title: [Analysis of high risk factors for patient death and its clinical characteristics on pregnancy associated with pulmonary arterial hypertension].
abstract: OBJECTIVE
  Study of pulmonary hypertension (PAH) during pregnancy has characteristics of the high risk factors for patient death and its clinical characteristics.
  
  
  METHODS
  Death in patients with clinical data was collected from January 2006 to October 2013 in Beijing Anzhen Hospital Affiliated to Capital Medical University treated 8 cases of pregnancy complicated with PAH in hospital. According to the mechanism of PAH patients will be divided into two categories, Idiopathic pulmonary arterial hypertension (IPAH) in 4 cases, 4 cases of secondary PAH [are secondary to congenital heart disease, also known as congenital heart disease associated PAH (CHD-PAH)]. Analyze the clinical features of 8 cases of patients and pregnancy outcome.
  
  
  RESULTS
  (1) In 8 patients, 4 cases were IPAH, none of them with primary diseases, and they were complicated with severe tricuspid regurgitation. 4 cases were CHD-PAH, all with Eisenmenger's syndrome. 8 patients were not preconception counseling and regular prenatal examination. (2) The pregestational cardiac function of 8 cases was grade I-II, and it was grade III-IV on admission. The estimation pressure (sPAP) of pulmonary artery systolic by echocardiography was 101 mmHg (1 mmHg = 0.133 kPa). In 8 patients, 7 cases were in pregnancy 27 weeks and beyond for treatment since the clinical symptoms increased, 1 case of pregnant 18 weeks for treatment caused by the increased clinical symptoms. (3) In 8 patients, 1 patient with CHD- PAH secondary to patent ductus arteriosus, its sPAP was 170 mmHg, dead at 12 hours after admission; the remaining 7 cases termination with cesarean section. 4 patients with IPAH were continuous epidural anesthesia, including 1 case for the intraoperative PAH crisis and respiratory and cardiac arrest with general anesthesia, 3 cases of CHD- PAH patients in 1 case with continuous epidural anesthesia, 2 cases of general anesthesia.(4) In 8 patients, 7 cases of median death time were 3 days after delivery, including 4 cases of IPAH patients death for 2.5 days after delivery; the causes of death were PAH crisis and heart failure. Time of death in 4 cases of CHD-PAH, 1 case was dead at 12 hours after admissions, the remaining 3 cases median death time were 13 days after delivery; the death causes for 4 cases of CHD-PAH were PAH crisis and multiple organ failure. (5) In 8 patients, 1 patient with CHD-PAH secondary to patent ductus arteriosus in gestational week 31 stillbirths occur. 1 case of pregnant 19 weeks had treatment of caesarean operation, the remaining 6 cases respectively at 28-30 weeks of gestation live birth, neonatal survival. (6) Before delivery, 4 cases of IPAH and 3 cases of CHD-PAH patients treated with alprostadil, iloprost, sildenafil, reduction of pulmonary artery pressure treatment, 1 case of CHD-PAH patient was dead after 12 hours in hospital, no drug treatment.
  
  
  CONCLUSIONS
  (1) PAH in patients need for consultation prior to conception, pregnancy must conduct regular prenatal examination, symptoms occur during pregnancy, the cardiac function was significantly decreased, and no improvement of drug treatment should be early terminated the pregnancy. (2) Compared with the pregnant women with CHD- PAH, faster progress and poor prognosis in patients with IPAH disease. (3)The patients during cesarean operation or intrapartumare easy to cause PAH crisis and heart failure or multiple organ failure. Taking active measures to maintain stability of hemodynamics is the key to prevent the occurrence of death of pregnant women with PAH.
authors.affiliation: Department of Obstetrics and Gynecology, Beijing Anzhen Hospital Affiliated to Capital Medical University, Beijing 100029, China.
authors.forename: Zhaoliang
authors.initials: Z
authors.lastname: Bao
authors.affiliation: Department of Obstetrics and Gynecology, Beijing Anzhen Hospital Affiliated to Capital Medical University, Beijing 100029, China. Email: drzhangj@outlook.com.
authors.forename: Jun
authors.initials: J
authors.lastname: Zhang
authors.affiliation: Department of Obstetrics and Gynecology, Beijing Anzhen Hospital Affiliated to Capital Medical University, Beijing 100029, China.
authors.forename: Dong
authors.initials: D
authors.lastname: Yang
authors.affiliation: Department of Obstetrics and Gynecology, Beijing Anzhen Hospital Affiliated to Capital Medical University, Beijing 100029, China.
authors.forename: Xiaohui
authors.initials: X
authors.lastname: Xu
mesh_terms: D000768:Anesthesia, General; D002585:Cesarean Section / Q000379:methods; D036861:Delivery, Obstetric / Q000379:methods; D004452:Echocardiography; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005865:Gestational Age; D006330:Heart Defects, Congenital / Q000150:complications* / Q000473:pathology; D006333:Heart Failure / Q000209:etiology; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D008428:Maternal Mortality; D010879:Piperazines; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D011256:Pregnancy Outcome; D011651:Pulmonary Artery; D011687:Purines; D012307:Risk Factors; D000068677:Sildenafil Citrate; D013449:Sulfonamides
chemical_list: D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D000068677:Sildenafil Citrate