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Cardiomyopathy Restrictive

A disorder of the myocardial musculature, restrictive cardiomyopathy is characterized by restricted ventricular filling (the result of left ventricular hypertrophy) and endocardial fibrosis and thickening. If severe, it's irreversible.

Restrictive cardiomyopathy is the least common form of cardiomyopathy. It has several similarities  with hypertrophic cardiomyopathy. Restrictive cardiomyopathy is rare in the United States, Canada, and Europe. Other forms of include dilated cardiomyopathy, in which the heart muscle stretches out, and hypertrophic cardiomyopathy, in which the heart muscle is thicker than usual.

Causes

Primary restrictive cardiomyopathy is an extremely rare disorder of unknown cause. However, restrictive cardiomyopathy syndrome, a manifestation of amyloidosis, results from infiltration of amyloid into the intracellular spaces in the myocardium, endocardium, and subendocardium.

In both forms of restrictive cardiomyopathy, the myocardium becomes rigid, with poor distention during diastole, inhibiting complete ventricular filling. It fails to contract completely during systole, resulting in low cardiac output.

Signs and symptoms

Shortness of breath during exercise may be the first symptom you notice. As the condition progresses and heart failure develops, you may have difficulty breathing with even slight exertion. You may feel weak and tired. Many people have swollen legs from fluid retention (edema). A buildup of fluid around your liver, stomach, and intestines can cause bloating, nausea, and poor appetite.

Many people with primary restrictive cardiomyopathy first learn they have the disease when they develop complications, including stroke , heart attack , blood clot in the lung ( pulmonary embolism ), and other conditions related to blood clots

Diagnosis

• Chest X-ray shows massive cardiomegaly in advanced stages of this disease, affecting all four chambers of the heart; pericardial effusion; and pulmonary congestion.
• Echocardiography rules out constrictive pericarditis as the cause of restricted filling by detecting increased left ventricular muscle mass and differences in end diastolic pressures between the ventricles.
• Electrocardiography may show low voltage complexes, hypertrophy, atrioventricular conduction defects, or arrhythmias.
• Arterial pulsation reveals blunt carotid upstroke with small volume.
• Cardiac catheterization demonstrates increased left ventricular end diastolic pressure and rules out constrictive pericarditis as the cause of restricted filling

Treatment

Although no therapy currently exists for restricted ventricular filling, digitalis glycosides, diuretics, and a restricted sodium diet ease the symptoms of heart failure.

Oral vasodilators-such as isosorbide dinitrate, prazosin, and hydralazine-may control intractable heart failure. Anticoagulant therapy may be necessary to prevent thrombophlebitis in the patient on prolonged bed rest.

Prevention

Obtaining early treatment for diseases that might cause restrictive cardiomyopathy might prevent or slow the development of heart wall stiffness. Anyone experiencing symptoms of shortness of breath, tiredness, and weakness should see a physician.