Modified Starnes procedure as a bridge to 2-ventricle repair in neonatal Ebstein's anomaly: A paradigm shift

Severe tricuspid valve regurgitation secondary to Ebstein's anomaly represents several challenges in neonates. It can result in significant respiratory and/or hemodynamic compromise that mandates urgent interventions. When conservative management fails, 2 surgical options are available: tricuspid valve repair or single ventricle palliation. The overall results of neonatal tricuspid valve repair are  unsatisfactory especially in sick neonates and those with preoperative hemodynamic instability. Single ventricle palliation utilizing the Starnes procedure with right ventricular exclusion provides a quicker way to improve hemodynamics and allows rapid decompression of the right ventricle but carries the long-term disadvantages of the single ventricle pathway. We were recently faced with a challenging case of neonatal Ebstein's anomaly resulting in severe tricuspid valve regurgitation (TR) and significant hemodynamic and respiratory instability. We performed an initial stage I palliation with a modified Starnes' procedure, which allowed stabilization and rapid recovery of the patient to be followed 5 months later with conversion to 2-ventricle repair using the cone technique. We believe combining these 2 strategies for suitable neonatal candidates may be a useful technique that should be considered in the algorithm for neonatal Ebstein's anomaly.