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Using DaSilva Cone Operation to Establish 1.5 or 2 Ventricle Circulation After Initial Single Ventricle Palliation
Craig P Dobson1, Adam B Christopher2, Mario Castro-Medina3
1Division of Pediatric Cardiology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA. DobsonCP@upmc.edu.
Pediatric Cardiology
|December 1, 2023
Summary
The Da Silva Cone operation can successfully reroute Ebstein anomaly patients from single ventricle palliation to a 1.5 or 2 ventricle repair after the Starnes procedure, offering a durable, mid-term solution with no deaths.
Area of Science:
- Congenital Heart Surgery
- Pediatric Cardiology
- Cardiac Surgery
Background:
- Ebstein anomaly (EA) is a congenital tricuspid valve defect causing right ventricular (RV) dysfunction and tricuspid regurgitation.
- Severe neonatal EA often necessitates the Starnes procedure, typically leading to single ventricle (SV) palliation.
- The Starnes procedure, while life-saving, has historically committed patients to a SV pathway.
Purpose of the Study:
- To evaluate the feasibility and outcomes of converting patients from a Starnes procedure to the Da Silva Cone operation.
- To assess the potential for achieving 1.5 or 2 ventricle circulation in EA patients previously treated with the Starnes procedure.
- To determine if the Da Silva Cone operation can offer an alternative to SV palliation in selected EA cases.
Main Methods:
- A single-center retrospective study of consecutive EA patients undergoing the Da Silva Cone operation after a Starnes procedure.
- Analysis of patient data from 2019 to 2023, focusing on conversions from Starnes to Cone reconstruction.
- Assessment of clinical outcomes, including circulation type achieved, intensive care and hospital stays, and long-term follow-up.
Main Results:
- Six EA patients were converted from Starnes to Da Silva Cone reconstruction.
- Two patients were successfully rerouted to a two-ventricle repair, and four to 1.5 ventricle circulation.
- Post-conversion outcomes included brief intensive care (median 5 days) and hospital stays (median 6 days), with mild tricuspid regurgitation and no deaths at 1 month to 4 years follow-up.
Conclusions:
- The Da Silva Cone operation is a feasible and effective strategy for redirecting Ebstein anomaly patients post-Starnes procedure.
- This approach allows for the achievement of 1.5 or 2 ventricle circulation, avoiding SV palliation in suitable candidates.
- The decision for initial Starnes procedure does not preclude conversion to a more favorable biventricular pathway using the Cone reconstruction.
