RAPID PROGRESSION FROM HYPERTROPHIC CARDI OMYOPATHY INTO A DILATIVE FORM - AN UNUSUAL CLINICAL COURSE IN A YOUNG MALE - A CASE-REPORT

Hypertrophic cardiomyopathy is a disorder which primarily affects the myocardium. It is characterized by a hypertrophic left ventricle that shows normal systolic function but impaired diastolic relaxation. The most important complication of HCM, sudden cardiac death, is mainly re sponsible for a mortality of approximately 3.5% in childhood. While mo st patients remain asymptomatic for years, there are some reports abou t patients progressing from hypertrophic cardiomyopathy to a dilative form within years. We report on a boy who was at the age of twelve whe n hypertrophic cardiomyopathy was diagnosed first. He remained stable for the first years, but at the age of sixteen his cardiomyopathy prog ressed to a dilative form with reduced systolic function and severe ca rdiac failure. The possible pathogenesis is discussed and an overview of similar cases reported earlier is given.