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Adult Congenital Heart Disease (Dr Krasuski 8 31 11)

Wednesday, August 31, 2011 - Noon

Richard Krasuski, MD
Staff Cardiologist and Adult Congenital Heart Disease Services Director, Department of Cardiovascular Medicine, Miller Family Heart & Vascular Institute


It is estimated that approximately one million adults and are living with congenital heart defects in the United States. While there are risks, with proper medical care, adults with congenital heart disease can live long and full lives. Dr. Krasuski answered questions about adult congenital heart disease.

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Cleveland_Clinic_Host: Welcome to our "Adult Congenital Heart Disease" online health chat with Richard Krasuski, MD. He will be answering a variety of questions on the topic. Unfortunately, Dr. Gosta Pettersson has been detained in surgery and will not be able to join us. Thank you for joining us, let's begin with the questions.

Dr__Richard_Krasuski: Thanks for having me today.

Patent Foramen Ovale (PFO)

Trish: I'm interested in learning if there have been any longterm (10+ years) health issues/symptoms (chest pain) recorded that are related to patching of a PFO.

Dr__Richard_Krasuski: There are no studies that I am aware of examining the long-term outcomes after PFO repair. There are a few case reports of device erosion (the device working its ways through the heart tissue) occurring late (up to 5 years out) in patients who received an Amplatzer device to close a PFO percutaneously. This appears to be a very rare event but can be a very serious complication when it occurs. I have seen rare patients who has noted chest pain after device closure, but I have never been able to isolate the device as the cause of their symptoms.

Karen: i was diagnosed with a congenital heart defect in 1998. i have annual echocardiograms. this year there was a change in the echo. i was told by a local hospital it was an ASD; after i started having symptoms my primary had me go to st. marys in madison,wi. i had an angiogram and TEE. the dr. wants to close the PFO but i am allergic to nickel; one of the alloys in the '"{plug". i am scared now because the right side of my heart has enlarged a little. is there an alternative other than open heart to close the hole? I am a 47 year old female.

Dr__Richard_Krasuski: There are currently 2 devices that are available to close atrial septal defects percutaneously (through a heart catheterization), the Amplatzer and the Helex device.

The Amplatzer is made of nitinol (a combination of a variety of metals which include nickel). The Helex has a nitinol wire that is completely enclosed by gortex fabric. The central lock, which is tiny, also contains a trace amount of nickel. This would be the only nickel that would contact the bloodstream. Skin reactions to nickel are common, though systemic (body) reactions are very rare. It is interesting to note that the cells involved in the immunologic reaction against nickel that occurs on the skin are not present in the bloodstream. So we really have no idea how systemic reactions occur. I did have a single patient in who we implanted an Amplatzer septal occluder come back with a rash. The rash went away with an anti-histamine (Zyrtec). The rash resolved but then came back a month later when he tried to stop the Zyrtec. After a year we stopped the Zyrtec and the rash did not come back. I suspect this could have been a reaction. There are studies that have shown trace amounts of nickel in the bloodstreams of patients getting Amplatzer devices but this goes away as the device heals into place (when a lining grows over the device).

An option for you may therefore be to implant a Helex device, particularly if your reaction to nickel is mild. Of note the surgeons are now able to close ASDs using a robot which results in a much smaller incision and more rapid recovery afterwards.

Evans: my baby has downs syndrome .he has gone through a lot of medical test includes chest x-ray,echo,ecg and TFT.I want an advice from an expect.the specific conclusion was that,he has small PFO (0.4CM) Is this something that I should be concerned about?

Dr__Richard_Krasuski: PFO is a very common lesion, occuring in ~25% of the population. In and of itself it rarely causes any problems. Other than repeating an echocardiogram in a few years I would not recommend any type of intervention for this.

Aortic Valve Disease

Sept14: Please discuss the prevalence of bicuspid aortic valve genetically. For example, if a patient with bicuspid aortic valve has children, what are the odds the children will have bicuspid also? If the children do NOT have bicuspid, would their own future children be at greater risk than the population at large of developing bicuspid...i.e. does it skip generations?

Dr__Richard_Krasuski: Bicuspid aortic valve is an incredibly common problem that appears to affect up to 2% of the general population. There are some families where bicuspid aortic valve appears more commonly. Some genetic studies are suggestive that this can be passed through generations. These still appear to be fairly rare cases. Most bicuspid aortic valves likely occur through spontaneous mutations and it would therefore not be expected to be passed down through generations. In general - congenital heart lesions affect approx 1% of the population and this usually excludes MV prolapse and bicuspid aortic valves. If one has a congenital heart defect, then the risk of a first degree relative having congenital heart defects is 3%.

christine: I had a ross procedure when I was 20 years old. I understand from a friend of mine that they are not really doing them any more. What are they doing in their place? How long will my valves last? Will I have problems in the future if I need valve surgery? Why did they stop doing it?

Dr__Richard_Krasuski: The Ross procedure although performed less frequently is still performed nation wide. It is an ideal surgery for younger individuals in whom the heart is still growing and developing. In a Ross procedure the native pulmonic valve is harvested and then implanted in to the aortic position. The pulmonic valve is then replaced with an artificial valve which is typically a treated human valve. The problems that have resulted from this surgery includes the creation of double valve disease from a single valve problem at the beginning. There can be problems that occur with the pulmonic valve as well as the tissue that sits either above or below the valve. As a result, patients can end up needing procedures or even reoperation for problems with the prosthetic valve. This is in addition to occasional failures of the aortic autograft.

In children, the benefits of keeping your native valve in an aortic position may outweigh the risks of needing double valve surgery, but in adults, it is a little more difficult to justify. It also appears that the Ross procedure takes a great amount of familiarity by the surgeon to perform successfully. And - so experience is absolutely essential. Dr. Pettersson has recently described the Ross reversal which entails reharvesting the pulmonic valve from the aortic position and putting it back where it started from - he then replaces the aortic valve with either a tissue or mechanical valve. In patients who have problems with both valves, this is a very ingenious solution.

Anno1: Is Aneurysm from the ascending aorta stretching to the arch common for congenital bicuspid aortic valve patients with severe regurgitation?

Dr__Richard_Krasuski: The presence of enlargement of the ascending aorta is a common problem in patients with bicuspid aortic valve. We used to believe that the aneurysm was the result of the flow coming out of the aorta being disrupted by an abnormally thickened aortic valve. We now recognize that there are inherent problems with the aortic tissue in patients with bicuspid valve that lead to the presence of this enlargement and leading to aneurysm formation.

Tetralogy of Fallot

KarenkP: Do you have many patients that are pacemaker dependent? I am a 53 y.o. female, TOF with a pulmonary valve replacement 10 years ago. What are the long term issues with being pacemaker dependent?

Dr__Richard_Krasuski: A pacemaker is a device that controls the heart rhythm in patients that are in need of this.There are a variety of different ways to program a pacemaker that can permit it to work less or more depending on the underlying problem. A pacemaker can either sense or pace the upper or lower chambers of the heart. In some patients the electrical system is abnormal so that they are pacemaker dependent - in such cases, the atria, the ventricles or both of these may be paced. This is certainly not uncommon. Tetralogy of Fallot typically does not result in patients being pacemaker dependent. But - I certainly have a number of patients with TOF who either have pacemakers or defibrillators that have been implanted.

We know from prior studies that pacing the heart in patients who otherwise would not require pacing may lead to heart dysfunction but we also know that it can be life saving in patients who require it. In general, it is very important for a cardiologist who specializes in rhythm disturbances to monitor and regulate your pacemaker on a regular basis to insure that it is functioning properly.

Right Ventricle Hypertrophy

CrystalSatin: 2a) What does it mean when an adult CHD patient, who is extremely symptomatic, has right ventricular volume overload and right ventricle hypertrophy that is seen on a resting echo, and is a new finding? 2b) Do elevated inflammatory markers such as ESR and CRP ever correlate with these findings and why or why not?

Dr__Richard_Krasuski: Enlargement and hypertrophy are the results of two different physiologic problems typically. When a heart chamber is under the stress of increased volume it typically dilates or enlarges in size. When the heart chamber is under pressure stress, the muscle has to work harder and as a result hypertrophies. Occasionally both chamber enlargement and hypertrophy can be seen. This would suggest the presence of both a volume problem and pressure overload. A hypertrophied right ventricle would suggest problems with obstruction to the outflow or pulmonary hypertension. Dilatation would typically suggest either the presence of a leaking pulmonic valve or the presence of an unrecognized shunt. A shunt is present when blood abnormally communicates between heart chambers. Examples include ASD and VSD. Inflammatory markers are typically suggestive of ongoing inflammation. When grossly elevated they can suggest the presence of infection but in lower levels they may just be indicative of low level inflammation. Such inflammation can increase the risk of development of CAD and may be associated with a variety of heart problems including rhythm disturbances such as atrial fibrillation.

Single Ventricle

ahazel: Hi Dr. Krasuski, I'm interested to see if you treat patients with complex forms of CHD, like single ventricle patients (my daughter has tricuspid atresia). I did not see that in your areas of interest in your bio. With more patients who have had Fontan surgery reaching adulthood, how do you expect treatment to evolve? Will they be your patients or still treated in peds cardiology?

Dr__Richard_Krasuski: Adults with single ventricle physiology are definitely an area of great interest for me and one which we very closely follow in the Adult Congenital Heart Disease Clinic. The adult population is steadily growing as a result of our pediatric colleagues success in surgical palliation. Fontan procedures, however, are not a cure in this case and are at best only palliative. Problems can result including issues with rhythm disturbances, blood clots and problems with the liver. The Fontan circuit can itself fail - there can be mechanical obstruction and the development of increased resistance in the pulmonary circuit.

Anomalous Coronary Artery

MaryK: Hi - My 14 year old son was diagnosed with anomalous coronary artery today after having chest pains for a few months. How serious is this? Does he need to restrict his activities? What about taking Ritalin along with this condition? He has had ADD for awhile and this helps him but now unsure if he should take it with this condition.

Dr__Richard_Krasuski: Coronary artery anomalies are a common condition that affect up to 1% of the population. The lesions that are thought to be the most dangerous are when the coronary artery comes off of the opposite cusp and passes between the great vessels. For instance, when the left main artery comes off of the right coronary cusp and passes between the aorta and pulmonary artery, to get where it needs to go. In such cases we believe that the vessel is compressed or compromised in some other way to limit blood flow to the front wall of the heart. In young adults this can lead to chest pain; passing out; or even sudden cardiac death. Without knowing the exact nature of your son's anomaly it is difficult to comment on his risk.

In terms of Ritalin - there have been some recent publications suggesting that there are adverse cardiovascular effects of this medication. In general, we believe that this is most troubling for patients who have underlying coronary atherosclerosis. How this would affect someone with a coronary anomaly is not clear but certainly any medication that could lead to excess effort on the part of the heart could be problematic - this should be discussed further with your son's physician.

Coarctation of the Aorta

Thidwick50: Is Losartan likely to stop the growth of the aortic root in an adult w/o Marfan's? Specifically in an adult who had patch repair of coarctation of aorta as a baby and has a bicuspid but well-functioning aorta valve, and whose aortic root has grown from 4.0 to 4.4 in the last 3 years?

Dr__Richard_Krasuski: The use of losartan in patients with aortic enlargement but without marfans would be considered experimental at best. In Marfans we believe that losartan may be interfering with one of the genetic mechanisms of disease progression. At this time, it is unclear whether this same process occurs in patients with bicuspid aortic valve and ascending aortic enlargement. At best, any medication which reduces blood pressure likely has some effect on reducing the enlargement of the aorta but for patients who do not have Marfans - there is likely nothing magical about losartan compared to other blood pressure medications.

Of note - a large randomized trial in marfans patients is currently ongoing and we should learn soon about the impact of this drug on this population of patients.

JIM-JU: If you have repair of coarctation of the aorta when you are little - does it last for life?

Dr__Richard_Krasuski: Coarctation of the aorta is a narrowing that occurs in the descending aorta and can lead to problems such as the development of high blood pressure and also decreased blood flow to the lower extremities. When coarctation is recognized early in life, it can be repaired operatively or in some cases, the location can be opened with a stent. The surgical repair has most often been to resect the narrowed area and to sew the two parts of the aorta together. We call this end to end anastomosis.

There are other procedures which can operatively repair coarctation - including patch aortoplasty where the lesion is cut out and a patch is placed over the aorta. And bypass grafting, where a tube graft is used to bypass the area of narrowing. The type of surgery that is performed determines the eventual complications. For someone with end to end anastamosis renarrowing can occur. In such cases we can often open the area with a stent and forgo the need for repeat surgery. Renarrowing is suspected when the same symptoms that led to the initial surgery recur. This includes high blood pressure in the arms; and symptoms resulting from low blood pressure in the legs. In patients with patch aortoplasty, aneurysms can occur at the site of repair. These can be tricky to diagnose and I recommend that each of these patients undergo a MRI scan at some point to look for aortic enlargement.

Multiple or Complex Congenital Abnormalities

Sonia: Hello Doctor, I am 35 yrs old female with Congenital Heart Defect. I have Atrial Septal Defect, Sinus Venosus / Partial Anomalous Pulmonary Venous with secondary pulmonary hypertension. I just discovered this defect when I delivered my first child in 2008. The doctors are suggesting open heart surgery. I wanted to know if there is any non or minimal invasive procedure to cure the defect. Thanks Sonia

Dr__Richard_Krasuski: The repair of choice is surgical as there is no catheter based way to fix this defect. During the surgery the anomalous vein is brought through the septal defect and back to the left atrium where it normally belongs. There is no way to do this without a surgical approach. At the Cleveland Clinic, we are always trying to find ways to make surgery less invasive. My colleagues try to keep the incision small. And - in a few cases, we are able to repair this robotically. The most important thing is to get a good operative repair and never should success of the procedure be compromised by trying to keep it less invasive.

sonia: I don’t think here in LA they have robotic surgeries for heart or may be other hospitals have I just wanted to know if its the only option or how long can I put off the surgery...

Dr__Richard_Krasuski: The problems that can arise from unrepaired sinus venosus ASD include heart failure; arrhythmias; and pulmonary hypertension. Since this was recognized during pregnancy; I suspect that you have had very little in the way of symptoms before this. It is not unusual for patients to be recognized with congenital heart defects for the first time during pregnancy. This is a period of considerable stress for the heart and that is why problems can arise. When congenital heart defects are known, beforehand in women of childbearing age - we try to counsel them about the risks of pregnancy and we have them meet with our high risk obstetrical colleagues.

More than likely, your ASD has not resulted in a number of problems and therefore the repair would be considered to be elective. As such, it would not be unreasonable to seek a second opinion in a place that provides additional surgical options.

CrystalSatin: 1a) Is it becoming more common with adults who have complex heart defects to be symptomatic with minor changes in the actual numbers when looking at echos? (Do adults with complex issues find it harder to tolerate little changes in blood flow or does it vary depending on the person and defect)?1b) For adults who are symptomatic, maybe class 3 or 4 level of heart failure what are your recommendations to improve Quality of Life? What about if the patient is not able to tolerate medications either due to no improvement in symptoms or the side effects are just too much to tolerate, what would you recommend in this situation?1c) Even though the patient with significant CHF symptoms may not meet the number criteria for surgery, are there any instances in which surgery would be appropriate?

Dr__Richard_Krasuski: Echocardiography is an easily obtained and non invasive way to look at the heart, both in patients with congenital heart and acquired heart disease. In some patients symptoms can develop with little in the way of changes in their echo, while in other patients they may have little in the way of symptoms but their echos can change dramatically. This is why follow up with both an office visit and echocardiogram is essential in patients with CHD. The best example of the differences between symptoms and echo would be for a valvular heart lesion. If a patient has a stenotic or narrowed valve we typically wait until symptoms develop to intervene in that particular valve. If the valve is leaking however, the heart chamber can begin to enlarge in size a long time before the patient actually has any symptoms. In the latter case, waiting too long can have a detrimental effect on the heart. The echo can guide us to intervene before the damage is permanent.

Other Medical Conditions

patmcg: would cardiomyopathy be considered an adult congenital heart disease?

Dr__Richard_Krasuski: Cardiomyopathy is a term that means weakening of the heart muscle. Typically when we refer to cardiomyopathy it involves the left ventricle and occasionally, the right ventricle as well. In general, we do not group this under congenital heart disease, as in most cases, there is a cause found - these causes include coronary artery disease; viruses; and endocrinologic problems. We do know however in patients for whom no clear cause if found, that there are genetic causes as well. Thus it can be an inherited problem. Interestingly, the problem can manifest itself during adulthood. It is not clear what triggers it.

MacSniff: I was diagnosed last yr with right side heart failure & congestive heart failure...I'm not certain if either is correct as they have taken me off almost al meds , and I have never had a heart cath. What would be the reason for no heart cath?

Dr__Richard_Krasuski: Without knowing most of the details of your case, it is very difficult for me to comment on the risks and benefits of heart catheterization. If your heart failure is related to pulmonary hypertension I generally recommend that all patients undergo heart cath for a thorough assessment before initiating any specific therapy for this. There are some risks assoc with right heart catheterization including vascular problems; bleeding; and arrhythmia, but in general these risks are small.

MacSniff: I'm 57, diagnosed with right side heart failure, congestive heart failure. I have been going to a cardiologist for a yr now and am not doing any better. My legs are so heavy it is hard to walk. The Dr. has cut off almost all meds. with no plan of action...I'm not certain about these diagnoses, as there has been no heart cath. I need some advice.

Dr__Richard_Krasuski: I would recommend seeking a second opinion with a cardiologist as it sounds like a heart cath is reasonable given your description.

TroyR: As you both know, my heart's circumflex artery is closed off and now the exterior wall is dead tissue. Currently my ejection fraction is still in the 30's. However, I am still having a lot of problems with exercise. After several attempts to start walking during the last three years (even with cardio rehab clinics), I am still not able to exercise at all. I start off slow, work my way up to a moderate level of exercise and then I start to go downhill. I go so far down hill that I usually have to rest for weeks before I feel normal. The cardio rehab nurses don't know what to think of me. It seems like every cardiologist says its not my heart...but no one has any suggestions of what it could be or where to start looking. Recently I have had to reduced my work week down to 20-30 hours (from 40-50 hours) because of my need to sleep. My weight is under control, and to look at me you would think I'm the picture of health. Do either of you have any suggestions on how I can improve my quality of life?

Dr__Richard_Krasuski: It is very nice to hear from you. I get updates on your business endeavors through Linkedin - you are an inspiration to us all being able to accomplish what you have (nice magazine pictorial!) despite your level of disability. I would disagree with the assertion that your fatigue and exercise limitation is "not your heart." In fact I strongly believe that it is your heart. A considerable position of your heart muscle is scarred due to you prior heart attack (from the vessel being oversewn early in life). An ejection fraction of 30% is about half of the normal amount and is undoubtedly leading to your limited reserve. My recommendation to you would be to remain as active as you can - keep a log of what you can tolerate and try to keep that up. If there are activities that you do that wipe you out on the following day, I would take that as your body telling you that you are trying to do too much. It is important, however, not to shut everything down as this will lead to deconditioning and further problems.

I will say that in some cases if there is considerable exercise limitation despite aggressive medical therapy we will consider heart transplantation. This may be something we will need to discuss further in the future.

JMG9620: What effect does ventricular bigeminy have on the ejection fraction (EF)?

Dr__Richard_Krasuski: The presence of premature heart beats from the ventricular chamber also known as PVCs can occasionally lead to a reduction in the overall heart function - why this occurs and how it occurs is not completely clear. A lot of it depends on how frequently these abnormal beats occur. If bigeminy is occurring most of the time, then I suspect the risk of this would be increased. If the heart function is reduced we would try to suppress the premature beats with medications. Or - possibly even consider ablation therapy to try to cure the problem.

Cleveland_Clinic_Host: I'm sorry to say that our time is now over. Thank you again for taking the time to answer our questions about adult congenital heart disease.

Dr__Richard_Krasuski: Thanks for having me today.

Reviewed: 09/11

This information is provided by Cleveland Clinic as a convenience service only and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. Please remember that this information, in the absence of a visit with a health care professional, must be considered as an educational service only and is not designed to replace a physician's independent judgment about the appropriateness or risks of a procedure for a given patient. The views and opinions expressed by an individual in this forum are not necessarily the views of the Cleveland Clinic institution or other Cleveland Clinic physicians.

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