2013/ MB_cgi?field=uid& term=D01491 Symptoms, sounds, and a video (http:/ / www. whoopingcough. net/ symptoms. htm) WhoopingCough.net Pertussis (whooping cough) (http:/ / www. health. ny. gov/ diseases/ communicable/ pertussis/ fact_sheet. htm), New York State Department of Health, Updated: January 2012, retrieved 8 June 2013. [10] http:/ / apnews. myway. com/ article/ 20120222/ D9T2I3JG4. html [11] Disease Control Priorities Project. (2006). Vaccine-Preventable Diseases (Table20.1, page390 (http:/ / files. dcp2. org/ pdf/ expressbooks/ vaccine. pdf)). International Bank for Reconstruction and Development, World Bank. Washington DC (www.worldbank.org). [12] http:/ / www. cdc. gov/ pertussis/ surv-reporting/ cases-by-year. html [13] Whooping Cough - Causes, Symptoms, Treatment, Diagnosis - - C-Health (http:/ / chealth. canoe. ca/ channel_condition_info_details. asp?disease_id=217& channel_id=2026& relation_id=18305& rot=4) [14] Kate Murphy. "Enduring and Painful, Pertussis Leaps Back" (http:/ / www. nytimes. com/ 2005/ 02/ 22/ health/ 22cough. html). The New York Times. 22 February 2005.

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External links
Pertussis at Todar's Online Textbook of Bacteriology (http://textbookofbacteriology.net/pertussis.html) Centers for Disease Control and Prevention (2012). "Ch. 15: Pertussis" (http://www.cdc.gov/vaccines/pubs/ pinkbook/pert.html). In Atkinson W, Wolfe S, Hamborsky J. Epidemiology and Prevention of Vaccine-Preventable Diseases (http://www.cdc.gov/vaccines/pubs/pinkbook/table-of-contents.html) (12th ed.). Washington DC: Public Health Foundation. pp.215232. Whooping Cough: A Stealthy Illness (http://well.blogs.nytimes.com/2012/09/24/ whooping-cough-a-stealthy-illness/) by Jane E. Brody, New York Times, September 24, 2012 Listen to the sounds of pertussis (http://www.cdc.gov/pertussis/pubs-tools/audio-video.html) Centers for Disease Control and Prevention (CDC) View personal stories of pertussis (http://shotbyshot.org/story-gallery/#Pertussis) ShotbyShot.org, California Immunization Coalition (CIC)

Article Sources and Contributors

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Article Sources and Contributors

Heart sounds Source: https://en.wikipedia.org/w/index.php?oldid=581849283 Contributors: 777sms, Aboosh, Aceofhearts1968, Adambein, Adoarns, AlexanderM, Andres, Andrewr47, Arcadian, ArcadianOnUnsecuredLoc, Bluedustmite, Brightguy88, Bryan Derksen, Butwhatdoiknow, CanadianLinuxUser, Caspian, Catcollier, Chihowa, Chmee2, Chris the speller, Cloudysusan, DarkWar4Ever, Darkwind, Darthfader77, Deadgnome, DemonicPartyHat, DerEikopf, Diachang, Diberri, Doctorjmz, Draeco, Drilnoth, ELLusKa 86, Eleassar, Eleassar777, EoGuy, Eric119, Escape Orbit, Eug, Everyking, F.chiodo, Faigl.ladislav, Figma, Furqan Tejani, Graham87, Grendelkhan, HBNayr, Hovea, Huckfinne, II MusLiM HyBRiD II, Inferior Olive, Jack-A-Roe, Jfdwolff, Jmh649, Joedaddy09, Johnnythefridge, Joseph.c.murray, KanylenDK, Kauczuk, KevinFox2010, Ksheka, Kyoko, Lawgers, Lbeben, Lenitudent, Lsmjake, MAlvis, Maderchoud113234, Madhero88, Med student2010, Mikael Hggstrm, Mikeylane, Mimihitam, Mr Stephen, MrDolomite, MrOllie, Mysid, NawlinWiki, NeilN, Nephron, Ninly, Nymccal, Omar sok, Omnipaedista, PFHLai, Patrixpax, Pch123, PhilipMW, Pkubin, R'n'B, RetsRftsugua, S h i v a (Visnu), SFK2, Saucepan, Scapermoya, Schmausschmaus, Sfahey, Shishew, Shoecream, Sonusona, Spiffulent, Splintax, Spundun, Stevenfruitsmaak, Tannim101, Tellyaddict, ThatPeskyCommoner, The Thing That Should Not Be, Thingg, Tjajkovskij, Tortie tude, Tristanb, Una Smith, Varunatla, Vedran12, VengeancePrime, Vinne2, Violinchic, Wayne Hardman, Wolfmankurd, Woohookitty, Wouterstomp, XWikiWhatx, Ywith, 280 ,12 ,55 anonymous edits Heart murmur Source: https://en.wikipedia.org/w/index.php?oldid=579220682 Contributors: 12wikiwiki, 147221help, 147221me, 15turnsm, ABF, Aircorn, Aitias, Alephbethgimel, Andmek, Andrew Benton, Andrewr47, AnglIesha2, Ano-User, Anon user, Arcadian, Arcadie, Axl, Badgettrg, Benji33, Billyrubin2008, Birdman1, Bobjgalindo, Buqing, Captain-tucker, Chantal Cooper, Charles Matthews, Chester Markel, Clarince63, Countincr, CyberSach, DOCtraind, Danjeffers, Darthfader77, Davidruben, Deathlasersonline, Diptanshu.D, Discospinster, Dr. Friendly, Drchriseades, Drgarden, Drjnk, Drphilharmonic, Ealicialeigh, EclecticDonor, Edward, Einstein76, ElKevbo, Erfil, Fabykot, Facts707, Fillanfloppy, Frankj1978, Gak, Gaussgauss, George Ho, Graham87, Hoverflysmiles, Idunno271828, Immunize, Ixfd64, Jack-A-Roe, Jerry teps, Jmh649, JohnManuel, JustAGal, Keivanmilani, Ksheka, Kuralyov, Kyoko, La Pianista, Laportechicago, Leftauricle, Liore, Madhero88, Magioladitis, Mahahahaneapneap, Manco Capac, Mani1, Marilynniisflyyy, Markbenjamin, Matthew Yeager, Mike Rosoft, Mollyhatzandco, MrOllie, Naniwako, NeilN, Nephron, Nltate, Nono64, Nrsmoll, Pch123, Ph.eyes, Pkubin, PrestonH, Ptolemy Caesarion, Reaper X, Rishigupta02445, Rjwilmsi, Roksonarim, Rror, S, SJP, Scarian, Schmausschmaus, Schneelocke, Sista40, Squids and Chips, SwisterTwister, The profit mohammed, Thusz, Tide rolls, TimSauder, Tommy2010, Transity, Tristanb, Ultimaga, Uncle Dick, Veggies, WikHead, Wwallacee, Z0OMD, Zebediah49, Zeeyanwiki, Zero sharp, 270 ,55 anonymous edits Aortic valve stenosis Source: https://en.wikipedia.org/w/index.php?oldid=581953495 Contributors: Akory99, Arcadian, Barek, Barticus88, Biosthmors, Bobblewik, Bobjgalindo, Borowiec, BruceBlaus, Cardioeditor, Catgut, Chill Factor Five, ChrisHodgesUK, Chuck369, Chuckster73, Colm1116, DMG413, Darrel francis, Ddnile, Deli nk, Diberri, Diomedea Exulans, Diptanshu.D, Dlodge, Doctormatt, Drphilharmonic, Dubliner, Easteralexa, Edward, Ers, Faigl.ladislav, Fluffernutter, Gaussgauss, Gongshow, Greg L, Hermitlife, James Foster, Jburns31780, Jfdwolff, Jmh649, Joelmills, Keja27, Kenmaietta, Ksheka, L Kensington, Lenitudent, Lenny Kaufman, Look2See1, Lupo, MER-C, MarcoTolo, Martian, McSush, Mcstrother, MedtronicDave, Michael Hardy, Mik777, Mikael Hggstrm, MrOllie, Najafhaider, Nbauman, Ndsg, Neparis, Nephron, Niceguyedc, Oogapark, Open2universe, PapayaSF, RDBrown, Rasmus Faber, Rich Farmbrough, Rishigupta02445, Rjwilmsi, Rm197800, Robasen, Rockytop62, Sanclaud, Saxbryn, Sbertazzo, Selket, Shaolin md, Sillyfolkboy, Sluzzelin, Sonyack, Spariant, Statkit1, Sun Creator, T-borg, TimBentley, Tonyliepert, Tristanb, TylerDurden8823, Urod, Vanessaezekowitz, Versageek, Wawot1, Woohookitty, Y2Vguy, , 05, 162 anonymous edits Mitral regurgitation Source: https://en.wikipedia.org/w/index.php?oldid=579795000 Contributors: AAAAA, Alexkoo38, Angelito7, Anim8cme, AnjaManix, Arcadian, Argon233, Avreenshah, BD2412, Baa, Barek, Biosthmors, Bob K31416, Bobblewik, Bobjgalindo, Briansal, Burfordc, Catleeu1, Chill Factor Five, Chwats, Cpt ricard, Danny B-), Ddnile, Dexcel, Doodle77, Ecgtocardiology, Edmondtw, Edward, Evwiki, Facts707, Flewis, Fwiesbauer, Goodnightmush, Happy B., Hertz1888, Hu12, Imagologist, Inferior Olive, Jfdwolff, Jimbobolaffsson, Jimw338, Jmh649, Ks64q2, Ksheka, L Kensington, Lenitudent, LeonardoRob0t, LilHelpa, Lisatwo, Mandarax, Meodipt, Mikael Hggstrm, Mpotse, MrOllie, MuffledThud, Ohnoitsjamie, RDBrown, Rejnal, RexxS, Rishigupta02445, Rjwilmsi, SJP, Sam Hocevar, Skrucher, Sloaneguy, Someguy1221, Suprcel, Swamp Ig, Tlabshier, Topbanana, Vdelso, Wafry, Weedwhacker128, Woohookitty, Wouterstomp, Xris0, 80 ,55 anonymous edits Pulmonary valve stenosis Source: https://en.wikipedia.org/w/index.php?oldid=567781522 Contributors: Alansohn, Andrewjlockley, Arcadian, Chill Factor Five, Chmee2, Diptanshu.D, DrAndrewJames, E0steven, Elf, Erindee14, Flowanda, Ghefley, Iron Dragon91, Jaredroach, Jburns31780, Jmh649, Ksheka, LadyofHats, Leeheonjin, Sculpher, Shire Reeve, VandalCruncher, Wouterstomp, 31 anonymous edits Tricuspid insufficiency Source: https://en.wikipedia.org/w/index.php?oldid=563183227 Contributors: Arcadian, Carthu15, Chill Factor Five, Discospinster, Facts707, Filip em, Gaussgauss, Hertz1888, Hojasmuertas, Jfdwolff, Jmh649, Leyo, Mikael Hggstrm, NLMOCPL, Winchelsea, Woohookitty, 27 ,55 anonymous edits Atrial septal defect Source: https://en.wikipedia.org/w/index.php?oldid=582538623 Contributors: .Koen, Aitias, Allyssabeth, Andrewjlockley, Antandrus, Antilived, Arcadian, Atlant, Axl, Barek, Bastet13, BjarteSorensen, Boud, BruceBlaus, CFCF, Cardiac Morph, Cardiac Morphology, Chantal Cooper, Cmdrjameson, CommonsDelinker, Contains Mild Peril, Craigsjones, Crystallina, DMG413, Danjeffers, Davidruben, Ddnile, Deadbeef, Deflective, Diberri, Diptanshu.D, Dlodge, Donsmokem, Dpilchard, DrAndrewJames, Drmanukrishnan, Droll, Drtriple, Dthomsen8, E4043, Editor randy, Ego White Tray, Ejdjr, Ekko, Emact, Ente75, Enuja, Equazcion, Erich gasboy, ExoSynth, Feezo, Fmalan, Fplay, Fredrik, FunkyDuffy, Gene Hobbs, Gogo Dodo, Hairy Dude, Haza-w, Head, Headbomb, Hu12, Hgsippe Cormier, Icarusgeek, Im.a.lumberjack, Irishbugs, ItalianDeception, Jaganath, Jaksmata, JamesMLane, Jason Recliner, Esq., Jbamb, Jburns31780, Jfdwolff, Jfurr1981, Jmccfip, John of Reading, KBi, Kerowyn, Ksheka, Kwamikagami, La goutte de pluie, Leonard Finger, Luckyherb, MER-C, Manco Capac, Mark.murphy, Meddevicefan, Miracle Pen, Moleskiner, Momhoff, MrOllie, NLMOCPL, Nephron, Nippoo, No Guru, Number774, Ojbooker, Paradoctor, Pbsouthwood, Penny Green, Philip Trueman, Ppntori, RJFJR, Redmond111, ReviewDude, Rishigupta02445, Rjwilmsi, RoyBoy, Royalguard11, Rytyho usa, Sachinvenga, Sbharris, Scriberius, Signalhead, Skittleys, Slappy83, Smyth, Smzocco2, Speciate, Srich32977, Stevenfruitsmaak, Sun Creator, Tailor70, Teles, Theodore Kloba, Tmaguild, ToothingLummox, Topbanana, Trabelsiismail, UCLABruin, Verbal, Vladaig, Vyn, Wavelength, Wiki Raja, Woohookitty, Wouterstomp, Yintan, Zachlipton, Zenek.k, , 146 anonymous edits Functional murmur Source: https://en.wikipedia.org/w/index.php?oldid=541752692 Contributors: Arcadian, Aswang, Bobjgalindo, Brianyoumans, Chris the speller, Dr Oluwabusayo, Ixnacious, Nephron, Yworo, 10 anonymous edits Aortic insufficiency Source: https://en.wikipedia.org/w/index.php?oldid=578834245 Contributors: Adavidb, Ageekgal, Angela, Angelito7, Ankit-7, Aqua lem, Arcadian, Aswang, Ataru, Atlant, Barticus88, BenFrantzDale, Bob K31416, Bobjgalindo, Brim, BruceBlaus, Burair, Cmradu, Cpt ricard, Daveharris3737, Davidruben, Ddnile, Deli nk, Dirkbb, Dr galant, Drc79, Dubliner, Eleassar, Ers, Everyking, Facts707, Firsfron, Fitnesseducation, Frank Lofaro Jr., Fwiesbauer, Gaussgauss, Goldenband, Graham87, Guhanramamurthy40, Headbomb, Horatiot burns, Iridescent, Jfdwolff, Jjz3d83, JmCor, Khazar2, Kimathi, Ksheka, Lanternix, Lbeben, Lkinkade, Magioladitis, Mgiganteus1, Mikael Hggstrm, Mike.lifeguard, Mikhailov Kusserow, MrOllie, Nephron, NovaDog, Omnipaedista, Rich Farmbrough, Rishigupta02445, Rytyho usa, Sanclaud, Saxbryn, Send513, SimonP, Skagedal, Snowmanradio, THEN WHO WAS PHONE?, ToNToNi, Trsalmon, Welsh, Woohookitty, Zvar, 72 ,12 anonymous edits Mitral stenosis Source: https://en.wikipedia.org/w/index.php?oldid=581104466 Contributors: Abrahamavich, Akmalhaziq, Akory99, Andthu, Arcadian, Astronautics, Aviados, BD2412, Bobjgalindo, Bongwarrior, BruceBlaus, Chill Factor Five, Chris Capoccia, Chris the speller, Danielmarksheehan, Dcoetzee, Deli nk, Dlodge, Dubliner, Edward, Expergefactionist, Fingers-of-Pyrex, Furqan Tejani, Giraffedata, Graham87, Hermitlife, Jmh649, Khazar, KiranRampersad, Koene, Ksheka, Lenitudent, Lkinkade, Lordmetroid, MYS medicin, Mean as custard, Mikael Hggstrm, Mikr18, Milind27, Mr.alhuwaykim, MrOllie, Nephron, Osm agha, Puldis, RJFJR, Rich Farmbrough, Rishigupta02445, Rytyho usa, Sbertazzo, Skagedal, Tagtagtag, Victorlukankin, Wouterstomp, Ypacara, 49 ,55 anonymous edits Tricuspid valve stenosis Source: https://en.wikipedia.org/w/index.php?oldid=540965027 Contributors: Arcadian, BurwashG, Chill Factor Five, Cxckabin, Darksamus8, Marwan soft, MrOllie, MuZemike, Nonnos333, Nono64, Rishigupta02445, Wouterstomp, 7 ,55 anonymous edits Pulmonary valve insufficiency Source: https://en.wikipedia.org/w/index.php?oldid=555062666 Contributors: Arcadian, Ashleyleia, Bearcat, DoctorKubla, Iridescent, Malcolma, Raimundo Pastor, Redmond111, Riley Huntley, 3 anonymous edits Patent ductus arteriosus Source: https://en.wikipedia.org/w/index.php?oldid=570246967 Contributors: 7mike5000, AbigailAbernathy, Adsllc, Al.locke, Andante, Aneon8, Arcadian, Ayttony, Bgwhite, Bobsagat, BrownCow, Buse69, Chantal Cooper, Chmee2, Chwats, Correogsk, Davidruben, Diberri, Diptanshu.D, Dr.queso, Draeco, Drahkrub, Drrafat1, Ekko, Ers, Flatjosh, Frank Lofaro Jr., Gadfium, Greensburger, Hallbrianh, Happy B., Hechung, Jburns31780, John of Reading, Johnlohmr, Karenjc, Ksheka, Lisarieden, MediguySutton, Mikael Hggstrm, MrOllie, Naidim, Nkiruka, Pulmonological, Registreernu, Renkate, Rich Farmbrough, Rishigupta02445, RupertMillard, SRS32, Saintrain, SeventhHell, Sgpsaros, Shadowfaxes, THB, Tannim101, The Anome, TimBentley, Tushain, Vanished user qkqknjitkcse45u3, Venkatsmf, Vlad, Vladaig, WadeSimMiser, Wiki.saeid, Woodward98, Woodwdan, Wouterstomp, XCatherwood, Zigger, 101 anonymous edits Wheeze Source: https://en.wikipedia.org/w/index.php?oldid=578234464 Contributors: Andycjp, Angelito7, Arcadian, Barticus88, Ceyockey, Chaldor, Chela47, ChrisGualtieri, Cosmocoen, Deli nk, Doc glasgow, Encephalon, Facts707, Flewis, Graham87, Hagaib, Hires an editor, Iamawesome123, Is is Is, J.delanoy, Jmh649, Johann Wolfgang, JustSomeKid, Kwekubo, LadyofShalott, Lladews1, Magioladitis, MrOllie, Mysid, Oddben, Paul Erik, Physchim62, Pkubin, Prunesqualer, Quothquhan, RDBrown, Rambam rashi, Reinthal, Rishigupta02445, SMC, Slightsmile, SonicAD, TheEgyptian, TimBentley, ToddFincannon, Uanfala, Upholder, WhatamIdoing, Wouterstomp, Zakawer, Zhatt, 82 ,55 anonymous edits

Article Sources and Contributors

Stridor Source: https://en.wikipedia.org/w/index.php?oldid=582774525 Contributors: 16@r, AndrooUK, Angelito7, Arcadian, Arjayay, AuburnPilot, Barek, Chowbok, CliffC, DiverDave, Djdoyle, Dr meetsingh, Earthmanweb, FlyHigh, Fredrik, Gchandy, Gonococcus, Graham87, Hudson Stern, JeanandJane, Jfdwolff, Jmh649, Ketyner, Kgrad, Kingpin13, LadyofShalott, Longhair, Lpstubbs, Magioladitis, Mikael Hggstrm, MrOllie, Ncbark, Ninad 1999, Pkubin, Rishigupta02445, Rl, Stjohncj, The Anome, Til Eulenspiegel, Umbumbumbumb, Vindigenous, Watplay, 66 ,55 anonymous edits Rhonchi Source: https://en.wikipedia.org/w/index.php?oldid=581207877 Contributors: Andrewjlockley, Arcadian, Artlung, Fountains of Bryn Mawr, Honbicot, J.delanoy, JWaters, Jfdwolff, Jmh649, Khazar2, Kilo-Lima, Kwiki, LadyofShalott, M1ss1ontomars2k4, Magioladitis, MrOllie, Niceguyedc, Nmg20, Nono64, Phoebe, Pkubin, Rishigupta02445, Rutigor, Shashikiranu, Transverse, Uthbrian, Viper875, 26 anonymous edits Crackles Source: https://en.wikipedia.org/w/index.php?oldid=577603108 Contributors: Anthonyhcole, Arcadian, Boonshofter, Courcelles, Dcfleck, DePiep, Devil Master, Doctortomwiki, Dreamfrolica, Drgarden, DuLithgow, Euryalus, Everard Proudfoot, Facts707, Fl295, Flyer22, Fountains of Bryn Mawr, Gak, Gene Nygaard, Gobonobo, GreenReaper, Jfdwolff, Jmh649, Kate Carter, LittleT889, Magioladitis, Maxxicum, Merovingian, Mikael Hggstrm, MrOllie, Pearle, Phil5329, Philip Trueman, Pkubin, Queequeg804, Rishigupta02445, SnowFire, Sparky the Seventh Chaos, Susfele, Tewfik, The Thing That Should Not Be, TheParanoidOne, Tinton5, Transverse, TylerDurden8823, Varlaam, Vegaswikian, W.Ross, WhatamIdoing, 49 anonymous edits Pertussis Source: https://en.wikipedia.org/w/index.php?oldid=581527790 Contributors: (jarbarf), 0kmck4gmja, 14kbowen, 28bytes, 5 albert square, Abeg92, Adashiel, Agateller, Agentscott00, Ahoerstemeier, Alansohn, Alerante, Alex.tan, AlphaEta, Ambark80, Amplusquem, Andrewman327, Andylepper, Anetode, Anil.sharma085, Antandrus, Aptenodytes, Arcadian, Aristiana, AtxApril, Aubreyd, Ayudante, Azhyd, Babbage94, Bart133, BenAveling, Benjamintchip, Beyond My Ken, BigrTex, Blanchardb, Bluebasket, Bluerasberry, Bobo192, Bubblesismycat, BuckwikiPDa535, Buddhafinder, Butwhatdoiknow, CDN99, CWii, Calton, Can't sleep, clown will eat me, CanadianLinuxUser, Cardboard911, CattleGirl, Cdcon, Cdlw93, Chaos Wolf, Choess, ChrisGualtieri, Colonies Chris, Coma28, ComfyKem, Copernicus151, Corecttman, Corinne68, Countincr, Courcelles, Cpt ricard, Crazysane, Cyan, D-Rock, DanieleProcida, Davidruben, Dawnseeker2000, Dcsohl, Deflective, Derekt75, Discospinster, DocWatson42, Dogbreath123456, Donner1122, Draeco, Drilnoth, Dustinlull, Dysepsion, EbenVisher, Eleassar, Electronsoup, Elembis, Elizabeth Blandra, Eluchil404, Eras-mus, Ethel the aardvark, Eubulides, Evanherk, Exert, Fenwayguy, Ffreisinger, Firegoat, Fixmacs, Fl1n7, Fotter, Frecklefoot, Freddyd945, FrenchIsAwesome, Frustro, Fuzbaby, F, GPHemsley, Gabbe, Gaius Cornelius, Gandydancer, Geneb1955, GeorgeLouis, Gilliam, Ginger Maine Coon, Ginkgo100, Gloriamarie, Gogo Dodo, Gongshow, GorillaWarfare, Graham87, GrahamColm, GrantGannon, Grim23, Grover cleveland, Gullywhumper, Gurps npc, Hadal, Henkeldg, Herorev, Heuvel41, Hmrox, Hughreid, Hunterkb, IGeMiNix, IINoScopeII, IanManka, Ibrmrn3000, Icairns, Idwal2000, Igoldste, Ikeday10021, Imesj, J.delanoy, JForget, Jacob3456, James McNally, JamesAM, JamesBWatson, Jasper Deng, Javert, Jesanj, Jimp, Jivecat, Jmh649, Jmlk17, John Nevard, Johngsndhdsfgds, Johnwcowan, Josh Grosse, Jpatokal, Jpz109, Jtpjtpjtp, Jvaigl, Karlhahn, Kelisi, Killiondude, Kingturtle, KnowledgeOfSelf, KoolerStill, Krakkles, Kshieh, Kwamikagami, L Kensington, Lacrimosus, Lew.Vad, Lights, LilHelpa, Loqi, LuK3, Lucien504, MD Prof, MER-C, Mani1, Manuel Anastcio, MarcoTolo, Marechal Ney, Marek69, Martianman9, Marx01, Master of Puppets, Mathwhiz 29, Matt Deres, Mdf, Meaningful Username, Mendalus, Mikael Hggstrm, Minnsurfur2, MistyMorn, Mkativerata, Moe Epsilon, Mojo Hand, Moppet65535, Mpulier, Mr Stephen, MrChupon, Msgarrett, Muchness, Mumia-w-18, Myocastor, Nakon, Naniwako, Narom, Nde789, Nicholas28, NighthawkJ, Nintendo, Noobishness, Novangelis, Nox noctis vicis, Ntsimp, Numbo3, Nunquam Dormio, Nuplex, Nyenyec, Nyq, Officiallyover, Onco p53, Ondewelle, Oxymoron83, PaleWhaleGail, Patar knight, Pb30, Persekka, Persian Poet Gal, Pflags, Pgan002, Phgao, Philip Trueman, Pietrow, Pongidae, Ppaulojr, Ppfk, Pulmonological, Quebec99, Quinnanya, RDBrown, Rachel Pearce, Raduga, Ramaksoud2000, RandomBagcheck, Raph, Relly Komaruzaman, Renaissancee, RexNL, Rich Farmbrough, Richard Arthur Norton (1958- ), Riffle, Rjwilmsi, Rmhermen, Rmt2m, Rohanww, Roulph, Rrburke, SMB McFizz, SN1P3RFR34K, Samkung86, Samw, Scjessey, Sebras, Shanask, Shipmaster, Sk19842, Skidude9950, Slon02, Snorlax Monster, Snow Blizzard, Snowolf, Solicitationsbox, Solomonfromfinland, Someone else, Sonelle, Spiritia, Stevenfruitsmaak, Steviedpeele, Sun Creator, Teejayess, Tesscass, ThatsNotFunny, The Thing That Should Not Be, TheJJJunk, Theleftorium, Thempp, Tide rolls, Timvasquez, Tobus2, Tommy2010, Trusilver, Turzh, Tydude187, Ugen64, Ukexpat, Ultra Venia, Uncle Dick, Uncle Milty, User27091, Utcursch, Vedran12, Vicki Rosenzweig, Victor Engel, Vrenator, Warofdreams, Waterthedog, Wayne Slam, WeightyWords, Werdnawerdna, Wham Bam Rock II, WhatamIdoing, Wheasley, Whollygrayle, Whywhenwhohow, Wiki alf, WikiDao, Wikihund, Wilsonchas, Wimt, Wknight94, Wolfmankurd, Wouterstomp, Xandumx, Yangosplat222, Yosri, Yvesnimmo, Zukogray, Zzyzx11, , 700 anonymous edits

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