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...

Berardinelli-Seip congenital lipodystrophy (BSCL) is a rare autosomal recessive condition associating insulin resistance, absence of subcutaneous fat and muscular hypertrophy. Disease-causing mutations have been described in AGPAT2 and BSCL2 genes. Hypertrophic cardiomyopathy is a classical late (third decade) complication which has only been occasionally described in childhood. We report on a ...

A patient with hypertrophic cardiomyopathy was found to have a chronic intravascular haemolytic anaemia. After investigation this was attributed to the abnormal haemodynamics of the cardiac disease. Treatment with propranolol was associated with an improvement in the anaemia. No further cases of haemolytic anaemia were found in 37 other patients with hypertrophic cardiomyopathy. It is concluded...

T he incidence of sudden death in hypertrophic cardiomyopathy is 2-4% a year in adults and 4-6% a year in children and adolescents.1 These data have been generated from referral cardiac centers and may reflect a bias to the more severe patients.2 The identification and management of patients with hypertrophic cardiomyopathy who are at increased risk of sudden death remains a major problem, part...

Abstract Purpose of review There are numerous health benefits to exercise, including improved cardiovascular, metabolic, and mental health. Despite these many benefits, children with cardiomyopathy have been traditionally restricted from engaging in exercise or participating organized sports. The purpose this is summarize the current literature regarding utility cardiopulmonary testing, discuss...

Hypertrophic cardiomyopathy is a condition where the muscle cells of the heart become big. This can make the walls of the heart thicker than normal, and because the walls surrounding the heart’s pumping chambers get thicker, the chambers become smaller than they should be. Hypertrophic cardiomyopathy is usually inherited, and is caused by changes to one or more of the person’s genes. The muscle...

Midventricular hypertrophic cardiomyopathy is a rare form of cardiomyopathy that may be associated with an apical aneurysm. The mechanism of aneurysm formation is uncertain, but it may be related to subendocardial ischemia. In this report, we describe a 57-year-old man with recurrent ventricular arrhythmias that were refractory to medical treatment because of midventricular hypertrophic cardiom...

A relatively infrequent form of hypertrophic cardiomyopathy is the isolated apical variant and it is more common in oriental people, especially in the Japanese. It contributes 25% of cases of hypertrophic cardiomyopathy in Japan but only 1% to 2% in the non-Japanese population. It may occasionally present in the elderly and classically involve the apex of the left ventricle. An isolated apical ...

The relation to the pressure gradient as assessed by echocardiography and the CMR-derived planimetry of the LVOT is not known, no values for the differentiation of obstruction exist. We studied 37 patients with hypertrophic cardiomyopathy and 14 healthy controls using standard sequences with 3D coverage of the left ventricular outflow tract. A cutoff value of 2.7 cm2 identified obstruction as d...

In order to examine the association between arrhythmia and subsequent prognosis, 72-hour ambulatory electrocardiographic monitoring was performed in 86 unselected patients with hypertrophic cardiomyopathy. During monitoring 23 patients experienced at least one episode of supraventricular tachycardia and 24 had ventricular tachycardia (of whom 10 had more than three episodes). The patients were ...