Eisenmenger syndrome (ES) is considered an absolute contraindication for pregnancy. ES is characterized by a congenital heart abnormality that results in a significant anatomical shunt. Hemodynamic forces generate a left-right shunt, leading to severe pulmonary arterial hypertension (PAH). Eventually, the shunt will become a right-to-left shunt due to increased pulmonary vascular resistance, leading to significant hypoxemia and cyanosis. Pregnant women with ES experience volume overload as a result of the syndrome and the physiological response of pregnancy. The decrease in systemic vascular resistance that occurs during pregnancy also increases the right-to-left shunt, resulting in left ventricular failure. Due to the significant risk to both the mother and the fetus, women are advised to terminate their pregnancy during the first trimester. However, with all the odds, very few cases show positive neonatal and maternal outcomes. Appropriate management of ES includes a multidisciplinary team assembled to monitor and manage the patient carefully and thoroughly. In this paper, we present a case of ES secondary to an atrial septal defect with severe PAH in a 32-year-old woman who underwent a cesarean section at 33 weeks of gestation. She delivered a healthy baby girl. On the seventh postoperative day, she was discharged with no complications.
Keywords: atrial septal defect secundum; eisenmenger syndrome; multidisciplinary decision-making; pregnancy; pulmonary hypertension.
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