The coexistence of multiple complex congenital cardiac anomalies in a single infant - particularly unbalanced Atrioventricular Septal Defect; Double Outlet Right Ventricle; Infracardiac Total Anomalous Pulmonary Venous Connection, situs inversus, and pulmonary hypertension - is exceptionally rare and presents significant surgical and physiological challenges. A full-term male neonate was diagnosed at 25 days of age with this rare combination of defects. Despite low-flow oxygen, the baseline oxygen saturation remained at 60%-65%, and growth was severely impaired. Initial management at a tertiary center included diuretics, captopril, and high-calorie feeding, with discharge on home oxygen therapy. Recurrent cyanotic spells worsened, leading to readmission at 7 months due to profound hypoxemia (SpO2 35%). Escalated respiratory and nutritional support, along with optimized heart failure therapy, achieved only transient stability. Two multidisciplinary teams concluded that local surgical or palliative options were infeasible and recommended urgent referral to a supraregional center for treatment. Before transfer could occur, the infant suffered a terminal cyanotic spell at 8 months. Resuscitation efforts were unsuccessful, and the infant succumbed to death at 8 months of age. This case illustrates how anatomical and physiological complexity, coupled with limited regional surgical infrastructure, can preclude curative repair. Early antenatal or neonatal detection, rapid multidisciplinary coordination, and timely transfer to specialized cardiac centers are essential for improving survival in such cases.
Taha et al. (Tue,) studied this question.