Earlier Repair of the Ebstein’s Anomaly Results in Better Long-term Right Ventricular Function
Presented During:
Saturday, May 3, 2025: 4:15PM - 4:30PM
Seattle Convention Center | Summit
Posted Room Name:
Ballroom 2, Level 5
Abstract No:
114
Submission Type:
Abstract Submission
Authors:
Federica Caldaroni (1), Edward Buratto (1), Diana Zannino (2), Gavin Wheaton (3), Terry Robertson (4), Patrick Disney (5), Bryn O. Jones (6), Igor Konstantinov (7), Stephanie Perrier (8), Michael M. H. Cheung (9), Christian Brizard (8)
Institutions:
(1) Royal Children's Hospital Melbourne, Parkville, Victoria, (2) Murdoch Children's Research Institute, University of Melbourne, Melbourne, VIC, (3) Women and children's hospital, Adelaide, NA, (4) Department of Cardiology, Women’s and Children’s Hospital, Adelaide, NA, (5) Royal Adelaide Hospital, Women's and Children's Hospital, Adelaide, NA, (6) Royal Children's Hospital, Murdoch Children's Research Institute, University of Melbourne, Melbourne, VIC, (7) Royal Children's Hospital, Melbourne, Victoria, (8) Royal Children's Hospital, Parkville, Victoria, (9) The Royal Children's Hospital Melbourne, Parkville, NA
Submitting Author:
Federica Caldaroni
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Royal Children's Hospital Melbourne
Royal Children's Hospital Melbourne
Co-Author(s):
Edward Buratto
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Royal Children's Hospital Melbourne
Royal Children's Hospital Melbourne
Diana Zannino
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Murdoch Children's Research Institute, University of Melbourne
Murdoch Children's Research Institute, University of Melbourne
Gavin Wheaton
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Women and children's hospital
Women and children's hospital
Terry Robertson
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Department of Cardiology, Women’s and Children’s Hospital
Department of Cardiology, Women’s and Children’s Hospital
Patrick Disney
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Royal Adelaide Hospital, Women's and Children's Hospital
Royal Adelaide Hospital, Women's and Children's Hospital
Bryn O. Jones
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Royal Children's Hospital, Murdoch Children's Research Institute, University of Melbourne
Royal Children's Hospital, Murdoch Children's Research Institute, University of Melbourne
*Igor Konstantinov
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Royal Children's Hospital
Royal Children's Hospital
Stephanie Perrier
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Royal Children's Hospital
Royal Children's Hospital
Michael M. H. Cheung
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The Royal Children's Hospital Melbourne
The Royal Children's Hospital Melbourne
*Christian Brizard
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Royal Children's Hospital
Royal Children's Hospital
Presenting Author:
Federica Caldaroni
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The Royal Melbourne Hospital
The Royal Melbourne Hospital
Abstract:
Objectives
The Cone procedure (CP) is now the procedure of choice for Ebstein's anomaly (EA). Follow-up studies of surgical treatment of EA demonstrate increased right ventricular (RV) and left ventricular stroke volume, albeit with reduction of the RV ejection fraction in comparison to pre-op assessment. The optimum timing of surgical intervention is unclear.
We advocate for surgery at an earlier age to preserve RV function. This study aims to evaluate the impact of age at surgery on RV functional recovery.
Methods
Patients with EA undergoing surgery were identified in our database. Retrospectively, we measured the postoperative RV fractional area change (FAC) on echo after discharge and at the latest follow up and calculated the delta (∆).
Results
Fifty-five patients underwent operation between 1996-2024 at a median age of 8 years (interquartile range (IQR) 2.6 to 14 y). Preoperative tricuspid regurgitation (TR) was severe in 43 and moderate in 12. EA was an isolated lesion in 49. 22 patients were asymptomatic and 25 were cyanosed at rest or during exercise. Transition from the Carpentier procedure to the CP occurred in 2009. Associated surgical resection of the atrialized portion as opposed to plication was introduced in 2006 and performed in 20 patients. Five patients required a cavopulmonary shunt and 6 underwent a repeat TV repair. Median follow-up was 7.5 years (IQR 3.6 to 13 years). There was no early death, 2 late deaths (patients with complex associated lesions), one proceeded to Fontan completion (Carpentier type D), one underwent heart transplant. Long-term follow up TR: absent or trivial n=10, mild n=31, moderate n=10.
Postoperative FAC improved significantly between early and late follow-up (p=0.0001) and the ∆ between assessments was inversely correlated with age at surgery (n=43) with an average decrease in ∆ FAC of 0.4% per year (95% CI -0.7 to -0.1) p=0.008. An operation at 4.5 years or younger was associated with a 5.8-fold increase in the odds of at least a 10% increase of the FAC over time compared to surgery at an older age (95% CI 1.4 to 26.8) p=0.015. We observed no significant difference between A, B, and C Carpentier classification.
Conclusion
Our data suggest that restoring normal preload and afterload of the RV at an early age in Ebstein's anomaly increases the potential for recovery of the residual RV function. Further prospective studies with iterative post operative cardiac MRI are warranted.
The Cone procedure (CP) is now the procedure of choice for Ebstein's anomaly (EA). Follow-up studies of surgical treatment of EA demonstrate increased right ventricular (RV) and left ventricular stroke volume, albeit with reduction of the RV ejection fraction in comparison to pre-op assessment. The optimum timing of surgical intervention is unclear.
We advocate for surgery at an earlier age to preserve RV function. This study aims to evaluate the impact of age at surgery on RV functional recovery.
Methods
Patients with EA undergoing surgery were identified in our database. Retrospectively, we measured the postoperative RV fractional area change (FAC) on echo after discharge and at the latest follow up and calculated the delta (∆).
Results
Fifty-five patients underwent operation between 1996-2024 at a median age of 8 years (interquartile range (IQR) 2.6 to 14 y). Preoperative tricuspid regurgitation (TR) was severe in 43 and moderate in 12. EA was an isolated lesion in 49. 22 patients were asymptomatic and 25 were cyanosed at rest or during exercise. Transition from the Carpentier procedure to the CP occurred in 2009. Associated surgical resection of the atrialized portion as opposed to plication was introduced in 2006 and performed in 20 patients. Five patients required a cavopulmonary shunt and 6 underwent a repeat TV repair. Median follow-up was 7.5 years (IQR 3.6 to 13 years). There was no early death, 2 late deaths (patients with complex associated lesions), one proceeded to Fontan completion (Carpentier type D), one underwent heart transplant. Long-term follow up TR: absent or trivial n=10, mild n=31, moderate n=10.
Postoperative FAC improved significantly between early and late follow-up (p=0.0001) and the ∆ between assessments was inversely correlated with age at surgery (n=43) with an average decrease in ∆ FAC of 0.4% per year (95% CI -0.7 to -0.1) p=0.008. An operation at 4.5 years or younger was associated with a 5.8-fold increase in the odds of at least a 10% increase of the FAC over time compared to surgery at an older age (95% CI 1.4 to 26.8) p=0.015. We observed no significant difference between A, B, and C Carpentier classification.
Conclusion
Our data suggest that restoring normal preload and afterload of the RV at an early age in Ebstein's anomaly increases the potential for recovery of the residual RV function. Further prospective studies with iterative post operative cardiac MRI are warranted.
CONGENTIAL:
Atrioventricular valve (mitral/tricuspid) surgery
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