Definition:

The least-encountered form of cardiomyopathy, restrictive cardiomyopathy is a disorder in which the myocardium (muscular layer of the heart) changes to where it becomes stiffer, which affects the bloodflow and contractility of the heart.  It mimics constrictive pericarditis in presentation in some cases.  It is caused by: amyloidosis, hemochromatosis, idiopathic eosinophilia, carcinoid syndrome, sarcoidosis, and endomyocardial fibroelastosis.  

Symptoms:

• Gradual shortness of breath and fatigue
• Weakness
• Edema of legs (swelling caused by fluid)
• Ascites (fluid collection in the abdomen)
• Chest pain
• Palpitations
• Syncope
• More comfortable sitting than lying down
• Neck vein distension

Diagnosis:

• Physical exam
  • Stethoscope hearing loud diastolic murmur with mitral/tricuspid insufficiency
  • Decreased breath shoudsn
• EKG - low QRS voltage
• Chest X-Ray - to look for congestion in the vessels
• Echocardiography - to look at heart chambers; ventricle walls thickened, atria size increased
• Cardiac Catheterization - to assess coronary vessel anatomy and to distinguish restrictive cardiomyopathy from constrictive pericarditis; can also do endomyocardial biopsy to establish diagnosis

Treatment:

• Treat etiology first
• Symptomatic therapy - diuretics and vasodilators to reduce volume and congestion