Objective To examine the impact of severe idiopathic pulmonary arterial hypertension (IPAH) on pregnancy outcome in modem era, and to investigate the effect of multidisciplinary approach during pregnancy on the pregnancy outcome in pregnant woman with severe IPAH.Methods We retrospectively evaluated consecutive ten pregnant women with severe IPAH between March 2007 and November 2013 in Beijing Anzhen Hospital.Hemodynamic measurements, medical therapy, manner of delivery, anesthetic administration, multidisciplinary management and outcomes were assessed.Results Ten patients were induced in this study, and all cases were first diagnosed during the pregnancy, and they were hospitaled at (24.5±3.1) weeks.The systolic pulmonary artery pressure (sPAP) estimated by transthoracic echocardiography was (95.6±1.3) mmHg, and the World Health Organization functional class was class Ⅲ for seven, and class Ⅳ for three patients.One patient underwent pregnancy termination at gestational age of 21 weeks with no maternal death or complications, nine patients continued pregnancy and all the patients underwent caesarean section.The mean pregnancy length was (30.9±5.0)weeks.Nine had cesarean deliveries during continuous epidural anesthesia, and one during general anesthesia.There were three maternal deaths in hospital (five, two, three days postpartum), and seven patients were alive.One fetus lost with cesarean section.Two were term delivery, and seven cases were premature delivery.Nine were alive and no malformation.Conclusions Because of maternal mortality in patients with severe IPAH remains prohibitively high, patients should continue to be counseled to avoid pregnancy.Women with severe IPAH who become pregnancy should be followed by multidisciplinary approach , and cesarean deliveries during continuous epidural anesthesia is a relatively safe way for pregnancy termination in patients with severe IPAH.