Primary pulmonary hypertension: modes of diagnosis and aggressive treatment with vasodilators

In the last decade we have made great strides in our understanding of the clinical presentation and findings, natural history, and treatment of patients with primary pulmonary hypertension. Recognizing the tendency for in situ thrombosis in many of these patients now gives strong support to the recommendation that anticoagulants be used for patients who are felt to have underlying pulmonary artery thrombosis. Vasodilators in adequate doses can be demonstrated to cause substantial reductions in pulmonary artery pressure and pulmonary vascular resistance which causes regression in right ventricular hypertrophy and marked clinical improvement. There is still much to be accomplished in this field: a major challenge is to diagnose pulmonary hypertension in patients earlier, at a more treatable stage, and to develop effective treatments for patients with severe right ventricular dysfunction refractory to vasodilator treatment.