BMJ 1996;313:1418-1419 (7 December)

Editorials

Thromboembolism in primary pulmonary hypertension

No clear increased risk from oral contraceptives

Premature death in patients with clinical evidence of severe right ventricular strain and no obvious predisposing condition has been recognised for over a century. In 1952 Paul Wood distinguished this syndrome of primary pulmonary hypertension from the pulmonary vascular disease that complicates some congenital heart lesions,1 recognising the very poor prognosis that continues to characterise this rare disorder. Its yearly incidence is between 1/200 000 and 1/1000 000, with the result that progress in understanding its pathology and interrupting its natural course has been slow. The overall mean survival remains under five years despite a variety of new treatments.

As in many systemic arterial diseases, there is increasing evidence that primary pulmonary hypertension is an endothelial disorder. The characteristic vascular obliteration that is observed as the condition progresses is thought to be the end stage of intense vasospasm and thrombosis, in different proportions . . . [Full text of this article]


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