Cone Reconstruction for Ebstein Anomaly: Effect on Tricuspid Valve Growth and Biventricular Remodelling in Children.

OBJECTIVES

This study aimed to evaluate the surgical outcomes of cone reconstruction (CR) in paediatric patients with Ebstein anomaly (EA).

METHODS

We retrospectively reviewed seventeen paediatric patients (<18 years) with EA (Carpentier types A, B, and C in 3, 12, and 2, respectively) who underwent CR without prosthetic annuloplasty materials between January 2014 and December 2024. Clinical outcomes, including tricuspid valve (TV) and biventricular function and size, were evaluated using echocardiography and cardiac magnetic resonance imaging (CMR).

RESULTS

The median age and body weight at surgery were 7.8 years and 21.2 kg, respectively. No operative mortality occurred; however, 3 patients required permanent pacemaker implantation before discharge. No late deaths, reoperations, or significant tricuspid regurgitation (TR, ≥moderate) or stenosis (mean pressure gradient ≥5 mmHg) occurred during the 8.2-year median follow-up. TR was significantly reduced postoperatively (odds ratio 1.34, 95% CI, 1.10-1.64, P = .008) and remained stable without recurrence of significant TR during follow-up. The mean TV annulus z-score decreased significantly after CR (1.81 [1.19] preoperatively versus -1.74 [0.92] at discharge, mean difference: -3.55, 95% CI, -4.30 to -2.80, P < .001) and remained within the normal range during follow-up (-1.74 [0.92] at discharge versus -1.61 [1.11] at the last follow-up, mean difference: 0.04, 95% CI, -0.02-0.10, P = .207). Postoperative CMR demonstrated significantly reduced TR fraction and right heart volumetric parameters, except functional right ventricular end-systolic volume and pulmonary forward flow volume indices. Based on CMR, left ventricular end-diastolic (change 2.40, 95% CI, 0.52-4.27, P = .014) and systolic (change 1.75, 95% CI, 0.74-2.76, P = .002) volume indices significantly increased over time.

CONCLUSIONS

Long-term outcomes after CR without prosthetic annuloplasty materials were favourable in paediatric patients with EA. CR can achieve durable reduction of TR with sustained TV growth, potentially facilitating biventricular reverse remodelling.