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 ...

A critical appraisal of diastolic time intervals as a measure of relaxation in left ventricular hypertrophy ABSTRACT Diastolic time intervals (DTIs) were calculated from simultaneous recordings of a phonocardiogram, apexcardiogram, and echocardiogram in 84 patients with hypertrophic cardiomyop-athy and 35 patients with hypertrophy secondary to chronic pressure and volume loading and were compar...

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 ...

BACKGROUND Patients with hypertrophic obstructive cardiomyopathy have left ventricular (LV) diastolic dysfunction due, in part, to temporal heterogeneity in regional function. The acute effect of the relief of LV outflow tract obstruction is unknown. Therefore, we investigated the effects of nonsurgical septal reduction therapy (NSRT) on regional function. METHODS AND RESULTS Twenty-two patie...

BACKGROUND For more than 20 years in Italy, young athletes have been screened before participating in competitive sports. We assessed whether this strategy results in the prevention of sudden death from hypertrophic cardiomyopathy, a common cardiovascular cause of death in young athletes. METHODS We prospectively studied sudden deaths among athletes and nonathletes (35 years of age or less) i...