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. Results indicated the cone reconstruction had a higher success rate and lower incidence of tricuspid regurgitation when compared to valve replacement or other surgical interventions. Specialists and surgeons have continued to analyze and refine the procedure, which has become the gold standard to treat Ebstein’s Anomaly.Ī retrospective study published in May 2020 in the Journal of Thoracic and Cardiovascular Surgery compared outcomes from 151 patients with Ebstein’s Anomaly. Since its initial unveiling, results of this autologous procedure have continued to show incredible promise with no chance of rejection. Cone reconstruction is possible at any age, however, when performed in children, the new valve continues to grow with the child. 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. Jose Pedro da Silva, a pediatric cardiovascular surgeon. History of the surgeryĪn alternative option was developed and first implemented in 1993 by Dr. Velez and his team have completed ten cone repairs since 2017 on patients ranging from neonates to adults. da Silva at the Children’s Hospital of Pittsburgh so that he could bring this life-saving service back to Phoenix Children’s. Velez, MD, received his formal surgical training from prominent cardiovascular programs around the world, but to master cone reconstruction, Dr. 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.Ĭhief of the Division of Cardiothoracic Surgery and Co-Director of the Phoenix Children’s Heart Center, Daniel A. Cone procedure offered at Phoenix Children’s provides hope 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. Traditional management of Ebstein’s Anomaly has involved tricuspid valve replacement. 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. It is also typical of children diagnosed with Ebstein’s Anomaly to experience other cardiac abnormalities including septal defects or pulmonary valve stenosis. All rights reserved.Ebstein's Anomaly, pre-surgery, showing severe tricuspid regurgitation Additional abnormalities Heart transplantation should be considered in patients with associated left ventricular dysfunction.Įbstein’s anomaly (EA) Knott-Craig procedure Modified Starnes procedure da Silva Cone repair.Ģ020 Journal of Thoracic Disease. If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Adding a Bidirectional Glenn to a valvuloplasty may help salvage marginal risk patients or marginally repairable valves. If the chance of a successful TV valvuloplasty is high, it should be offered early in childhood to prevent further cardiac dilation. The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. Some neonates initially committed to a single ventricle pathway may be converted to a biventricular repair by a delayed TV valvuloplasty. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Presentation varies from a severe symptomatic form during the neonatal period to an incidental detection later in life due to the wide morphological variation of the condition. It is a disease at a minimum of the tricuspid valve (TV) and the right ventricular myocardium. Ebstein's anomaly (EA) is a rare congenital cardiac anomaly.
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