114. Earlier Repair of the Ebstein's Anomaly Results in Better Long-term Right Ventricular Function
Presented During: Congenital Innovation Session: Hot Topics in Congenital Heart Surgery
Mayo Clinic - Rochester
Rochester, MN
United States - Contact Me
Elizabeth Stephens received her MD/PhD from Baylor College of Medicine and Rice Unviversity, with a PhD in Bioengineering (tissue engineering heart valves). She then did a post-doctoral fellowship with Dr. Craig Miller at Stanford University in valve-sparing aortic root replacement. She completed her cardiothoracic surgery training at Columbia University and cogenital fellowship at Northwestern's Lurie Children's Hospital. She is now an Associate Professor at Mayo Clinic as a congenital heart surgeon for pediatric and adult patients.
The Royal Melbourne Hospital
RICHMOND, Victoria
Australia - Contact Me
Pediatric and Congenital Cardiac Surgeon, Researcher
Federica is a Cardiothoracic Surgeon currently working at the Royal Melbourne Hospital and at the Royal Children's Hospital, in Melbourne. Her interests involve pediatric and adult congenital cardiac surgery, together with acquired cardiac diseases. She is currently pursuing a PhD at the University of Melbourne and her research interests include congenital valve repair, Ebstein's anomaly and Ross operation.
Saturday, May 3, 2025: 4:15 PM - 4:30 PM
Seattle Convention Center | Summit
Room: Ballroom 2, Level 5
Description
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.
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
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.
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
Presentation Duration
7 minute presentation, 8 minute discussion with an assigned commentator
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- 114 - Earlier Repair of the Ebstein’s Anomaly Results in Better Long-term Right Ventricular Function