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Congenital Heart Disease (Dr Krasuski 2 16 12)

Thursday, February 16, 2012 – Noon


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. Take advantage of this rare opportunity to chat live about adult congenital heart disease with a cardiologist and heart surgeon in a secure online setting

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Cleveland_Clinic_Host: Welcome to our "Congenital Heart Disease" online health chat with Dr. Richard Krasuski. He will be answering a variety of questions on the topic. We are very excited to have him here today! Let's begin with the questions.

Dr__Krasuski: Thank you for having me.

Aortic Valve Surgery

jbrandeen: Is a BAV usually associated with a problem with the aorta (ascending, descending)? If so, do these problems arise because of the BAV defect or because of a defect of the aorta?

Dr__Krasuski: The normal aortic valve has 3 leaflets or cusps as they are called. The patient with a bicuspid valve has only 2 leaflets. Over time this can lead to either narrowing or leaking depending on the shape and orientation of these cusps as they close and open. We used to believe that the ascending aorta increased in size as a consequence of narrowing of the aortic valve. We now recognize that this is a problem related the aorta itself and that when you study the tissue of the aorta under microscope, it appears abnormal.

I have personally seen several patients with aortic aneurysms and bicuspid valves in whom their bicuspid valve was neither leaking nor narrowed at the time they needed surgical repair of their aorta. Patients with bicuspid valve who have their valves either repaired or replaced need to be followed very closely for any evidence of aortic enlargement over time.


Dr__Krasuski: Yes - see the above answer.


Dr__Krasuski: Early on the major problem with bicuspid valves is narrowing and this often requires some type of intervention, either a balloon procedure or surgery during childhood. Patients then do relatively well unless the valve is leaking.

Most folks present in their 50s and 60s with narrowed bicuspid aortic valves. It seems unlikely that you would have had problems earlier in life that were not recognized and if I had to classify you into one of these 3 groups, you seem to fit the third group the best. As such, I don't believe your bicuspid valve would have affected your teenage years.

WilliamS: What is your opinion about the recently revised Ross procedure, whereby the pulmonary valve is reinforced after replacing the aortic? What is your opinion about the femoral access method for aortic valve replacement? What is your opinion about the new 320-line CT-MRI machine? Does it afford enough resolution to substitute for the relatively invasive catheterization procedure in preparation for aortic valve replacement?

Dr__Krasuski: The Ross procedure has generally fallen out of favor in the adult population, mainly because it results in problems potentially with two valves rather than just one. The Ross procedure requires harvesting the native pulmonary valve and implanting it into the aortic position. A cadaveric valve is then implanted into the pulmonary position. We have certainly seen a number of patients who have problems with either the aortic valve or the pulmonic valve after the Ross,

One of my colleagues, Dr. Pettersson, has begun doing the reverse Ross procedure in some of these patients. What he does is harvest the pulmonic valve back from the aortic position and reimplants it into the pulmonary position and then replaces the aortic valve with either a bioprosthesis or a mechanical valve.

Regarding the femoral access for aortic valve replacement, you must be referring to TAVR or TAVI - transcatheter aortic valve replacement. During this procedure a valve is inserted into the aortic position via an incision into the femoral artery. Another approach to TAVI is to insert it directly into the heart through the apex. The latter technique is typically only done in the operating room. It appears that the femoral route has fewer complications than the apical route. But a study just recently published, suggests that as surgeons and cardiologists become more familiar with the transapical route, their outcomes improve. It is important to recognize that TAVI requires a relatively healthy and reasonably sized femoral artery to facilitate insertion of the valve. As the technology changes, however, and the size of the tubes used to deliver the valve become smaller, more patients will be able to have the procedure done via femoral route.

In terms of the newer CT or MRI technology, we are getting better and better at assessing the heart and even the coronary arteries. The problem with assessing the coronary arteries with either technology is the fact that the heart is a moving object. As such, we always have to account for this motion, otherwise, there is considerable motion artifact. Some of the new pictures from the latest machines are quite remarkable. I still feel, however, that when we are making decisions about the need for bypass in patients undergoing open heart surgery, that coronary angiography is still mandatory in patients of appropriate age.

conniet: My husband was diagnosed 2 years ago with a bileaflet w/intra-coronary infusion aortic valve at northwestern university. he has moderate aorta leakage, and now mild mitral valve leakage. He also was diagnosed with aortic regurgitation secondary to an incompletely coapted bicuspid aortic valve resulting in mild left ventricular chamber enlargement & asymmetric basal septal hypertrophy. His current med's are captopril 12.5 mg/day, furosemide 20 mg/day. question - our local dr. says to continue with med's he is not ready for valve replacement, do you agree or are you more aggressive with valve transplant while he is still as good as he is.

Dr__Krasuski: In general, earlier valve repair and replacement has become more popular as the surgeries have become less invasive and more successful. We typically do not replace valves unless they are severely regurgitant (leaking). A moderately leaking aortic valve should not result in left ventricular enlargement. If that is truly present, then other potential causes such as coronary artery disease should be more fully investigated. The use of ace inhibitors such as captopril seems to be a reasonable strategy in patients with leaky aortic valves, though recent studies have been less supportive for this strategy. Certainly if his blood pressure is elevated, it is smart to keep this under good control.

Aorta Disease

jbrandeen: Once the ascending or descending aorta becomes dilated, what is generally the progression of this disease? In other words, does the dilation increase at a certain rate before medical procedure is recommended? Obviously, lifestyle habits can impact this rate of this dilation or is it a time factor?

Dr__Krasuski: There is usually gradual enlargement over time. Occasionally this enlargement can occur more rapidly. Alternatively when the aorta gets quite large, it can tear. This is considered a medical emergency and is generally associated with severe chest pain that patients describe as a tearing going to the back. This usually does not occur unless the aorta has enlarged beyond 5 cm in diameter.

A factor that we believe increases the rate of expansion is anything that increases the force on the walls. One such factor is elevated blood pressure. Ideally patients with abnormal aorta is should keep her systolic blood pressure at least under 140 mmHg and preferably less than 130 mmHg. I also recommend that patients avoid weight lifting; in general aerobic exercise (walking and jogging) is very good for the heart and the cardiovascular system as a whole.

Tricuspid Valve Disease

SusanB: I am having an echo next week for my congenital heart murmur which relates to tricuspid valve regurgitation. I had an echo in March 2009 and the finding was Severe tricuspid regurgitation with trace aortic and trace mitral valve regurgitation. The tricuspid is 3+. My primary care wants me to also do a sleep study. I have recently learned that I have borderline heart enlargement. I am 52 years old and a breast cancer survivor. What are your thoughts on my situation?

Dr__Krasuski: The most common congenital lesion which causes TR is Ebstein's Anomaly. In this condition, the tricuspid valve is apically displaced and the leaflets are malformed resulting in regurgitation. If this condition is present in your case, it should be obvious by the echocardiogram. In rare cases, where we cannot be certain, we perform a cardiac MRI to more fully assess where the leaflets are attached. In your condition, one thing that comes to mind is whether you received either radiation therapy or chemotherapy for your breast cancer. Either of these could have potentially damaged the tricuspid valve and resulted in regurgitation.

Another question I would have is what the estimated right ventricular pressure is by echocardiogram or the actual pressure is by catheterization. TR can also be due to pulmonary hypertension. The latter condition is very different and has a number of potential medical therapies and therefore needs to be excluded.

Septal Defects

Genes: My mother didn't find out until late in her life that she had a hole in her heart. My brother recently had a stroke which was attributed to a hole in his heart as well. Can this condition be something that is hereditary or is this something that my other siblings and I should not worry about?

Dr__Krasuski: Holes in the heart can be of a variety of different types. The most likely defects are either a patent foramen ovale (PFO) or atrial septal defect (ASD). PFO is extremely common, occurring in approx. 25% of the population.

ASD is present in one in 300 - 400 patients and is therefore less common. ASDs do appear to run in families and there are some genetic defects in which ASD is a major component.

We know less about the genetics of PFO. To have a stroke with either an ASD or PFO presumably requires a blood clot to develop in the venous system and then travel across the defect into the arterial circulation, where it is able to get to the brain and block up a vessel and thus cause as stroke.

Some clotting disorders clearly run in families, and in some, we know the genetics extremely well. Not knowing your details specifically, it is hard to comment on this. I would certainly recommend finding out whether a clotting disorder was responsible as this is very easy to screen for.

tamin: A seven month old baby who's asymptomatic was discovered incidentally to have ASD II 18mm defect (like a common atrium), fine rim of the septum, all pulmonary veins drain to the left atrium. What's the best way of management at your center and When to operate? Thank you

Dr__Krasuski: I would refer you to a pediatric cardiologist to better answer this question. In general, to close an atrial septal defect requires adequate septal rim to be able to hold the device properly. From your description, it does not appear that this baby has adequate rim present. As such, surgical repair would be the only option.

Spunky: I have a 20 year old son who suffered a stroke 2 years ago, subsequently we found out he has a PFO and borderline anticardiolipin antibodies. He is in college and takes 4 baby aspirin daily. We have had numerous consultations and some of the physicians have said they use CardioSeal or Gore-Helix to close the PFO's, others believe we should use the Amplatzer. My son was concerned that he needed the device in for 60 years and there were not any long term studies on its effectiveness. !. What device do you use and why? 2. How long has the device been used for the closure of PFO'S? 3. Are there any studies you recommend for us to read? Thank you.

Dr__Krasuski: As I mentioned above, patients with PFO and stroke have to have a clot develop in the venous system for a stroke to occur. It is important to mention that the only randomized study of device closure vs. medical therapy failed to show any difference between the two therapeutic strategies.

It is also important to mention that a hypercoagulable state such as having a lupus anticoagulant is a contra-indication to implanting a device as per the labeling of the devices. The reason for the latter is that there may be an increased risk of device thrombosis. Whether there is any large difference between the two available devices for device thrombosis is extremely controversial. I personally have never witnessed a device thrombosis, but have certainly heard a lot of such cases presented. This is certainly something we would like to try to prevent. In our own studies, at the Cleveland Clinic, we found that patients having PFO closed surgically when the defect was found incidentally actually increased the risk of stroke over the short term. Thus, surgery is not an adequate alternative for patients who are hypercoagulable. In such cases, medical therapy with either anti-platelet therapy or anti-coagulation may be the best option.

In terms of my own practice, I am trained in implantation of all three devices. Unfortunately, as you may be aware, the Cardioseal or Starflex has been pulled off the market as NMT medical has gone bankrupt.Thus, the remaining devices are the Helex device made by Gore Medical and the Amplatzer septal occluder device made by St. Jude Medical. For PFOs I typically choose the Helex device as my first choice because of concerns over device erosions in patients receiving Amplatzer devices. Device erosion is a very rare complication, but one which can be fatal when it occurs. Devices have been around for over 25 years and the durability appears to be excellent. We believe that the device simply functions as scaffolding for cells to grow over and completely close the defect. As such, they should remain stable over the long term.

Transposition of the Great Arteries

Kristenlo85: I'm a 26yr F and have TGA. I had a senning procedure when i was 2.5yrs. I tend to sleep a lot, like I need nine hours a sleep/night. I'm just wondering if that is normal behavior for someone with TGA. I also have periods where I will sleep almost an entire day even when i'm not sick, just really tired and incapeable of waking up fully.

Dr__Krasuski: Everybody differs in terms of their requirements for sleep. In general it is recommended that the average person receive at least 6 hours a night and preferably 8. Some of us sleep far less and can function normally, though I don’t recommend it.

I am more concerned by your level of fatigue. Patients with transposition of the great arteries can develop enlargement and progressive dysfunction of their systemic ventricle. The correction that you underwent has placed a morphological right ventricle in the position of a normal left ventricle. While this can work efficiently for a number of years, eventually that heart chamber can start to fail.

Also the atrioventricular valve, which in your case is the morphologic tricuspid valve, can begin to leak. This can also contribute to worsening heart failure. Profound fatigue can be a cardinal symptom of congestive heart failure and when it occurs in someone like you should be investigated in expeditious fashion. If you have not seen a cardiologist in some time, I would recommend that he do so. You should be followed at least yearly for your congenital heart disease by a specialist who is trained in managing adults with congenital heart disease. You should certainly have an echocardiogram, if he had not if he has not had one in greater than a year.

The other problem to be aware of in transposition of the great arteries is heart block. This is particularly true in patients with congenitally corrected transposition of the great arteries but also applies to your type. It is estimated that up to a third of such patients will need a pacemaker during her lifetimes. It is possible that your own intrinsic pacemaker may not be functioning properly on the days when you are particularly tired. This can be assessed by having you wear a heart monitor.

TahirH: Hi. I am Tahir from Pakistan. My son, aged 6-1/2 yrs, is dextrocardia (Situs Solitus) and has Congenitally Corrected Transposition of Great Arteries (Isolated). So far, he is asymptomatic but his recent ECHO findings (19 Jan 2012) showed Trivial TR. His Cardiologist has advised that my son should not perform strenuous physical activities and maintains that there is no need of any intervention at present. May you please offer insight with respect to risks of future complications and tretament options. I shall be grateful if you could also advise care with respect to diet and his general routine life. Thanks and Regards. Tahir (Pakistan)

Dr__Krasuski: As I mentioned above, congenitally corrected transposition results in a morphological right ventricle being positioned in the systemic circulation. This is usually well tolerated for several decades before the ventricle starts to fail and/or the valve begins to leak. It is not yet known whether we can do anything to prevent eventual deterioration in this condition. It remains unclear whether enlargement of the heart leads to the valve leaking or whether the valve leaking is what leads to the heart enlargement.

If the later is true, then earlier repair or replacement of the valve may prevent the heart from enlarging. This would only be indicated if the valve were severely leaking. Fortunately in your son's case, this leaking is only trivial. In terms of activity, I would certainly recommend against competitive athletics or weight lifting, but most aerobic activity should be ok. As in any heart patient, a heart healthy diet is reasonable… but, not an absolute necessity.

Tetralogy of Fallot

floridaheart: Have you seen a patient with Tetralogy of Fallot and Sarcoidosis that has gone to their heart?

Dr__Krasuski: Both of these conditions are unusual, but yes, I have seen a patient with both of these. Sarcoidosis generally does not affect the heart directly; and when it does, the disease is fairly advanced. Tetralogy of Fallot is generally a disease of the right side of the heart. The lesions of tetralogy include an overriding aorta, obstruction of the right ventricular outflow tract, a ventricular septal defect and right ventricular hypertrophy. In patients with repaired tetralogy of Fallot, a major concern is leaking or renarrowing of the pulmonic valve. Therefore if I saw a patient who had both these conditions and right-sided heart enlargement, I would be more suspicious that the tetralogy was responsible for the heart enlargement rather than sarcoidosis.

One exception to this is in the presence of pulmonary hypertension. This can also affect the right heart. We are still learning about pulmonary hypertension in sarcoidosis and my friend and terrific colleague in the Pulmonary Institute, Dr. Dan Culver, and I are doing research in this area. If I saw the patient with both these conditions and enlargement of the left side of the heart, then the sarcoidosis may be a more likely culprit. Patients with tetralogy occasionally develop left-sided heart enlargement, but in general this is seen in fairly advanced stages.

floridaheart: What are the typical EF's values for Tetralogy of Fallot in patients 50 years and older who only had one surgery. My right ventricle is 28 and left 46 is this normal? I know most Tets worry about the right EF but my valves are leaking only a trace to mild levels?

Dr__Krasuski: The right ventricle is often dilated and occasionally dysfunctional in patients with Tetralogy of Fallot who have undergone prior operative repairs. We have evidence that if surgery is performed at an earlier stage, heart function and size can return to near normal levels. If surgery is performed at a later stage, however, heart enlargement is extremely common and some dysfunction is the norm. The left ventricle we are only learning now can also be dysfunctional in patients with Tetralogy of Fallot. This likely has to do with the complex interactions between the two ventricles, since they are close neighbors of one another. An EF less than 50% for the right ventricle and under 55% for the left ventricle would be considered to be abnormal. How to approach this should be discussed more with your adult congenital heart disease specialist.

Shones Complex

shones: I am a 30-year-old male born with Shones Complex. I had my co-arc repaired near my 2nd birthday and 7-8 weeks later I had open-heart surgery to repair my mitral valve. Now my Cardiologist informs me that I have moderate regurgitation in the mitral valve. He contacted Stanford to see if they would accept me for balloon valvotomy. They told him I am not a candidate. Now my Cardiologist has told me that my current Echo indicates that there may be some calcification of the mitral valve and that I should undergo surgery sooner rather then later. He is worried that a piece of calcification might come loose and travel to my brain. I do tire out quickly from exercise. I have conducted hour upon hour of research on the Internet. It seems that successful repair versus replacement of severely calcified mitral valves is very uncertain. My questions to you are: should I put off the surgery and is it possible for my parachute mitral valve to be repaired despite possible severe calcification?

Dr__Krasuski: Shones complex consists of aortic coarctation, aortic stenosis-often a subaortic membrane, and parachute deformity of the mitral valve that leads to stenosis. In your case, you had aortic coarctation which was appropriately repaired and mitral stenosis that was also intervened upon.

You are correct that calcified mitral valves are more difficult to repair and often require replacement. Ideally, to answer whether your valve is reparable a surgeon would need to review your echocardiographic (ideally transesophageal) images. In terms of the risk for stroke or other embolic phenomena from a calcified mitral valve, this would be controversial at best.

I am not sure that I would recommend surgery for this indication alone. It does sound however, that you have symptoms which could be related to leaking and or narrowing of this valve, and this would be another potential indication for surgery. In general, narrowed valves lead to symptoms and we perform interventions when these symptoms develop. Leaking valves, however can lead to heart enlargement... even permanent damage before symptoms develop. Therefore, waiting for symptoms when a leaking valve is present is a much trickier proposition. In such cases, the echocardiograms need to be closely followed for evidence of chamber enlargement. We would be happy to review your films with one of your surgeons should you wish.

Eisenmenger Syndrome

Melonlady17: I am an Eisenmenger patient who was on Coumadin for many many years before asking if I could try a daily aspirin instead. Is there much information as to why Coumadin isn't highly suggested in Eisenmenger patients? I'm glad to be off of it, but I was on it for 9 years before changing to aspirin, and wonder why I wasn't advised to get off Coumadin earlier!

Dr__Krasuski: Patients with Eisenmenger syndrome have a risk of pulmonary hemorrhage. This risk increases when they are placed on coumadin. In general, patients with pulmonary hypertension are treated with coumadin because we believe that thrombosis of the pulmonary vessels is part of the intrinsic disease process. This is probably so in Eisenmengers as well. But, in Eisenmenger patients we are not sure if the benefits outweigh the risks for Coumadin use. If an imaging study suggests you have clots in your blood vessels, then the benefits of coumadin outweigh the risks and nuisances.

Adult Congenital Heart Disease Symptoms

CrystalSatin: Why are more ACHD patients developing exercise induced symptoms or even symptoms of heart failure and not meeting the requirements to repair valves according to their defect issue? In some patients, these symptoms seem to progress; yet in heart caths and echos, there is nothing obvious to identify the cause of the symptoms. Also in some patients, there are minor changes to the data, but nothing that would indicate the significance of heart failure that they have. Why does this seem to be an increasing issue in adults with CHD?

Dr__Krasuski: I have not this to be an issue in my average patient that I see with ACHD, there are rare exceptions. In general, we have to approach surgery and percutaneous intervention for what they are, which is a risky proposition in certain patients. For each patient, we weigh the risks and benefits before proceeding to any such intervention. There are a number of reasons why people with prior heart surgeries and interventions develop chest pain or shortness of breath. In some cases, the chest pain is simply incision pain or the distortion of the anatomy resulting from the surgery. It is also important to recognize that exercise limitation is extremely common in congenital heart patients and although we can improve patient's symptoms, in some cases we can only improve and not eradicate all symptoms.

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

Dr__Krasuski: Thank you for having me today.

Reviewed: 02/12

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