The cardiomyopathies

Abstract

Cardiomyopathies are heart muscle diseases that are classified by pathophysiology: (i) dilated, (ii) hypertrophic, (iii) restrictive. Specific cardiomyopathies are those with a distinct disease association. Most dilated cardiomyopathy in childhood is idiopathic, but specific causes must be excluded as treatment can be directed towards the cause. Dilated cardiomyopathy has a poor prognosis with approximately 60% of children surviving 5 years from presentation. Medical and surgical therapy is improving but ultimately transplantation may be required. Idiopathic hypertrophic cardiomyopathy is a disease of the sarcomere relating to familial defects in genes encoding contractile proteins. Sudden death is common but can be prevented by implanting cardioverter defibrillators in high-risk cases. Restrictive cardiomyopathy is uncommon, it appears more rapidly progressive when presenting in younger patients although the prognosis and molecular genetic causes are not well defined.