Cone Procedure for Ebstein's Anomaly

Ebstein’s Anomaly: General Information About the Da Silva Cone Technique

Ebstein’s anomaly is a congenital heart malformation that involves the tricuspid valve and the right ventricle. The tricuspid valve is displaced into the right ventricle and presents variable degrees of insuffiency. In general, the disease harms the heart in a progressive way and most patients with Ebstein’s anomaly presents poor prognosis without surgical treatment. There is a classic follow-up study on a large number of Ebstein’s anomaly patients showing that their mean survival age was about 32 years of age, without surgery.

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To address Ebstein’s anomaly on its variable and large spectrum of anatomical presentation, many surgical techniques have been proposed. In USA, the technique developed by Dr. Danielson was the most used. Dr. Danielson repair was very successful but was limited to certain anatomical types of the disease, and tricuspid valve replacement was necessary in a substantial number of patients (65% valve replacement in patients younger than 12 years). In 2006, we presented the Cone technique concepts, showing a series of 40 consecutives patients with excellent results and no need for tricuspid valve replacement. Since then, many centers have adopted my technique. The Cone technique is a more anatomical repair procedure and has become the gold standard surgical treatment for Ebstein’s anomaly.

I created the Cone technique for Ebstein’s anomaly repair in 1993. In the same year, I operated on a six-year-old girl who presented echocardiographic anatomical features of Ebstein’s anomaly that were not possible to be repaired using the Danielson’s operation, which was the most used surgical procedure at that time. As an alternative to tricuspid valve replacement, I offered the cone repair. After the appropriated consents were obtained, we performed the cone procedure for the first time. She did well but required a second intervention a few years later to repair a residual regurgitation. Afterwards, she did fine and had a successful pregnancy. Her recent echocardiogram shows a tricuspid valve with good anatomy and function after 26 years since the first cone repair.

Since 1993, we have operated on over 260 patients. The initial results were 2.5% hospital mortality and about 5% of reoperations (re-repair). In the subset of patients younger than 12 years, encompassing over a hundred cases, there were no deaths, either early or in the long-term follow-up, except for a two years girl who died accidentally of non-cardiac cause. Since I moved to Pittsburgh, in 2016, we have done the cone procedure in 27 consecutives patients with Ebstein’s anomaly. The number is gradually growing, and we have operated in 8 patients in the first semester of 2019. Additionally, I have operated on 24 patients in the last 12 months in Israel, India, England and Brazil.

The results have improved over the years with improvement in life support technology, postoperative care and better timing for the surgical repair. In the last five years we haven’t lost any patient after the cone repair, excluding the newborns who have presented higher risk if operation becomes necessary.

The valve repair results have been excellent and our recent results at the UPMC Children’s Hospital of Pittsburgh (34 patients from 2016 to October 2020) shows that about 80% of the patients present only trivial tricuspid regurgitation. The other 20% present either mild or moderate tricuspid insufficiency. Our overall results are consistent and durable. To date, we could repair any anatomical presentation of Ebstein’s anomaly, and never replaced the valve in patients younger than 30 years old. It was necessary to replace the tricuspid valve in only two adult patients. In the first patient it was a planned option as a first operation for a 50 years old lady. In the second patient, the tricuspid valve replacement was necessary due to residual insufficiency one year after the cone operation.

Regarding the right ventricle size and function, there is a tendency to decrease the enlargement and to improve its function in most patients, after the cone repair. This is called reverse remodeling of the right ventricle. We have found better results in patients younger than 12 years when compared with older patients. It seems like the growing process benefits the tricuspid valve and right ventricle remodeling.

The clinical outcome after the cone repair is excellent for the vast majority of patients. They usually, return to normal physical activities and some can perform very well in sports.