Heart Surgery

Down Syndrome Center at UPMC Children’s Hospital of Pittsburgh

An interview with Cardiothoracic Surgeon Peter Wearden, MD, PhD, of the Heart Institute at UPMC Children’s Hospital of Pittsburgh.

Released: 2/3/14


Dr. Vellody: Hello again to everyone and welcome to the Down Syndrome Center of Western Pennsylvania podcast. As always, I am your host Dr. Kishore Vellody. Today, I am grateful to have Dr. Peter Wearden joining me on the podcast. Dr. Wearden is a pediatric cardiothoracic surgeon here at Children’s Hospital of Pittsburgh. Pete, thanks for joining us today on the podcast. Could you please give us a brief background of your training?

Dr. Wearden: I grew up in Morgantown, West Virginia and I trained in, at that time we had a complete training and general surgery first, so I trained as a general surgeon. I then came to Pittsburgh and trained as an adult heart surgeon. I then went to Toronto, Canada and trained at the Hospital for Sick Children as a pediatric and congenital heart surgeon.

Dr. Vellody: Ok, well that’s a lot of training and we’re glad that it eventually brought you back here to Pittsburgh. As you know, we had Dr. Russo on our last podcast and she filled us in on the most common cardiac issues in Down syndrome. If you don’t mind, could you give our listeners a brief overview of the most common issues with the heart seen in Down syndrome?

Dr. Wearden: The most common things that we see are holes between the chambers of the heart. A hole between the top chambers of the heart we call an atrial septal defect, and a hole between the bottom chamber of the heart we call a ventricular septal defect. The septum is sort of the wall. It is the term we use to describe the wall between the chambers. Very common in children with Down Syndrome is what we call an atrioventricular septal defect or a complete canal defect. Sometimes we call this an AV canal defect. And that’s a hole, its really one hole, but it is essentially a hole between the top chambers, the bottom chambers, and where there is supposed to be two heart valves there is only one heart valve. Other conditions that we treat in kids with Down syndrome would be Tetralogy of Fallot and also patent ductus arteriosus.

Dr. Vellody: Thanks. So, if a baby has one of these conditions, what’s next? Do they rush immediately to surgery or is there a period of waiting?

Dr. Wearden: Generally, there is a period of waiting, and it is always some decision making as to the appropriate time for surgery. A lot of it depends upon how the child is doing. So we’d prefer to wait for many of these things for two, three, four months of age. Occasionally, our hand is forced based upon their condition, and we need to operate sooner. Some of these defects may actually close on their own and not necessitate any surgery in which case we would just watch them very carefully, making sure that the children do not get into any sort of trouble with their heart and hope that these holes could close on their own.

Dr. Vellody: A question that comes up frequently is “why wait?” After all, if you know they have a heart condition that will need surgery, why not go in and do that right away?

Dr. Wearden: I think that that is a good question. Most of the time the kids will do a little better if we have waited a period of time. Sometimes I like to tell the parents that they like to be on earth a little while before we drag them into the operating room to fix these conditions. So generally, by three or four months of age, they really do better than they would in the first month of life. That is not to say that we do not operate on many children in the first month of life. They do fine too, but, for instance, they may end up staying in the hospital two weeks where the same operation done on a child at four months of age, they may be in the hospital for five to seven days. So really the advantage of waiting is just for them to become a little bigger and perhaps a little more mature physiologically.

Dr. Vellody: That makes sense. Could you fill in our listeners on how exactly the decision to do surgery on a baby is made?

Dr. Wearden: One of the great things about our heart institute is that we truly function as a team. So while you may see the cardiologist and then the cardiac surgeon, behind the scenes, we have all talked together about the patient. The patient may see the cardiologist, and the echocardiogram may be done. We meet once a week, and we discuss all of the patients under our care as to the appropriate timing of surgery. Sometimes the child is born, and there is not the time for the week to go by for the conference to be held. In which case, several of the cardiologists and the cardiac surgeons would meet in an adhoc basis to discuss the patient. And again, the consensus is formed as to the appropriate timing of surgery and what the appropriate surgery is. So really it is never a decision made by one doctor in isolation but really as a team of doctors caring for the child.

Dr. Vellody: Can you give us an idea of what might make the cardiac team more concerned about a patient? What would make you want to operate sooner?

Dr. Wearden: What happens in these patients as the time for surgery approaches is that they essentially develop heart failure – not quite the same heart failure that we think of in an adult and certainly not a heart failure that cannot be reversed. But if you think about it for a baby, things like eating are the most exercise they do. If their heart is not working as well as it could they may eat and become tired. They may stop eating because that is exercise for them. And along those lines we watch for is their inability to gain weight, if they are not putting on weight at the rate that we expect them too. Sometimes these holes in the heart cause too much blood flow to the lungs and it can keep the lungs ‘wet’ as we call it, and these kids may be more prone to respiratory infections and so forth. All of those things would be the things to look for but really it is common sense things – you can tell if your baby is continuing to get better and move along in the right track or if they seem to have plateaued or maybe even have worsened a little bit. It is all fairly obvious things.

Dr. Vellody: That’s good to know. Ok, so let’s say the decision has been made for the surgery and the date is set. What can the families expect on the day of surgery?

Dr. Wearden: Sure, that is another good question. Obviously that is a day of high anxiety for the families. We usually start in the operating room at a certain time, and they will ask the families to have presented themselves to the same-day surgery center approximately one hour prior to that. The nurses will then meet the family, review some things with them, go over any of the blood work tests that may have been done in the outpatient setting. Usually, depending upon the child and their age, they may receive a pre-anesthetic or a little bit of medicine to calm their nerves. Almost always you will meet one of the surgeons before you go back to the operating room. The surgeons will review the procedure that will be performed on the child, and it is the one last chance to ask any questions by either of the parents or family members if there are any. At Children’s Hospital of Pittsburgh, the parents are welcome to accompany their child back to the operating room at the time of surgery. The parents will be asked to put on what we call a bunny suit, which is a funny name for a white suit they would wear over their clothes because of the sterile environment of operating room. They can accompany their child back to the operating room. Generally in pediatrics, the children go to sleep with an inhalational anesthetic at that point in time before there is any needle sticks or anything like that. Once their child is safely asleep, the parents leave the operating room, and the anesthesiologists begin preparing the child for the surgery. This includes the insertion of some special IVs – generally one in the neck and one in the wrists so that we can more acutely monitor the blood pressure and other cardiac functions during the surgery. Once the child is all ready for surgery, they will have a sterile prep done which is usually a betadine or chlorhexidine prep, and then the patient is draped. At that point in the time surgeon will begin the operation. We always have a time out first, and again, everyone in the operating room reviews the planned procedure and agrees that that is what we are planning to do on that child. The surgery gets underway at that point.

Dr. Vellody: Thanks for that walk-through. Could you now give us a broad overview of what happens during the surgeries?

Dr. Wearden: Certainly cardiac surgery is a very unique thing and in some ways a kind of miraculous thing because I do have to stop the heart, open the heart up and go inside there and patch these holes. The way we approach this is we open the breast bone, or the sternum, down the middle so it is a straight incision and the bone is open. A retractor is inserted to open the chest and then we place the child on the heart/lung machine. This is done by placing a canula, or tubes, into the aorta as well as into the superior vena cava and the inferior vena cava, where the unoxygenated blood drains back to the heart. We take all of the unoxygenated blood from the heart, and it goes through our heart/lung machine where it receives oxygen and it comes back to the body. At that point in time, the child’s heart and lungs can be excluded from the circulation. They no longer really have to be doing anything because the heart/lung machine is doing all of the work. That allows me to administer a high potassium solution to the heart. We give it a fancy name called Cardioplegia. The potassium basically causes the cells in the body to what we call ‘depolarize’, which means the heart cells will not contract at that point in time. With the heart stopped, a clamp on, and no blood going into the heart, I can now go in and open it. There is no blood in there so I can see the holes and patch them. Once we are done, we close up the heart and allow the blood to enter the heart. We are very careful to make sure we get all of the air out of the heart before we allow it to beat again because we would not want any air to go to the brain which could cause a stroke, one of the risks of heart surgery, be it very small. It is fascinating to watch as the high potassium solution is washed out of the heart. The heart immediately, and every time, starts to beat again.

Dr. Vellody: That really is amazing when you step back and think about it. After the heart starts beating again, then what happens?

Dr. Wearden: Once we allow the heart to beat again, we always do echocardiograms again in the operating room to make sure we have achieved the intended result. If the echocardiogram demonstrates that everything is ok and we have repaired everything that we have set out to repair, we then close the child’s chest. We leave behind some temporary pace making wires and what we call chest tubes. The blood has to be completely thinned to go through the heart/lung machine so there can be some bleeding for a period of time after the surgery. Those tubes remove that blood until the bleeding stops. Then the child is brought out to the cardiac intensive care unit. Generally we try to extubate all the children prior to going to the cardiac ICU and that means that we remove the breathing tube from their throat. I would say that about 80% of the time, I would expect the parents to see their child off the ventilator when they see them in the cardiac ICU. It can still be extremely overwhelming and as a parent myself, I can not imagine what it would be like to see your child connected to these tubes and wires. They also frequently have become swollen. The heart/lung machine causes a side effect of inflammation that goes away over the next couple of days. So when they first see their child in the ICU there will usually be a chest tube, some temporary pace maker wires, perhaps a ventilator, and then obviously some swelling from the time of surgery.

At that point in time, the cardiac intensive care unit doctors begin to care for the child. Of course the surgeons remained involved throughout this time. Over the next several hours as they recover from the surgery, things begin to settle down. The nurses will adjust their sedation and pain medicine to keep them comfortable. The parents, after about a half an hour, can go back and be with their child and remain with them continuously if they care to do so. Over the next 24 hours, the recovery begins in earnest. Quite frankly, these children bounce back far more quickly than most parents expect that they will. Really, by the day after surgery, they are starting to come around to seem like themselves again. At this point in time, if they are able to take liquids or solids by mouth, we begin to feed them. We can begin to remove some of the special tubes and wires. Usually by the second day after surgery, they may have left the ICU and gone to a regular floor bed. At that point in time, the amount of recovery is variable, and it depends upon the child and the heart lesion that we corrected. But most of our patients have left the hospital by five to seven days after surgery.

Dr. Vellody: All right. How about after they leave the hospital and go back home? Are there any precautions that the parents need to be aware of in that stage of recovery?

Dr. Wearden: This is a very common question and concern of the family, and I always tell them that there is really not much that the child or they can do to hurt them at that point in time. Everyone is very concerned that they will do something wrong and will hurt the child - that is really not the case. The children in fact limit themselves by their discomfort so if they do something that causes them discomfort, they will stop. Generally, the operation will take the wind out of their sails a little bit so they are not quite as active as they were before surgery, but, within a week or so, that has returned to normal. The only thing that really has to heal is the breast bone (sternum). It has been wired together with stainless steal wires. It is a bone just like an arm or a leg so it heals in the same fashion. If we broke our arm or leg, we would have a cast for six weeks or so, but we cannot put a cast on the sternum so we use the wires and they remain forever. It takes about six weeks or so for the bone to heal so in older children that may be riding a bike or something, we ask them not to do so for two months until their sternum has fully healed.

Dr. Vellody: What about for a baby who might roll over and onto their chest – is that a concern for their breast bone healing?

Dr. Wearden: Generally no. We use a liquid bandage on the incision so the incision is protected. After a week or so the liquid bandage starts to look like peeling sunburn and can peel off. We do tell the families to leave them on their back and have no real tummy time until they see the cardiologist. They would generally see their cardiologist around two weeks after surgery. At that point in time they can go back to being on their stomach.

Dr. Vellody: Ok. Thanks. What about long term issues? Do the children ever require a second surgical repair?

Dr. Wearden: There are from time to time things that can happen. I tell all the families that we should always be cognizant that the child has a repaired heart. While we hope that it is perfectly repaired, it still is a repaired heart. It should be followed by their cardiologist at the interval that they recommend, generally once a year or every other year. Nothing happens quickly or acutely. Many families are afraid that something will happen very fast, but most of the things that we see that come up as problems requiring a second surgery happen very slowly over time. It may be something that is discussed for several years before they proceed to surgery. Most commonly in a child with Down Syndrome, that would be regurgitation of the atrioventricular valves and a complete AV canal defect. If you remember, I said in that condition it is really one valve that has turned into two valves. At the time of surgery, we want it to have as little a leak as possible. Some of these children, and it is reported into literature, about 10% of these kids may need a second operation to repair leaking of those valves, particularly the left sided valves. We currently really strive to decrease that, and I think in our practice there is probably less but there is the chance that a child with an AV canal defect could need surgery in the future to further repair the valve. With Tetralogy of Fallot, a similar condition happens in that when we patch the outflow of the right heart, there is no valve small enough to put in where we put the patch. In the processing of patching, we render the valve they were born with unusable. While many people have lived forty or more years without having a valve there, I think as time goes on we realize that there was a reason there is a valve intended to there in the first place so many of these kids would need valve replacement surgery but not really until their late teens or twenties.

Dr. Vellody: Thanks. Well, Pete, we’ve reached the end of our podcast. This has been a really informative podcast. Thanks for joining us today.

Dr. Wearden: Thank you. I appreciate the opportunity and these are wonderful kids and families to care for.

Dr. Vellody: I couldn’t agree more. Thanks again for sharing your time with us. Families, please stay tuned for our next podcasts. Until then, send us any questions or requests to DownSyndromeCenter@chp.edu. Bye for now.

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