Surgical Management of Congenital Non-Ebstein Tricuspid Dysplasia

Introduction

Non-Ebstein tricuspid dysplasia (NTD) is a rare congenital malformation of the tricuspid valve, characterized by the absence of delamination defects and normal leaflet insertion without apical displacement. The valvular lesions present as thickening and restriction, leading to significant tricuspid regurgitation (TR). The anatomical and morphological variability of this condition makes surgical management complex, and there are no established surgical guidelines.

Method

We conducted a retrospective, single-center study from November 2010 to March 2023, assessing the surgical management of NTD in pediatric and adult patients with short-term follow-up. Patients with single-ventricle heart disease, Ebstein anomaly, or any congenital heart defect involving the tricuspid valve were excluded. The surgical techniques employed (valve reconstruction, annuloplasty) and their post-operative outcomes were analyzed (TR, right ventricular (RV) function, post-operative complications, and short- to medium-term survival).

Results

Sixteen patients (six children and ten adults) were included in this analysis, most of whom were symptomatic at the time of surgery. The mean age at surgery was 28 years. Tricuspid valve reconstruction combined with annuloplasty was performed in 80% of adults but only in two children. Patients who underwent valve reconstruction with annuloplasty were more likely to have TR grades I and II at discharge (p = 0.026). At discharge, 70% of patients who underwent valve reconstruction and annuloplasty had only mild TR. At one-year follow-up, TR grade progression was not significantly different between surgical techniques. However, in the annuloplasty group, TR tended to progress to grade III in 37.5% of cases. In contrast, 80% of post-operative grade II TR cases remained stable in the reconstruction-only group. No deaths were reported, and the rate of major complications was low (5%, mainly transient arrhythmias).

Conclusion

Our study evaluates the surgical management of biventricular physiology NTD in 16 adult and pediatric patients at a specialized center. This congenital heart defect is often misdiagnosed as Ebstein anomaly and lacks standardized diagnostic criteria. Our findings suggest that NTD surgery provides encouraging short-term outcomes, with excellent survival rates and limited complications, including in pediatric patients. Early surgical intervention, before RV dilation or dysfunction develops, is crucial to preserving right ventricular function and achieving optimal TR outcomes. Valve reconstruction combined with annuloplasty seems to effectively reduce TR in the immediate post-operative period.