Pediatric Restrictive Cardiomyopathy Treatment & Management

Updated: Sep 18, 2019
  • Author: Kimberly Y Lin, MD; Chief Editor: Syamasundar Rao Patnana, MD  more...
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Approach Considerations

Therapy for idiopathic restrictive cardiomyopathy (RCM) is limited to symptomatic treatment and is often ineffective in improving outcome. Secondary arrhythmias, when they occur, may require pharmacologic and/or device-related therapies that generally require evaluation by an electrophysiologist. Surgical options in children with RCM are limited to heart transplantation. The results of heart transplantation are generally quite good but depend on the degree of pulmonary hypertension and resultant postoperative complications. [39]

A normal diet is recommended. The degree of hemodynamic abnormality and the risk of sudden death are significant, and patients should be restricted from competitive athletics, despite a paucity of data on the subject. [40]


Pharmacologic Therapy

Diuretics reduce pulmonary or systemic venous congestion; however, some patients may require high ventricular filling pressures to maintain cardiac output and may actually feel worse after diuresis.

Digoxin has not been shown to be beneficial with normal systolic function and should be used with caution.

Anticoagulation should be considered because of the significant risk of thromboembolic complications. [28, 41, 3] If chosen, anticoagulation agents should be carefully administered with close supervision of coagulation parameters. Some pediatric cardiologists prefer platelet-inhibiting doses of aspirin, because no extensive monitoring of coagulation parameters is necessary.

Previous studies in pediatric patients have suggested that angiotensin-converting enzyme inhibitors (ACEIs) may acutely reduce systemic blood pressure without increasing cardiac output; therefore, they should probably be avoided. [42] Oral vasopressin–receptor antagonists may be helpful in selected cases. [43]

Experimental studies in mice suggest that catechin (epigallocatechin-3-gallate) may reverse diastolic dysfunction associated with restrictive cardiomyopathy, [44] which may hold promise for future. Clinical studies to test this hypothesis are warranted.


Cardiac Transplantation

Children with RCM should be considered early for heart transplantation, particularly if they are symptomatic or have evidence of pulmonary hypertension. Children with RCM are at higher risk for the development of pulmonary hypertension than children with dilated or hypertrophic cardiomyopathy and therefore need to be closely monitored for progression of pulmonary hypertension. [45]

Among the greatest challenges in the management of pediatric RCM are risk stratification and the decision regarding when to list a patient for heart transplantation. Some advocate immediate transplant evaluation and listing upon diagnosis of RCM. [46] However, patients with normal or only mildly elevated pulmonary vascular resistance may remain stable for years and may not require urgent listing for heart transplantation.

In one series, longer survival from diagnosis was correlated with lower left atrial–to–aortic root ratios by echocardiography and lower cardiac filling pressures by cardiac catheterization. [22] A recent study from Mayo Clinic indicated that early referral for cardiac transplantation may improve overall survival of RCM patients [47] .

Standard techniques for heart transplantation are used in children with RCM. Results are generally good. In those with pretransplant evidence of pulmonary hypertension, special attention must be paid to monitoring and treating this complication. [48, 49, 50] Sildenafil may be beneficial in helping to manage postoperative pulmonary hypertension, even in those patients who require heterotopic heart transplantation. [51]

Some patients may require such treatments as high levels of inspired oxygen or hyperventilation. Pulmonary vasodilators, such as prostacyclin, sodium nitroprusside, and nitric oxide, have all been used with some success in this situation. In extreme cases, mechanical circulatory assistance (eg, extracorporeal membrane oxygenation [ECMO]) may be necessary. Static balloon dilatation [52] following transseptal puncture may become necessary to decompress the left atrium and reduce pulmonary hypertension. [53]

Ventricular assist devices (VADs) may be used as a bridge to transplantation. [54, 55] However, small left ventricular size may cause problems; in such situations, left atrial cannulation may be a safer in children. [56]