UPMC Children’s Heart Institute Team Publishes Technique of Surgical Repair of Ebstein Anomaly Variant with Anterior Leaflet Displacement

A team from the Heart Institute and the Da Silva Center for Ebstein’s Anomaly at UPMC Children’s Hospital of Pittsburgh published details in JTCVS Techniques in September 2024 on a recent case of Ebstein anomaly and a variant involving anterior leaflet displacement.

The senior author of the paper was Luciana Da Fonseca Da Silva, MD. Also contributing to the case were first author Syed Faaz Ashraf, MD (CT Surgery Resident), Irsa Hasan, MD, Jose Pedro Da Silva, MD, Mario Castro Medina, MD, and Victor O. Morell, MD, all from the Division of Pediatric Cardiothoracic Surgery at UPMC Children’s.

Brief Overview

The case handled by the UPMC Children’s team involved a rare presentation of Ebstein's anomaly characterized by extreme anterior leaflet displacement of the tricuspid valve. Usually, in Ebstein anomaly, the anterior leaflet is not displaced. 

The patient, a 17-month-old female from Europe, had marked cardiomegaly, moderate tricuspid valve insufficiency, significant right atrial enlargement, and an atrial septal defect. Preoperative imaging confirmed these findings, prompting the need for a specialized surgical approach.

The surgical team used a two-layer plication technique to reposition the tricuspid valve leaflets and preserve the coronary artery branches. The procedure also involved reduction annuloplasty and primary closure of the atrial septal defect to optimize the patient’s valve function. The patient recovered well, and follow-up evaluations showed a durable repair with only mild residual valve regurgitation.

The full report is available for open access reading and includes a supplementary video showing aspects of the surgical repair.

About the Da Silva Center for Ebstein’s Anomaly at UPMC Children’s

Ebstein’s anomaly is a rare congenital heart defect, with variable clinical presentation and variable anatomical presentation. 

The cone procedure, created in 1993 by Jose Pedro Da Silva, MD, founding director of the Da Silva Center for Ebstein’s Anomaly at UPMC Children’s Hospital of Pittsburgh, has become the standard surgical repair for Ebstein’s anomaly.

Dr. da Silva and Luciana da Fonseca da Silva, MD, lead the surgical team and perform a large number of Ebstein's anomaly repair every year using the cone procedure. They are able to repair the valve in any type of anatomical presentation. Their expertise is one of the reasons that families from all over the globe seek care at the Heart Institute at UPMC Children’s for Ebstein’s anomaly.

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Reference

Ashraf SF, Hasan I, Da Silva JP, Viegas M, Morell VO, Da Fonseca Da Silva L. Ebstein Anomaly Variant with Anterior Leaflet Displacement. JTCVS Techniques. 2024; 28: P120-P123.