Eisenmenger Syndrome Hidden by Unrepaired Patent Ductus Arteriosus: Diagnosis After Delivery

Pregnancy in Eisenmenger syndrome (ES) confers high risk of fetal, obstetric, and maternal complications including death.

A previously healthy nulliparous 22-year-old woman underwent a caesarean section at 37 weeks because of intrauterine growth restriction and preeclampsia. Postpartum, she developed hypoxia and was found to have differential cyanosis and suprasystemic pulmonary pressures because of an unrepaired patent ductus arteriosus. Pulmonary vasodilator therapy was commenced. She was discharged from hospital at day 12 postpartum and remained stable when reviewed as an outpatient 6 months postdischarge.

Decompensation postpartum is common given hormonal and hemodynamic changes in patients with ES and requires meticulous management. This case highlights clinical findings that should prompt investigation for ES including digital clubbing, secondary erythrocytosis, and right ventricular hypertrophy on electrocardiogram.

Pregnancy may unmask undiagnosed ES. Risk of complications remains high in the postpartum period.