Atrioventricular septal defect and aortic arch obstruction: A challenging combination.
5 affiliations
- 1Division of Congenital Heart Surgery, Texas Children's Hospital, Houston, Tex.
- 2Department of Surgery, Baylor College of Medicinem, Houston, Tex.
- 3NewYork-Presbyterian Weill Cornell Medical Center, New York, NY.
- 4Division of Cardiology, Texas Children's Hospital, Houston, Tex.
- 5Department of Pediatrics, Baylor College of Medicine, Houston, Tex.
Abstract
Objective
The combination of atrioventricular septal defect and aortic arch obstruction (AVSD-AAO) is rare and associated with significant morbidity and mortality. We characterized our institutional experience to identify variables influencing the surgical strategy and outcomes in this heterogeneous patient population.Methods
A retrospective review was performed on all patients undergoing biventricular AVSD repair at Texas Children's Hospital between January 1995 and August 2024. Patients with AVSD-AAO were identified and stratified by age at first intervention (neonates vs infants) and surgical strategy (staged vs concurrent repair). Kaplan-Meier survival estimates, competing risks analysis, and Wilcoxon rank-sum tests were employed in statistical analysis.Results
Of 790 AVSD patients, 33 (4.2%) had concurrent AAO. Compared to AVSD without AAO, AVSD-AAO patients had significantly higher cumulative incidence of a left atrioventricular valve reoperation (32.2% vs 14.4%, P = .0006), left ventricular outflow flow tract obstruction repair (18% vs 4.7%, P = .0015), and the mortality (15-year survival, 77.6% vs 94.2%, P < .0001). Staged repair with an initial arch repair followed by AVSD repair trended toward improved survival compared to concurrent repair (87.8% vs 67.0%, P = .13). Survival was not different between patients who underwent an initial intervention as neonates versus infants (81.9% vs 73.3%, P = .50).Conclusions
AVSD-AAO carries substantially higher rates of reoperation and mortality compared with AVSD alone. AAO is the primary driver of surgical timing due to the frequent duct-dependent systemic blood flow; however, neonatal AVSD repair is challenging. Temporizing surgical interventions that optimize systemic and pulmonary blood flow may allow for somatic growth, the valve maturation, and the normalization of pulmonary vascular resistance prior to definitive repair.
Related
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BibTeX
@article{villar2026atrioventricular,
title = {{Atrioventricular septal defect and aortic arch obstruction: A challenging combination.}},
author = {Santiago Villar and Alisa Lu and Thao T Wu and Tam T Doan and Temitope Olarinde and Junsang Cho and Sarah K Clunie and Michiaki Imamura and Jeffrey S Heinle and Yishay Orr},
journal = {JTCVS open},
year = {2026},
month = aug,
doi = {10.1016/j.xjon.2026.102082},
url = {https://europepmc.org/article/MED/42948136},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Atrioventricular septal defect and aortic arch obstruction: A challenging combination.
AU - Santiago Villar
AU - Alisa Lu
AU - Thao T Wu
AU - Tam T Doan
AU - Temitope Olarinde
AU - Junsang Cho
AU - Sarah K Clunie
AU - Michiaki Imamura
AU - Jeffrey S Heinle
AU - Yishay Orr
PY - 2026
DA - 2026/08/26
JO - JTCVS open
DO - 10.1016/j.xjon.2026.102082
UR - https://europepmc.org/article/MED/42948136
AB - Objective The combination of atrioventricular septal defect and aortic arch obstruction (AVSD-AAO) is rare and associated with significant morbidity and mortality. We characterized our institutional experience to identify variables influencing the surgical strategy and outcomes in this heterogeneous patient population.Methods A retrospective review was performed on all patients undergoing biventricular AVSD repair at Texas Children's Hospital between January 1995 and August 2024. Patients with AVSD-AAO were identified and stratified by age at first intervention (neonates vs infants) and surgical strategy (staged vs concurrent repair). Kaplan-Meier survival estimates, competing risks analysis, and Wilcoxon rank-sum tests were employed in statistical analysis.Results Of 790 AVSD patients, 33 (4.2%) had concurrent AAO. Compared to AVSD without AAO, AVSD-AAO patients had significantly higher cumulative incidence of a left atrioventricular valve reoperation (32.2% vs 14.4%, P = .0006), left ventricular outflow flow tract obstruction repair (18% vs 4.7%, P = .0015), and the mortality (15-year survival, 77.6% vs 94.2%, P < .0001). Staged repair with an initial arch repair followed by AVSD repair trended toward improved survival compared to concurrent repair (87.8% vs 67.0%, P = .13). Survival was not different between patients who underwent an initial intervention as neonates versus infants (81.9% vs 73.3%, P = .50).Conclusions AVSD-AAO carries substantially higher rates of reoperation and mortality compared with AVSD alone. AAO is the primary driver of surgical timing due to the frequent duct-dependent systemic blood flow; however, neonatal AVSD repair is challenging. Temporizing surgical interventions that optimize systemic and pulmonary blood flow may allow for somatic growth, the valve maturation, and the normalization of pulmonary vascular resistance prior to definitive repair.
SN - 2666-2736
ER -