Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infants

V Reed LaSala; Edward Buratto; Halil Beqaj; Isabel Aguirre; Julian Maldonado; Nimrod Goldshtrom; Andrew Goldstone; Matan Setton; Ganga Krishnamurthy; Emile Bacha; David M Kalfa

doi:10.1016/j.xjon.2023.08.007

Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infants

JTCVS Open. 2023 Aug 22:16:629-638. doi: 10.1016/j.xjon.2023.08.007. eCollection 2023 Dec.

Affiliations

¹ 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.
² College of Physicians and Surgeons, Columbia University, New York, NY.
³ Division of Neonatology, Department of Pediatrics, New York-Presbyterian Morgan Stanley Children's Hospital, Columbia University Medical Center, New York, NY.
⁴ Division of Cardiology, Department of Pediatrics, New York-Presbyterian Morgan Stanley Children's Hospital, Columbia University Medical Center, New York, NY.

Abstract

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.

Keywords: Ebstein anomaly; Starnes; cone; congenital heart disease; single ventricle; tricuspid valve; tricuspid valve dysplasia.