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Contemporary Outcomes And Management Of Atrioventricular Septal Defect-Tetralogy Of Fallot.
Connor P. Callahan, MD1, David J. Barron, MD2, Brian W. McCrindle, MD, MPH2, Anusha Jegatheeswaran, MD, PhD2, Osami Honjo, MD, PhD2, Anastasios C. Polimenakos, MD, MBA3, Joseph W. Turek, MD, PhD4, Robert J. Dabal, MD5, James K. Kirklin, MD5, Madison B. Argo, MD6, William M. DeCampli, MD, PhD7, Pirooz Eghtesady, MD, PhD8, David M. Overman, MD9.
1CHSS Data Center, Toronto, ON, Canada, 2The Hospital for Sick Children, Toronto, ON, Canada, 3The Methodist Children's Heart Institute, San Antonio, TX, USA, 4Duke Children's Medical Center, Durham, NC, USA, 5The University of Alabama at Birmingham, Birmingham, AL, USA, 6University of Wisconsin-Madison, Madison, WI, USA, 7Arnold Palmer Hospital for Children, Orlando, FL, USA, 8St. Louis Children's Hospital, St. Louis, MO, USA, 9Children's Minnesota, Minneapolis, MN, USA.

Objective: We sought to describe the management and outcomes of atrioventricular septal defect-tetralogy of Fallot (AVSD-TOF) in a contemporary multicenter cohort. Methods: Of 739 patients in the Congenital Heart Surgeonsí Society AVSD cohort (Jan 2012-May 2021), 40 were identified with AVSD-TOF. Propensity scores were used to match AVSD-TOF to AVSD-nonTOF patients for survival comparison. Survival and freedom from reoperation were also compared between staged (n=16) and primary repair (n=24) AVSD-TOF patients. Results: Staged patients all survived to complete repair (12 BT shunts, 3 RVOT stents, and 1 ductal stent). Staged patients had smaller pulmonary valve annulus on baseline echocardiogram (4.6 vs. 6.3 mm, p=0.006). For all AVSD-TOF, 5-year survival was 81%. Staged patients had worse survival (Figure-A). Five-year survival was similar between AVSD-TOF and matched AVSD-nonTOF patients (81%, p=0.9). The staged group had reduced freedom from reoperation post-complete repair (Figure-B). From baseline to pre-discharge post-repair echocardiograms, primary repair patients had greater increases in severity of left and right atrioventricular valve (AV) regurgitation (left mean +0.4 grade, p=0.02, right mean +0.5, p=0.02). Conclusions: There was no survival disadvantage after AVSD-TOF repair compared to matched AVSD-nonTOF patients. Following complete repair, AVSD-TOF staged patients carry higher risk for reoperation, whereas primary repair was associated with poorer AV valve performance. Continued enrollment in our cohort is needed to understand who may benefit from early primary vs. staged repair.


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