Changing Course-Starnes Takedown in High-Risk Children and Review of the Current Literature

Neonates with Ebstein anomaly or severe tricuspid valve dysplasia represent a high-risk patient cohort. The modified Starnes procedure was historically performed for cases incompatible with biventricular (BV) repair, leading them towards single-ventricle palliation. However, in recent years, a paradigm shift has emerged in which the Starnes procedure in the acute setting can provide a means for right ventricular (RV) rehabilitation, by providing relief of RV volume overload and time for recovery of RV function, thus serving as a bridge to subsequent one and a half ventricle or BV repair. Herein, we present two cases at our institution of successful transition to BV repair after the Starnes procedure, despite an initially failed index BV repair as a neonate. Both patients presented with severe tricuspid valve dysplasia. The first patient underwent Starnes procedure at 10 days of life and takedown at 4 years of age after bilateral cavopulmonary shunt (BCPS) failure from recurrent bilateral pulmonary artery stenoses. The 2nd patient received Starnes at 4 months of age and successfully underwent takedown at 18mo after being deemed an unsuitable candidate for BCPS. Improvement in RV function was noted throughout the Starnes course. By providing our center's experience in the context of other case reports and series of BV conversion post-Starnes procedure, summarizing clinical and surgical considerations advocated in recent guidelines, and highlighting the topical areas of management that remain to be elucidated, we hope this discussion can lead to appropriate adoption of this new treatment strategy.