Paediatrics: Cardiomyopathy

Cardiomyopathy

Cardiomyopathy may be primary, or secondary to systemic or metabolic disease. Primary cardiomyopathy may be classified as:

·hypertrophic;

·dilated (congestive);

·restrictive.

Dilated (congestive) cardiomyopathy

This condition is rare and characterized by massive dilatation of the ven-tricles and cardiomegaly. The cause is unknown in most cases, although it is seen in association with other conditions:

·post-viral infection phenomenon;

·metabolic (e.g. mitochondrial disease).

Clinical features

·Insidious onset of progressive congestive cardiac failure is common.

·The course is usually progressive and the prognosis poor.

Management

Mainly directed at treating heart failure and, where possible, any underly-ing cause. Heart transplantation if severe heart failure.

Restrictive cardiomyopathy

This condition is rare and characterized by poor ventricular compliance and inadequate ventricular filling. The clinical features are similar to con-strictive pericarditis. It is sometimes seen in the Löffler hypere-osinophilic syndrome (multisystem disorder of skin, lungs, nervous system, and liver) and results in endocardial fibrosis of the AV valves and the ventricles. Prognosis is poor and cardiac transplantation often required.