妊娠合并先天性心脏病相关肺动脉高压预后及影响因素分析

Long-term outcome and prognostic factors in pregnant women with pulmonary arterial hypertension associated with congenital heart disease

目的 探讨妊娠合并先天性心脏病（CHD）相关肺动脉高压（PAH）患者围产期结局、相关影响因素及远期转归.方法 回顾性分析2004—2013年北京安贞医院妇产科及综合外科ICU住院并诊断为CHD相关PAH孕产妇的临床资料,并对患者的生存和诊治等进行随访.结果 入选110例CHD相关PAH孕产妇,PAH轻度者11例,PAH中度者33例,PAH重度者66例.孕期纽约心脏病协会（NYHA）心功能分级下降≥2级、呼吸衰竭、PAH危象、心律失常发生率分别为25.5%（28/110）、7.3%（8/110）、10.0%（11/110）、10.0%（11/110）,其中孕期NYHA心功能分级下降≥2级、呼吸衰竭在PAH轻中度和重度者间比差异有统计学意义.院内死亡8例（7.3%）,均为重度PAH患者.多因素logistics分析显示,肺动脉收缩压是孕产妇围术期死亡的危险因素（OR=1.042,P=0.005）.足月产55例（50.0%）,医源性流产35例（31.8%）.PAH重度者足月产比例降低,医源性流产、小于胎龄儿（SGA）比例明显升高.新生儿畸形发病率为8.0%（6/75）.随访率61.8%（63/102）.1例患者于出院数天后猝死,其余62例患者均存活.随访时NYHA心功能分级Ⅰ-Ⅱ级53例（85.5%）,Ⅲ-Ⅳ级9例（14.5%）.孕期心功能恶化与远期心功能恶化相关性不显著（P=0.767）.结论 妊娠合并CHD相关PAH患者围术期病死率、母婴不良事件发生率高,肺动脉收缩压是孕产妇围产期死亡的主要危险因素.CHD相关PAH孕产妇产褥期后总体转归良好.

Objective To investigate the perinatal outcome , risk factors and long-term outcome of pregnancy complicated with pulmonary arterial hypertension （PAH） and congenital heart diseases （CHD）. Methods Clinical data of 110 pregnant women who were diagnosed as PAH-CHD were retrospectively analyzed in the Department of Obstetrics and Gynecology and Surgical Intensive Care Unit at Beijing Anzhen Hospital from 2004 to 2013.The survival and treatment status were followed up .Results 110 subjects consisted of 11 mild PAH, 33 moderate and 66 severe ones .The incidences of deterioration in New York Heart Association （ NYHA ） classes （≥2 ） during pregnancy , respiratory failure , pulmonary hypertension crisis and arrhythmia were 25.5% （28/110）,7.3% （8/110）,10.0% （11/110）,10.0% （11/110） respectively.Among them, the difference of deterioration in NYHA classes （≥2） during pregnancy among the three groups was statistically significant .A total of 8 （ 7.3%） maternal deaths occurred during hospitalization , all of whom were severe PAH cases .Multivariate analysis showed that pulmonary artery systolic pressure was a risk factor of perioperative death （OR=1.042, P=0.005）.There were 55 cases （50.0%） of term delivery, and 35 cases （31.8%） of iatrogenic abortion.The proportion of term delivery in the severe PAH group was significantly lower . The proportion of iatrogenic abortion and small for gestational age infant （ SGA ） were higher in severe group .The incidence of neonatal malformations was 8.0%（6/75）.The follow-up rate was 61.8%（63/102）.Sudden death was reported in a parturient a few days after discharge .The remaining 62 patients survived during follow-up, while 53 patients （85.5%） were functional class （ FC ） Ⅰ -Ⅱ, 9 （ 14.5%） were FC Ⅲ -Ⅳ at follow-up.The cardiac function deterioration during pregnancy was not significantly correlated with long-term deterioration （P =0.767）. Conclusions Perinatal mortality and the incidence of maternal and fetal adverse events were high in pregnancy with PAH-CHD.Pulmonary artery systolic pressure is a major risk factor for perioperative mortality in pregnant women .PAH-CHD woman had good overall outcome after puerperium .