Webinar: Congenital Heart Disease – What Patients Need to Know

Fri, Feb 13, 2015
12:00pm to 1:00pm ET


During this one-hour interactive Q&A, Dr. Joseph Dearani and Dr. Carole Warnes provide an overview of congenital heart disease, including transition of care into adulthood, pregnancy, surgical risks, valve repair or replacement, innovation, and lifelong follow-up. A question and answer period followed the presentation.

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I am 33 and I was diagnosed with a vascular ring and had the “repair” surgery when I was 29. I have had little to no relief since the surgery. (I had no symptoms until I was 28). There is very little research that I have found for Vascular Ring. We are currently going through the IVF process to start a family and I am concerned about my breathing being labored significantly during pregnancy and my baby being affected. Is there any precaution I should take or anything I can or should do to prevent any issues? Do Vascular ring patients typically digress? Or multiple surgeries recommended? Thank you!

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From Dr. Carole Warnes: I think it should be determined why you have residual symptoms and if there is residual airway obstruction and how severe it is. Usually one surgery is sufficient to relieve the symptoms. Only if your airway is severely obstructed is it likely to affect the baby but all of this should be discussed with your adult congenital heart specialist.


My 1 1/2 year old son has Ebstiens Anomally of the tricuspid valve and WPW. He has been on Propanolol since 2 months after birth. When should we be looking at ablation? He also has an atrial septal defect. Will this get larger; because it has yet to close on its own? Are strokes very likely to happen in Ebstiens patients?

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From Dr. Carole Warnes: The decision about surgery on Ebstein’s anomaly is based on a number of things, particularly on the degree of tricuspid regurgitation (leaky tricuspid valve) and how big the right ventricle is and whether or not it is dysfunctional. Symptoms also have to be considered. The atrial septal defect typically does not get larger but at 11 ½, it is very unlikely to close on its own. Patients with an atrial septal defect tend to have more limitation than those without since they may shunt blue blood back into the left side of the heart with exercise and the lack of oxygen in the body circulation usually is associated with more symptoms. If there is WPW, this can be addressed with an ablation procedure before surgery, sometimes it is dealt with at the time of surgery.

Strokes are not very likely to happen in Ebstein’s patients, but when there is an atrial septal defect, there is always a chance that a clot in the right-sided circulation (for example in the leg veins) could cross the atrial septal defect and get into the body circulation and go to the head and cause a stroke. Would recommend that he be seen in a center where there is considerable expertise in Ebstein’s anomaly.


With regard to 3 month old with right anomolous artery will she have to also go through CS during pregnancy

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From Dr. Carole Warnes: Most patients with an anomalous right coronary artery never require surgery. Frequently it is found incidentally because a CT scan has been performed for another reason. It is only when patients have symptoms or high risk features that surgery is necessary.

Indeed it has been estimated that there may be more than half a million Americans with an anomalous right coronary and so generally, for the majority the prognosis is excellent.


My 5 year old adopted daughter was diagnosed with a PDA after birth, which we were told had closed on it’s own by the time she turned one (she was followed with ECHO’s every 3 months until it showed that it had closed). Since we were told it was closed, I was wondering if this is something we will need to mention to her physicians as she grows, and for her to keep in mind when she is an adult, or is she really in a place where she does not need to ever think about it again?

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From Dr. Carole Warnes: If the PDA really closed and there are no residual problems, i.e. the heart function is normal and there are no residual murmurs, then she may be in the happy situation of never having to think about it again. A PDA is one lesion that, when it is closed, if there are no residual problems, it can be forgotten about.


My husband is 32 years old and was operated in Mayo Clinic at the age of 13 because of Ebstein anomaly. We live in Germany and his cardiologist is planning a catheter operation to replace his biological porcine prothesis because of a tricuspid stenosis with a bovine prothesis. Do you recommend this kind of operation or are there alternatives?

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From Dr. Carole Warnes: The biological valves in the tricuspid position do degenerate with time, usually after about 15 or 18 years and it may be that this has happened in your husband with his tissue prosthesis. The valves can be replaced using a catheter procedure but alternatively another operation to put in another valve could be performed. The decision about one intervention versus another has to be individualized. The long term durability of tissue prostheses (about 15-18 years on average) versus the new catheter valve have to be considered. Certainly we have many patients who have had a reoperation to replace the tricuspid valve for a second time and this is an alternative which could be considered versus a catheter procedure.


Is there a limit to the amount of homografts you can receive in a lifetime, or is it more dangerous the more you have? My 29yo son (DORV, Taussig Bing anomaly) is due for 3rd pulmonary homograft. Has had 6 OHS so far. Thank you for the nice comment about families!

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From Dr. Carole Warnes: There is not an absolute limit to the number of homografts you can have in a lifetime but every kind of open heart surgery carries a slightly increased risk. Homografts may not last long as other kinds of tissue prostheses and it may be that a different kind of biological valve could be considered. Sometimes in an effort to avoid another operation if there have been many, in the future, a mechanical pulmonary valve may be considered but this requires lifelong anticoagulation (blood thinners) so the risks and benefits have to be considered in each individual case.


my chd was diagnosed late in life – I have now been told I have what is called eisenmengers syndrome. can you explain exactly what this is

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From Dr. Carole Warnes: Eisenmenger syndrome is a condition named after Viktor Eisenmenger who first described it many years ago. Your congenital heart specialist should explain this to you carefully. Basically it usually involves being born with a hole in the heart connecting the left and right circulations. This may be an atrial septal defect or a ventricular septal defect and it allows blood from the high pressure side (the left ventricle) to enter the right sided circulation and raise the pressure in the right ventricle and the lungs. When the pressure in the lungs goes up, this is called “pulmonary hypertension” and this then reverses the shunt so that blue blood is shunted backwards into the left side of the circulation produces “cyanotic heart disease” meaning that the skin gets a dusky blue color.

Many therapies are now available to treat this condition to help to lower the pulmonary pressure which improves exercise capacity and quality of life. There are many important issues related to the “syndrome” which you should completely understand. Your congenital cardiologist should explain all of them to you so you have a comprehensive understanding what the situation is.


I have moderate to severe aortic stenosis and an ascending aortic aneurysm at 4.4 cm. Would it be possible to repair the valve and the aneurysm during the same surgery?

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From Dr. Carole Warnes: With aortic stenosis and an ascending aortic aneurysm, yes it is certainly very possible (and frequently done) to replace the valve and the dilated part of the aorta during the same surgery. This may not be necessary now if the aortic stenosis is not severe, but generally if the valve is being operated upon and the ascending aorta is 4.5 cm or larger, the aorta would be replaced at the same time.


For ACHD patients,VSD, Eisenmenger’s, PH: I question other routine screenings such as Colonoscopy etc, going under anesthesia is something my Congenital heart cardiologist(Dr. Warnes) warns me about. Do we still do these? And under the supervision of who present?
Thank you for your time and knowledge!

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From Dr. Carole Warnes: For congenital heart patients with Eisenmenger syndrome, yes it certainly is possible to do screening such as colonoscopy. Depending on how blue or “cyanotic” you are, however, having an anesthetic can be problematic and we usually recommend that the procedure is done with very careful sedation, sometimes using our cardiac anesthesia colleagues to make sure you do not drop your oxygen saturation and to make sure the procedure is a safe as possible. This is not a procedure we recommend doing in a center where congenital cardiac expertise is not available.


If a patient has a malfunctioning mechanic valve what is likelyhood, you would replace it with another type of mechanical valve or would you put in a different type of valve ?

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From Dr. Carole Warnes: If a patient has a malfunctioning mechanical valve which does not respond to appropriate anticoagulation therapy, then it may need to be replaced. Depending on why the mechanical valve was malfunctioning, it is possible to replace the valve with a tissue prosthesis and if you are in normal rhythm, it may be that you might not require anticoagulation. The decision needs to be made on things like how many operations you had in the past, whether this was an old type of valve more prone to dysfunction, or whether there was an issue with maintaining optimum anticoagulation that caused the mechanical valve to malfunction.


The melody valve for pulmonary valve replacement only has a limited range of sizes available. What is the likelihood that more sizes will be available for patients who need a broader range of sizes, such as those who currently have a bovine valve? If so, is it possible for this to happen within the next 2-3 years?

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From Dr. Carole Warnes: I think it is possible with all of these transcatheter valves that different sizes and improved technology will allow the valves to be more broadly applicable within the next three to five years.


My 1 1/2 year old son has Ebstiens Anomally of the tricuspid valve and WPW. He has been on Propanolol since 2 months after birth. When should we be looking at ablation? He also has an atrial septal defect. Will this get larger; because it has yet to close on its own? Are strokes very likely to happen in Ebstiens patients?

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Hi my son is 15 years old, was the 1st ever case of Ebstein’s in Scotland. He was born with moderate/severe EA, also with an ASD. However at 3yrs old they decided to go in a close it with a Helex device, as my son was exhausted all the time and had reocurring episodes of cyanosis. After they closed it my son came on leaps and bounds. As we are in Scotland and there is lack of experience with EA my sons case was always a wait and see if. He got 5 yrs and then ectopic beats started to present. He got another 6 years before he started to get really sick. Both atrial and ventricular arrhythmias started to present, causing many other symptoms. My sons files were taken over to the mayo in 2011 and Dearani told our team my son needed surgery ASAP. For me to take my son to the mayo we would have needed to raise somewhere in the region of £250,000. Fortunately for us a charity stepped in and met cost of sending our team back and forth to the mayo on educational visits to see the cone procedure being done. The also met costs of GMC registration for American surgeon to operate in our country, again they met costs of Americans, flights, food, and accommodation whilst in Scotland. We were told at my sons yearly appointment in January 2012 my son was being prepared for surgery. I believe Dearani told our team exactly how long they would get before my son got really I’ll, he got 5 months after that appointment and his health started to rapidly decline. Due to red tape this and red tape that my sons op was cancelled 3 times. Eventually Dearani was unable to attend at the time of my sons op so links had then to be established at Boston’s sick kids hospital with professor Pedro Del Nido. My son was so sick he was here within a fortnight. Fortunately for us the professor was able to talk our surgeon and team through the full procedure without having to take over or intervene at anytime, which now means the cone procedure can be done in Scotland by an awesome surgeon who goes by the name of Mr Mark Danton. Dearani opted for the bi directional shunt and Glen, Del Nido went with the maze and cone procedure instead ( maze procedure does not work in every cardiac patient) again fortunately it worked for my son and is a lot less riskier than the shunt and glen. My son had an ablation procedure carried out 2 weeks before op to see if he had any extra pathways etc, again fortunately he had none. He eventually got his op on 17th of April 2014. He is now 10 months post cone and maze procedures and is doing very well. He was on Amiodarone for 6 months after op for his rhythm issues, this was stopped end of October and not as much as an ectopic beat has been picked up on his ECG’s. If you could have seen how ill my son was before his op and then see him now it looks like 2 different people. He still has trivial leakage but the atrium looks good and the ventricle is still functioning well. I know in the future he will need more surgery but for now I’m quite happy holding onto everything is all good, 1 day at a time. I thank god, the charity the Americans and our team and all who were involved during and after with my sons care, everyday for giving me that gift, the gift is of that my son is still with me something that back then I was told, prepare yourself for worst case senario as today may not have been possible.
I hope reading my sons storey gives you hope, god bless and take care xx

I’m in Scotland, my son has EA. My son had last appointment under pediatric cardiologist last month. As he is 16 by the time his next appointment he has been transferred over to adult services. He is also 10 months post cone and maze procedure. Not looking forward to adult services at all. My son had been under the same cardiologist for 12 years. Think you may be safe fir now and have another 4 years before the transfer. Hope your son is well xx

Sorry just seen this, all I can say is that I am glad I am in Scotland. I don’t think my son or me would have coped with being transferred over to adult services when he was 12. I hope everything goes well and I wish all the very best of luck xx


My 1 1/2 year old son has Ebstiens Anomally of the tricuspid valve and WPW. He has been on Propanolol since 2 months after birth. When should we be looking at ablation? He also has an atrial septal defect. Will this get larger; because it has yet to close on its own? Are strokes very likely to happen in Ebstiens patients?

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My son is 10 months post cone and maze procedures, carried out in Scotland under the supervision of Professor Pedro Del Nido of Boston’s sick children. Dearani originally assessed my sons case and opted for the bidirectional shunt and glen. Just wondered why? And if you could shed any light. Also i would be interested to hear what you have to say as I feel my sons circulation is very poor. He always had very mottled skin I thought this would get better after his op but if any thing it is worse. Thank you if you reply

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