IMAGES IN PEDIATRIC CARDIOLOGY Closure of a Superior Vena Cava Baffle Leak in a Patient with D-Transposition After Mustard Palliation: Importance of Both Angiography and Echocardiography for Confirmation of Closure Kanwal Farooqi Æ Leo Lopez Æ Nicole J. Sutton Æ Robert H. Pass Received: 4 February 2009 / Accepted: 3 August 2009 / Published online: 25 August 2009 Ó Springer Science+Business Media, LLC 2009 An 18-year-old boy with dextrotransposition of the great arteries (D-TGA) after Mustard palliation at the age of 6 months had a 2 year history of increasing cyanosis with exertion. Echocardiography identified a large leak in the superior limb of the Mustard repair. He was catheterized in an attempt at transcatheter closure of the leak. Figure 1 shows an angiogram of the superior vena cava (SVC) demonstrating a large baffle leak with significant right-to-left shunting. This defect measured approximately 18 mm on the angiogram and by transesophageal echo- cardiogram (TEE) measurements performed at the cathe- terization. The aortic saturation with room air during this procedure was 93%. Using TEE and angiographic guidance, a 34-mm-long Numed (Numed Corp., Hopkinton, NY, USA) polytetra- fluoroethylene (PTFE)-covered stent was mounted on a Numed BiB balloon catheter with a diameter of 22 mm and advanced to the level of the defect from the SVC. Angi- ography demonstrated the stent/balloon apparatus in good position, and the stent was implanted at the level of the defect. Figure 2a shows an SVC angiogram demonstrating the stent to be in good position without any further left-to- right shunting. However, Fig. 2b exhibits the simultaneous TEE image that clearly demonstrates a residual defect (asterisks). Color Doppler mapping showed right-to-left shunting now through this small residual defect that was superior to the top edge of the stent. Because of the residual defect, a second stent/balloon of the same caliber and size as the first was positioned more superiorly in the SVC, with roughly one-third of its length overlapping the first stent. The stent was implanted at this position, and Fig. 3 demonstrates the final angio- gram, which shows no left-to-right shunting at the level of the defect. Importantly, the simultaneous TEE now showed complete occlusion of the defect, with no further left-to-right or right-to-left shunting. At this writing, K. Farooqi Á L. Lopez Á N. J. Sutton Á R. H. Pass (&) Pediatric Cardiac Catheterization Laboratory, Division of Pediatric Cardiology, Department of Pediatrics, Children’s Hospital at Montefiore and Albert Einstein College of Medicine, 3415 Bainbridge Avenue, Rosenthal 1, Bronx, NY 10467-2490, USA e-mail: Pediheart@aol.com Fig. 1 Superior vena cava (SVC) angiogram demonstrating baffle leak (marked by asterisks). The SVC and inferior vena cava (IVC) limbs of the Mustard baffle are demonstrated 123 Pediatr Cardiol (2009) 30:1046–1047 DOI 10.1007/s00246-009-9517-z