Da Silva Cone Operation After Starnes Procedure for Ebstein's Anomaly

Severe neonatal Ebstein's Anomaly can present a surgical challenge, and the advent of the Starnes procedure has improved survival in these critically ill neonates. Importantly, however; this does not commit them to a single ventricle physiology for their entire lives, and they can be successfully converted to 1.5 or 2 ventricle physiology later in life.

Here we present a 2.5-year-old who initially underwent a Starnes procedure at day 3 of life for severe neonatal Ebstein's Anomaly with pulmonary atresia. Post-operatively the patient required support on venoarterial ECMO but was eventually weaned off. At age 4 months she underwent a bidirectional Glenn with takedown of her Blalock-Taussig-Thomas shunt.

For the next several months she struggled with pulmonary hypertension and pulmonary edema, but was eventually weaned off of oxygen with initiation of diuretics and sildenafil. Over the next two years she slowly gained weight and improved clinically. After undergoing a cardiac MRI, cardiac catheterization, and transthoracic echocardiogram, she was presented in a joint surgery and cardiology conference and the decision was made to pursue 1.5 ventricle palliation.

The procedure was performed through a midline sternotomy with bicaval cannulation and moderate hypothermia. The Starnes patch was taken down and the da Silva Cone tricuspid valve repair was performed. She underwent a radio frequency ablation of the cavo-tricuspid isthmus for a history of atrial arrhythmias. She had a prior atrial septectomy as a part of her Starnes procedure and a fenestrated ASD patch repair was performed. Finally, a pulmonary artery, pulmonic valve, and right ventricular outflow tract augmentation was performed with a monocusp valve fashioned from cardio-cell.

References

1. Silva J.P. da Silva L.F. Ebstein's anomaly of the tricuspid valve: the cone repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2012; 15: 38-45
2. Silva J.P., Viegas M., Castro-Medina M., Da Fonseca Da Silva L. The Da Silva cone operation after the Starnes procedure for Ebstein's anomaly: New surgical strategy and initial results. JTCVS Techniques. 2020. 3:281-283
3. Starnes V.A., Pitlick P.T., Bernstein D., Griffin M.L., Choy M., Shumway N.E. Ebstein's anomaly appearing in the neonate: a new surgical approach. J Thorac Cardiovasc Surg. 1991; 101: 1082-1087

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