Dual-Drainage Infradiaphragmatic TAPVR With Splenic Venous Connection: What You See Is Not What You Get

Infradiaphragmatic total anomalous pulmonary venous return (TAPVR) is an uncommon subtype of anomalous pulmonary venous drainage; dual, nonconfluent pulmonary venous pathways are extraordinarily rare. Such atypical configurations increase the complexity of diagnosis and operative planning, requiring meticulous anatomic evaluation in critically ill neonates.

A term 2-day-old neonate presented with hypoxemic respiratory failure and pulmonary congestion with suspected infradiaphragmatic TAPVR. Emergent repair revealed a right-sided confluence draining via a vertical vein with a blind connection to the left atrium; the left pulmonary veins were not visualized. Postoperative computed tomography demonstrated a separate left-sided confluence draining into the splenic vein. Although initially unobstructed, progressive narrowing led to readmission and redo sternotomy with sutureless anastomosis to the left atrium. The patient recovered with unobstructed bilateral pulmonary venous return and decreased right ventricular pressures.

Dual, nonconfluent infradiaphragmatic TAPVR in a biventricular heart is exceptionally rare. This case highlights the limitations of echocardiography and the essential role of cross-sectional imaging in delineating complex venous anatomy.

Despite optimal echocardiography, there is always some degree of uncertainty regarding total anomalous pulmonary venous return anatomy. If risks are acceptable, efforts should be made to obtain advanced imaging for preoperative management and surgical planning.