Supracardiac total anomalous pulmonary venous drainage is commonly associated with a left-sided ascending vein draining into innominate vein. We present a case of a newborn with a right-sided ascending vein, draining into the right superior caval vein with stenosis at the SVC-ascending vein junction, posing a surgical dilemma in corrective surgery. Usage of three-dimensional computed tomographic scan was essential in delineating the anatomy and aiding surgery. The case demonstrates the rarity of this type of cardiac disease and the complications that develop.
From April 2002 to March 2005, 18 patients having undergone bidirectional Glenn shunt (BDG) without cardiopulmonary bypass (CPB) [off-pump BDG] were retrospectively reviewed. During BDG anastomosis, a temporary bypass was established between superior vena cava (15) or innominate vein (3) and main pulmonary artery (16) or right atrium (2). Hemodynamics and oxygenation were maintained well throughout the temporary bypass time. There was no emergent use of CPB. Mean transpulmonary pressure gradient immediately after and 24 hours after the BDG were 6.7 and 5.6 mmHg, respectively. Echocardiography showed mild flow turbulence at the anastomosis in 1 case. This simple and inexpensive technique provided good surgical view with stable hemodynamics enabling satisfactory BDG in selected cases. Furthermore, it could avoid adverse effects of CPB such as lung injury and possible blood transfusion. This experience would encourage off-pump BDG combined with more challenging procedures.