Total anomalous pulmonary venous return with mixed drainage and double connection: a rare case report not previously described

Abstract

Background Total anomalous pulmonary venous return is a rare cyanotic congenital heart disease in which pulmonary veins connect to the systemic veins, right atrium or coronary sinus. Given its variability, it is essential to define the pathway and drainage site. Case summary We describe a 3-day-old patient with total anomalous pulmonary venous return, in which the four pulmonary veins drained into a common vertical vein that had a double connection at the cardiac (coronary sinus) and infracardiac (portal vein) levels. The echocardiographic diagnosis was confirmed by CT scan. At 1.5 months she underwent surgery by anastomosis of the venous collector with the left atrium, unroof of the coronary sinus and clousure of its comunication with right atrium. The vertical vein was ligated. There were no complications after one year of follow-up. Discussion This is an unusual anatomy that has not been described in the literature. In this article, we discuss the embryological origin. Furthermore, we highlight the importance of an accurate diagnosis of the venous pathway and its impact on surgical planning and patient prognosis.

Four‐dimensional imaging of fetal infracardiac total anomalous pulmonary venous connection with double drainage

“…According to the Darling classification, there are four types of TAPVC: supracardiac, cardiac, infracardiac and mixed 5 . In mixed TAPVC, the pulmonary veins terminate at two or three body levels (supracardiac–cardiac, supracardiac–infracardiac, cardiac–infracardiac and supracardiac–cardiac–infracardiac) 6 . Multiple connections are more common at supracardiac and cardiac levels, less common at supracardiac and infracardiac levels and rare at cardiac and infracardiac levels.…”