Cardiomyopathy in children

Abstract

| Cardiomyopathy in children is a rare but serious disease leading to heart failure with a high mortality rate. Dilated cardiomyopathy (DCM) and hypertrophic cardio-myopathy (HCM) are more common than restrictive cardiomyopathy (RCM). Arrhythmogenic right ventricular dysplasia (ARVD) is extremely rare in pediatric age groups. Treatment should be targeted at the cause of the cardiomyopathy, if identified. Nutritional deficiencies, metabolic disorders, and inborn errors of metabolism should be corrected if possible. Immunosuppressive therapy may be considered in patients with lymphocytic myocarditis. Maximal medical therapy with inotropic drugs or cardiac glycosides, diuretic drugs, and angiotensin-converting enzyme inhibitors improve heart failure symptoms in patients with DCM. Beta-blocker therapy in adults with DCM is well established and is being evaluated in children with DCM. High-dosage beta-blocker or calcium channel blocker therapy improves the survival rate in children with HCM. The cautious use of diuretics and angiotensin-converting enzyme inhibitors in patients with RCM improves symptoms. Anticoagulation to prevent thromboembolism in patients with DCM and RCM is indicated. Antiarrhythmia therapy is needed in patients with DCM- or HCM-associated dysrhythmia. Cardiac transplant is indicated in patients with progressive deterioration.