What is Ebstein’s Anomaly?
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 ventricle. Therefore, blood can regurgitate back into the right atrium which can lead to myocardial hypertrophy and fluid congestion.
Ebstein's Anomaly, pre-surgery, showing severe tricuspid regurgitation
Additional abnormalities
It is also typical of children diagnosed with Ebstein’s Anomaly to experience other cardiac abnormalities including septal defects or pulmonary valve stenosis. Without prompt treatment, life-threatening complications can occur including arrythmias, cyanosis and oxygenation issues, reduced physical activity, fluid accumulation, slower growth, and even heart failure.
Management of Ebstein’s Anomaly
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.
Cone procedure offered at Phoenix Children’s provides hope
The Adult Congenital Heart Disease Program in Phoenix Children’s nationally ranked Heart Center now offers cone reconstruction surgery of the tricuspid valve as an alternative treatment for patients with congenital heart defects.
Repaired tricuspid valve, post-cone procedure.
It is the first full-range program of its kind in the state of Arizona to offer medical, surgical, and interventional therapies to manage Ebstein’s Anomaly and proudly stands among only a small number in the entire country. Any patient with Ebstein’s Anomaly 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.
Chief of the Division of Cardiothoracic Surgery and Co-Director of the Phoenix Children’s Heart Center, Daniel A. Velez, MD, 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 the Children’s Hospital of Pittsburgh so that he could bring this life-saving 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.