Hypertrophic cardiomyopathy is a heart muscle disease with an obscure aetiology. Data from four generations of a large family (71 members) are presented. The occurrence of hypertrophic cardiomyopathy among members of the two oldest generations was compatible with a pattern of autosomal dominant inheritance. Seven out of 14 siblings in the second generation had definite signs of or were clinical...

This study was designed to assess the prevalence of cardiac arrhythmias in a group of relatives of patients who had come to necropsy with hypertrophic cardiomyopathy. Another aim of the study was to assess the validity of an interventricular septal thickness of 1.3 cm or more, measured by echocardiography, as a diagnostic criterion of hypertrophic cardiomyopathy among relatives of cases proven ...

The morphologic determinants of subaortic obstruction in patients with hypertrophic cardiomyopathy are not completely understood. To define the relation between left ventricular outflow tract orifice size and presence or absence of subaortic obstruction, we studied 65 patients with hypertrophic cardiomyopathy and 16 normal controls by quantitative two-dimensional echocardiography. Left ventricu...

of late gadolinium enhancement detected by cardiovascular magnetic resonance correlates with the inducibility of ventricular tachyarrhythmia in hypertrophic cardiomyopathy. J Cardiovasc Magn Reson 2010;12:30. 37. Teraoka K, Hirano M, Ookubo H, Sasaki K, Katsuyama H, Amino M et al. Delayed contrast enhancement of MRI in hypertrophic cardiomyopathy. Magn Reson Imaging 2004;22:155–61. 38. Maron BJ...

INTRODUCTION AND OBJECTIVES Differences between anatomical severity and clinical manifestations are frequent in patients with hypertrophic cardiomyopathy. Our objective was to assess functional capacity in a consecutive group of patients with hypertrophic cardiomyopathy measuring exercise aerobic parameters, as well as clinical and echocardiographic variables. PATIENTS AND METHOD We studied 9...

A beneficial effect of verapamil on left ventricular relaxation has been reported in patients with hypertrophic cardiomyopathy. The effect of 0.1 mg/kg intravenous verapamil on left ventricular relaxation and diastolic mechanics was studied in 10 patients with hypertrophic cardiomyopathy and 13 patients with aortic stenosis. M mode echocardiograms and left ventricular high-fidelity pressure mea...

To identify the chromosomal location of a gene responsible for familial hypertrophic cardiomyopathy, we used clinical and molecular genetic techniques to evaluate the members of a large kindred. Twenty surviving and 24 deceased family members had hypertrophic cardiomyopathy; 58 surviving members were unaffected. Genetic-linkage analyses were performed with polymorphic DNA loci dispersed through...

Familial hypertrophic cardiomyopathy is a genetically and phenotypically heterogeneous disease caused by mutations in seven sarcomeric protein genes. It is known to be transmitted as an autosomal dominant trait with rare de novo mutations.A French family in which two members are affected by hypertrophic cardiomyopathy was clinically screened with electrocardiography and echocardiography. Geneti...

BACKGROUND Conventional Doppler indices of left ventricular diastolic function do not correlate with symptoms or exercise capacity in patients with hypertrophic cardiomyopathy, because of their dependence on loading conditions. Diastolic mitral annular velocity measured using Doppler tissue imaging has been reported to be a preload independent index of left ventricular diastolic function. OBJ...