Primary pulmonary hypertension and pregnancy.

Primary pulmonary hypertension (PPH) is a disorder intrinsic to the pulmonary vascular bed characterized by sustained elevation in pulmonary artery pressure and pulmonary vascular resistance with normal pulmonary artery wedge pressure, in the absence of a known cause. Cardiovascular disorders are the most common cause of morbidity and mortality in pregnant women. Risk is so great with some cardiovascular abnor-malities that recommendation of avoidance or interruption of pregnancy is supportable, one of these conditions is pulmonary hypertension. In this article two patients with primary pulmonary hypertension who sustained two pregnancies each are described. Both patients had uneventful pregnancies and deliveries, with all neonates surviving. When evaluating primary pulmonary hypertension and pregnancy, early studies reported a maternal mortality rate as high as 50%. More recent studies report a maternal mortality of 30%. In this article, cases of primary pulmonary hypertension undergoing pregnancy from 1978 to 2005 were reviewed, revealing a 22% maternal mortality for the total number of pregnancies. Despite advances in treatment, primary pulmonary hypertension continues to carry considerable maternal morbidity and mortality, and prevention of pregnancy is still the main recommendation. Early diagnosis and treatment is critical and a multi-disciplinary approach is required when dealing with a patient with PPH who desires to continue pregnancy.