Clinical Spectrum of Total Anomalous Pulmonary Venous Drainage: A Case Series

Abstract Total anomalous pulmonary venous drainage (TAPVD) represents a critical congenital heart defect requiring urgent surgical intervention. This case series presents two patients with different TAPVD presentations and outcomes from a tertiary cardiac center. Case 1 involved a 15-month-old boy with cardiac TAPVD and severe pulmonary hypertension (tricuspid regurgitation gradient: 84 mm Hg). He required aggressive management with multiple pulmonary vasodilators (nitroglycerin, milrinone, nebulized iloprost), triple inotropic support (dopamine, adrenaline, noradrenaline), and experienced prolonged intensive care with mechanical ventilation, peritoneal dialysis for acute kidney injury, and nosocomial pneumonia. Case 2 presented a 6-year-8-month-old boy with supracardiac TAPVD and only mild pulmonary hypertension (tricuspid regurgitation gradient: 24 mm Hg). He required minimal hemodynamic support with milrinone and a single inotrope (adrenaline), achieving an uneventful recovery with extubation on postoperative day 2 and intensive care unit discharge on day 4. Both patients underwent successful surgical correction with rerouting of pulmonary veins to the left atrium and atrial septal defect (ASD) closure using different techniques. The key determinants of outcome were the size of the ASD, the degree of pulmonary hypertension, and the prevention of postoperative pulmonary venous obstruction. A comprehensive multimodal diagnostic evaluation, utilizing echocardiography, cardiac catheterization, and computed tomography angiography, provided detailed anatomical characterization. These cases highlight the importance of early detection through pulse oximetry screening, timely surgical intervention, individualized perioperative pulmonary hypertension management based on severity, and consideration of sutureless techniques in high-risk patients. Long-term surveillance for pulmonary venous obstruction and arrhythmias remains essential for optimal outcomes in resource-limited settings.