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Cardiomyopathy: An Overview

Lori Ann Daughenbaugh


ABSTRACT Cardiomyopathies and their resultant systolic and diastolic heart failure remain the main cause of cardiovascular morbidity and mortality in both children and adults and are a frequent indication for cardiac transplantation.According to the American Heart Association 2005 Heart and Stroke Update, more than 26,000 deaths each year in the United States are caused by cardiomyopathy. Cardiomyopathy is second to coronary artery disease for the most common direct cause of sudden death in the United States and is a leading cause of heart failure.This article provides an overview of the pathophysiology, causes, signs and symptoms, diagnosis, and treatment of the different types of cardiomyopathies. Newer therapeutic modalities and pharmacologic interventions are discussed, with an emphasis on improving symptoms and long-term survival. Keywords: cardiomyopathy, diastolic dysfunction, dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, systolic, systolic dysfunction

Cardiomyopathies and their resultant systolic and diastolic heart failures remain the main cause of cardiovascular morbidity and mortality in both children and adults and are a frequent indication for cardiac transplantation.According to the American Heart Association 2005 Heart and Stroke Statistical Update more than 26,000 deaths each year in the United States are caused by cardiomyopathy. Cardiomyopathy is second to coronary artery disease for the most common direct cause of sudden death in the United States.1 In 1995, the World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of the Cardiomyopathies defined cardiomyopathies as diseases of the myocardium associated with cardiac dysfunction.2 The purpose of this article is to provide the advanced practice nurse with an overview of the pathophysiology, causes, signs and symptoms, diagnosis, and
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management of the different types of cardiomyopathies. Newer therapeutic modalities and pharmacologic interventions are discussed, with an emphasis on improving symptoms and long-term survival. SYSTOLIC VERSUS DIASTOLIC DYSFUNCTION Systolic dysfunction is characterized by a decrease in myocardial contractility.A reduction in the left ventricular ejection fraction (LVEF) results when myocardial contractility is decreased throughout the left ventricle. Cardiac output is maintained in two ways: left ventricular enlargement results in a higher stroke volume, and the Frank-Starling relationship (an increase in contractility in response to an increase in stretch). However, these compensatory mechanisms are eventually exceeded and cardiac output decreases, resulting in the physiologic manifestations of heart failure.3 The left heart cannot pump with enough force to push a
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Figure 1. Diagrams of a normal heart and a heart with dilated cardiomyopathy. Adapted from: www.nscardiology.com/factscardiomyopathy.htm.

Normal Heart

Heart with Dilated Cardiomypathy

Left ventricle Right Ventricle

Heart chambers relax and fill, then contract and pump.

Muscle fibers have stretched. Heart chamber enlarges.

sufficient amount of blood into the systemic circulation. This leads to fluid backing up into the lungs and pulmonary congestion. Systolic dysfunction is a characteristic of dilated cardiomyopathy (DCM). It is also seen in some patients with hypertrophic cardiomyopathy (HCM) who develop progressive left ventricular dilatation and a decrease in LVEF. In general terms, systolic dysfunction is defined as an LVEF less than 40%.4 Diastolic dysfunction refers to cardiac dysfunction in which left ventricular filling is abnormal and is accompanied by elevated filling pressures.The diastolic phase of cardiac function includes two components. Left ventricular relaxation is a process that takes place during isovolemic relaxation (the period between aortic valve closure and the mitral valve opening) and then during early rapid filling of the ventricle. Later in diastole, after relaxation is complete, further left ventricular filling is a passive process that depends on the compliance, or distensibility, of the myocardium.The ventricles are unable to relax, and subsequent muscle hypertrophy occurs which then leads to inadequate filling.3 Diastolic dysfunction may lead to fluid accumulation, especially in the feet, ankles, and legs, and some patients may also have pulmonary congestion. For patients with heart failure but without systolic dysfunction, diastolic dysfunction is the presumed cause. Diastolic dysfunction is characteristic of both HCM and restrictive cardiomyopathy (RCM).3 However, some component of diastolic dysfunction is also common in patients with DCM. In general terms, diastolic dysfunction is defined as an LVEF of greater than 40%. Diastolic dysfunction is more difficult to identify with echocardiograph scanning than systolic dysfunction, and it may be missed or underestimated in many cases.
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Doppler scan assessment of transmitral flow is the standard approach to detect diastolic dysfunction, although a variety of other measurements can be used.3 It is important to understand that some of the symptoms of systolic and diastolic heart failure are similar. DILATED CARDIOMYOPATHY Anatomic and Physiologic Classification DCM, the most common form of cardiomyopathy, is characterized by enlargement of one or both ventricles accompanied by systolic and diastolic contractile dysfunction and symptoms of heart failure.5 In DCM, myocardial muscle mass is increased and ventricular wall thickness is reduced.The heart assumes a globular shape, and there is pronounced ventricular chamber dilatation, diffuse endocardial thickening, and atrial enlargement often with thrombi in the appendages.6 The heart muscle becomes thin and weakened and is unable to pump the blood efficiently.The heart muscle stretches and dilates so that it can hold more but in time becomes even weaker, leading to symptoms of heart failure.These structural changes decrease the amount of blood ejected from the ventricle with systole and allow more blood in the ventricle after contraction.A smaller volume of blood enters the ventricle during diastole and increases end-diastolic pressure and pulmonary pressures.The enlarged stretched ventricle alters valvular function, usually resulting in regurgitation. Left ventricular dilatation occurs as venous return and systemic vascular resistance rise. Eventually, the atria also dilate as more work is required to pump blood into the full ventricles. Cardiomegaly occurs as a consequence of dilatation of the atria and ventricles. Blood pooling in the ventricles increases the risk of emboli (Figure 1).
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DCM has an incidence of 5 to 8 cases per 100,000, with a prevalence of 36 per 100,000.4 These figures may underestimate the disease because so many patients with DCM are asymptomatic. Men and blacks are at greatest risk. Other risk factors include coronary artery disease, hypertension, pregnancy, viral infections, and use of alcohol or illegal drugs. Because DCM is not diagnosed until its advanced stages, the prognosis is generally poor.2 Cause The complete list of causes of DCM is extensive. DCM can be caused by a variety of disorders (Table 1).The most common causes are ischemic cardiomyopathy, valvular cardiomyopathy, viral cardiomyopathy and genetic cardiomyopathy.3 In many cases, however, no cause can be found, and the cardiomyopathy is deemed idiopathic. In the United States and all industrialized countries, the most common form of DCM is termed ischemic, and it reflects left ventricular dilatation after myocardial infarction.The next largest category is idiopathic, in which no definitive cause can be found. Of patients diagnosed with idiopathic cardiomyopathy, approximately 25% have a familial component.5 Clinical Presentation The patient with DCM usually has progressive symptoms of heart failure. Most patients present between 20 and 60 years of age, but DCM can also occur in children and the elderly.7 Symptoms often develop insidiously with exercise intolerance, dyspnea, orthopnea, paroxysmal nocturnal dyspnea, fatigue, and a dry cough at night as a result of left-sided heart failure.The physical examination provides a useful estimate of the severity of hemodynamic dysfunction.The blood pressure is variable, and the pulse pressure tends to narrow as the disease progresses. Jugular venous distention, peripheral edema, hepatomegaly, and weight gain caused by right-sided heart failure are also common. Peripheral cyanosis, cool extremities, decreased blood pressure, and tachycardia may be present as a compensatory response to low cardiac output. Palpation indicates evidence of cardiac enlargement. S3 and S4 gallops associated with heart failure are typically found with auscultation. Murmurs of mitral and tricuspid regurgitation secondary to cardiomegaly and weak papillary muscles are common. Bibasilar rales, an indication of pulmonary congestion, may be heard with auscultation of the lungs.
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Table 1. Main Causes of Dilated Cardiomyopathy


Ischemia Infectious diseases
Viral Coxsackie virus Cytomegalovirus HIV Varicella Hepatitis Epstein-Barr virus Echovirus Other Bacterial Streptococci, rheumatic fever Typhoid fever Diphtheria Brucellosis Psittacosis Rickettsial disease Lyme disease Mycobacteria, fungal Histoplasmosis Cryptococcosis Parasitic Toxoplasmosis Trypanosomiasis Schistosomiasis Trichonosis

Deposition diseases
Hemochromatosis Amyloidosis

Medications
Chemotherapeutic agents Anthracyclines (doxorubicin) Cyclophosphamide Trastuzumab Antiretroviral drugs Zidovudine Didanosine Zalcitabine Phenothiazines Chloroquine

Table 1 Continued on next page.

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Table 1. Main Causes of Dilated Cardiomyopathy


Toxins
Alcohol Cocaine Amphetamines Cobalt Lead Mercury Carbon monoxide Beryllium

Electrolyte abnormalities
Hypocalcemia Hypophosphatemia Uremia Nutritional deficiencies Thiamine Selenium Rheumatologic diseases Systemic lupus erythematosus Scleroderma Giant cell arteritis Endocrinologic disorders Thyroid hormone excess or deficiency Growth hormone excess or deficiency Pheochromocytoma Diabetes mellitus Cushing disease

Sometimes DCM is diagnosed as a result of family or routine medical screening. It is important to take a careful family history, because family screening accounts for a large number of patient referrals. Less common presentations include systemic embolization, arrhythmias, and sudden death.8 The clinical course is largely unpredictable in each individual patient and may depend in part on the cause of the heart disease.The most important diseaseindependent predictors of survival are New York Heart Association (NYHA) functional class, LVEF, and maximal oxygen consumption.9 Diagnosis The initial evaluation of the patient suspected of having DCM should include an electrocardiogram (ECG), echocardiogram, and chest x-ray.The results of the ECG are occasionally normal, but more often they show nonspecific abnormalities or bundle branch block.Abnormal Q waves suggest that prior myocardial infarction is the cause of left ventricular dysfunction.An echocardiogram will assess chamber sizes, wall thickness, and ventricular function. LVEF can be measured, and valvular regurgitation and left ventricular mural thrombi can be detected. The results of the chest x-ray show evidence of cardiomegaly and possibly pulmonary congestion, pulmonary venous hypertension, and pleural or pericardial effusion. Cardiac catheterization is seldom of specific value unless myocardial ischemia or left ventricular aneurysm is suspected. Medical Management The fundamental goals of drug therapy for DCM are to relieve symptoms, improve functional status, prevent disease progression, and reduce the rates of morbidity and mortality.10 The medical management of symptomatic patients is similar to that for heart failure for any cause. Currently, DCM is not curable, although some patients improve spontaneously. Management of DCM is multifaceted.The underlying cause should be identified and treated if possible.The relief of symptoms such as dyspnea and improvement in exercise tolerance is best accomplished by using a combination of diuretics and vasodilators to reduce fluid retention. Several trials have shown that vasodilator therapy with either angiotensin-converting enzyme (ACE) inhibitors (to reduce afterload through vasodilatation) or the combination of nitrates (isosorbide dinitrate) and
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Neuromuscular diseases
Duchenne muscular dystrophy Myotonic dystrophy Friedreich ataxia

Miscellaneous
Peripartum cardiomyopathy Persistent tachycardia Sarcoidosis Familial cardiomyopathies Sleep apnea Autoimmune myocarditis Radiation Calcium overload Oxygen free radical damage Myocarditis, idiopathic
Reproduced with permission from Weigner, M, Morgan JP. Causes of dilated cardiomyopathy. In: Up To Date, Rose, BD, (Ed), Waltham, MA. 2006. Copyright 2006 Up To Date, Inc. For more information visit www.uptodate.com.

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hydralazine, to produce vasodilatation, improves survival in patients with impaired left ventricular systolic function.Angiotensin II receptor blockers should be considered for patients intolerant of ACE inhibitors as a result of cough or angioedema because they have a similar therapeutic effect. -Blockers also play an important role in the treatment of DCM.These agents should be prescribed for all patients with stable heart failure as a result of systolic dysfunction of the left ventricle unless contraindicated.According to the practice guidelines of the American College of Cardiology/American Heart Association, patients with mild symptoms should receive a blocker to reduce the risk of disease progression, future clinical deterioration, and sudden death.11 As a class they reduce the rate of mortality and the frequency of hospitalization in patients with heart failure and were shown to increase LVEF. Currently, only carvedilol and long-acting metoprolol are approved by the US Food and Drug Administration for use in heart failure.These drugs must be started at low doses and gradually uptitrated.12 Digoxin has long been used for the treatment of DCM. It improves contractility and slows certain fast heart rhythms, which improves circulation. Digoxin is the only available oral agent with mild, positively inotropic activity that has not been shown to affect mortality in patients with heart failure. Spironolactone, an aldosterone-blocking agent, reduces mortality in patients with chronic heart failure as a result of left ventricular systolic dysfunction.Treating patients with spironolactone in conjunction with an ACE inhibitor, loop diuretic, and digoxin, decreases atrial natriuretic peptide concentrations and does not lead to serious hyperkalemia.The Randomized Aldactone Evaluation Study found that treatment with spironolactone reduced the risk of death, decreased hospital stays, improved symptoms of heart failure, and was well tolerated.12 Other treatment considerations include the following: (1) anticoagulants (warfarin sodium) to reduce the risk of emboli in patients at high risk, which include those with prior embolic events, advanced heart failure, atrial fibrillation, and echocardiographic evidence of left ventricular thrombus13; (2) antiarrhythmics, such as amiodarone, used cautiously, to control arrhythmias, although empiric therapy is generally not advised. Patients who are characterized with a high risk of sudden death may require an implantable cardioverterdefibrillator that is used to treat ventricular arrhythmias
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and for prophylaxis (because of the high incidence of sudden death in patients with NYHA class III or IV heart failure.) A biventricular pacemaker for cardiac resynchronization therapy is advocated if symptoms continue despite optimal drug therapy and if the patient has NYHA class III or IV heart failure. Biventricular pacing has been used with success, especially in patients with a QRS duration greater than 150 ms, prolonged PR interval, or ejection fraction of 35% or less.8,14 Revascularization, such as coronary artery bypass graft surgery, may be needed if DCM is due to ischemia. Valvular repair or replacement may be necessary if DCM is due to valvular dysfunction. Referral for heart transplantation or use of a left ventricular assist device should be considered for the patient refractory to medical therapy and with advanced heart failure. Patient education on lifestyle modifications should include smoking cessation; low-fat, low-sodium diet; physical activity; abstinence from alcohol; and restriction of fluid intake. After the initial diagnosis is made, all patients should be seen for regular checkups to monitor progress and to adjust treatment as necessary. HYPERTROPHIC CARDIOMYOPATHY HCM is a complex and relatively common autosomal dominant genetic disorder that affects 1 in 500 persons.13 The disorder occurs twice as often in men than in women and is relatively more common in persons younger than 35 years. HCM is the most common cause of sudden death in those younger than 30 years and accounts for a high proportion of athlete-associated sudden deaths.15 Anatomic and Physiologic Classification HCM is a primary disease of the cardiac muscle that is characterized by a hypertrophied and nondilated left ventricle in the absence of other cardiac or systemic diseases such as systemic hypertension or valvular aortic stenosis that is capable of producing left ventricular hypertrophy (LVH). HCM is a disorder characterized by ventricular hypertrophy, diminished cavity dimensions of the left ventricle, normal or enhanced contractile function, and impaired ventricular relaxation. In HCM, the heart muscle increases in size and mass, especially along the intraventricular septum.The
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Figure 2. Diagrams of heart with hypertrophic cardiomyopathy and heart with restrictive cardiomyopathy. Adapted from: www.nscardiology.com/factscardiomyopathy.htm.

Heart with Hypertropic Cardiomyopathy

Heart with Restrictive Cardiomyopathy

Growth and arrangement of muscle fibers are abnormal. Heart walls thicken, especially in the left ventricle.

Ventricle walls stiffen and lose flexibility.

heart muscle is often not dilated, but instead it is rather stiff with a normal pumping capacity.The heart muscle thickness decreases the size of the ventricular cavities and causes them to take longer to relax.Thickened muscle walls also cause the atrial and ventricular cavities to hold a smaller volume of blood.The loss of ventricular elasticity results in abnormal filling pressures, thus increasing left ventricular end-diastolic pressure and eventually causing pulmonary congestion and edema.To assist ventricular filling the atria contract more forcefully. Atrial contraction is exceptionally important for these patients because the stiffness of the left ventricle impairs passive filling.The decrease in cavity size of the left ventricle results in an exaggerated systolic function in patients with HCM.When the ventricle contracts, the smaller volume is rapidly expelled against a comparatively decreased afterload. Therefore, the ventricle appears more hyperdynamic than hypercontractile.The left ventricular volume is normal or reduced in HCM, and diastolic dysfunction is usually present. Systolic dysfunction can be normal or high, which results in a higher than normal ejection fraction. Patients with HCM typically have mitral regurgitation because the septal hypertrophy interferes with normal valvular function.The hypertrophy causes the papillary muscle to move out of alignment, thus causing mitral regurgitation (Figure 2). Various terms have been used to describe the pathophysiology of HCM.These terms include hypertrophic
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obstructive cardiomyopathy, idiopathic hypertrophic subaortic stenosis, asymmetric septal hypertrophy, and muscular subaortic stenosis.5 Cause The idiopathic form of HCM has an early onset (as early as the first decade of life) without associated hypertension. HCM is inherited as a mendelian autosomal dominant trait and is caused by genetic mutations in any of 10 genes.16 Autosomal dominant means that the condition may be passed from one generation to the next and does not skip a generation.There is a 50% chance of transmission to offspring.An acquired form also occurs in elderly patients with chronic hypertension.16 A long history of hypertension can lead to the overgrowth of heart muscle. The mechanisms by which disease-causing mutations cause LVH and the HCM disease state are unresolved, although several hypotheses are proposed.13 Clinical Presentation The associated hemodynamic consequences of HCM depend on the severity of ventricular hypertrophy, the extent of left ventricular diastolic and systolic dysfunction, and the presence or absence of left ventricular outflow obstruction and mitral regurgitation.17 HCM is a unique cardiovascular disease with the potential for clinical presentation during any phase of life.The clinical course is typically variable, and patients may remain stable over long periods with up to 25% of
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patients with HCM achieving normal longevity (75 years of age or older).16 Most patients with HCM have few symptoms, if any, and the diagnosis is made incidentally or during family screening.18 Many patients are asymptomatic, whereas other patients have dyspnea, paroxysmal nocturnal dyspnea, angina, fatigue, or syncope. Dyspnea is usually due to elevated left ventricular filling pressure. Angina is caused by the inability of the coronary arteries to supply enough blood to meet the increased demand of the hypertrophied heart. Fatigue is associated with a decreased cardiac output. Syncope results from arrhythmias or decreased ventricular filling, leading to decreased cardiac output. A few patients with HCM may die of cardiac failure. Patients with the presence of a left ventricular outflow tract obstruction can manifest several findings on physical examination, including a left sternal border murmur from outflow obstruction across the aortic valve.This murmur is preload dependent and intensifies with maneuvers that decrease preload, such as standing and the Valsalva maneuver. It will decrease with squatting (which increases preload and afterload) and handgrip (which increases afterload). Because only one third of patients manifest obstruction, most patients have no murmur on examination. Other findings include pulsus bisferiens (twice beating second peak after initial rapid carotid upstroke), carotid pulse, and a large wave on jugular venous pulse associated with an S4.18 Atrial fibrillation is a long-term consequence and a poor prognostic sign.Ventricular arrhythmias are common, and sudden death may occur. Sudden death can occur in athletes after extraordinary exertion. Diagnosis The clinician should first begin by obtaining a detailed and accurate family history, especially noting any sudden unexplained deaths. Clinical screening of firstdegree relatives and other family members should be encouraged. Diagnosis is often made on the basis of heart murmur or abnormal results from an ECG or screening echocardiogram. To make a diagnosis of HCM, other conditions resulting in hypertrophic alterations such as hypertensive heart disease and valvular or supravalvular aortic stenosis need to be ruled out.The initial evaluation of the patient
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suspected of having HCM should include an ECG, chest x-ray, and an echocardiogram.The detection of an abnormal finding on an ECG is often the first clue to the presence of HCM.The most frequent changes include left atrial enlargement, repolarization abnormalities, and pathologic Q waves, most commonly in the inferolateral leads.18 Cardiomegaly may be noted on chest x-ray, along with left atrial enlargement and occasionally interstitial edema. Generally, an unexplained left ventricular wall thickness greater than 15 mm (normal, 12 mm)8 on two-dimensional echocardiogram in any myocardial segment is sufficient to make the diagnosis of HCM in an adult.18 Results of Doppler ultrasound scanning show turbulent blood flow across the aortic valve and commonly mitral regurgitation. Holter monitoring should be performed in patients with palpitations or isolated syncope. Exercise testing with simultaneous respiratory gas analysis objectively assesses disease severity and helps with the differential diagnosis of unexplained hypertrophy. Patients with HCM usually have some reduction in peak oxygen consumption compared with healthy persons even when asymptomatic. Up to 25% of patients have an abnormal blood pressure response during upright exercise; systolic blood pressure fails to rise by more than 20 to 25 mm Hg from baseline values or falls.18 Medical Management Although HCM is a chronic disease without a known cure, a number of treatments are available to alter its course.The management of patients with HCM is complex and can change during the course of the disease (Figure 3).18 The goal in HCM is to reduce the effects of the hypercontractile heart and to alleviate the symptoms related to heart failure. Management of HCM should include the use of medications such as -blockers and calcium channel blockers to control signs and symptoms. -Blockers are used to slow the heart rate, reduce myocardial oxygen demands, and increase ventricular (diastolic) filling time by relaxing the muscle, thereby increasing cardiac output. If -blockers are discontinued for any reason, patients must be weaned off the medications slowly. Non-hydropyridine calcium channel blockers such as verapamil or diltiazem may also be used to treat HCM.Verapamil improves diastolic filling time by improving relaxation of the heart muscle, decreasing septal stiffness, and also increasing exercise intolerance in many patients.19,20 The use of ACE inhibitors is not recommended unless there is significant hypertension, because
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Figure 3. Key aspects of disease management in patients with hypertrophic cardiomyopathy. AF indicates atrial fibrillation; ICD, implanted cardioverter-defibrillator; LVOTO, left ventricular outflow tract obstruction; VF, ventricular fibrillation; VT, ventricular tachycardia. Reprinted from The Lancet, 363 (9424). Elliott, P, Mckenna WJ. Hypertophic Cardiomyopathy, 18811891, 2004, with permission from Elsevier.
Hypertrophic cardiomyopathy Exclude secondary causes: Amyloidosis Metabolic disease Phaeochromocytoma

Counselling

Symptoms

Syncope

Complications

Genotyping

LVOTO

Non-obstructive

Family screening

blockers disopyramide (verapamil)

blockers calcium antagonists

Assess risk of sudden cardiac death: History of VF/VT Family history Syncope Exercise blood pressure Non-sustained VT Wall thickness

Progressive heart failure

AF

ACE inhibitors Diuretics blockers

Direct current cardioversion Amiodarone Rate control Anticoagulation

Patient information Insurance Exercise Employment Pregnancy

Myotomy-myectomy Septal alcohol ablation Dual chamber pacing

ICD (amiodarone)

Heart transplant

these drugs may worsen the gradient by peripheral vasodilatation.There is no role for digoxin, because this agent acts as a positive inotropic and can increase the outflow obstruction. Nitrates are also contraindicated, because they decrease preload and thereby increase the gradient across the outflow tract of the left ventricle. Refractory Symptomatic Management It is of clinical importance to distinguish between the obstructive or nonobstructive forms of HCM, according to the presence or absence of an outflow gradient of the left ventricle under resting or provocable conditions or both.When patients develop congestive symptoms that are refractory to maximum medication therapy and have outflow obstruction, there are three other options that exist. Septal myectomy (Morrow procedure) is still reported as the standard for relief of obstructive symptoms.21 In this procedure, the hypertrophied part of the septum is removed in an attempt to relieve the obstruction and decrease the outflow gradient.This procedure has been successful in patients with severe symptoms. Nonsurgical percutaneous transluminal septal ablation (alcohol septal ablation) is performed by injecting alcohol into septal branches of the left anterior
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descending coronary artery.This causes a therapeutic infarction within the septal myocardium to reduce the hypertrophy of the septum and associated obstruction. As a result, the area involved becomes thin and contractile dysfunction develops.This reduces the left ventricular outflow tract gradient by expanding the left ventricular outflow tract. Atrioventricular block requiring a permanent pacemaker occurs 30% of the time.20,22 After some initial enthusiasm for the role of the dualchamber pacemaker, consensus now is that its role in HCM is limited and its use is still being evaluated.18,22 According to Maron et al,16 the failure to achieve gradient reduction with temporary pacing suggests that permanent pacing is probably not indicated. Pacing for severely symptomatic and medically refractory patients with HCM with a left ventricular outflow obstruction is designated as a class IIB indication.16 Patients with a history of ventricular arrhythmias and unexplained syncope in the presence of a positive family history of sudden death are probably best managed with an implantable cardioverter-defibrillator. In patients with nonobstructive HCM who are symptomatic despite maximal medical therapy, heart transplantation is a viable option.13
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RESTRICTIVE CARDIOMYOPATHY Anatomic and Physiologic Classification RCM is the least common type of cardiomyopathy.The hallmark of RCM is diastolic dysfunction that results in impaired ventricular filling, normal or decreased diastolic volume of either or both ventricles, and increased left ventricular wall thickness.14,21 In other words, there is stiffness of the ventricle, caused by LVH, endocardial fibrosis, and thickening, thus reducing the ability of the ventricle to relax and fill during diastole. Systolic function usually is normal depending on the underlying cause. RCM is less common than either DCM or HCM outside the tropics, but it is a common cause of death in Africa, India, South and Central America, and Asia, primarily because of the high incidence of endomyocardial fibrosis in these regions23 (Figure 2). Causes RCM commonly results from myocardial or endomyocardial disease of diverse causes which stiffen the heart by infiltration or fibrosis. RCM may be classified as primary or secondary.The primary RCMs include endomyocardial fibrosis, Loeffler endocarditis, and idiopathic cardiomyopathy.The secondary forms of RCM are more common and include specific heart muscle diseases in which the heart is affected as part of a multisystem disorder.These can be subclassified as noninfiltrative (eg, carcinoid heart disease), infiltrative (eg, amyloidosis, sarcoidosis, or postirradiation therapy), or as storage disorders (hemochromatosis, glycogen storage disease, Fabry disease).6 Clinical Manifestations In RCM, the inability of the ventricle to fill limits cardiac output and raises filling pressure.Therefore, exercise intolerance and dyspnea are usually the most prominent symptoms. Patients have symptoms of dyspnea, orthopnea, paroxysmal nocturnal dyspnea, peripheral edema, ascites, fatigue, and weakness.Angina does not occur except in amyloidosis.As a result of persistently elevated venous pressure, these patients commonly have dependent peripheral edema, ascites, and an enlarged, tender, and pulsatile liver.The jugular venous pressure is elevated and does not fall normally, or it may rise with inspiration (Kussmaul sign). Initial evaluation should attempt to differentiate from constrictive pericarditis, which has similar symptoms.Atrial fibrillation is common, and heart block may be particularly evident in patients with amyloidosis
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or sarcoidosis. Up to one third of patients may have thromboembolic complications. Right-sided heart failure is often more prominent than left-sided heart failure early in the course of RCM.5 On physical examination, Kussmaul sign may be present, there may be a prominent apical impulse, and a loud S3 or murmurs of tricuspid or mitral regurgitation may be heard with auscultation. Diagnosis Most patients have right-sided heart failure out of proportion to left-sided heart failure and have normal or nearnormal cardiac size on examination and chest x-ray.The differential diagnosis of RCM includes constrictive pericarditis, chronic right ventricular infarction, right ventricular dysfunction from right ventricular pressure or (less likely) right ventricular volume overload, intrinsic myocardial disease of the right ventricle, or tricuspid valve disease. Results of the examination and echocardiogram usually narrow the differential diagnosis to RCM and constrictive pericarditis, which affect hemodynamics differently.5 The ECG often shows low voltage, nonspecific ST-T wave changes, and various arrhythmias. Echocardiographic findings include biatrial dilatation, hypertrophied ventricles with decreased compliance, initially small cavities of the left ventricle, and normal-to-depressed systolic function.24 Medical Management Treatment options for RCM are limited.The treatment of RCM is palliative and is similar to that of DCM and heart failure.The underlying cause should be treated if possible. Medical intervention includes the use of diuretics,ACE inhibitors, and antiarrhythmic and anticoagulant medications. Diuretics can help, but excessive diuresis can produce worsening symptoms.A pacemaker may be used to treat atrioventricular conduction block. Cardiac transplantation may be considered in patients with refractory symptoms in idiopathic or familial RCM.14 Caution should be used with all medications to avoid decreasing ventricular filling pressures and cardiac output. Implications for Practice Treatment options for the patient with cardiomyopathy are mainly focused on treating heart failure, managing symptoms and progression, and preventing thromboembolism and sudden cardiac death.The types and classifications of cardiomyopathies are listed in Table 2. The cause, pathophysiology, structure and function,
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Table 2. Types and Classifications of the Cardiomyopathies


Dilated Common causes
Idiopathic, ischemia, alcohol, HTN, myocarditis, postpartum, doxorubicin, endocrinopathies, genetic diseases Myocardial fibers degenerate Increase in fibrous tissue

Hypertrophic
Idiopathic, hereditary, possibly chronic hypertension

Restrictive
Idiopathic, amyloidosis, sarcoidosis, endomyocardial fibrosis, radiation fibrosis, diabetes

Pathophysiology

Disproportionate thickening of the interventricular septum

Myocardium becomes rigid and noncompliant Ventricular filling is impeded

Myocardial structure and function

Chamber size Impaired contractility, EF

Nondilated ventricles LVH Thickened and rigid interventricular septum (HOCM) Myocardial muscle mass Contractility

Ventricular chamber size Atrial chamber size LVH Contractility

Symptoms

Similar to CHF Dyspnea with exertion Orthopnea Fatigue Palpitations Auscultation: S3, systolic murmur of MR

Dyspnea with exertion Angina Syncope Palpitations Left-sided heart failure Sudden death Auscultation: normal, S4, double apical impulse (vigorous atrial contraction), cresc-decres systolic murmur (obstructive)

Fatigue Weakness Dyspnea with exertion Anorexia Exercise intolerance Left- and right-sided heart failure Cardiac output JVD Kussmaul sign

Recommended diagnostic tests and results

CXR: cardiomegaly, pulmonary congestion ECG: ST-T abnormalities, ventricular ectopy Echo: LV dilatation, dysfunction; systolic dysfunction; EF Cath: LV dilatation, dysfunction; left- and often right-sided filling pressures; cardiac output

CXR: mild cardiomegaly ECG: LVH (thick septum), ST-T abnormalities, abnormal Q waves Echo: asymmetric septal hypertrophy, LVH, diastolic dysfunction, EF normal or Cath: vigorous systolic function, dynamic LVO obstruction, right- and left-sided filling pressures Maneuvers -Blockers Afterload reducers: calcium channel blockers Diuretics

CXR: mild-to-moderate cardiomegaly ECG: low-voltage conduction defects Echo: LV wall thickness, normal or mildly systolic function Cath: normal or mildly systolic function, right- and left-sided filling pressures

Treatments

Inotropic agents such as dobutamine or digoxin Diuretics Vasodilators -Blockers Sodium-restricted diet, fluid restriction Anticoagulants, transplantation

Treat underlying cause, infiltrative disease of the myocardium Diuretics Afterload reducers

HTN indicates hypertension; , increased; , decreased; EF, ejection fraction; LVH, left ventricular hypertrophy; HOCM, hypertrophic obstructive cardiomyopathy; CHF, congestive heart failure; MR, mitral regurgitation; JVD, jugular venous distention; CXR, chest x-ray; ECG, electrocardiogram; Echo, echocardiogram; Cath, cardiac catheterization; LVD, left ventricular dysfunction; LVO, left ventricular outlet; LV, left ventricular.

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symptoms, diagnostic tests, and treatment of each type of cardiomyopathy are given. It is important for the advanced practice nurse to understand the importance of heart failure prevention. It is also important to remember that you may encounter patients living with cardiomyopathy at various stages of disease progression. Understanding the different types of cardiomyopathy and management strategies will enable the clinician to offer valuable patient support, education, and advice. Clinicians need to be diligent about promoting habits for a healthy lifestyle such as heart-healthier diets, weight control, and smoking cessation. Resources such as The Cardiomyopathy Association25 and the Heart Failure Society of America26 provide patients with access to current literature on cardiomyopathy and a network of regional support.
References 1. American Heart Association. Heart Disease and Stroke Statistics2005 Update. Dallas, TX: American Heart Association; 2004. 2. Richardson P, McKenna W, Bristow M, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation. 1996;93(5):841-842. 3. Cooper LT. Definition and classification of the cardiomyopathies. Up to Date. 2004 May. Available at: www.uptodate.com. Accessed May 4, 2005. 4. Henry LB. Left ventricular systolic dysfunction and ischemic cardiomyopathy. Crit Care Nurs Q. 2003;26(1):16-21. 5. Runge MS, Ohman M. Netters Cardiology. 1st ed. Teterboro, NJ: Icon Learning Systems LLC; 2004. 6. Hughes SE, McKenna WJ. New insights into the pathology of inherited cardiomyopathy. Heart. 2005;91(2):257-264. 7. Dec GW, Fuster V. Idiopathic dilated cardiomyopathy. N Engl J Med. 1994;331(23):1564-1575. 8. Elliott P. Dilated cardiomyopathy. Heart. 2000;84(1):106-112. 9. Weigner M, Morgan JP. Causes of dilated cardiomyopathy-I. Up to Date. 2005 January. Available at: www.uptodate.com. Accessed May 4, 2005. 10. Humes HD. Kelleys Essentials of Internal Medicine. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2001. 11. Hunt, SA, Barker DW, Chin MH. ACC/AHA Guidelines for the Evaluation and Management of Chronic Heart Failure in the Adult: Executive Summary A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee to Revise the 1995 Guidelines for the Evaluation and Management of Heart Failure): Developed in Collaboration With the International Society for Heart and Lung Transplantation; Endorsed by the Hearth Failure Society of America. Circulation. 2001;104(24):2996-3007. 12. Tarolli K. Left ventricular systolic dysfunction and nonischemic cardiomyopathy. Crit Care Nurs Q. 2003;26(1):3-15. 13. Maron BJ. Hypertrophic cardiomyopathy: a systematic review. JAMA. 2002;287(10):1308-1320. 14. Cruickshank S. Cardiomyopathy. Nurs Stand. 2004;18(23):46-52, 54-56. 15. Maron BJ, Gardin JM, Flack JM., Gidding SS, Kurosaki TT, Bild DE. Prevalence of hypertrophic cardiomyopathy in a general population of young adults: echocardiographic analysis of 4111 subjects in the CARDIA Study. Circulation. 1995;92(4):785-789. 16. Maron BJ, McKenna WJ, Danielson GK, et al. American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. J Am Coll Cardiol. 2003;42(9):1687-1713. 17. Evangelista LS, Chen J. Hemodynamic benefits of treatment modalities for hypertrophic cardiomyopathy: a case study. J Cardiovasc Nurs. 2001;15(2):33-40. 18. Elliott P, McKenna WJ. Hypertrophic cardiomyopathy. Lancet. 2004;363(9424):1881-1891.

19. Braunwald E, Seidman CE, Sigwart U. Contemporary evaluation and management of hypertrophic cardiomyopathy. Circulation. 2002;106(11):1312-1316. 20. Steinbis S. Hypertrophic obstructive cardiomyopathy and septal ablation. Crit Care Nurse. 2003;23(3):47-50. 21. Crawford PA, Lin TL, editors. The Washington Manual Cardiology Subspecialty Consult. Philadelphia. PA: Lippincott Williams & Wilkins; 2004. 22. Betocchi S, Elliott PM, Briguori C, et al. Dual chamber pacing in hypertrophic cardiomyopathy: long-term effects on diastolic dysfunction. Pacing Clin Electrophysiol. 2002;25(10):1433-1440. 23. Kushwaha SS, Fallon JT, Fuster V. Restrictive cardiomyopathy. N Engl J Med. 1997;336(4):267-276. 24. Wood MJ, Picard MH. Utility of echocardiography in the evaluation of individuals with cardiomyopathy. Heart. 2004;90(6):707-712. 25. The Cardiomyopathy Association. Available at: www.cardiomyopathy.org. Accessed July 17, 2005. 26. The Heart Failure Society of America. Available at: www.hfsa.org/. Accessed July 17, 2005.

Lori Daughenbaugh, MSN,ACNP-BC, CCRN, is employed at Lexington Hospitalists Inc,Altoona Regional Health System, in Altoona, Pennsylvania. She can be reached at ldemko@atlanticbb.net. She has no relationship with business or industry to disclose. Acknowledgment I thank Robyn Daniels, RN, MSN,ACNP-BC, for reviewing this article.
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