Home > PUM One on One: Jose da Silva, MD, Children's Hospital of Pittsburgh of UPMC

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PUM One on One Jose Da Silva, MD -Pioneering the way of performing cardiac surgeries in children.

Dr. Jose Pedro da Silva Developed the Cone Technique, an innovative procedure for tricuspid valve repair in Ebstein’s anomaly.

Surgical Director, Center for Valve Therapy, Division of Cardiothoracic Surgery
Visiting Professor, Cardiothoracic Surgery, University of Pittsburgh School of Medicine


Biography Summary

 

Dr. da Silva comes to Pittsburgh to help establish the new Center for Valve Therapy at Children’s Hospital of Pittsburgh of UPMC. Dr. da Silva is internationally recognized as the creator of the cone procedure for Ebstein’s anomaly. The cone procedure has been the worldwide standard for the surgical treatment of Ebstein’s anomaly for years. As part of the Heart Institute, the Center for Valve Therapy is dedicated to the care of children and adults with congenital heart defects that involve absent or poorly functioning heart valves.

Clinical Specialties
Developer of the Cone Technique, an innovative procedure for tricuspid valve repair in Ebstein’s anomaly

 
 
 
 
 
Dr. da Silva Interview
 
PUM: Dr. da Silva you moved to Pittsburgh to help establish the new Center for Valve Therapy at Children’s Hospital of Pittsburgh of UPMC in 2016. You are internationally recognized as the creator of the cone procedure for Ebstein’s anomaly. Tell us more about what your focused at Children's Hospital since you moved to Pittsburgh from Hospital Beneficencia Portuguesa de Sao Paulo, Brazil.

Dr. da Silva:  About 2 years ago, Dr. Victor Morell, chief of the Division of Pediatric Cardiothoracic Surgery at Children’s Hospital of Pittsburgh of UPMC, recruited me to come to Pittsburgh. He was interested in an expert that could develop a program on valve surgery and valve repair techniques. The main reason that he recruited me is to continue my work on the cone procedure. 

The cone procedure is a technique that I developed to treat Ebstein’s anomaly, a congenital heart defect of one of the heart’s valves. In every patient, it is different anatomically. Because of that, it can be very difficult to treat this type of disease.

Currently, I direct the heart valve program that deals with the repair many types of valves of the heart but specially to apply the cone technique, and on top of this, to make it available to the most difficult cases of Ebstein’s anomaly.

 
PUM: What about your experience at Children's Hospital, what do like so far working with your medical team?

Dr. da Silva: My experience here has been very good; actually, it has been awesome. And the reason for that is that we have an excellent team, with amazing surgeons and intensivists. We have it all. I have all the support and equipment needed to treat these kids with this very serious condition. 

 
PUM: As a pioneering heart surgeon worldwide, in 1989, you and your team developed a procedure for repair of Ebstein’s anomaly, a congenital heart defect of one of the heart’s valves. This surgical technique, known as the cone procedure, reconstructs the tricuspid valve and the right ventricle. In this procedure, the extra tissue on the overgrown right side of the heart is folded up, and the defective valve is then reshaped into a cone.

Dr. da Silva: Yes, I have developed this specific technique to repair defective heart valves that is now the standard of care around the world.

In Ebstein’s anomaly, one of the heart’s four valves –– the tricuspid valve –– doesn’t work properly. The defect prevents blood from flowing smoothly from the heart’s upper right chamber, the right atrium, to the lower right chamber, the right ventricle.
In 1989, I started to design a new surgical technique, cone reconstruction of the tricuspid valve, to repair Ebstein’s anomaly. After four years of work to refine and standardize the technique, in 1993 we started using the cone reconstruction, or cone procedure, routinely to treat patients with the anomaly.
Since then, I have performed over 230 cone procedures with 13 being done here at Children’s Hospital of Pittsburgh of UPMC. 

 
More info on Cone Procedure:
In a healthy heart, the tricuspid valve acts as a one-way gate between the two right chambers, allowing blood to flow from the upper chamber (the right atrium) to the lower chamber (the right ventricle). Then the gate closes tightly so blood can’t flow backward.

In children born with Ebstein’s anomaly, the tricuspid valve is malformed and in the wrong place. As a result, blood leaks backward from the right ventricle to the right atrium. The right ventricle shrinks and the right atrium becomes abnormally large. Other heart defects often occur along with Ebstein’s anomaly. 

Many techniques used to correct Ebstein’s anomaly before the cone technique. However, they failed to achieve consistent results due the vast variety of anatomical types in Ebstein’s anomaly. Therefore, the tricuspid valve had to be replaced with a mechanical valve or one made of animal tissue (bioprosthetic valve).  Such artificial valves, however, have limitations. The tissue valve has limited duration due to either calcification or tissue degeneration (failure). Also — important when the patient is a child — an artificial valve doesn’t grow with the child and eventually must be replaced. Therefore, after valve replacement, the patients will need reoperations over time. Regarding the mechanical valves, they require the use of blood thinners fir life, and still carry the risk of thrombosis. 

I devised a new technique, using tissue from the patient’s own malfunctioning tricuspid valve to create a new, cone-shaped valve. Unlike previous techniques to repair Ebstein’s anomaly, cone reconstruction — also known as the cone procedure — closely mimics the normal anatomy of the tricuspid valve. The reconstructed valve is made of the patient’s own tissue, so the body doesn’t reject it. In children, the reconstructed valve grows with the patient, so it doesn’t need to be replaced in a few years.

In the last 10 years, since the technique has been perfected and the best age determined, the results have improved substantially with very low mortality and nearly no need for valve replacement.

The cone procedure has been performed around the world on patients ranging from newborns a few days old to adults, and is considered the “gold standard” for repairing Ebstein’s anomaly.
 
 

 
PUM: Dr. da Silva you have performed more than 200 cone procedures and you continue to refine the procedure even as he traveled around the world speaking about it and training other cardiovascular surgeons to perform it. How are you improving your technique, what are some of your results?

Dr. da Silva: I have continued to refine the procedure even and travel around the world speaking about it and training other cardiovascular surgeons to perform it.

 
PUM: Dr. da Silva, your technique to repair defective heart valves is now the standard of care around the world, bring us up to speed on how other surgeons are utilizing the cone procedure to save lives?

 
Dr. da Silva: I am very glad to know, very honored to know that very great surgeons from the United States, and throughout the world have adopted this technique. And they are doing a good job, you can see the publications from those institutions – they have excellent results.

 
PUM: Does the age of the patient matter when it comes to these types of surgeries?  Replacing the valve tell us more about the difficulty in mastering this process.  

Dr. da Silva: The age of the patient matters somehow but the reason for that is if the patient had the disease that need to be operated before and was postponed because of the risk of having the valve replaced when they were young. They present to you a very enlarged and dysfunctional right ventricle. If they are older and the heart is in good shape, then the risk is very low. In the past because, we did not have a good technique to repair the valve, so the operation would be postponed. Now that we have the cone, the replacement will not have to be re-operated. That’s the big difference. 

Right now, we are trying to operate in patients 3 to 5 years of age routinely, but sometimes we must operate in newborns if they are not doing well, cannot go home or their condition is bad. Ideally, we should operate on a younger patient because they recover much better and the cone makes it possible because with the cone technique valve replacement is rarely necessary. Personally, I have never replaced the tricuspid valve in Ebstein’s anomaly. 

 
PUM: Tell us more about some of your concerns regarding how some surgeons who may be using artificial tissues during these very serious operations, what should families be aware of with these delicate surgeries?

Dr. da Silva: Families should be aware of the limitation of the cone technique because we don’t have enough tissues to contrast the cone, but that is a concept that can be seen in different ways. For me very few patients less than 2% need additional tissue to complete the cone. My concern is people are using artificial tissues and we don’t know the future we don’t know how long those will last in a patient. 

We don’t have reliable artificial tissues that we can use and be confident that they will work well years from now. The technique to get the tissues is not that easy, you have extensively take other tissues. People are afraid to come back because they don’t have enough experience. The disease is uncommon – about 1% of all congenital heart surgeries. That’s the reason I have traveled so much – I have got invitations to perform operations and they see me operate and they say ‘Oh now I think I can do it.’ And I think they really can do it.

The pediatric cardiovascular surgery program at Children’s is tied with one other institution on having the lowest overall four-year surgical mortality rate among all high-volume programs with a rate of 1.5 percent, according to the latest data compiled by the Society of Thoracic Surgeons<http://www.sts.org/>(2011-2015). Nationally, the average mortality rate for all pediatric cardiovascular programs was 3.4 percent during the same reporting period. Tell us more about how your innovation and procedure is helping to save lives.

 
PUM: Growing up in Brazil, why did you want to become a surgeon? Who were some of your earliest role models? You are also known to be a great painter, a skill you used to put yourself through medical school.

Dr. da Silva: I decided to be a doctor because I saw my father with a chronic lung disease, at that time, he had tuberculosis and I had accompanied him to his long-time doctor and I could see that they had very good communication. My father was very happy to see him. I realized you can do good things for people being a doctor. I was very good at mathematics and my professors gave me a book and expected me to become a mathematician or maybe an engineer. But, I chose one day in high school when my teacher asked what we wanted to be. And I said well I’ll try to be a doctor. The teacher said well that’s very difficult because in Brazil at the time there was about 30 candidates for 1 opening for a good medical school in Brazil. 

After they said that, I decided to do it because I like the challenge. It was exciting and I don’t regret that I became a doctor.

In Brazil there were two great surgeons, one was Dr. Euryclides de Jesus Zerbini, internationally known for performing the first heart transplantation in Latin America. The other was Dr. Adib Jatene who did the first successful arterial switch operation for transposition of the great arteries. In the US, I admired Dr. John Kirklin. He was a great person and a great teacher. He was initially from the Mayo Clinic and went to Alabama, where he ended his career.  He performed the first ventricular septal defect closure utilizing a heart-lung machine for cardiovascular bypass. He did a great job, by applying scientific concepts to heart surgery.

When I was in middle and high school and then in medical school, I used to paint. I would also sell my paintings. That was very important for my financial support while in the medical school and in the design of new surgical techniques.


 
PUM: Moving to Pittsburgh how have you adjusted to the snow and the Steelers? Has the city recruited you yet to become a fan of both yet?

Dr. da Silva: I used to live in Cleveland, so it’s not hard to adapt to the Pittsburgh weather, especially the winter. But for me it’s something different to see the snow. I like to have well defined seasons. We don’t have that in Brazil. Here we have the colors so I enjoy being here very much.

I must learn a lot about American football and baseball as I don’t understand very well. Now I know what a fumble is.

 
PUM: As an innovator and creator of the cone procedure what motivates you to achieve success?

Dr. da Silva: You must think that you aren’t done yet. As a doctor, as a scientist, we always have something new to invent or create, and there is always some room for improvement and keeping that in mind, anything that we do we have to ask questions.  Can you do it in a better way? And by asking that question you can come up with ideas, some of them are not good so we have to scrap but we use common sense to select the right direction and work from there.

 
PUM: What sort of big goals for 2018 do you have in store?

Dr. da Silva: I’m very interested to publish the surgical maneuvers that I have used  as  a way to improve the cone technique in  the most extreme anatomical subset of Ebstein’s anomaly. The one, which the valve is rotated too much inside the right ventricle, enough for it to be very near the pulmonary valve. Bringing it to the right position at the atrioventricular junction was considered undoable at the initial publication on the cone technique and it was predictive of valve replacement with the Mayo clinic Danielson’s technique.   

 
PUM: When you hear from the families expressing their gratitude and heartfelt appreciation for your ability to help save lives, how do you feel?

Dr. da Silva: I feel very good. I think it’s a privilege to be able to help families and their children.
 
 
Pictured: Dr. da Silva with wife Luciana and son Pedro. 









 
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