Pregnancy in a patient with uncorrected total anomalous pulmonary venous connection (TAPVC) – A case report and literature review

Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart defect in which all pulmonary veins drain anomalously into the systemic venous circulation and right atrium. Uncorrected, it carries high mortality in childhood, making survival to adulthood and pregnancy exceptional. The profound hemodynamic demands of pregnancy pose a significant risk, often leading to consideration for termination. This case report discusses the perioperative and anaesthetic management of a pregnant patient with uncorrected total anomalous pulmonary venous connection (TAPVC) who successfully delivered a healthy infant via lower segment cesarean section (LSCS). For life to be compatible with TAPVC, an intracardiac shunt (e.g., atrial septal defect) must exist to allow partially oxygenated blood to reach the systemic circulation. This report and review highlight that successful pregnancy continuation and delivery in this high-risk population mandates meticulous, multidisciplinary care involving cardiology, obstetrics, anaesthesia, and neonatology specialists.