Hypertrophic cardiomyopathy occurs as an autosomal dominant familial disorder or as a sporadic disease without familial involvement. While missense mutations in the beta cardiac myosin heavy chain (MHC) gene account for approximately half of all cases of familial hypertrophic cardiomyopathy, the molecular causes of sporadic hypertrophic cardiomyopathy are unknown. To determine whether beta card...

Apical hypertrophic cardiomyopathy is an uncommon variant of non-obstructive hypertrophic cardiomyopathy with low prevalence outside East Asia. A case is reported of a non-Asian (European) 51 year old man with characteristic ECG and morphological changes of apical hypertrophic cardiomyopathy. Although the patient underwent catheterisation three years previously because of suggested coronary isc...

Transmural myocardial infarction in the absence of epicardial coronary arterial narrowing is fairly common at necropsy in patients with hypertrophic cardiomyopathy. Although difficult to diagnose clinically in patients with hypertrophic cardiomyopathy, acute myocardial infarction occasionally has a typical presentation, as in the patient presented in this report. More often, however, transmural...

Affecting approximately one in every 500 people, hypertrophic cardiomyopathy is a unique disease in which there is an abnormal increase in the thickness of the heart muscle. Although the cause of hypertrophic cardiomyopathy was unknown in the past, it is now recognized that people with hypertrophic cardiomyopathy have a gene mutation that causes this abnormality. Most people with hypertrophic c...

BACKGROUND Hypertrophic cardiomyopathy, a familial myocardial condition caused by sarcomere protein mutations, is usually recognized by early adulthood. Hypertrophic cardiomyopathy of the elderly has similar clinical features but, notably, a later age of onset and noncontributory family history. Causes of elderly-onset hypertrophic cardiomyopathy are unknown. METHODS AND RESULTS Eighteen wome...

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...

Apical hypertrophic cardiomyopathy is an uncommon variant of non-obstructive hypertrophic cardiomyopathy with low prevalence outside East Asia. A case is reported of a non-Asian (European) 51 year old man with characteristic ECG and morphological changes of apical hypertrophic cardiomyopathy. Although the patient underwent catheterisation three years previously because of suggested coronary isc...

The topography and specificity of fibre disarray and fibrosis in hypertrophic obstructive cardiomyopathy were determined in a histological study comprising 40 necropsy hearts--10 with hypertrophic cardiomyopathy, 10 with congestive cardiomyopathy, 10 with aortic valve stenosis, and 10 normal hearts. Seven standard regional sections were sampled from each heart and graded "double-blind" (tissue ...

1. Sigwart U. Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy. Lancet 1995;346:211–214. 2. Lakkis N, Nagueh SF, Kleiman NS, Killip D, He ZX, Verani MS, Roberts R, Spencer WH III. Echocardiography-guided ethanol septal ablation for hypertrophic obstructive cardiomyopathy. Circulation 1998;98: 1750–1755. 3. Knight C. Alcohol septal ablation for obstructive hypertroph...

BACKGROUND There are limited data on the prevalence, pathophysiology, and management implications of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy and advanced heart failure. METHODS AND RESULTS To assess the clinical significance of measured cardiopulmonary hemodynamics in hypertrophic cardiomyopathy patients with heart failure, we retrospectively assessed r...