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Cone Procedure to Treat Ebstein's Anomaly

Daniel A. Velez

Advancing modern medicine can improve long-term outcomes for pediatric congenital heart defects. Although relatively rare, Ebstein's anomaly (EA) is one type of cardiac congenital malformation that can have many different anatomical and physiological presentations, which can range in severity from mild to severe. The anomaly directly affects the functionality of the tricuspid valve because it is displaced deeper into the right ventricle, the leaflets are malformed and can be larger than normal, and they may be abnormally attached to the walls of the ventricle. Therefore, blood can regurgitate back into the right atrium, which can lead to myocardial hypertrophy and fluid congestion. For children diagnosed with EA, it is also common for them to experience cardiac abnormalities, including septal defects or pulmonary valve stenosis. Without prompt treatment, life­ threatening complications can occur, including arrhythmias, cyanosis, and oxygenation issues, reduced physical activity, fluid accumulation, slower growth, and even heart failure.

Graph 1

Traditional management of Ebstein's anomaly has involved tricuspid valve replacement. While this surgery can alleviate symptoms, long-term outcomes have been poor because the patient either outgrows the valve or it degenerates, thereby prompting additional medical or surgical interventions.

An alternative option was developed and first implemented in 1993 by Dr. Jose Pedro da Silva, a pediatric cardiovascular surgeon. His groundbreaking surgical procedure involved using the patient's own tissue to reconstruct the tricuspid valve through mobilization and rotation of the leaflets into a cone shape. Cone reconstruction is possible at any age; however, when performed in children, the new valve continues to grow with the child. Since its initial unveiling, results of this autologous procedure have continued to show incredible promise without the possibility of rejection. Specialists and surgeons have continued to analyze and refine the procedure, which has become the gold standard to treat Ebstein's anomaly.

The Adult Congenital Heart Disease Program at Phoenix Children's recently added cone reconstruction surgery of the tricuspid valve as an alternative treatment for patients with congenital heart defects. It is the first full-range program of its kind in the state of Arizona to offer medical, surgical, and interventional therapies to manage EA, and proudly stands among only a small number in the entire country. Patients with EA can choose cone reconstruction as their treatment of choice over valve replacement; however, the surgery is complex and should only be performed by cardiothoracic surgeons who have specialized training and firsthand experience. Dr. Daniel A. Velez, Chief of the Division of Cardiothoracic Surgery and Co-Director of the Phoenix Children's Heart Center, received his formal surgical training from prominent cardiovascular programs around the world, but to master cone reconstruction, Dr. Velez spent valuable time alongside Dr. da Silva at UPMC Children's Hospital of Pittsburgh so that he could bring this lifesaving service back to Phoenix Children's. To date, Dr.

Velez and his team have completed ten cone repairs since 2017 on patients ranging from neonates to adults.

Graph 2

A retrospective study published in May 2020 in The Journal of Thoracic and Cardiovascular Surgery compared outcomes from 151 patients with EA. Results indicated that cone reconstruction had a higher success rate and lower incidence of tricuspid regurgitation when compared to valve replacement or other surgical interventions.

According to Dr. Velez, "We've reached the point where we can say with confidence that the cone reconstruction procedure is a safe and highly effective treatment for children with Ebstein's anomaly... Evidence has confirmed we can expect consistent results and long-term durability across all variations in tricuspid valve migration and dilation.”

It is important to note that patients with EA will require lifelong monitoring and cardiovascular therapy to manage any and all lasting complications.

Fortunately for the patients who live in Arizona, Phoenix Children's Heart Center is poised to provide the comprehensive care that is necessary across the lifespan through its Adult Congenital Heart Disease program. Congenital heart patients are supported and guided as they transition from pediatric to adult care.

The team at Phoenix Children's consults with providers in the Southwestern United States to share their experience and expertise. Dr. Velez believes that "by working together, we can help ensure every child has access to the life­ changing or lifesaving cardiovascular care they need."