Outcomes after Cone Repair for Ebstein Anomaly: CT Volumetric and Functional Outcomes
Presented During:
Saturday, May 3, 2025: 4:30PM - 4:45PM
Seattle Convention Center | Summit
Posted Room Name:
Ballroom 2, Level 5
Abstract No:
115
Submission Type:
Abstract Submission
Authors:
Dong Hee Jang (1), Chun Soo Park (1), Tae-Jin Yun (1), Bo Sang Kwon (1), Eun Seok Choi (1), Dong-Hee Kim (1)
Institutions:
(1) Asan Medical Center, Seoul, Korea
Submitting Author:
Dong Hee Jang
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Asan Medical Center
Asan Medical Center
Co-Author(s):
Chun Soo Park
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Asan Medical Center
Asan Medical Center
Tae-Jin Yun
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Asan Medical Center
Asan Medical Center
Bo Sang Kwon
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Asan Medical Center
Asan Medical Center
Eun Seok Choi
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Asan Medical Center
Asan Medical Center
Dong-hee Kim
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Asan Medical Center
Asan Medical Center
Presenting Author:
Dong Hee Jang
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Asan Medical Center
Asan Medical Center
Abstract:
Objective: This study aimed to investigate the short- and mid-term clinical outcomes of cone repair (CR) in patients with Ebstein's anomaly (EA). In addition, we evaluated the ventricular volumetric and functional outcomes after CR using cardiac CT.
Methods: From 2017 to 2023, 28 consecutive patients with EA who underwent CR performed by a single surgeon were included. The outcomes of interest included development of significant tricuspid valve (TV) regurgitation, reintervention or reoperation for TV dysfunction, and changes in conventional echocardiographic and cardiac CT volumetric measures. CT-derived cardiac strains were measured using multiphase images by the commercially available software to assess the postoperative function of the atrialized right ventricle (RV).
Results: The median age was 14.5 years (IQR: 3.75-51 years). The median follow-up duration was 34 months (IQR: 21-58 months). Atrialized RV plication was not performed during CR in all but one patient. There were no early or late deaths. One patient required reoperation for TV dehiscence 7 months after CR, and the freedom from TV reoperation at 3 years was 90.8%. At the latest echocardiography, the degree of tricuspid regurgitation was mild or less in 26 patients (92.9%). While tricuspid annular plane systolic excursion (TAPSE) significantly decreased immediately postoperatively (p<0.001), it improved over time (p<0.001) at a median interval of 35 months (IQR: 23-57 months) post-operation. In 23 patients (82.1%), CT volumetry was performed both preoperatively and postoperatively (median interval: 31 months, IQR: 34-55 months). The indexed systolic and diastolic RV volumes and stroke volume (SV) significantly decreased after CR (p<0.001), whereas indexed systolic and diastolic left ventricular (LV) volumes and LV SV significantly increased (p<0.001). Multiphasic images were obtained in 10 patients, none of whom underwent atrialized RV plication. The average strain of the atrialized RV was -14.3 ± 4.13%, indicating a reasonably well-functioning chamber.
Conclusions: Cone repair for EA consistently resulted in favorable clinical outcomes, characterized by extremely low mortality and reoperation rates, as well as significantly improved tricuspid valve function. Although RV function decreased immediately following CR, it recovered over time. Post-CR, RV volume decreased while LV volume and SV increased. The atrialized RV was functioning after CR, contributing to the total RV function.
Methods: From 2017 to 2023, 28 consecutive patients with EA who underwent CR performed by a single surgeon were included. The outcomes of interest included development of significant tricuspid valve (TV) regurgitation, reintervention or reoperation for TV dysfunction, and changes in conventional echocardiographic and cardiac CT volumetric measures. CT-derived cardiac strains were measured using multiphase images by the commercially available software to assess the postoperative function of the atrialized right ventricle (RV).
Results: The median age was 14.5 years (IQR: 3.75-51 years). The median follow-up duration was 34 months (IQR: 21-58 months). Atrialized RV plication was not performed during CR in all but one patient. There were no early or late deaths. One patient required reoperation for TV dehiscence 7 months after CR, and the freedom from TV reoperation at 3 years was 90.8%. At the latest echocardiography, the degree of tricuspid regurgitation was mild or less in 26 patients (92.9%). While tricuspid annular plane systolic excursion (TAPSE) significantly decreased immediately postoperatively (p<0.001), it improved over time (p<0.001) at a median interval of 35 months (IQR: 23-57 months) post-operation. In 23 patients (82.1%), CT volumetry was performed both preoperatively and postoperatively (median interval: 31 months, IQR: 34-55 months). The indexed systolic and diastolic RV volumes and stroke volume (SV) significantly decreased after CR (p<0.001), whereas indexed systolic and diastolic left ventricular (LV) volumes and LV SV significantly increased (p<0.001). Multiphasic images were obtained in 10 patients, none of whom underwent atrialized RV plication. The average strain of the atrialized RV was -14.3 ± 4.13%, indicating a reasonably well-functioning chamber.
Conclusions: Cone repair for EA consistently resulted in favorable clinical outcomes, characterized by extremely low mortality and reoperation rates, as well as significantly improved tricuspid valve function. Although RV function decreased immediately following CR, it recovered over time. Post-CR, RV volume decreased while LV volume and SV increased. The atrialized RV was functioning after CR, contributing to the total RV function.
CONGENTIAL:
Atrioventricular valve (mitral/tricuspid) surgery
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