Introduction Hypertrophic cardiomyopathy (HCM) is a common disorder of cardiac muscle associated with sudden cardiac death (SCD). HCM is defined by increased left ventricular wall thickness or mass, in the absence of abnormal loading conditions to account for the observed abnormality. In most adults, the disease is inherited as an autosomal dominant trait and is caused by mutations in cardiac s...

PURPOSE OF REVIEW Hypertrophic cardiomyopathy is the most common identifiable cause of sudden death in the young. This review details the history of hypertrophic cardiomyopathy, recent discoveries in its genetic underpinnings and important genotype-phenotype relationships described in recent studies. RECENT FINDINGS Since the discovery of the genetic underpinnings of hypertrophic cardiomyopat...

Hypertrophic cardiomyopathy is an autosomal dominant inherited disease characterized by ventricular hypertrophy and myofibril disarray. Mutations responsible for hypertrophic cardiomyopathy have been identified in 11 genes that encode for cardiac sarcomere proteins. Traditionally, hypertrophic cardiomyopathy due to mutation of the myosin-binding protein C gene (MYBPC3) has been thought to follo...

Endochondral bone formation is characterized by the progressive replacement of a cartilage anlagen by bone at the growth plate with a tight balance between the rates of chondrocyte proliferation, differentiation, and cell death. Deficiency of matrix metalloproteinase-9 (MMP-9) leads to an accumulation of late hypertrophic chondrocytes. We found that galectin-3, an in vitro substrate of MMP-9, a...

Hypertrophic cardiomyopathy is a fascinating disease of marked heterogeneity. Hypertrophic cardiomyopathy was originally characterized by massive myocardial hypertrophy in the absence of known etiology, a dynamic left ventricular outflow obstruction, and increased risk of sudden death. It is now well accepted that multiple mutations in genes encoding for the cardiac sarcomere are responsible fo...

By definition, myocardiopathies or cardiomyopathies (CM) are heart muscle diseases that cause structural and functional myocardial abnormalities in the absence of coronary artery disease, hypertension, valvular congenital disease., CM constitute a very heterogeneous group one leading causes death either from end-stage failure sudden due to arrythmias. According phenotype, may be classified into...

N Engl M Med 2004;350:1320-7. Copyright © 2004 Massachusetts Medical Society. A 28-year-old man presents with a two-year history of increasing dyspnea on strenuous exertion and is found to have hypertrophic cardiomyopathy, with a septal thickness of 23 mm and a left ventricular outflow gradient of 80 mm Hg. There is no family history of hypertrophic cardiomyopathy or sudden death. Forty-eight-h...

Hypertrophic cardiomyopathy is a genetic cardiac disease with a particularly heterogeneous presentation and diverse natural history. Sudden and unexpected death has been recognized as a prominent and devastating consequence of hypertrophic cardiomyopathy since the initial description of this disease over 40 years ago. Many authors have emphasized that these catastrophic events occur not uncommo...