Robert Pass, MD, Division Chief of Pediatric Cardiology at Mount Sinai Hospital in New York City talks about COA.
Interview conducted by Ivanhoe Broadcast News in July 2019.
What is COA for our viewers who have never heard that before?
PASS: Coarctation is a narrowing of the aorta. Typically it’s just below the part of the aorta that sends blood to the left arm. The problem with an aortic coarctation is that it blocks blood flow that the heart is trying to send to the entire body, things such as the kidneys, the liver, the arms, the legs. Because of this obstruction the heart has great difficulty in getting blood where it needs to go. And this could cause many bad outcomes obviously. And so this needs to be repaired. And depending on how severe the obstruction is it sometimes even needs to be repaired in the newborn period.
Is surgery the only option then for most patients?
PASS: It’s in most cases surgery in the present era is the preferred approach particularly in young infants.
Talk to me a little bit about the way surgery used to be performed? We’ve got a father and son with the same condition.
PASS: Yes. The surgery has always been a central part of repair of the obstruction but the way in which a surgeon repairs the obstruction has changed over time. Today the most common technique and the one that doctor Pastuszko uses is called an end to end anastomosis. So they literally cut out or resect the area that is narrow and they take the two normal ends and they literally sew them together. That approach is technically not very difficult but in the past was considered to be very difficult. And in the hands of an excellent surgeon like Peter it’s typical that patients will be cured of their coarctation from this type of repair. In the past surgeons weren’t as skillful at doing that type of repair. And what they would do is take the subclavian artery which is an artery that sends blood to our left arm and is very physically close to the area where the narrowing is, and they would use the tissue from that artery in order to improve the diameter or augment the repair that they were doing. That approach worked fairly well but as you would imagine by taking the actual artery that goes to your left arm, sometimes in late follow up patients had problems with their arm which makes good sense obviously if you’re disrupting the artery to your left arm in order to do the repair. In the present era pretty much surgeons have abandoned that approach.
What is the benefit of the end to end approach?
PASS: Well the benefit is that it is a proven approach that has worked extremely effectively and reliably probably for the last 20 to 30 years. It is the preferred approach by most surgeons. And in the newborn period I would say virtually 100% of the time it’s the approach that cardiac surgeons use when there is a discrete coarct, meaning that there is a discrete narrowing in the aorta.
What kind of symptoms would newborns be having? Is it something that’s detected ahead of time?
PASS: Well coarctation can be diagnosed by fetal echocardiography, by a special type of cardiologist called a fetal cardiologist. And in our program here at Sinai we have quite a few. It happens to be a very difficult diagnosis in the prenatal period. So occasionally it is not diagnosed prenatally but in the hands of an excellent fetal cardiologist in the majority of cases it can be diagnosed even in the newborn period. I mean, I’m sorry in the prenatal period.
Charlie had a stretching of the aorta, and he was operated on when he was two days old. Was he diagnosed prenatally?
PASS: The likelihood is that he probably was diagnosed prenatally. I can almost guarantee he was diagnosed prenatally because we know that this particular form of congenital heart disease does tend to run in families. If you or a first-degree relative has an aortic coarctation there could be as high as a 5 to 10% chance of a similar type of heart defect in a family member. And so the fact that Dad had this problem as a young child at least put it as a higher risk for his son. So it’s not shocking to a pediatric cardiologist that the son of a person who had an aortic coarctation might also have an aortic coarctation.
These days kids who have these serious surgeries so early on, can they be expected to live a normal life and the life expectancy? Do they have any restrictions?
PASS: That’s a very good question. Most patients who’ve had an aortic coarct repair do extremely well. It is rare to have serious long term side effects as a result of having a coarct repair. And we believe that the earlier in life that you’re repaired and the better the repair is the more likely it is that you will not suffer serious complications. We do continue to follow these patients because sometimes high blood pressure can be a problem later in life in patients who’ve had a coarctation repair but oftentimes if the surgery is good and it’s done early in life most patients live a completely normal life with the one exception that they have to see the cardiologist once a year.
So this is something that Charlie, as he gets older, he can play ball?
PASS: Absolutely. He will have absolutely no restrictions whatsoever.
What are some of the early symptoms, doctor? And my other part of that question is if this is not treated and you should treat it with surgery, is this uniformly fatal?
PASS: As in most things everything is a matter of degrees. And the devil is in the detail. If someone has a severe aortic coarctation meaning that there’s a very severe narrowing of the aorta that is a truly life threatening problem. If you do not treat that, a patient can literally have a cardiac arrest at home. And so one of the things that all pediatricians do in the well baby nursery is assess children for that exact diagnosis of coarctation because it is so easy to treat surgically if we know it’s there. And you don’t want to miss it.
How long is the surgery? How difficult of a procedure is it?
PASS: Well it’s hard for me as a non-surgeon to tell you exactly how difficult it is. As I often say I speak with the courage of the non-combatant. However surgery for this amongst good congenital heart surgeons like Dr. Pastuszko is generally considered very easy, very straightforward. I think in general the actual cutting and sewing and repair of this is probably under an hour. Child might be in the operating room for a few more hours because of all the monitoring and proper positioning, et cetera, by the anesthesiologist, et cetera, the nursing staff. But generally speaking this is a very straightforward operation. And when we send patients for this operation we are very optimistic that the patient is going to do well.
If it hasn’t been picked up on the fetal echocardiogram, what are some of the symptoms?
PASS: Well typically in a newborn the symptoms might just be rapid breathing, very poor feeding. Unfortunately as I often joke with some of the parents of my patients with congenital heart disease, it’s almost like being a veterinarian because children that young don’t talk. But generally speaking when you have a coarctation that is unrepaired children become very ill very rapidly.
The fact that Patrick the father had surgery and he’s doing very well. And his son will have a normal childhood, what does this say about pediatric cardiology?
PASS: People often ask me why do I love being a pediatric cardiologist. And it’s stories exactly like this one. Patients who have congenital heart lesions in the present era with rare exception can be treated so effectively that the vast majority well over 95% of our patients today live very full normal lives. I would say that if you were to walk into our office today and into our waiting room I would defy you to tell me who it is that has congenital heart disease and who does not because the vast majority of these patients are completely normal in terms of how they appear, their exercise tolerance and their lifestyles.
How many cases of COA are there in the United States?
PASS: Well it’s hard for me to say exactly but I think that the incidence is supposed to be about 4 to 6 per 10,000 live births. And it’s roughly 4 to 5% of all congenital heart disease is coarctation. So it is a very common problem in our field.
END OF INTERVIEW
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