A 36 year old woman, with an 18 year history of syncope, became pregnant shortly after a cardiac catheterization demonstrated a high pulmonary arterial pressure and resistance and a low cardiac output. During pregnancy she remained stable at NYHA FC III, on nifedipine, apresoline, isosorbide, aspirin and bed rest. At 28 weeks, catheterization showed a decreased pulmonary pressure and an increased cardiac output. At 38 weeks, she was submitted to an elective caesarean section, and delivered a healthy newborn of 2820 g. After 5 months, her catheterization showed a pulmonary artery pressure similar to the pre-pregnancy study. Her condition deteriorated, leading to death 10 months later. Urinary 6-keto-PGF1[symbol: see text], nitrates/nitrites, kallikrein and angiotensin-(1-7) were increased from 13 to 33 weeks, to drop in week 35 of pregnancy. The safe maternal and fetal outcome, and the intragestational hemodynamic improvement are attributed to a close multidisciplinary surveillance, and to the effects of the endogenous vasodilators of pregnancy on the reversible component of the pulmonary hypertension. Reports in the literature show a decrease in maternal mortality rate, from 56% for the period previous to 1963, to 34 and 30% for those spanning between 1978-1996 y 1997-2001 respectively.