Bao Zhaoliang, Zhang Jun, Yang Dong, Xu Xiaohui
Department of Obstetrics and Gynecology, Beijing Anzhen Hospital Affiliated to Capital Medical University, Beijing 100029, China.
Department of Obstetrics and Gynecology, Beijing Anzhen Hospital Affiliated to Capital Medical University, Beijing 100029, China. Email:
Zhonghua Fu Chan Ke Za Zhi. 2014 Jul;49(7):495-500.
Study of pulmonary hypertension (PAH) during pregnancy has characteristics of the high risk factors for patient death and its clinical characteristics.
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.
(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.
(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.
研究妊娠合并肺动脉高压(PAH)患者的死亡高危因素及临床特点。
收集2006年1月至2013年10月在首都医科大学附属北京安贞医院住院治疗的8例妊娠合并PAH患者的临床资料。根据PAH发病机制将患者分为两类,特发性肺动脉高压(IPAH)4例,继发性PAH 4例[继发于先天性心脏病,又称先天性心脏病相关性PAH(CHD-PAH)]。分析8例患者的临床特征及妊娠结局。
(1)8例患者中,IPAH 4例,均无基础疾病,合并重度三尖瓣反流。CHD-PAH 4例,均合并艾森曼格综合征。8例患者均未进行孕前咨询及规律产前检查。(2)8例患者孕前心功能为Ⅰ-Ⅱ级,入院时为Ⅲ-Ⅳ级。超声心动图估测肺动脉收缩压(sPAP)为101 mmHg(1 mmHg = 0.133 kPa)。8例患者中,7例因临床症状加重于孕27周及以后入院治疗,1例孕18周因临床症状加重入院治疗。(3)8例患者中,1例继发于动脉导管未闭的CHD-PAH患者,sPAP为170 mmHg,入院后12小时死亡;其余7例行剖宫产终止妊娠。4例IPAH患者采用连续硬膜外麻醉,其中1例术中发生PAH危象,改行全身麻醉后出现呼吸、心搏骤停,3例CHD-PAH患者中1例采用连续硬膜外麻醉,2例采用全身麻醉。(4)8例患者中,7例中位死亡时间为产后3天,其中4例IPAH患者产后2.5天死亡,死亡原因均为PAH危象及心力衰竭。4例CHD-PAH患者中,1例入院后12小时死亡,其余3例中位死亡时间为产后13天,死亡原因均为PAH危象及多器官功能衰竭。(5)8例患者中,1例继发于动脉导管未闭的CHD-PAH患者于孕31周发生死胎。1例孕19周行剖宫产手术,其余6例分别于孕28-30周顺产,新生儿存活。(6)分娩前,4例IPAH患者及3例CHD-PAH患者采用前列地尔、伊洛前列素、西地那非降低肺动脉压力治疗,1例CHD-PAH患者入院后12小时死亡,未进行药物治疗。
(1)PAH患者孕前需咨询,妊娠期间必须进行规律产前检查,孕期出现症状、心功能明显下降且药物治疗无改善者应尽早终止妊娠。(2)与CHD-PAH孕妇相比,IPAH患者病情进展快,预后差。(3)剖宫产术中或分娩时患者易发生PAH危象及心力衰竭或多器官功能衰竭。采取积极措施维持血流动力学稳定是预防PAH孕妇死亡的关键。