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

Abstract

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. 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 computed tomography 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 communication with right atrium. The vertical vein was ligated. There were no complications after 1 year of follow-up. 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.