Many patients with hypertrophic cardiomyopathy die suddenly and unexpectedly, a significant number perhaps due to arrhythmia. Of 100 patients initially evaluated for signs or symptoms suggestive of heart disease or a family history of hypertrophic cardiomyopathy, 51 were selected solely because they met the echocardiographic criteria for the disease, and 49 patients were selected primarily beca...

Andrew Maslow MD Department of Anesthesiology Rhode Island Hospital, Providence Rhode Island Introduction Cardiomyopathy is generally defined as a “disease of the myocardium associated with cardiac dysfunction”. 1 Primary cardiomyopathies are divided into three major classifications: a) dilated cardiomyopathy (DCM), b) hypertrophic cardiomyopathy (HCM), and c) restrictive (or infiltrative) card...

Although idiopathic cardiomyopathies are prognostically important and are a common indication for cardiac transplantation in all age groups, the incidence and age distribution of idiopathic cardiomyopathies in a well-defined pediatric population have been poorly characterized. A retrospective study was carried out in Finland in 1980-1991 to obtain information on the epidemiology of childhood ca...

In the recent past, the mitral valve was referred to as the cornerstone of any echocardiographic examination. Changes in its echocardiographic pattern were considered, in many cases, to be specific for the diseases in which they were described. Among such diseases was hypertrophic cardiomyopathy. Three abnormalities were successively described in the M-mode echocardiograms of patients with the ...

Originally described by Brock and Teare, today hypertrophic cardiomyopathy is clinically defined as left (or right) ventricular hypertrophy without a known cardiac or systemic cause, such as systemic hypertension, Fabry's disease or aortic stenosis.Also appreciated today is the enormous genotypic and phenotypic heterogeneity of this disease with more than 300 mutations over more than 24 genes, ...

In this journal in 1958 Donald Teare reported the pathological findings in a series of eight unrelated young adults who died suddenly and who at necropsy had manifested gross asymmetrical hypertrophy of the heart.' In a footnote, he noted the recent sudden death of the brother of one of the original cases, thus identifying the familial nature of this condition. The subsequent report2 of two gen...

Hypertrophic cardiomyopathy is typically inherited in an autosomal dominant pattern and has a variable age of onset and prognosis. Mutations in the myosin-binding protein C (MYBPC3) gene are one of the most frequent genetic causes of the disease. Patients with MYBPC3 mutations generally have a late onset and a relatively good prognosis. We report here more than 20 Old Order Amish children with ...

BACKGROUND Population-based studies have provided insight into the natural history of adult hypertrophic cardiomyopathy, but comparable information for affected children is lacking. METHODS AND RESULTS All Australian children who presented with primary cardiomyopathy at 0 to 10 years of age between January 1, 1987, and December 31, 1996, were enrolled in a longitudinal cohort study. A single ...