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

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

V Reed LaSala  1 , Edward Buratto  1 , Halil Beqaj  2 , Isabel Aguirre  2 , Julian Maldonado  2 , Nimrod Goldshtrom  3 , Andrew Goldstone  1 , Matan Setton  4 , Ganga Krishnamurthy  3 , Emile Bacha  1 , David M Kalfa  1
Affiliations

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

V Reed LaSala et al. JTCVS Open. .

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.

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Conflict of interest statement

The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.

Figures

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Graphical abstract
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Kaplan–Meier plot of mortality by surgical strategy after neonatal surgery for EA and TVD.
Figure 1
Figure 1
Flow diagram of patient outcomes. The dashed box/line indicates 1 patient who initially underwent a TVr at 5 days of age but was converted to a Starnes procedure at 33 days of age during the same hospitalization due to hemodynamic instability. TVr, Tricuspid valve repair.
Figure 2
Figure 2
Kaplan–Meier plots of mortality stratified by diagnosis, type of surgery performed, and POD 1 lactate level. Log rank test used to calculate P values. TVr, Tricuspid valve repair; POD, postoperative day.
Figure 3
Figure 3
Graphical abstract illustrating the main implications of the study. EA, Ebstein anomaly; TVD, tricuspid valve dysplasia; TVr, tricuspid valve repair; POD, postoperative day; TV, tricuspid valve.
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