Comparative outcomes and risk analysis after cone repair or tricuspid valve replacement for Ebstein's anomalyCentral MessagePerspective
Rebekah Boyd, MD,
David Kalfa, MD, PhD,
Stephanie Nguyen, MD,
Matan Setton, MD,
Amee Shah, MD,
John Karamichalis, MD,
Matthew Lewis, MD,
Noa Zemer Wassercug, MD,
Marlon Rosenbaum, MD,
Emile Bacha, MD
Affiliations
Rebekah Boyd, MD
Section of Pediatric and Congenital and Cardiac Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Department of Surgery, Morgan Stanley Children Hospital New York-Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
David Kalfa, MD, PhD
Section of Pediatric and Congenital and Cardiac Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Department of Surgery, Morgan Stanley Children Hospital New York-Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
Stephanie Nguyen, MD
Section of Pediatric and Congenital and Cardiac Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Department of Surgery, Morgan Stanley Children Hospital New York-Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
Matan Setton, MD
Division of Pediatric Cardiology, Morgan Stanley Children Hospital New York-Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
Amee Shah, MD
Division of Pediatric Cardiology, Morgan Stanley Children Hospital New York-Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
John Karamichalis, MD
Section of Pediatric and Congenital and Cardiac Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Department of Surgery, Morgan Stanley Children Hospital New York-Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
Matthew Lewis, MD
Division of Cardiology, New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
Noa Zemer Wassercug, MD
Division of Cardiology, New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
Marlon Rosenbaum, MD
Division of Cardiology, New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
Emile Bacha, MD
Section of Pediatric and Congenital and Cardiac Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Department of Surgery, Morgan Stanley Children Hospital New York-Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY; Address for reprints: Emile Bacha, MD, Columbia University College of Physicians and Surgeons, New York-Presbyterian Morgan Stanley Children's Hospital, 3959 Broadway, CHN-274, New York, NY 10032.
Objective: Ebstein's anomaly is a rare congenital heart malformation for which surgical and medical management are still controversial. The cone repair has transformed surgical outcomes in many of these patients. We aimed to present our results on the outcomes of patients with Ebstein's anomaly who underwent a cone repair or tricuspid valve replacement. Methods: A total of 85 patients who underwent a cone repair (mean age, 16.5 years) or tricuspid valve replacement (mean age, 40.8 years) between 2006 and 2021 were included. Univariate, multivariate, and Kaplan–Meier analyses were used to evaluate operative and long-term outcomes. Results: Residual/recurrent greater than mild-to-moderate tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement (36% vs 5%; P = .010). However, at last follow-up, the risk of greater than mild-to-moderate tricuspid regurgitation was not different between groups (35% in the cone group vs 37% in the tricuspid valve replacement group; P = .786). The tricuspid valve replacement group had a higher risk of tricuspid valve reoperation (37% vs 9%; P = .005) and tricuspid stenosis (21% vs 0%; P = .002) compared with the cone repair group. Kaplan–Meier freedom from reintervention was 97%, 91%, and 91% at 2, 4, and 6 years after cone repair, respectively, and 84%, 74%, and 68% at 2, 4, and 6 years after tricuspid valve replacement, respectively (P = .0191). At last follow-up, right ventricular function was significantly worse from baseline in the tricuspid valve replacement group (P = .0294). There were no statistical differences between age-stratified cohorts or surgeon volume in the cone repair group. Conclusions: The cone procedure offers excellent results, with stable tricuspid valve function and low reintervention and death rates at last follow-up. The rate of greater than mild-to-moderate residual tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement, but this did not expose the patient to a higher risk of reoperation or death at last follow-up. Tricuspid valve replacement was associated with a significantly higher risk of tricuspid valve reoperation and tricuspid valve stenosis, and worse right ventricular function at last follow-up.
