title: [Severe idiopathic pulmonary arterial hypertension in pregnancy: a review of 10 cases].
abstract: OBJECTIVE
  To study the impact of severe idiopathic pulmonary arterial hypertension (IPAH) on pregnancy outcome and to investigate the effect of multidisciplinary approach during pregnancy on the pregnancy outcome in pregnant woman with severe IPAH.
  
  
  METHODS
  Between March 2007 and November 2013, 10 pregnant women with severe IPAH undergoing treatment in Beijing Anzhen Hospital were studied retrospectively.Hemodynamic measurements, medical therapy, manner of delivery, anesthetic administration, multidisciplinary management and outcomes were assessed.
  
  
  RESULTS
  All 10 cases were first diagnosed at the mean of (24 ± 3) weeks during the pregnancy. The systolic pulmonary artery pressure (sPAP) estimated by transthoracic echocardiography was (95.6 ± 1.3) mmHg (1 mmHg = 0.133 kPa) .Seven cases of class III and 3 cases of class IV were recorded by World Health Organization functional class.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 cesarean section. The mean pregnancy length was (31 ± 5) weeks.Nine had cesarean deliveries during continuous epidural anesthesia, and one during general anesthesia. There were three maternal deaths in hospital (5, 2, 3 days postpartum), and seven patients were alive, and the average hospitalization days was (8 ± 4) days.One fetus lost with cesarean section. Two were term delivery, and seven cases were premature delivery. The average weight is (1 948 ± 731) g and 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 are a relatively safe way for pregnancy termination in patients with severe IPAH.
authors.affiliation: Department of Obstetrics and Gynecology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.
authors.forename: Xiaohui
authors.initials: X
authors.lastname: Xu
authors.affiliation: Email: drzhangj@outlook.com.
authors.forename: Jun
authors.initials: J
authors.lastname: Zhang
authors.forename: Hui
authors.initials: H
authors.lastname: Wang
authors.forename: Yanna
authors.initials: Y
authors.lastname: Li
authors.forename: Haofeng
authors.initials: H
authors.lastname: Zhang
authors.forename: Duomao
authors.initials: D
authors.lastname: Lin
mesh_terms: D000028:Abortion, Induced; D000767:Anesthesia, Epidural; D062186:Arterial Pressure / Q000502:physiology; D002585:Cesarean Section; D036861:Delivery, Obstetric; D004452:Echocardiography; D065627:Familial Primary Pulmonary Hypertension / Q000175:diagnosis* / Q000401:mortality; D005260:Female; D005865:Gestational Age; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D008428:Maternal Mortality; D011247:Pregnancy; D011248:Pregnancy Complications; D011256:Pregnancy Outcome; D047928:Premature Birth; D011651:Pulmonary Artery / Q000000981:diagnostic imaging*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome