title: Pregnancy outcome in women with Eisenmenger's syndrome: a case series from west China.
abstract: Eisenmenger's syndrome (ES) consists of pulmonary hypertension with a reversed or bidirectional shunt at the atrioventricular, or aortopulmonary level. The cardiovascular changes that occur during the pregnancy contribute to the high maternal morbidity and mortality in patients with ES. This study is to assess maternal and fetal outcomes in patients with ES.
  
  
  
  This study is a retrospective analysis of 11 pregnancies in women with ES who delivered at a tertiary care center in west China between 2010 and 2014. Cases were divided into group I (maternal survival) and group II (maternal death). Clinical data were noted and analyzed.
  
  
  
  All ES patients presented with severe pulmonary arterial hypertension (PAH). Four maternal deaths were recorded (maternal mortality of 36%). Only one pregnancy continued to term. Ventricular septal defect diameter in group II was larger than that in group I (2.93 ± 0.76 cm vs. 1.90 ± 0.54 cm, p < 0.05). Arterial oxygen saturation and pre-delivery arterial oxygen tension during oxygen inhalation were significantly lower in group II (p < 0.05). Pulmonary arterial blood pressure (PABP) in both groups were high while ejection fractions (EF) were significantly lower in group II (p < 0.05). The incidence of pre-delivery heart failure in group II was substantially higher than in survivors (100 vs.14.3%, p < 0.05). Fetal complications were exceptionally high: preterm delivery (88%), small for gestational age (83%), fetal mortality (27%) and neonatal mortality (25%).
  
  
  
  In west China,the perinatal outcome of pregnant women with ES is poor, especially when complicated with high pulmonary arterial hypertension (PAH). Pregnancy remains strongly contraindicated in ES. Effective contraception is essential, and the option of terminating pregnancy in the first trimester should be presented to pregnant women with ES.
authors.affiliation: Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.|Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.
authors.forename: Ruiqi
authors.initials: R
authors.lastname: Duan
authors.affiliation: ICU of Gynecology & Obstetrics, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.|Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.
authors.forename: Xiumei
authors.initials: X
authors.lastname: Xu
authors.affiliation: Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.|Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.
authors.forename: Xiaodong
authors.initials: X
authors.lastname: Wang
authors.affiliation: Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China. fanjy422@163.com.|Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China. fanjy422@163.com.
authors.forename: Haiyan
authors.initials: H
authors.lastname: Yu
authors.affiliation: Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.|Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.
authors.forename: Yong
authors.initials: Y
authors.lastname: You
authors.affiliation: Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.|Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.
authors.forename: Xinghui
authors.initials: X
authors.lastname: Liu
authors.affiliation: Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.|Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.
authors.forename: Aiyun
authors.initials: A
authors.lastname: Xing
authors.affiliation: Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.|Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.
authors.forename: Rong
authors.initials: R
authors.lastname: Zhou
authors.affiliation: Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.|Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, China.
authors.forename: Mingrong
authors.initials: M
authors.lastname: Xi
mesh_terms: D000293:Adolescent; D000328:Adult; D002681:China; D004541:Eisenmenger Complex / Q000150:complications / Q000401:mortality*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality; D007231:Infant, Newborn; D063130:Maternal Death / Q000209:etiology; D008428:Maternal Mortality; D066087:Perinatal Death / Q000209:etiology; D054238:Perinatal Mortality; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000209:etiology / Q000401:mortality*; D011256:Pregnancy Outcome; D012189:Retrospective Studies; D055815:Young Adult
keywords: Eisenmenger’s syndrome (ES); Fetal outcome; Maternal outcome; Pregnancy; Pulmonary arterial hypertension (PAH)