• Connor P Callahan, Anusha Jegatheeswaran, David J Barron, S Adil Husain, Pirooz Eghtesady, Karl F Welke, Christopher A Caldarone, David M Overman, James K Kirklin, Marshall L Jacobs, Linda M Lambert, William M DeCampli, Brian W McCrindle, and Congenital Heart Surgeons' Society Tricuspid Atresia Working Group.
• Division of Cardiovascular Surgery, The Hospital for Sick Children, Toronto, Ontario Canada.
• J. Thorac. Cardiovasc. Surg. 2022 Feb 1; 163 (2): 399-409.e6.

ObjectiveTricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS-Fontan interstage death/transplantation versus transition to Fontan in TA.MethodsA total of 417 infants younger than 3 months of age with TA were enrolled (January 1999 to February 2020) from 40 institutions into the Congenital Heart Surgeons' Society TA cohort. Parametric competing risk methodology was used to determine factors associated with the competing end points of death/transplantation without Fontan completion, and transition to Fontan.ResultsCPS was performed in 382 patients with TA; of those, 5% died or underwent transplantation without transition to Fontan and 91% transitioned to Fontan by 5 years after CPS. Prenatal diagnosis (hazard ratio [HR], 0.74; P < .001) and pulmonary artery band (PAB) at CPS (HR, 0.50; P < .001) were negatively associated with Fontan completion. Preoperative moderate or greater mitral valve regurgitation (HR, 3.0; P < .001), concomitant mitral valve repair (HR, 11.0; P < .001), PAB at CPS (HR, 3.0; P < .001), postoperative superior vena cava interventions (HR, 9.0; P < .001), and CPS takedown (HR, 40.0; P < .001) were associated with death/transplantation.ConclusionsThe mortality rate after CPS in patients with TA is notable. Those with preoperative mitral valve regurgitation remain a high-risk group. PAB at the time of CPS being associated with both increased risk of death and decreased Fontan completion may represent a deleterious effect of antegrade pulmonary blood flow in the CPS circulation.Copyright © 2021 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

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