PRIMARY PULMONARY HYPERTENSION: REPORT OF SIX CASES AND REVIEW OF LITERATURE

Abstract

Six female patients with primary pulmonary hypertension were studied. Exertional dyspnea and fatigue were present in all. Cardiac murmurs were non-specific. P2 was always accentuated. Electrocardiograms and radiologic examinations disclosed right ventricular hypertrophy. There was no left atrial enlargement and the pulmonary functions were usually normal. Five patients who developed right ventricular failure all died within 1 year of the onset of failure. Cardiac catheterization revealed precapillary pulmonary hypertension, high pulmonary vascular resistance, and low cardiac output in patients with failure. The response of the pulmonary vascular resistance to pharmacologic agents suggests importance of structural changes of the pulmonary vessels. These are characterized by marked reduction and frequent occlusion of the lumen of small arteries and arterioles in the lungs.