TABLE 514 Common Causes of Restrictive Cardiomyopathy

CLINICAL FEATURES AND DIAGNOSIS Symptoms are typical of CHF and include dyspnea, orthopnea, and pedal edema. Right-sided manifestations may predominate and result in hepatomegaly, right upper quadrant pain, and ascites. Chest pain is uncommon, except in amyloidosis.

Findings on physical examination depend on the stage or severity of myocardial involvement. An S 3 is almost always present, and an S4 is often heard if the patient is in sinus rhythm. Pulmonary rales, jugular venous distention, Kussmaul sign (jugular venous pulse rises during inspiration rather than falling), hepatomegaly, pedal edema, and ascites are also typical findings.

The chest x-ray may reveal signs of CHF in the absence of cardiomegaly. Chamber enlargement due to wall thickening, but not dilatation, and nonspecific ST-T-wave changes are usually noted on the ECG. Cardiac conduction disturbances are common in amyloidosis and sarcoidosis. Atrial fibrillation may occur in the setting of atrial enlargement. Low-voltage QRS complexes (QRS amplitude less than 0.7 mV) have been frequently described in patients with restrictive cardiomyopathy secondary to amyloidosis and hemochromatosis.12

TREATMENT AND DISPOSITION Symptoms and signs of CHF, particularly right-sided failure, with a normal-size cardiac silhouette on chest x-ray should prompt a suspicion of underlying restrictive cardiomyopathy, constrictive pericarditis, or diastolic LV dysfunction (most commonly due to ischemic heart disease, hypertension, or age-related changes in ventricular diastolic compliance). Doppler echocardiographic studies and cardiac catheterization with hemodynamic assessment are often required to differentiate between the above-mentioned entities. Computed tomography and magnetic resonance imaging of the heart have also been shown to be of value in differentiating constrictive pericarditis and restrictive cardiomyopathy. 11 Timely diagnosis is important because constrictive pericarditis can be surgically corrected and diastolic LV dysfunction not due to restrictive cardiomyopathy usually responds well to drug therapy (b blockers or calcium-channel blockers). The medical management of restrictive cardiomyopathy is less effective and symptom directed (diuretics and angiotensin-converting enzyme inhibitors) unless due to sarcoidosis (corticosteroid therapy) or hemachromatosis (chelation therapy). The need for admission is usually determined by the severity of symptoms and the availability of a timely and usually invasive workup.

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