Evaluation of Outcomes of Pregnancy in Women with Eisenmenger Syndrome: Is There Any Prognostic Criterion?

Objective: 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.

Methods: 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.

Results: 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.

Conclusion: 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.