Delayed Clinical Presentation of Infracardiac Total Anomalous Pulmonary Venous Return with Portal Venous Drainage

Background: Infracardiac total anomalous pulmonary venous return (TAPVR) is a rare congenital cardiac anomaly that is usually associated with pulmonary venous obstruction and presents early in the neonatal period with severe respiratory distress and hemodynamic compromise. Delayed clinical presentation is uncommon, particularly in cases with portal venous drainage.

Case Presentation: A 19-day-old female neonate presented with sudden cyanosis following feeding after remaining asymptomatic during the first 18 days of life. Chest radiography demonstrated pulmonary congestion. Echocardiography revealed a small left atrium, right-sided cardiac chamber dilatation, severe tricuspid regurgitation, and a posterior pulmonary venous confluence. Subcostal imaging identified a dilated vascular structure at the portal hilum, raising suspicion of anomalous pulmonary venous drainage. Cardiac computed tomography confirmed infracardiac TAPVR, with all pulmonary veins draining through a large vertical vein into the portal venous system. The vertical vein showed marked aneurysmatic dilatation measuring 18 mm. Surgical repair consisting of confluence-to-left atrial anastomosis was successfully performed, while the vertical vein was intentionally left patent. The postoperative course was uneventful, and the patient recovered without major complications.

Conclusion: This case demonstrates that infracardiac TAPVR may occasionally present later than expected despite anatomically severe disease. Awareness of atypical presentations, careful echocardiographic assessment, and prompt confirmation with advanced imaging are essential for timely diagnosis and surgical intervention. Early recognition remains critical to prevent rapid clinical deterioration and potentially fatal outcomes.