Treatment of Primary Pulmonary Hypertension — The Next Generation

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Abstract
Primary pulmonary hypertension predominantly affects women, frequently in the prime of life, and usually leads to death from right ventricular failure within a few years after diagnosis. It is a vascular disease but is oddly confined to the small pulmonary arterioles, where intimal fibrosis and medial hypertrophy lead sequentially to vascular obstruction, elevated pulmonary vascular resistance, pulmonary hypertension, and right ventricular overload. Coagulation at the endothelial surface contributes to obstruction, and thromboembolism may occur as a secondary event. The right ventricle compensates through hypertrophy, and although it can sustain function at high pressures for months to years, decompensation is ultimately . . .