Introduction:
Total anomalous pulmonary venous connection (TAPVC) is one of the rarest
congenital cardiac diseases with 7 per 100000 neonates’ approximate
incidence (1,2). It accounts for about 1% of all congenital heart
diseases (3). In this anomaly, all the pulmonary veins drain into right
atrium (or one of its tributaries) instead of the normal drainage into
left atrium (4). Anatomically, TAPVC can be divided into 4 major types
depending on the level of pulmonary veins’ drainage: supracardiac (into
the innominate vein or right superior vena cava), cardiac (into the
coronary sinus or directly to the right atrium), infracardiac (most
commonly into the portal vein or into the ductus venosus, hepatic vein,
or directly into the infradiaphragmatic inferior vena cava), and mixed
(in which there is a combination between types above) (1-4). The
separation of both pulmonary and systemic circulations in TAPVC makes
the presence of right-to-left shunt obligatory for survival, and any
restriction in this communication represents an ”obstruction”. The
obstruction may be at any level of the drainage, and is encountered in
about 25% to 50% of TAPVC patients. The degree and level of
obstruction are remarkable factors affecting the pathophysiology and
presentation (5, 6). In the presence of obstruction, the patient is
usually symptomatic in the neonatal period, symptoms progressively
worsen, and the neonate may present with profound acidosis and hypoxemia
despite the aggressive management and oxygen supplement (4). Herein, we
report a rare case of an obstructed mixed-type TAPVC in a neonate in
association with large VSD in whom successful surgical repair was
performed.