Seven unrelated patients with atypical variants of hemizygous Fabry's disease were found among 230 men with left ventricular hypertrophy (3 percent). Fabry's disease should be considered as a cause of unexplained left ventricular hypertrophy.

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MCI-154 improved not only left ventricular systolic function but also diastolic relaxation and distensibility in a chronic heart failure model.

ypertrophic cardiomyopathy (HCM) is hemodynamically characterized by abnormal ventricular diastolic function: impaired isovolumic relaxation, 1-4 abnormal diastolic filling pattern, [3][4][5][6] and decreased diastolic distensibility. 3,4 These abnormalities have been well demonstrated in the left ventricle (LV), but little information has been available concerning the right ventricle (RV) in HCM patients. Suzuki et al using cine magnetic resonance imaging, showed decreased RV early diastolic filling in HCM patients. 7 However, there has been no systematic study of RV isovolumic relaxation, diastolic filling, or compliance in HCM patients, because invasive studies are needed to analyze high-fidelity ventricular pressures and volumes.The purpose of the present study was to systematically investigate RV isovolumic relaxation, diastolic filling, and diastolic distensibility in HCM patients and to compare those variables with other hemodynamic variables and those of the LV. We did this by analyzing high-fidelity RV and LV pressures using catheter-tipped manometers and biplane RV cine-angiograms in HCM patients. Methods Study SubjectsThe study group comprised 19 patients with HCM and a control group of 13 patients investigated for chest pain who proved to have angiographically normal coronary arteries and normal LV function. The HCM patients were 18 men Japanese Circulation Journal Vol.63, September 1999 and 1 woman with an average age of 45 years (range, 20-60 years). Among the normal control subjects were 7 men and 6 women with an average age of 50 years (range, 36-72 years). The diagnosis of HCM was made by clinical, echocardiographic, and angiographic evaluation. Based on the echocardiographic classification, all patients had asymmetrical septal hypertrophy with a septal-to-posterior wall ratio of 1.3:1 or more. The thickness of the interventricular septum and posterior free wall was 20.5±4.5 and 12.2±2.6 mm, respectively. All patients had normal sinus rhythm. No patient with HCM had a resting RV or LV outflow tract pressure gradient. Patients with apical hypertrophic cardiomyopathy were not included in this study. All medications, including calcium antagonists, were terminated at least 5 days before cardiac catheterization. Informed consent was obtained from each patient, and there were no complications as a result of this study. Study ProtocolCardiac catheterization was performed via the percutaneous femoral approach in the fasting state. RV and LV pressures were measured using a catheter-tip manometer (RV: Goodtec, USA; LV: Millar Instruments, Houston, TX, USA) and recorded at a paper speed of 150-200 mm/s (Electronics for Medicine VR-12, Pleasantville, NY, USA). After routine right and left heart catheterization, we inserted another catheter via the femoral vein to perform right ventriculograms. Simultaneous biplane cineventriculograms were obtained in the 30°right anterior oblique and 60°left anterior oblique projections by injecting 40 ml of iohexol at a rate of 10-12 ml/s. The film speed was 50 frames/s...