Open Access

ARTICLE

Pulmonary Valve Preservation without Right Ventriculotomy in Biventricular Repair of Atrioventricular Septal Defect with Tetralogy of Fallot or Double-Outlet Right Ventricle

Jae Hong Lee¹, Seung Min Baek², Hye Won Kwon³, Sungkyu Cho^3,4, Jae Gun Kwak^3,4, Woong-Han Kim^3,4,5,*
1 Department of Thoracic and Cardiovascular Surgery, Korea University Anam Hospital, Korea University College of Medicine, Seoul, 02841, Republic of Korea
2 Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, 03080, Republic of Korea
3 Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul, 03080, Republic of Korea
4 Department of Thoracic and Cardiovascular Surgery, Seoul National University College of Medicine, Seoul, 03080, Republic of Korea
5 JW Lee Center for Global Medicine, Seoul National University College of Medicine, Seoul, 03087, Republic of Korea
* Corresponding Author: Woong-Han Kim. Email:

Congenital Heart Disease https://doi.org/10.32604/chd.2025.075046

Received 23 October 2025; Accepted 30 December 2025; Published online 05 February 2026

Abstract

Background: We evaluated surgical outcomes of biventricular repair for atrioventricular septal defect (AVSD) with tetralogy of Fallot (TOF) or double-outlet right ventricle (DORV). Methods: This retrospective pilot study included 12 patients who underwent biventricular repair of AVSD with TOF (n = 6) or DORV (n = 6) between 2004 and 2023. Right ventricular outflow tract (RVOT) reconstruction was performed using transannular patch (TAP, n = 4) or pulmonary valve preservation (PVP, n = 8). Clinical outcomes, including longitudinal pulmonary valve growth, RVOT obstruction, and pulmonary regurgitation, were reviewed descriptively, with particular focus on the feasibility of PVP. Results: The median age and body weight at the time of surgery were 11.7 (8.3–18.8) months and 8.6 (7.3–10.5) kg, respectively. The median follow-up duration was 67.9 (58.7–174.3) months. The two-patch technique (n = 10) was most commonly used for AVSD repair. There were no early mortalities and one late mortality. At discharge, significant (≥36 mmHg) RVOT obstruction was observed in two patients who underwent PVP. During follow-up, one patient required reoperation for significant (moderate or greater) atrioventricular valve regurgitation, and two patients in the PVP group underwent transcatheter intervention for significant RVOT obstruction. At the last follow-up, significant RVOT obstruction was present in two patients. Significant (moderate or greater) pulmonary regurgitation occurred in three patients in the TAP group. In patients who underwent PVP, the pulmonary valve annulus z-score remained within acceptable ranges, and the RVOT pressure gradient predominantly decreased to acceptable levels during follow-up. Conclusions: Mid-term outcomes of PVP in the biventricular repair of AVSD with TOF or DORV are acceptable. In selected patients, the PVP strategy may be considered a reasonable and feasible option for RVOT reconstruction.

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Keywords

Endocardial cushion defects; tetralogy of Fallot; double outlet right ventricle; pulmonary stenosis; pulmonary valve

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