Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infantsCentral MessagePerspective
V. Reed LaSala, MD,
Edward Buratto, MD, PhD,
Halil Beqaj, MD,
Isabel Aguirre,
Julian Maldonado,
Nimrod Goldshtrom, MD,
Andrew Goldstone, MD, PhD,
Matan Setton, MD,
Ganga Krishnamurthy, MD,
Emile Bacha, MD,
David M. Kalfa, MD, PhD
Affiliations
V. Reed LaSala, MD
Section of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NY
Edward Buratto, MD, PhD
Section of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NY
Halil Beqaj, MD
College of Physicians and Surgeons, Columbia University, New York, NY
Isabel Aguirre
College of Physicians and Surgeons, Columbia University, New York, NY
Julian Maldonado
College of Physicians and Surgeons, Columbia University, New York, NY
Nimrod Goldshtrom, MD
Division of Neonatology, Department of Pediatrics, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NY
Andrew Goldstone, MD, PhD
Section of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NY
Matan Setton, MD
Division of Cardiology, Department of Pediatrics, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NY
Ganga Krishnamurthy, MD
Division of Neonatology, Department of Pediatrics, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NY
Emile Bacha, MD
Section of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NY
David M. Kalfa, MD, PhD
Section of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NY; Address for reprints: David M. Kalfa, MD, PhD, Section of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, Department of Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, 3959 Broadway, Babies North, Suite 274, New York, NY 10032.
Objective: To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD). Methods: Retrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution from January 2005 to February 2023. Results: Fifteen symptomatic neonates and infants who had surgery for EA or TVD were included, 8 with EA and 7 with TVD. Eleven patients (73%) and 3 patients (20%) required preoperative inotropes and extracorporeal membrane oxygenation, respectively. Nine patients (60%) had a Starnes procedure and 6 patients (40%) had tricuspid valve repair (TVr). Mortality at last follow-up was 27% overall (n = 4/15), 22% after Starnes (n = 2/9) and 33% after TVr (n = 2/6), without a significant difference despite a greater-risk profile in the Starnes group. Postoperative day 1 lactate level was associated with mortality on Cox regression (hazard ratio, 1.45; P = .01). Three of 9 patients who had a Starnes procedure were or will be converted to a cone repair (1.5/2-ventricle repair). Conclusions: Mortality after surgery for EA or TVD during the index hospitalization after birth is still significant in the current era and is associated with a greater lactate level at postoperative day 1. The Starnes procedure and TVr had comparable outcomes despite a greater-risk profile in the Starnes group. An initial single-ventricle approach does not preclude conversion to biventricular or 1.5-ventricle repair.
