Update in Pediatric Cardiothoracic Surgery: New Research Shows Efficacy for Cone Procedure After Starnes Procedure

Under the leadership of Victor Morell, MD, the pediatric cardiothoracic surgery program at UPMC Children’s Hospital of Pittsburgh continues its pursuit of success and optimal outcomes for congenital heart patients, particularly in its treatment approaches for repairing complex congenital heart defects.

In May, Jose Pedro Da Silva, MD, director of the Da Silva Center for Ebstein’s Anomaly at UPMC Children's Hospital of Pittsburgh, published new research in the journal JTCVS Techniques on the efficacy of the cone procedure after a Starnes procedure for the surgical treatment of Ebstein’s anomaly. 

Dr. Da Silva is the inventor of the cone procedure to repair defective tricuspid heart valves that, since its perfection in the 1990s, has become a worldwide standard of surgical care for the correction of congenital heart valve defects and Ebstein’s anomaly. Since 1993, Dr. Da Silva and colleagues have performed the cone procedure on 208 patients. Since 2013, after Dr. Da Silva and colleagues modernized their overall care protocols, they have successfully performed the cone procedure on 74 non-neonatal patients without mortality.

With colleagues Melita Viegas, MD, Mario Castro-Medina, MD, and Luciana Da Fonseca Da Silva, MD, Dr. Da Silva published findings from the first two cases of neonatal patients who had previously undergone a Starnes procedure for surgical palliation of severe Ebstein’s anomaly, and who subsequently had surgery using the cone procedure to repair the tricuspid valve of the right ventricle and restore the patient's biventricular physiology. A third successful case has since been performed after while this manuscript was in publication.

In the setting of Ebstein’s anomaly, pulmonary atresia and circular shunt typically are managed with the Starnes procedure. The Starnes procedure is an effective surgical intervention for the critical neonate presenting with Ebstein’s anomaly. However, after a Starnes procedure, the patient is usually committed to a single ventricle repair pathway. The single ventricle repair pathway subsequently leads to the long-term complications associated with Fontan palliation: ventricular failure, exercise intolerance, arrhythmias, coagulopathy, venous shunts, and others. 

Because of these long-term morbidities, Dr. Da Silva and colleagues have searched for and developed a potential solution by demonstrating that right ventricular rehabilitation after the Starnes procedure is feasible. Their new approach of using the cone procedure after the Starnes procedure documented in the study results in a biventricular repair, which is likely to improve a patient’s long-term outcomes compared against the outcomes associated with a single ventricle repair strategy.

“We recognize the Starnes procedure as an excellent initial operation for the rescue of critically ill neonates with Ebstein’s anomaly. These patients usually present with a dilated right ventricle, which compresses the left ventricle, making its effective performance difficult. The procedure uses a patch with a small hole in its center to exclude the right ventricle. The severe reduction of the right ventricle's blood entrance reduces its size immediately, facilitating more space for the left ventricle, thereby improving its efficacy as a pump. Bearing in mind that the right ventricle undergoes progressive size reduction after the Starnes procedure, we reasoned that, at a point in this shrinkage process, the right ventricle might be a suitable pump for a biventricular repair. Based on this concept, we began to rehabilitate the right ventricle for a biventricular repair using the Da Silva cone technique. Theoretically, this new approach provides substantial long-term benefits versus a single-ventricle pathway,” says Dr. Da Silva

Findings and outcomes from the initial two patients to undergo cone procedure repair after the Starnes procedure show excellent promise for the technique. However, further research by Dr. Da Silva's team will be required to conclusively assess the benefits and long-term outcomes of this approach to treating severe cases of Ebstein’s anomaly.

Ebstein's anomaly with a circular shunt is a condition prompted by the combination of pulmonary valve leakage and tricuspid regurgitation, causing continuous retrograde ductal flow and blood circulating over and over inside the heart, which results in low tissue perfusion and a life-threatening condition. 

“Effective treatment of fetuses and neonates with Ebstein's anomaly with a sizeable circular shunt is an urgent task that should begin soon after fetal diagnosis. We manage neonates with Ebstein's anomaly who present with a circular shunt according to their clinical condition. Often these patients must undergo the Starnes procedure as the initial surgical intervention. In this situation, it appears possible to follow this approach with a cone repair, ultimately accomplishing a two-ventricle repair,” says Dr. Da Silva.

Additional details and videos clips from the study and surgical procedures are available at the journal website and reference below or by clicking here.

Reference

Da Silva JP; Viegas M; Castro-Medina M; Da Fonseca Da Silva. The Da Silva Cone Operation After the Starnes Procedure for Ebstein’s Anomaly: New Surgical Strategy and Initial Results. JTCVS Techniques. 2020; 3: 281-283.

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

Founded in 2016, the Da Silva Center for Ebstein’s Anomaly at UPMC Children’s provides world-class care for children and adults who have congenital defects of the heart valves, including Ebstein’s anomaly.

Referring physicians may contact the Da Silva Center for Ebstein’s Anomaly for a referral or consultation by calling the Heart Institute at UPMC Children’s at 412-692-5540.