Biventricular remodeling and function after Cone repair of Ebstein Anomaly: A review

Abstract

The Cone reconstruction is the prevailing repair strategy for Ebstein anomaly (EA), providing near-anatomical restoration of the tricuspid valve (TV) and reliable reduction of tricuspid regurgitation (TR). Most of the risk of Cone repair is seen in the early postoperative period from dehiscence, with very low rates of mortality and reintervention thereafter. By correcting TV dysfunction and abnormal right ventricle (RV) geometry, ventricular reverse remodeling has been demonstrated through decreased right atrial and atrialized RV size, along with improved antegrade pulmonary flow and consequent left ventricular filling. Early postoperative RV dysfunction is common due to increased afterload upon addressing TR, with suggestion of delayed recovery. Cone repair augments left ventricular preload and preserves left ventricular function. The contrasting effects of ventricular plication and superior repositioning of TV during the Cone repair has led to discrepant reports in the trajectory of RV remodeling, which is further confounded by the lack of standardization in imaging measurements and timing of surveillance. Consistency in imaging protocol and lengthening follow-up will hopefully provide a more robust understanding about the evolution of the postoperative RV. Improvement in functional status and aerobic capacity can also be appreciated post-Cone repair, particularly in highly symptomatic patients. Innovation to promote remodeling of the Ebsteinoid heart include stem cell therapy at the time of surgical repair, which has shown promise in phase I clinical trial. Lastly, the goal of biventricular repair through Cone reconstruction can be facilitated by Starnes palliation to stabilize critical presentations, a paradigm shift that is increasingly adopted.