Supracardiac Total Anomalous Pulmonary Venous Return With Extrinsic Compression of the Vertical Vein by the Left...

Supracardiac total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect. An anatomical variant where the vertical vein courses posterior to the left pulmonary artery, causing extrinsic compression and severe pulmonary venous obstruction, is exceptionally uncommon and life-threatening.

We describe the case of a male infant aged 2.5 months who exhibited severe central cyanosis, respiratory distress, and right heart failure. In the absence of computed tomography (CT), diagnosis was based exclusively on 2D echocardiography, which effectively detected obstructed supracardiac TAPVR with a vertical vein compressed behind the left pulmonary artery, as well as a secundum atrial septal defect (ASD). A small pulmonary venous confluence was discovered intraoperatively. A customized surgical method was used, making use of the cut vertical vein to enhance a broad anastomosis between the confluence and the left atrium. The postoperative course was complicated by severe respiratory failure and neurological events, such as a generalized seizure accompanied by a 3-min asystole and subsequent periventricular leukomalacia. Even with these serious challenges, thorough medical management resulted in complete recovery. At the 7-month follow-up, the patient demonstrated stability, with unobstructed pulmonary venous flow and normalized cardiac geometry.

In settings with limited resources, echocardiography alone can provide an accurate diagnosis of atypical and severely obstructed TAPVR. Even in the presence of serious neurological and respiratory complications during the perioperative phase, it is possible to obtain excellent long-term results by means of adaptive surgical methods aimed at widening a small venous confluence and thorough postoperative intensive care.