Cyanotic Congenital Heart Disease in Pregnancy: A Comprehensive Review of Pathophysiology, Maternal-Fetal Outcomes, and Contemporary Management.

Advances in pediatric cardiac surgery and medical management have enabled an increasing number of women with cyanotic congenital heart disease to reach reproductive age and contemplate pregnancy. Maternal complications occur in approximately 32% of pregnancies in women with uncorrected cyanotic heart disease, with heart failure, arrhythmias, and thromboembolism representing the primary adverse events. Fetal outcomes are critically dependent on maternal oxygen saturation, with live birth rates of 92% when saturation exceeds 90% but plummeting to 12% when saturation falls below 85%. This review synthesizes current evidence regarding the pathophysiology of major cyanotic lesions, maternal cardiovascular adaptations and risks, fetal and neonatal outcomes, risk stratification approaches, and contemporary management strategies from preconception through the postpartum period. Particular attention is given to lesion-specific considerations, guideline-based recommendations from the American Heart Association, American College of Cardiology, and the European Society of Cardiology, and the essential role of multidisciplinary cardio-obstetrics teams in optimizing outcomes for this high-risk population. The 2025 American College of Cardiology/American Heart Association/Heart Rhythm Society/International Society for Adult Congenital Heart Disease/Society for Cardiac angiography and Interventions Guideline for the Management of Adults with Congenital Heart Disease reinforces the importance of structured preconception counseling, individualized risk stratification, and coordinated multidisciplinary care in specialized ACHD centers.