Factors Associated With Mortality/transplant After Cavopulmonary Shunt For Tricuspid Atresia Versus Transition To Fontan: A Congenital Heart Surgeonsí Society Analysis
Connor P. Callahan1, Anusha Jegatheeswaran2, David J. Barron2, S. Adil Husain3, Pirooz Eghtesady4, Christopher A. Caldarone5, Tara Karamlou6, Peter J. Gruber7, Karl F. Welke8, James K. Kirklin9, Marshall L. Jacobs10, David M. Overman11, Linda M. Lambert3, Christian Pizarro12, Varsha Zadokar12, Aaron M. Abarbanell4, Karthik Ramakrishnan13, William M. DeCampli14, Brian W. McCrindle2.
1CHSS Data Center - The Hospital for Sick Children, Toronto, ON, Canada, 2The Hospital for Sick Children, Toronto, ON, Canada, 3Primary Children's Medical Center, Salt Lake City, UT, USA, 4St. Louis Children's Hospital, St. Louis, MO, USA, 5Texas Children's Hospital, Houston, TX, USA, 6Cleveland Clinic, Cleveland, OH, USA, 7Yale New Haven Children's Hospital, New Haven, CT, USA, 8Levine Children's Hospital, Charlotte, NC, USA, 9University of Alabama at Birmingham, Birmingham, AL, USA, 10Johns Hopkins School of Medicine, Baltimore, MD, USA, 11The Children's Heart Clinic, Minneapolis, MN, USA, 12Alfred I. duPont Hospital for Children, Wilimington, DE, USA, 13Children's National Health System, Washington, DC, USA, 14Arnold Palmer Hospital for Children, Orlando, FL, USA.
Objectives: Tricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplant after cavopulmonary shunt (CPS) are underappreciated. We aim to describe factors associated with CPS-Fontan interstage death/transplant, versus transition to Fontan in TA.
Methods: A total of 417 infants under 3 months of age with TA were enrolled (Jan 1999-Feb 2020) from 40 institutions into the Congenital Heart Surgeonsí Society TA cohort. Parametric competing risk methodology was used to identify factors associated with the endpoints of death/transplant prior to Fontan and transition to Fontan.
Results: CPS was performed in 382 patients; of those 5% died or underwent transplant without transition to Fontan, and 91% transitioned to Fontan by five years after CPS (Figure). Prenatal diagnosis (HR: 0.75) and PA band at CPS (HR: 0.57) were associated with decreased transition to Fontan (both p<0.0001). Preoperative moderate or greater mitral regurgitation (HR: 22.5, p<0.0001) was the only factor associated with mortality/transplant. CPS flow was augmented with a systemic-pulmonary shunt in 9 patients; 5 died, and 3 transitioned to Fontan.
Conclusions: With TA, mortality after CPS is not inconsequential. Those with preoperative MR, and those who undergo postoperative systemic-pulmonary shunt represent high risk groups. The management of additional sources of pulmonary blood flow at the time of and after CPS merits further investigation.
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