PRIMARY PULMONARY-HYPERTENSION

A mysterious disease: Primary pulmonary hypertension is a serious thou gh uncommon condition. Short-term outcome may be fatal. The pathogenes is remains obscure. Abnormal interactions between the pulmonary vascul ar endothelium and platelets appears to be the key pathophysiological factor. Unknown cause: In some cases, primary pulmonary hypertension i s associated with other disease or cofactor; which may be inaugural or accelerate its development Cases involving portal hypertension, HIV i nfection and use of anorexic drugs have been described. Difficult diag nosis:The condition is usually seen in young adults. Early diagnosis i s exceptional because symptoms are nonspecific The main functional sig n is exercise-induced dyspnea and may remain the only manifestation fo r a considerable period. Echocadiography and catheterism for a hemodyn amic study are needed for positive diagnosis. Management: Early treatm ent with vasodilators usually improves patient comfort. Lung transplan tation can now provide cure. Adapted therapy and careful surveillance have helped improve prognosis somewhat. (C) 1998, Masson, Paris.