The peripartum management of a 32-year-old patient presenting at 34 weeks’ gestation with unrepaired cyanotic heart disease

Unrepaired cyanotic heart disease is considered a high-risk lesion owing to the consequent increase in maternal and fetal complications. In the presence of pulmonary hypertension, maternal mortality approaching 50% has been reported, and pregnancy is therefore considered contraindicated. We present a case of a 32-year-old woman presenting at 34 weeks’ gestation in heart failure due to newly diagnosed cyanotic complex cardiac disease. The diagnosis of left atrial isomerism with a common atrium, single atrioventricular valve, and a restrictive ventricular septum defect was made. Cyanosis was due to mixing at atrial level, without features of Eisenmenger’s syndrome. Her intrapartum multidisciplinary management is described in detail. The patient was discharged home in a condition similar to her baseline, together with her healthy neonate.