Department of Cardiology, Mersin University Medical Faculty, Mersin, Türkiye.
Department of Cardiology, Gazi University Medical Faculty, Ankara, Türkiye.
Turk Kardiyol Dern Ars. 2024 Oct;52(7):492-498. doi: 10.5543/tkda.2024.40963.
Pregnancy in women with Eisenmenger syndrome (ES) presents very high morbidity and mortality rates for both the mother and fetus. In this study, we aimed to evaluate the outcomes of pregnancies affected by ES.
This retrospective cohort study reviewed pregnancies in women with ES at two university hospitals over the past 10 years. The primary outcomes examined were maternal, perinatal, and pregnancy-related outcomes in women with ES.
The study enrolled eight pregnant women diagnosed with ES. The average maternal age was 26.7 years (range 21-36 years). The causes of ES included ventricular septal defect (five patients), patent ductus arteriosus (two patients), and atrial septal defect (one patient). Four patients had been diagnosed with ES before pregnancy. Two patients died in this group; one developed right heart failure in the third trimester and unfortunately died immediately after delivery due to cardiogenic shock, and another died on the 10th postpartum day from sudden cardiac arrest despite having had a successful delivery. Four patients were diagnosed with ES during the postpartum period; none in this group died, and there were no cases of fetal mortality. We could not identify any clinical or echocardiographic predictors for postpartum mortality. The two deceased patients showed tendencies of severe right heart failure and lower oxygen saturation during the third trimester and before delivery.
Despite advanced obstetric care and targeted treatments for pulmonary arterial hypertension, maternal mortality is still unacceptably high in patients with ES. No significant predictors of maternal mortality were identified.
患有艾森曼格综合征（Eisenmenger syndrome，ES）的女性妊娠会导致母婴发病率和死亡率非常高。本研究旨在评估 ES 患者妊娠的结局。
这是一项回顾性队列研究，分析了过去 10 年在两所大学医院中患有 ES 的女性的妊娠情况。主要观察指标为 ES 女性的母体、围产期和妊娠相关结局。
该研究纳入了 8 名被诊断为 ES 的孕妇。平均母体年龄为 26.7 岁（范围 21-36 岁）。ES 的病因包括室间隔缺损（5 例）、动脉导管未闭（2 例）和房间隔缺损（1 例）。有 4 名患者在妊娠前已被诊断为 ES。该组中有 2 名患者死亡；1 例在妊娠晚期发生右心衰竭，不幸在分娩后因心源性休克立即死亡，另 1 例在产后第 10 天因心脏骤停突然死亡，尽管分娩成功。4 名患者在产后期间被诊断为 ES；该组中无人死亡，也没有胎儿死亡的情况。我们无法确定产后死亡的任何临床或超声心动图预测因素。两名死亡患者在妊娠晚期和分娩前均表现出严重右心衰竭和低氧饱和度的趋势。
尽管产科护理先进，并针对肺动脉高压进行了靶向治疗，但 ES 患者的孕产妇死亡率仍然高得无法接受。未确定母体死亡率的显著预测因素。
