Cone Repair in Adult Patients with Ebstein Anomaly

Abstract

Ebstein anomaly is a rare congenital heart malformation typically involving the tricuspid valve and the right ventricle that has a wide range of anatomical and pathophysiological presentations. Various surgical repair techniques for Ebstein anomaly have been reported because of its near-infinite anatomical variability. Cone repair for Ebstein anomaly can achieve nearly anatomical reconstruction of the tricuspid valve with promising outcomes. In this article, the surgical techniques for cone repair in adult patients with Ebstein anomaly are described in detail, and clinical experiences and technically challenging cases are presented.

New trend: Surgical correction of Ebstein's anomaly using modified cone repair and a combination of different techniques

“…The results of this study indicate that tricuspid valve delamination, surgical valve rotation, right ventricular oblique plication, and tricuspid ring annuloplasty, when used to modify cone‐type repairs, have beneficial effects on preserving valve coaptation and functions in the early and midterm, as well as on right‐ventricle remodeling 7,13,15 …”

“…These repair techniques can provide excellent short‐ and medium‐term results, but even some regurgitation and a slight change in tricuspid coaptation and annulus geometry can cause greater valve regurgitation than we anticipated, leading to other changes that eventually result in severe regurgitation. However, in cone repair, the cone‐shaped valve opens to the central blood flow and closes with the complete union of the leaflets, resulting in central diastolic blood flow 9,15 . We believe that tricuspid rings will prevent the progression of tricuspid regurgitation by restricting the annulus and remodeling in cases of mild leakage.…”

“…However, in cone repair, the cone-shaped valve opens to the central blood flow and closes with the complete union of the leaflets, resulting in central diastolic blood flow. 9,15 We believe that tricuspid rings will prevent the progression of tricuspid regurgitation by restricting the annulus and remodeling in cases of mild leakage.…”

“…If surgical modifications are chosen appropriately, they are tolerated well by the patient and contribute positively to the outcome. In the presence of severe right ventricle dysfunction, uncontrollable arrhythmias, and cyanosis, the choice of 1.5 ventricles is preferred.The results of this study indicate that tricuspid valve delamination, surgical valve rotation, right ventricular oblique plication, and tricuspid ring annuloplasty, when used to modify cone-type repairs, have beneficial effects on preserving valve coaptation and functions in the early and midterm, as well as on right-ventricle remodeling 7,13,15.…”

Cone Reconstruction for Ebstein’s Anomaly: Is It Durable in Pediatric Patients?

“…Compared to various other procedures, favorable outcomes of cone reconstruction have been reported in adult patients. 2) Cone reconstructions are also applied for children and even for neonates and the outcomes are favorable. 3) 4) However, tricuspid reoperation is more frequent in young children.…”