History

Reasons for referral in restrictive cardiomyopathy (RCM) include the following:

Abnormal chest radiography findings during a respiratory illness
Abnormal physical findings (see Physical Examination)
Syncope
Positive family history
Peripheral edema
Ascites
General fatigue and weakness
Typical history of congestive heart failure
Respiratory symptoms, including dyspnea with exertion or at rest, paroxysmal nocturnal dyspnea, and orthopnea

Physical Examination

Physical examination findings usually reflect the degree of congestion from the diastolic dysfunction of the affected ventricle and the resultant degree of decreased cardiac output. In patients who are only mildly affected, findings may be normal. However, patients with significant left ventricular RCM have pulmonary venous congestion with tachypnea. Older patients in this category occasionally present with rales.

Murmurs and gallop rhythms are common. The S2 may become more prominent with the development of secondary pulmonary hypertension. Evidence of right-side congestion (manifested as hepatomegaly, jugular venous distention, or both) is usually present, either because of right-side RCM or as a secondary congestion from left-side RCM. Sometimes, the jugular venous pulse fails to fall during inspiration and may actually rise (Kussmaul sign).

In more severe forms, patients can present with peripheral edema or ascites and frank congestive heart failure.

Arrhythmias may occur in RCM, including atrial fibrillation, flutter, and ventricular tachycardia. In some infiltrative forms of RCM, complete heart block may also be present.^[29]

Complications

Complications include worsening congestive heart failure, arrhythmia (including sudden death), pulmonary hypertension, and thromboembolism.

If RCM results in advanced congestive heart failure, end-organ damage may result from low cardiac output.

Arrhythmias, both atrial and ventricular, may occur.

Pulmonary hypertension is more prevalent in this type of cardiomyopathy than in dilated or hypertrophic cardiomyopathy. Patients must be closely monitored because the main therapeutic intervention for RCM (cardiac transplantation) is generally contraindicated once irreversible pulmonary hypertension develops.

The incidence of thromboembolic complications is high enough that many investigators have recommended anticoagulation with warfarin or antiplatelet agents (eg, aspirin) as a preventive measure.^[20]

Differential Diagnoses

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Media Gallery

Echocardiographic 4-chamber view of a child with restrictive cardiomyopathy demonstrating characteristic marked enlargement of right atrium (RA) and left atrium (LA), which are larger than left ventricle (LV).

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