Cone Repair After Starnes Procedure: The Surgical Strategy To Rehabilitate The Right Ventricle In Severely Ill Ebstein's Anomaly Patients
Luciana D. Da Fonseca Da Silva, MD, Victor Morell, Craig Dobson, Adam Christopher, Mario Castro-Medina, Melita Viegas, Jose P. Da Silva.
UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
Objective(s): We present Da Silva Cone operation's results and surgical details, achieving two-ventricle or one-and-half ventricle repair' following Starnes's procedure in severe Ebstein's anomaly.
Methods: Since January 2019, six patients underwent Cone reconstruction of the tricuspid valve with take-down of the Starnes patch. All patients were critically ill (four were on ECMO) before their initial Starnes procedure; two had the Starnes procedure at another institution. The velocity of the regurgitant jet in the Starnes patch orifice was <2m/s in four patients, and all six had severely rotated tricuspid valves, Carpentier type D in three and type C in three patients. Results: Two patients achieved two-ventricle repair, one with additional Pulmonary valvuloplasty due to pulmonary atresia. Four patients underwent 1.5 ventricle repair, with intermediate Glenn anastomosis, all requiring surgical pulmonary valvuloplasty: two with pulmonary atresia, one stenosis, and one with a previously oversewn valve (circular shunt). None presented heart block. There were no deaths, with a median follow-up of 36 months (1-47m). None required reintervention in the Tricuspid valve after the Cone repair. One patient with biventricular repair is under evaluation for pulmonary valve replacement three years after the Cone and pulmonary valvuloplasty. All patients are thriving, despite persistent moderate/severe RV dysfunction in one patient in the one and half ventricle repair group. Tricuspid regurgitation was mild in all patients at the recent echo, without stenosis. Conclusion: Right ventricle re-recruitment after the Starnes procedure, achieving one-and-half or two-ventricle physiology is feasible with the Cone repair and presents good midterm results.
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