The Da Silva Center for Ebstein’s Anomaly: Refining the Cone Procedure While Providing Expert Specialty Care

Written by José Pedro da Silva, MD, and Luciana da Fonseca da Silva, MD, PhD.

The Division of Pediatric Cardiac Surgery within the UPMC Department of Cardiothoracic Surgery provides cardiothoracic surgical interventions for infants, children, and adults with congenital heart diseases, congenital abnormalities of the tracheobronchial tree, and abnormalities of the chest wall. The Da Silva Center for Ebstein’s Anomaly is a specialty center within the Division, founded by José Pedro da Silva, MD. Because Ebstein’s anomaly is a rare congenital condition, concentrating expert care for patients with Ebstein’s anomaly improves the quality of their treatment and encourages innovation.

In patients with Ebstein’s anomaly (EA), the tricuspid valve (TV) is malformed and displaced such that it is inside of the right ventricle (RV). This displacement and malformation cause tricuspid regurgitation with blood leaking backwards into the right atrium. The muscle of the RV is also abnormal, leading to a thin ventricular wall and dilation of the RV that can worsen over time. An atrial septal defect (ASD) accompanies EA in more than 80% of patients. These congenital abnormalities, which account for 1%–2% of all congenital heart disease, typically cause limited exercise capacity, cyanosis, and arrhythmia.

In the early 1990s, Dr. da Silva created the cone procedure for repair of EA. Now, almost 30 years later, we have performed more than 300 cone procedures and can complete the cone procedure in almost any patient with EA. With continued application and study of the cone technique, the long-term results are very good. The cone procedure creates a durable repair with a TV capable of appropriate growth. Optimal outcomes are obtained when the cone procedure is performed as an elective surgery in children under 12 years of age, and the ideal age to undergo the procedure is three to five years.¹

EA has several anatomic variations. Some patients are asymptomatic throughout their lifetime, while others experience severe heart dysfunction as neonates and require immediate surgery. Pulmonary valve atresia and severe pulmonary insufficiency are associated with poor outcomes in newborns with EA. If the neonate develops a condition known as a circular shunt, in which the blood is circulating inside of the heart instead of perfusing the tissues, the prognosis is still worse. This can lead to heart failure and death if palliative surgery cannot be performed within the first days of life.²

The Cone Repair After the Starnes Procedure: Our Recent Innovation

Although the cone procedure is durable and is the most anatomically accurate repair for EA, few newborns with EA are good candidates for the surgery. The friability of the neonatal valvular tissue may lead to a tenuous repair, and the infant may not be able to tolerate a prolonged procedure. In severely symptomatic neonates, the Starnes procedure, which imparts lower risk, can be used to improve circulation and serve as a bridge to additional procedures later in life, when the heart and the child are more capable of withstanding a complex surgery.³

When the RV is full and dilated, it compresses the ventricular septum and reduces ventricular output from the LV because it prevents the LV from filling completely. Sufficient LV output is crucial for most neonates with critical EA because the LV must propel both the pulmonary circulation and the systemic circulation. During the Starnes operation, the surgeon occludes the TV with a fenestrated patch, which keeps the RV from filling completely. As a result, the septum is decompressed during diastole, and the LV fills and functions better.

In the past, the Starnes procedure committed patients with EA to single ventricle physiology. The Starnes procedure is typically followed by a Glenn procedure and a Fontan procedure, after which the venous blood goes directly to the lungs without using the RV as a pump.⁴ The RV atrophies, and venous pressure on the right side of the heart is higher. In the long run, this is not ideal for the patient and can impact life expectancy.⁵

Recently, we expanded the patient population who could reap the benefits of biventricular repair of EA by performing the cone procedure after the Starnes procedure. We postulated that as the RV goes from being dilated to being hypoplastic over the course of several years, there must be a point at which the RV is an optimal size to restore biventricular circulation. In these patients, a Starnes procedure as a newborn, a Glenn procedure at four to five months of age, and then the cone procedure six months after the Glenn procedure should rescue the patient from neonatal circulatory insufficiencies and provide them with a long-lasting repair with the right ventricle pumping blood to the lungs. This sequence of operations should only be performed by highly experienced pediatric cardiac surgeons. Because these patients often present with associated pulmonary atresia and pulmonary insufficiency, the surgeon must possess the expertise to fix both the TV and the pulmonary valve.

We published the successful treatment of two patients using this sequential approach in 2020 and have since expanded this series to six patients in the United States and Brazil. When we needed to treat patients with contraindications for the Glenn procedure, we successfully completed the cone procedure in patients who were four to six months of age.^6,7

The Da Silva Center for Ebstein’s Anomaly

The experienced surgeons of the Da Silva Center for Ebstein’s Anomaly can repair almost any valve abnormality, including markedly rotated TVs in patients with EA. We receive patients of all ages from all over the world, often after their local care providers have given them no options or referred them for a heart transplant. We have treated these patients successfully with a cone procedure at UPMC Children’s Hospital of Pittsburgh. These patients typically have very challenging presentations of EA and allow us the opportunity to refine the cone procedure and perfect techniques for repairing different anatomic variations of EA.

Cone repair after a Starnes procedure. A. Removal of the fenestrated polytetrafluoroethylene patch. B. Tricuspid valve mobilization. C. Construction of the cone-shaped valve with the mobilized leaflets. AL, anterior leaflet. From reference (6), used with permission from Elsevier under a Creative Commons license.

With more than 50 years of experience, the Division of Pediatric Cardiac Surgery has a well-established mission to provide surgical care to patients with EA and other forms of congenital heart disease. The children who we treat at the Da Silva Center for Ebstein’s Anomaly benefit from a comprehensive Heart Center with expert surgeons, cardiologists, advanced practice providers, and support staff. Their perioperative care is enhanced by a dedicated cardiac anesthesiology team and a cardiac ICU where board-certified pediatric intensivists, cardiologists, specially trained nurses, respiratory therapists, and multiple other professionals work in close collaboration with the pediatric cardiothoracic surgeons.

The adults who we treat benefit from comprehensive medical care with a smooth transition in their care from childhood through adolescence and into adulthood.

Under the leadership of Victor Morell, MD, the Division of Pediatric Cardiac Surgery within the UPMC Department of Cardiothoracic Surgery has been a national leader in the surgical treatment of congenital heart disease for almost two decades with excellent surgical outcomes and surgical mortality rates far below national averages. At the Da Silva Center for Ebstein’s Anomaly, we continue to refine the cone procedure to improve its application in subsets of patients with challenging presentations of EA. The Division of Pediatric Cardiac Surgery is well-equipped to continue our long tradition of excellent care of patients with congenital heart disease.

References

1. Da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM, et al. The cone reconstruction of the tricuspid valve in Ebstein’s anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg. 2007;133(1):215-23.

2. Sainathan S, da Fonseca da Silva L, da Silva JP. Ebstein’s anomaly: contemporary management strategies. J Thorac Dis. 2020;12(3):1161-73.

3. Said SM, Sainathan S. Modified Starnes procedure as a bridge to 2-ventricle repair in neonatal Ebstein’s anomaly: A paradigm shift. Multimed Man Cardiothorac Surg (MMCTS). 2021;2021

4. Kumar SR, Kung G, Noh N, Castillo N, Fagan B, Wells WJ, et al. Single-Ventricle Outcomes After Neonatal Palliation of Severe Ebstein Anomaly With Modified Starnes Procedure. Circulation. 2016;134(17):1257-64.

5. Rychik J, Atz AM, Celermajer DS, Deal BJ, Gatzoulis MA, Gewillig MH, et al. Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association. Circulation. 2019;140:e234–e84.

6. Da Silva JP, Viegas M, Castro-Medina M, Da Fonseca Da Silva L. The Da Silva cone operation after the Starnes procedure for Ebstein’s anomaly: New surgical strategy and initial results. JTCVS Tech. 2020;3:281-3.

7. Lopes LM, Bezerra RF, da Silva JP, da Fonseca da Silva L. Critical Ebstein’s anomaly with circular shunt: from successful fetal therapy with non-steroidal anti-inflammatory drugs to biventricular repair using Da Silva cone technique. Cardiol Young. 2021;31(6):1039-42.