Pulmonary arterial hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a recognized manifestation of connective tissue diseases, particularly systemic lupus erythematosus and systemic sclerosis. It is associated with high rates of morbidity and mortality in general, and these risks increase substantially during pregnancy and delivery. Women with systemic sclerosis who wish to conceive should have an evaluation for PAH prior to pregnancy. Women with known PAH should be counselled about the high risks to maternal health associated with pregnancy, and permanent or long acting reversible contraception strongly recommended. Women who strongly desire pregnancy despite the risks should be supported and aggressive monitoring and management of PAH and congestive heart failure undertaken with vasodilators. Endothelin receptor antagonists and oral anticoagulants should be avoided due to risks of teratogenicity. Women with progressive or abrupt dyspnea should be evaluated for the development of venous thromboembolism (VTE) or PAH, and treated accordingly. o