Total anomalous pulmonary venous connection to the portal and splenic vein associated with unilateral hypoplasia of pulmonary veins

Abstract

Total anomalous pulmonary venous return (TAPVR) represents a rare congenital anomaly with wide anatomical and physiological variability. We report a case of a newborn with a challenging form of obstructed infracardiac TAPVR, in whom left and right pulmonary veins drained separately into the portal system. The right pulmonary venous sinus connected to the left branch of the portal vein, whereas the left venous sinus connected to the splenic vein. Surgical repair consisted of the creation of a common retrocardiac venous trunk which was anastomosed to the left atrium. The postoperative course was characterized by persisting congestion of the right lung. Two months later, right pulmonary vein hypoplasia was successfully enlarged with autologous pericardium.

Drenagem anômala total das veias pulmonares, em sua forma infracardíaca: desafio diagnóstico

“…Technological advances in echocardiography equipment, particularly Doppler color flow mapping, has made identification of this type of congenital heart disease both quicker and more straightforward [4][5][6][7][8] . Early recognition prevents inadequate management, which could in turn lead to severe adverse effects including death, or delayed surgical referrals, giving rise to clinical deterioration, respiratory tract infection, and severe pulmonary hypertension, which may have a major adverse effect on morbidity and outcome.…”