CONGENITAL HEART DISEASE

1. Winfrey Pangestu (07120080034 ) 2. Anita (0712008 3. Laura Wijaya ( 0712008 4. Pusparina (0712008
5.

Ruth ( 0712008

6. Andry ( 0712008 7. Mahavira Guntur ( 0712008 8. Amelia Tirta (0712008 9. Johnny Wijaya (0712008 10. Gabby (0712008

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VENTRICULAR SEPTAL DEFECT DEFINITION : Ventricular septal defect describes one or more holes in the wall that separates the right and left ventricles of the heart. Ventricular septal defect is one of the most common congenital (present from birth) heart defects. It may occur by itself or with other congenital diseases. PATHOLOGY : Before a baby is born, the right and left ventricles of its heart are not separate. As the fetus grows, a wall forms to separate these two ventricles. If the wall does not completely form, a hole remains. This hole is known as a ventricular septal defect, or a VSD. The heart is forming during the first 8 weeks of fetal development. It begins as a hollow tube, then partitions within the tube develop that eventually become the septa (or walls) dividing the right side of the heart from the left. Ventricular septal defects occur when the partitioning process does not occur completely, leaving an opening in the ventricular septum. Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Most ventricular septal defects occur sporadically (by chance), with no clear reason for their development. Ventricular septal defect is the most common congenital heart defect. The baby may have no symptoms, and the hole can eventually close as the wall continues to grow after birth. If the hole is large, too much blood will be pumped to the lungs, leading to heart failure. These babies often have symptoms related to heart failure and may need medicine to control the symptoms and surgery to close the hole. The hole can also be closed without surgery, through cardiac catheterization. The cause of VSD is not yet known. This defect often occurs along with other congenital heart defects. In adults, ventricular septal defects are a rare but serious complication of heart attacks. These holes are related to heart attacks and do not result from a birth defect. SYMPTOMPS : Patients with ventricular septal defects may not have symptoms. The most common symptoms, however, include:

Shortness of breath Fast breathing

Hard breathing Paleness Failure to gain weight Fast heart rate Pounding heart Sweating while feeding Frequent respiratory infections

CLASSIFICATION OF VSD : KLASIFIKASI :

1.VSD perimembran : a. Defek perimembran inlet (mengarah ke posterior ke daerah inlet septum ) b. Defek perimembran outlet (mengarah ke anterior, di bawah aortic root ke daerah septum pars muskularis) c. Defek trabekular ( mengarah kebawah , kearah septum trabekularis ) d.Defek perimembran konfluen (yang mencakup bagian septum muskularis defek sangat besar ) 2.Defek septum muskular : a.Defek muskular inlet b.Defek muskular trabekular c.Defek muskular outlet 3. Defek subarterial (m double commited subarterial defek )

Why is ventricular septal defect a concern?

If not treated, this heart defect can cause lung disease. When blood passes through the VSD from the left ventricle to the right ventricle, a larger volume of blood than normal must be handled by the right side of the heart. Extra blood then passes through the pulmonary artery into the lungs, causing higher pressure than normal in the blood vessels in the lungs. A small opening in the ventricular septum allows a small amount of blood to pass through from the left ventricle to the right ventricle. A large opening allows more blood to pass through and mix with the normal blood flow in the right heart. Extra blood causes higher pressure in the blood vessels in the lungs. The larger the volume of blood that goes to the lungs, the higher the pressure. The lungs are able to cope with this extra pressure for while, depending on exactly how high the pressure is. After a while, however, the blood vessels in the lungs become diseased by the extra pressure. As pressure builds up in the lungs, the flow of blood from the left ventricle, through the VSD, into the right ventricle, and on to the lungs will diminish. This helps preserve the function of the lungs, but causes yet another problem. Blood flow within the heart goes from areas where the pressure is high to areas where the pressure is low. If a ventricular septal defect is not repaired, and lung disease begins to occur, pressure in the right side of the heart will eventually exceed pressure in the left. In this instance, it will be easier for oxygen-poor (blue) blood to flow from the right ventricle, through the VSD, into the left ventricle, and on to the body. When this happens, the body does not receive enough oxygen in the bloodstream to meet its needs. Because blood is pumped at high pressure by the left ventricle through the VSD, tissue damage may eventually occur in the right ventricle. Bacteria in the bloodstream can easily infect this injured area, causing a serious illness known as bacterial endocarditis.

Some ventricular septal defects are found in combination with other heart defects (such as in transposition of the great arteries). TO DIAGNOSE VSD : Your child's physician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the opening from the left side of the heart to the right. A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. The location within the chest where the murmur is heard best, as well as the loudness and quality of the murmur (harsh, blowing, etc.) will give the cardiologist an initial idea of which heart problem your child may have. However, other tests are needed to help with the diagnosis, and may include the following:

chest x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. With a VSD, the heart may be enlarged because the right ventricle handles larger amounts of blood flow than normal. Also, there may be changes that take place in the lungs due to extra blood flow that can be seen on an x-ray. electrocardiogram (ECG or EKG) - a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle stress. echocardiogram (echo) - a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. An echo can show the pattern of blood flow through the septal opening, and determine how large the opening is, as well as much blood is passing through it. cardiac catheterization - a cardiac catheterization is an invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to more clearly visualize the structures inside the heart.

Treatment for ventricular septal defect:

Specific treatment for VSD will be determined by your child's physician based on:

your child's age, overall health, and medical history extent of the disease your child's tolerance for specific medications, procedures, or therapies expectations for the course of the disease your opinion or preference

Small ventricular septal defects may close spontaneously as your child grows. A larger VSD usually requires surgical repair. Regardless of the type, once a ventricular septal defect is 5

diagnosed, your child's cardiologist will evaluate your child periodically to see whether it is closing on its own. A VSD will be repaired if it has not closed on its own - to prevent lung problems that will develop from long-time exposure to extra blood flow. Treatment may include:

medical management Some children have no symptoms, and require no medication. However, most children may need to take medications to help the heart work better, since the right side is under strain from the extra blood passing through the VSD. Medications that may be prescribed include the following: o digoxin - a medication that helps strengthen the heart muscle, enabling it to pump more efficiently. o diuretics - the body's water balance can be affected when the heart is not working as well as it could. These medications help the kidneys remove excess fluid from the body. adequate nutrition Infants with a larger VSD may become tired when feeding, and are not able to eat enough to gain weight. Options that can be used to ensure your baby will have adequate nutrition include the following: o high-calorie formula or breast milk Special nutritional supplements may be added to formula or pumped breast milk that increase the number of calories in each ounce, thereby allowing your baby to drink less and still consume enough calories to grow properly. o supplemental tube feedings Feedings given through a small, flexible tube that passes through the nose, down the esophagus, and into the stomach, can either supplement or take the place of bottle feedings. Infants who can drink part of their bottle, but not all, may be fed the remainder through the feeding tube. Infants who are too tired to bottle feed may receive their formula or breast milk through the feeding tube alone. infection control Children with certain heart defects are at risk for developing an infection of the inner surfaces of the heart known as bacterial endocarditis. It is important that you inform all medical personnel that your child has a VSD so they may determine if the antibiotics are necessary before a procedure. surgical repair The goal is to repair the septal opening before the lungs become diseased from too much blood flow and pressure. Repair is indicated for defects that are causing symptoms, such as poor weight gain and rapid breathing. Your child's cardiologist will recommend when the repair should be performed based on echocardiogram and cardiac catheterization results. The operation is performed under general anesthesia. Depending on the size of the heart defect and your physician's recommendations, the ventricular septal defect will be closed with stitches or a special patch. Consult your child's cardiologist for more information.

interventional cardiac catheterization Your child's VSD may be repaired by a cardiac catheterization procedure. One method currently being used to close some small muscular VSDs is the use of a 6

device called a septal occluder. During this procedure, the child is sedated and a small, thin flexible tube is inserted into a blood vessel in the groin and guided into the heart. Once the catheter is in the heart, the cardiologist will pass the septal occluder into the VSD. The septal occluder closes the ventricular septal defect providing a permanent seal.

Common Atrioventricular Canal Defect

DEFINITION AV canal defects are characterized by defects in isolation or combination including an ASD in the lowermost part of the atrial septum (ostium primum), a cleft of the mitral valve (either alone in combination with a cleft of the tricuspid valve), or VSD. PATHOLOGY The ostium primum type of ASD is characterized by a crescent-shape upper border with no septal tissue forming the lower border. The lower aspect of the defect is bounded by the atrial surfaces of the AV valves and, in the complete type, in part by the upper edge of the ventricular septum.

ANATOMICAL TYPES Variations occur with respect to the nature of the AV valves. Partial type. The ostium primum ASD is associated with a cleft in the anterior mitral leaflet or probably more accurately, a septal commisure between the superior and inferior

leaflets of the left AV valve. The tricuspid valve is not cleft or shows a minor central deficiency. The ventricular aspects of the anterior mitral valve elements are fused to the upper edge of the deficient ventricular septum, precluding an interventricular communication. If there is no atrial septal tissue or if the atrial septum is sio rudimentary that is produces a common chamber involving both atria, the term common atrium or single atrium is applied. Complete type. The complete type of common AV canal is characterized by failure of partitioning of the primitive canal into separate AV orifices, The orifice between the atria and ventricles is guarded by a common valve with the anterior leaflet derived from the AV endocardial cushion and represents the anterior halves of the anterior mitral and septal tricuspid leaflets, The posterior leaflet originates from the dorsal AV endocardial cushion and represents the posterior halves of the atrial mitral and septal tricuspid leaflets. Associated Conditions. In the asplenia syndrome, the complete variety is almost universal; with polysplenia, it occurs in about one quarter of cases. An ASD of the secundum type is present in about half of these cases. A double orifice of the mitral valve may be associated with the incomplete type, and tetralogy of Fallot may be associated with the complete type. Abnormal Physiology. If the communication at the ventricular level is large, the right ventricular and pulmonary artery pressures will be elevated. These patients are are similar to those with large VSDs. Patients with a communication at the atrial level usually have only normal or slightly elevated systolic pressures in the right side of the heart and large pulmonary blood flow, as in the secundum type of ASD. Defects in the tricuspid valve, mitral valve, or both may result in severe regurgitation or direct shunting of blood from the left ventricle to the right atrium. CLINICAL MANIFESTATIONS 3 percent infant and children with congenital heart defects have AV canal defects. Female and men ratio is 13:1. over half patients with the complete form have associated with down syndrome. History Only if the mitral palveis incompetent do the symptom of the patients with partial AV canal differ from those associated with a secundum type of ASD. The complete form of AV canal or the partial form connected with significant nitral regurgitation may be associated with weight gain, easy fatique, tachypnea, repeated respiration infection and congestive heart failure. Patients with complete AV canal are almost invariably very sick 8

PHYSICAL EXAMINATION The finding with partial defects are those of an ASD. If the cleft anterior mitral leaflet is incompetent, the findings of mitral regurgitation also will be present. The physical findings with the complete AV canal defects are those very large VSD, usually with full brown congestive failure, but the second heart sound is split and fixed. The murmur of mitral regurgitation may not be heard or recognized as such. Chest Roetgenogram Overall cardiac enlargement that is out of proportion to the degree of pulmonary plethora or a cardiacsilhoettr, suggesting combined ventricular dilatation may serve to distinguish uncomplicated secumdum ASD from a primum defect with significant mitral regurgitation. Marked cardiac enlargement and severe pulmonary overcirculation are features of complete AV canal defects.

Electrocardiogram Helpful diagnostic to distinguish individual with Av canal defects from those with AV canal defects from those with isolated ASDs and VSDs is the charactheristic superior orientation of the mean QRS axis in the frontal plane, with a right bundle branch delay in the precordial leads. The patterns of atrial and ventricular hypertrophy reflect the underlying hemodynamic abnormalities. Echocardiogram Two dimensional echo is capable of visualizing the extend of septal defects and with Doppler study and color flow mapping, left to right shunting at the atrial and ventricular level and associated mitral or tricuspid valvular regurgitation. The anatomic features of the anterior AV leaflet and its connections may be visualized with sufficient clarity to permit subdivision of complete AV canal defects into types A,B and C. straddling AV valves, a double orifice mitral valves, single pappilary muscles and hypoplasia or outflow obstruction of the right or left ventricle also can be determined with this technique. Cardiac Catheterization. Cardiac catheterization is rarely performed if the echocardiogram is characteristic and if the history, clinical examination, and echo suggest a large left-to-right 9

shunt and low pulmonary resistance. When it is performed, a significant increase in oxygen saturation between the superior vena cava and the right atrium is present. A right ventricular or pulmonary arterial systolic pressure in excess of 60 percent of the systemic systolic pressure favors the presence of a complete canal. With a large communication between the two ventricles below the AV valves, the right ventricular, pulmonary arterial, and systemic arterial systolic pressure are virtually identical. Left ventricular angiography in the frontal view demonstrates the gooseneck deformity of the left ventricular outflow tract that is characteristic of AV canal malformations and allows a semiquantitative assessment of the degree of mitral regurgitation and shunting from the left ventricle to the right atrium. The left anterior oblique view with craniocaudal anglation is recommended for visualizing the interventricular defect and judging the extent of ventricular septal deficiency. Aortography is essential to eliminate the possibilityof a PDA if the echocardiogram was not diagnostic.

Patent Ductus Arteriosus

DEFINITION The most common type of extracardiac shunt, represents persistents patency of the vessels that normally connects pulmonary arterial systems and the aorta in the fetus. PATHOLOGY Ductus arteriosus usually closes within 2 or 3 days after birth and became ligamentum arteriosum but it will be patent for several month prior to spontaneous closure. It is from the origin of the left pulmonary artery below to the aspect of the aortic arch just beyond the level of the origin of the left subclavian artery above. The recurrent branch of the left vagus nerve hooks around the lateral and inferior. Ductus postnatally constriction involve a complex interaction and increase partial pressure of oxygen, decrease circulating and ductal receptors of PGE2 and finally decrease the pressure. The vessels wall hypoxia of the ductus promotes further closure through inhibition the prostaglandin and nitric oxide within ductal wall. Exogenous PGE1 has been used to keep the ductus open postnatally and indomethacin, a prostaglandin inhibitor, can close the ductus in many premature infants in whom persistent patency is disadvantageous. ABNORMAL PHYSIOLOGY

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Patients with PDA divided into groups according whether the vascular resistance through the ductus is low, moderate or high. The ductus resistance related not only to its cross-sectional area but also to its length. In patients with a very small ductus that offers high resistance, the flow across the ductus is relatively small. Extra volume of work of the left ventricle is small and the pulmonary pressure and resistance are not elevated. In patients with large patent ductus, the aorta and pulmonary artery are essential in free communication and the systolic pressure in the pulmonary artery equal to that in the aorta. Left ventricular volume overload result from recirculation through the lungs, with pulmonary congestion resulting from increased pulmonary flow and left ventricular failure. The left ventricle compensates by dilation followed in many cases by hyperthrophy, and pulmonary vasculature may respond to the high pressure. The right ventricle is subjected to a pressure load. If the pulmonary resistance equals or exceed the resistance of the systemic circulation, there is right to left shunting from the pulmonary artery to the aorta, resulting the hypoxemia especially in the lower body and legs.

CLINICAL MANIFESTATIONS History. The history of the mothers pregnancy and of perinatal events may provide clues associated with a high incidence of PDA, such as exposure to rubella in the first trimester. PDA is also most common in premature infants especially those with birth asphyxia or respiratory distress. Heart failure is most likely to develop in the first few weeks or month of life. The clinical presentation in a premature infant is usually very different from that in a full term infant, particularly in one with a birth weight under 15 kg.

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PHYSICAL EXAMINATION In full term in infant or child with PDA, there is frequently a systolic thrill over the pulmonary and in the suprasternal notch. A patient with elevated pulmonary vascular resistance and a right-to-left shunt will have differential cyanosis. The apical impulse may be increased or displaced in those with large shunts. The typical murmur is a continuous murmur best heard at the left upper sternal border and below the left clavicle. It is usually a rough murmur with eddy sounds, which are helpful in making a diagnosis. In patients with at least a moderate shunt, there is a mid diastolic rumble at the apex as a result of relative mitral stenosis from increased flow across the mitral valve. The second heart sound may be difficult to hear because of the continuous murmur, but it is usually normal. The pulmonary component is attenuated in those with pulmonary hypertension.

Chest Roentgenogram
Finding on chest roentgenography are also dependent on the magnitude of the shunt. In patients with a small shunt, the chest roetgenogram is normal. In the presence of heart failure, there are signs of pulmonary edema.

Electrocardiogram
Left atrial hypertrophy is probably the most common abnormality found, but left ventricular hypertrophy of the volume overload type with deep Q wave and increase R wave voltage in the left precordial leads, is also common as the shunts size increase and left ventricular dilation occur. Right ventricular hypertrophy is seen with pulmonary hypertension.

Cardiac catheterization
In those with typical, uncomplicated PDA, cardiac catheterization is not necessary unless closure in the catheterization lab is contemplated. When catheterization is perform usually passes preferentially from the left pulmonary artery into the descending aorta except when the ductus is too small. The saturation is increased in the pulmonary artery compare with the right atrium and ventricle to a degree relative to the size of the shunt. The pulmonary arterial and right ventricular pressure are elevated in those with a large ductus. The pulmonary vascular resistance is elevated in older patient who have changes in pulmonary vascular bed. The patient also diminished saturation in descending aorta once the pulmonary resistance reach a level that will reverse the shunt.

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References
http://www.emedicine.com/ped/ http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/avsd.htm http://www.he.net/~altonweb/cs/downsyndrome/index.htm?page=cardpda.html http://www.emedicine.com/ped/topic2834.htm http://www.healthsystem.virginia.edu/uvahealth/ http://images.google.co.id/imgres?imgurl=http://www.healthsystem.virginia.edu/ http://images.google.co.id/imgres?imgurl=http://www.cvimaging.northwestern.edu/ http://www.medicine.cmu.ac.th/dept/radiology/ http://myweb.lsbu.ac.uk/dirt/museum/ http://myweb.lsbu.ac.uk/dirt/ http://connection.lww.com/products/sadler/

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