Ebstein anomaly (EA) is a congenital dysplasia of the tricuspid valve resulting in reduced right ventricular (RV) volume and tricuspid regurgitation. Severe EA in the neonatal period is associated with high mortality. The Starnes procedure (fenestrated RV exclusion) is reserved for EA patients with cardiogenic shock and has previously committed patients to single ventricle (SV) palliation. In this report, we present the results of a strategy to redirect patients utilizing the Da Silva Cone operation to achieve a 2 or 1.5 ventricle circulation. Single-center retrospective study including all consecutive cases of Da Silva Cone operation after Starnes procedure. Between 2019 and 2023, six conversions from Starnes procedure to Cone reconstruction were performed. All were critically ill before their Starnes procedure; four on extracorporeal membrane oxygenation. Two patients were successfully rerouted to a two-ventricle repair; the remainder to 1.5 ventricle circulation. RV pressure estimates showed no correlation with success. Post-Cone intensive care and hospital stays were brief, median 5 and 6 days, respectively. All are between 2.5 and 6 years old, without indications for SV palliation. There were no deaths, with follow up ranging 1 month-4 years. No repeat interventions were performed on the tricuspid valves. One subject had a surgical pulmonary valve replacement. Tricuspid regurgitation was mild in all. The Da Silva Cone operation offers successful redirection of EA patients from a SV pathway to a 1.5 or 2 ventricle pathway after Starnes procedure. The approach is feasible and durable in midterm follow-up. The decision to initially proceed with Starnes need not be an irrevocable decision to continue down a SV palliation pathway.
Keywords: Cone procedure; Congenital heart disease; Ebstein anomaly; Starnes procedure.
© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.