妊娠合并肺动脉高压死亡患者临床特征分析

Analysis of clinical characteristics of dead pregnant women with pulmonary hypertension

目的分析妊娠合并肺动脉高压(PAH)死亡患者临床特征及高危因素。方法收集2000年1月至2019年6月北京协和医院妇产科收治的妊娠合并PAH死亡患者资料,对其临床特征以及妊娠结局进行分析。结果(1)9例妊娠合并PAH死亡患者,其中1例为特发性PAH(IPAH),4例继发于先天性心脏病室间隔缺损,4例继发于风湿免疫病。所有患者均未进行孕前咨询,均未规律产检。(2)9例患者中3例孕前已诊断PAH,6例为孕期首次发现。入院时,8例为心功能III^IV级,1例为心功能II级。超声心动图检查估测的肺动脉收缩压(sPAP)平均值为(104±15.4)mmHg(1 mmHg=0.133 kPa),中位数是102 mmHg。9例患者中8例就诊时有胸闷憋气症状,1例因下肢肿胀、口唇紫绀而就诊。(3)9例患者均以剖宫产术终止妊娠。其中8例采用全身麻醉,1例采用连续硬膜外麻醉。(4)9例患者中,4例为分娩后48 h内死亡,另5例患者死亡时间为分娩后4~26 d,中位死亡时间为14 d。(5)9例患者中,终止妊娠孕周为20^+1~35^+2周,中位孕周为28^+4周。其中3例为20~28周之间终止妊娠,流产儿分娩时即死亡;3例因社会经济原因放弃抢救新生儿,另外3例新生儿存活。结论(1)妊娠合并PAH风险极高,母儿结局预后不良。孕期出现自觉症状,心功能明显下降以及对药物治疗无改善者应尽早终止妊娠。(2)与妊娠合并继发于先天性心脏病的PAH患者比较,合并继发于风湿免疫病的PAH患者更早出现胸闷憋气症状,预后更差。(3)在剖宫产围手术期极易发生PAH危象及全心功能衰竭或多器官功能衰竭,死亡率高。

Objective:To analyze high risk factors and clinical characteristics of dead pregnant women with pulmonary hypertension(PAH).Methods:A retrospective review of medical records was conducted to dead pregnant patients with PAH who were treated in the Department of Obstetrics and Gynecology of Peking Union Medical College Hospital from January 2000 to June 2019.Their clinical features and pregnancy outcome were analyzed.Results:(1)Among 9 dead pregnant patients with PAH,one patient was idiopathic pulmonary arterial hypertension(IPAH),four patients were secondary to ventricular septal defect of congenital heart disease,and another four patients were secondary to rheumatic immune disease.None of the patients had any pre-pregnancy consultation or regular prenatal examination.(2)Among the nine patients,three patients had been diagnosed with PAH before pregnancy,and six patients were diagnosed for the first time during pregnancy.On admission,the cardiac function of eight patients was classified as grade III-IV,and one patient was classified as grade II.The mean systolic pulmonary arterial pressure(sPAP)was(104±15.4)mmHg(1 mmHg=0.133 kPa),the median was 102 mmHg,estimated by echocardiography.Eight patients presented with chest tightness and suffocation,and one patient presented with lower limb swelling and cyanosis.(3)All the nine patients delivered by cesarean section.Eight patients were operated under general anesthesia and one patient was operated under continuous epidural anesthesia.(4)Among the nine patients,four patients died within 48 hours after delivery.Another five patients died ranged from 4 to 26 days of postpartum,the median time of death was 14 days.(5)Among the nine patients,the gestational age of termination ranged from 20^+1 to 35^+2 weeks,and the median gestational age was 28+4 weeks.Among them,the pregnancies of three patients were terminated between 20 to 28 weeks,and the newborn died during delivery.Three cases gave up rescuing newborns due to social and economic reasons,and the other three newborns were alive.Conclusions:(1)The risk of pregnancy complicated with pulmonary hypertension is very high,the outcomes of the mother and child are poor.Once cardiac function significantly decreased and no improvement by drug treatment,the pregnancy should be terminated as soon as possible.(2)Compared the pregnancy patients with CHD-PAH,PAH patients secondary to rheumatic immune-disease preferred to present chest tightness and suffocation earlier and had a worse prognosis.(3)PAH crisis and total heart failure or multi-organ failure are prone to occur during the perioperative period of cesarean section,and the perinatal mortality of mother is high.