先天性心脏病伴重度肺动脉高压孕产妇12例死因分析

Death analysis of 12 pregnant patients with congenial heart disease with severe pulmonary arterial hypertension

目的:总结分析12例先天性心脏病(先心病)伴重度肺动脉高压孕产妇死亡的临床特点及相关因素、吸取经验,以便更好指导今后临床工作。方法:回顾性分析安贞医院2002年9月至2014年3月,收治12例先心病伴重度肺动脉高压孕产妇死亡病例的临床资料,并与同时期未死亡先心病伴重度肺动脉高压孕产妇86例做对比分析。先心病类型有:房间隔缺损、动脉导管未闭、室间隔缺损、复杂先心(心内膜垫缺损、巨大房间隔缺损)及右心室双出口。结果:12例死亡病例中:孕期死亡3例,9例终止妊娠后死亡,其中6例进入围产期行剖宫产术,1例晚期流产经阴道分娩,2例剖宫取胎终止妊娠,医疗干预终止妊娠率66.7%。12例死亡病例全部心功能≥III级,心功能III级1例(8.3%),心功能IV级11例(91.7%)。86例未死亡病例中:人工流产术28例,剖宫取胎术33例,剖宫产术24例,经阴道分娩1例,医疗干预终止妊娠率98.8%。心功能<III级46例(53.5%),心功能≥III级40例(46.5%)。两组在心功能及医疗干预终止妊娠率方面比较,差异有统计学意义(P<0.05)。在平均肺动脉压,平均氧饱和度,平均血氧分压方面,死亡病例与未死亡病例比较有加重趋势,但差异无统计学意义(P>0.05)。98例先心病伴重度肺动脉高压病例汇总分析:早期妊娠28例,无死亡病例;中期妊娠37例,死亡4例(10.8%);晚期妊娠33例,死亡8例(24.2%),死亡率随孕周增加明显升高。患者死亡原因主要为肺动脉高压危象、多器官衰竭、呼吸衰竭、心力衰竭及室性心动过速。结论:先心病伴重度肺动脉高压属妊娠禁忌证,妊娠、产后加重心肺负担,易引起致命性并发症、病死率极高,故此类患者发现妊娠应尽早进行医疗干预。

Objective：To summarize the clinical characteristics and related factors of death cases in pregnancy or postpartum women with congential heart disease （CHD） with severe pulmonary arterial hyperten- sion （PAH） , for better future clinical work. Methods：A retrospective analysis of 12 death and 86 survival cases of AnZhen hospital from september 2002 to march 2014. Tapes of congenital heart disease including ：atrial sep- tal defect（ASD） ,ventricular septal defect （ VSD ）, patent ductus arteriosus （ PDA）, complex congenital heart disease （CCHD）,double outlet right ventricular（DORV）. Results：The 12 death cases：three of them died in pregnancy,9 died after delivery. Modes of delivery：l vaginal delivery, 6 cesarean sections （ ~〉28 weeks） ,2 ce- sarean sections（ 〈 28 weeks）. The percent of medical termination of pregnancy was 66. 7%. There were 1 case （8. 3% ） with NYHA class III and 11 cases （91.7%） with NYHA class IV. The 86 survival cases included 28 artificial abortions,33 cesarean sections （ 〈 28 weeks） ,24 cesarean sections （ ~〉28 weeks） and 1 vaginal deliv- ery. The percent of medical termination of pregnancy is 98. 8%. There were 46 cases with NYHA class 〈III and 40 cases with NYHA class ≥ III. Compared with cardiac fucation and percent of medical termination of preg-nancy in two groups, the differences between the two groups were significantly （ P 〈 0. 05 ）. But pulmonary ar- terial hypertension,oxygen saturation and oxygen partial pressure were no difference between the two groups（P 〉 0. 05）. In all the 98 cases：no death cases in firse trimester pregnancy,4 death cases （10. 8% ）in second tri- mester pregnancy and 8 death cases （24. 2% ）in third trimester pregnancy, mortality was increased with the ges- tational weeks. The reasons of death were pulmonary hypertension crisis （ PHC ） , multiple organ dysfunction syndrome （ MODS）, respiration failure （ RF）, heart failure （ HF）, malignant arrhythmia. Conclusion： In preg- nancy or postpartum the functions of cardiopulmonary could be worse in women with CHD with severe PAH, e- ven lead to death, so medical interventions should be taken as early as possible.