Feb. 17, 2026
Mayo Clinic cardiac surgeons share new updates on managing Ebstein anomaly, one of the most complex and heterogeneous congenital heart defects. Previously considered an untreatable condition in its severe form, managing Ebstein anomaly has transformed to where biventricular repair is possible. The research was published in Expert Review of Cardiovascular Therapy.
Ebstein anomaly is characterized by tricuspid valve regurgitation and right ventricle myopathy. It presents with a broad spectrum of anatomy and clinical presentation from highly symptomatic neonates to adults without symptoms. Most patients with Ebstein anomaly require surgery at some point.
This review is critical to the field. "Our review has the most up-to-date information on the management of patients with Ebstein anomaly, including the most recent expert consensus documents from our specialty on the topic. As our understanding and experience has increased, best practices have changed and become more clearly defined," says Joseph A. Dearani, M.D., a cardiovascular surgeon and director of Cardiovascular Surgery at Mayo Clinic in Rochester, Minnesota. Dr. Dearani has specific expertise with Ebstein anomaly and is the senior author of the review.
Ebstein anomaly areas evolving
Managing patients with Ebstein anomaly has evolved significantly in several areas. Two key aspects include patients presenting with severe Ebstein anomaly early in life and the overall surgical repair strategy.
"We used to consider patients presenting as neonates and young infants to be committed to a 'single ventricle' strategy. Their right heart and the tricuspid valve would not be able to support their circulation," says Dr. Dearani. "However, we've now seen that these babies can undergo a neonatal palliation with a Starnes procedure. If the Starnes technique is performed in a certain way, the baby can later undergo a repair of the tricuspid valve and the right heart can be used to support at least some of the body's circulation."
For overall surgical repair strategy, there's an emerging area of interest in the cone procedure for older adults. The cone procedure is a type of valve repair that involves separating the heart muscle from the tissue that should have formed the tricuspid valve and gathering all remnant valve tissue to construct a "cone" that becomes a working tricuspid valve.
"Prior repair strategy of the tricuspid valve involved creating a 'two leaflet' type of valve instead of three," says Dr. Dearani. "Currently, the cone repair technique is used broadly. It utilizes all available tricuspid valve leaflet material and creates a valve more like a normal tricuspid valve."
Innovation's expanded reach
Innovation and a deeper understanding of disease progression, from advances in prenatal and neonatal care to refinement of the cone procedure, have led to updates in managing Ebstein anomaly.
"The cone repair has revolutionized repair techniques of the tricuspid valve not only for Ebstein anomaly but also for other tricuspid anomalies, which we did not expect. Principles of cone repair have great application to non-Ebstein abnormalities, increasing the probability of successful valve preservation," says Dr. Dearani. "In the past, many adults older than 50 typically underwent tricuspid replacement as opposed to tricuspid repair. Now, more older patients can undergo successful tricuspid repair, and it's generally preferred."
Treatment strategies
A center with high surgical expertise and surgeons specialized in Ebstein anomaly valve repair is key. Performing cone repair is demanding due to fragility of the leaflet tissue.
"The review includes an algorithmic approach to symptomatic neonates with Ebstein anomaly, which is the most challenging subset of patients. Given this is a rare entity, with the symptomatic neonate a small proportion, this algorithm offers guidance for a clinical situation infrequently seen," says Dr. Dearani. "Secondly, the current strategy of considering a patient who has undergone a Starnes procedure for tricuspid valve repair is important to be aware of to enable appropriate timing of referral for surgical evaluation."
Mayo Clinic specialists are continually refining surgical techniques with an individualized approach necessary for optimizing outcomes. Today's modern surgical advancements allow for earlier intervention, which can enhance biventricular remodeling and reduce long-term complications. Patients who may be candidates for surgery include:
- Symptomatic patients with severe tricuspid regurgitation (TR) and progressive right ventricular (RV) dilation or dysfunction.
- Asymptomatic patients with severe TR, moderate RV enlargement and favorable valve anatomy.
- Children ages 3 to 5 with severe TR and moderate RV enlargement, when a durable repair is likely.
Next steps
This review can impact clinical care with updates on:
- Timing for repair for an asymptomatic child with Ebstein anomaly and right heart dilation.
- Appropriate timing and evaluation for a patient who has undergone Starnes in whom tricuspid valve repair is considered.
- Prognostic indicators for patients who may need right heart unloading at the time of surgery.
The researchers share that another era of innovation may be in the future, with molecular medicine, advanced imaging and personalized care, helping to refine the approach to managing Ebstein anomaly. Stem therapy and regenerative medicine may be next in surgical management. A promising path ahead is the possibility of stem cell injections into the myocardium. This would be done during conventional tricuspid valve surgery to augment RV function.
"The management of Ebstein anomaly in the future will likely be increasingly personalized, blending surgical artistry with biologic insight," says Dr. Dearani. "Given this entity is rare with a large spectrum of anatomy, physiology and presentation, continued research is needed and ongoing."
For more information
Jamal T, et al. Ebstein anomaly update: The evolution of care and the revolution of surgical strategy. Expert Review of Cardiovascular Therapy. 2025;23:493.
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