Surgical repair of transposition of great arteries and total anomalous pulmonary venous return to the coronary sinus (TGA with TAPVR)

Abstract

The association of transposition of the great arteries (TGA) and total anomalous pulmonary venous return (TAPVR) is very rare; we report one case of this malformation with an intracardiac type of TAPVR draining into the coronary sinus. The surgical repair was performed directing the coronary sinus blood flow to the tricuspid valve. The proximity of the anatomical structures allowed an intra-atrial patch repair similar to a modified Mustard procedure. In the postoperative period the child developed low cardiac output for 2 days, requiring inotropic support and mechanical ventilation. Her recovery was otherwise uneventful. The postoperative echocardiogram showed an intact repair with perfect flow through the patch from the pulmonary veins to the right ventricle. Almost 2 years after surgery, the patient underwent cardiac catheterization that showed integrity of the surgical repair with normal pressures in all heart chambers. To our knowledge this is the first report in the medical literature of surgical treatment of this association.