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Adult Congenital Heart Disease (Dr Krasuski 09 12 13)

Thursday, September 12, 2013 - 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. Dr. Krasuski, Medical Director of the Adult Congenital Heart Disease Center at Cleveland Clinic answers your questions about adult congenital heart disease.

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Congenital Heart Disease - General

JackAttack: Hello, following a myocarditis 10 years ago, I have felt very poorly. My cardiac evaluations have been mostly unremarkable. One test that is abnormal is my VO2max which is very poor at 16ml/kg/min. Also a cardiac MRI in 6 years ago and then again last year shows a difference in my LV stroke volume and my RV stroke volume. The earlier one showed a LVSV = 88ml, RVSV = 65ml. The more recent MRI showed a LVSV=112ml and a RVSV of 75ml. Are there congenital defect which could account for the differences in stroke volume?

Richard_Krasuski,_MD: Patients with shunt lesions (ASD, VSD, PDA) have more pulmonary blood flow than systemic blood flow. These are left to right shunts. What you describe is more blood being sent to the systemic circuit than the pulmonary circuit, which would suggest a right to left shunt. These are unusual and usually result in such problems as cyanosis (blue lips, fingers, toes). More than likely, the numbers are the result of calculation error than a true shunt.

LAM62: I had a successful coarctation repair as a child and I had my bicuspid valve and aortic root replaced last year (age 49). I am very happy with my cardiologist but I notice that the recommendation is to see a cardiologist who is a specialist in treating adult congenital heart disease. If I'm doing well, is this really imperative? There aren't many cardiologists who actually specialize in adult congenital heart disease....mostly they are pediatric cardiologists.

Richard_Krasuski,_MD: You bring up a very interesting dilemma and that is the lack of cardiologists with specialized training in adult congenital heart disease. I am happy to say that we just received word from the American Board of Internal Medicine that congenital heart disease will be an accredited specialty. This should influence more trainees to take up a career in ACHD.

The ACHD guidelines in 2008 emphasized the importance of patients with ACHD to follow up with specialists. For simple lesions such as prior ASD repair this may only necessitate a single visit - for lesions of moderate complexity it is recommended that patients be assessed and then a follow up treatment plan should be developed. For complex lesions, follow up with an ACHD expert should be every 6 - 12 months.

Fortunately for you, aortic coarctation would fall under a moderately complex lesion. Ideally you should probably be seen in an ACHD center at least once for follow up and then every 3 or so years as long as the aorta and the valve remain stable. There are many cardiologists who are familiar with congenital heart disease but may have not specialized in this field. As an ACHD expert, one of our roles is to communicate and educate our colleagues to insure that proper follow up occurs.

It sounds like you have found a great physician whom you are confident in and this is often half the battle.

Atrial Septal Defect (ASD)

miamickey: Female diagnosed at age 55 w/large ASD, aFib and main coronary blockage. Had DaVinci surgery: one bypass that kinked, Maze procedure, and closure of ASD w/mammary tissue. Did have sternotomy because had complication of bleeding during surgery. Have had many issues of AFib since the surgery three years ago. Taking potassium and magnesium and baby aspirin, nothing else. Question: Is an enlarged heart ( a result of late discovery of ASD) more likely the reason for ongoing AFib problems? Would it indicate need for another Maze procedure? Thanks.

Richard_Krasuski,_MD: Atrial fibrillation is a well-known complication of an atrial septal defect, more common if the ASD is not fixed until after 40. It is a real nuisance and can recur, even after a maze procedure. Treatment options after maze include antiarrhythmics and ablation in addition to further surgery, which ideally we like to avoid.

Quest79: Can you talk about ASD and when they repair them with surgery vs. an interventional closure device?

Richard_Krasuski,_MD: There are several types of atrial septal defects (ASD). The most common ASD is the secundum ASD. But there are also primum ASDs, sinus venosus ASDs, and coronary sinus ASDs. Only the secundum ASD is amenable to a catheter based repair. Also, it is important that the defect have adequate septal rims so that an occluder device will sit well in the defect.

Another thing that needs to be excluded is the presence of anomalous pulmonary veins - this can happen in up to 10% of patients with secundum ASD. In this case, blood returning from the lungs gets sent to the right atrium. A catheter closure will not affect this and the patient will still be left with a left to right shunt. Such cases need a surgical repair where the vein can be baffled through the septum and to the left atrium.

Sometimes the ASD is too large to safely place an occluder device in the catheterization lab. Finally there can be other lesions that need to be repaired at the same time and surgery is the best choice for patients like this - like atrial fibrillation or leaky valves. That being said, catheter based closure is still considered the gold standard for repair of secundum atrial septal defect and I would recommend that you have someone skilled in catheter based closure review your imaging studies to assess whether catheter based closure is a possibility as this is a very safe and easier procedure to undergo.

Patent Foramen Ovale (PFO)

pfovent: Hi, my name is Cheryl, I used to be a patient of Dr. Krasuski and Dr. Minai, but my insurance forced me to find doctors here in Buffalo. I have Secondary Pulmonary Hypertension due to a PFO and a ventricular septal defect. Is there anything new in treating either of these so I can get rid of the PH, or is a lung transplant still the only option?

Richard_Krasuski,_MD: Pulmonary HTN is a common complication of congenital heart disease. It usually occurs in patients with shunt lesions - Atrial Septal Defects (ASD), ventricular septal defects (VSD), and patent ductus arteriosus (PDA). It happens in about 10% of cases. PFO does not cause pulmonary hypertension but can be present in patients with pulmonary hypertension. The impact of this is unknown. Typically, we treat with medications that are designed to impact the small vessels of the pulmonary vascular bed. There are a number of treatments currently available and several new agents are on the way or in development.

When to close a congenital heart defect in the presence of pulmonary hypertension is a very interesting and controversial topic. In general if the pulmonary hypertension is advanced, the defect should not be touched and patients appear to do better when the defect remains open. Sometimes we will treat with these medications and the pressure improves at which point we will re-assess whether repair is possible. In general, improvement to the point of repair is unusual. But...stay tuned as the therapies keep getting better.

Ventricular Septal Defect (VSD)

Jeff R: What is the treatment of choice for a large VSD?

Richard_Krasuski,_MD: There are four types of ventricular septal defects (VSD). The most common VSD is called a perimembranous VSD, which accounts for about 85% of all VSDs. There are also muscular VSDs; atrial ventricular canal defects; and supracristal (sub-pulmonic or sub-aortic) VSDs. In general, catheter based closure of VSD is not feasible and for large VSDs, we usually recommend surgical repair. At present, the VSD which is most amenable to repair with a catheter is a muscular VSD because there are no other sensitive structures near by (such as valves).

Transposition Repair

Sharonk: What are your thoughts about exercise for a 12 year old boy who had transposition repair done when younger - can he participate. He is doing really well and he wants to play sports.

Richard_Krasuski,_MD: Transposition of the great arteries is a lesion where the patient is born with the aorta attached to the right ventricle and the pulmonary artery attached to the left ventricle. There are two types of transposition - D and L. In D transposition, the atria are attached to their corresponding ventricles. These patients will require surgery as children in order to survive to adulthood. The L transposition patients are "congenitally corrected" and so surgery is generally not necessary unless there are other defects present such as pulmonic stenosis and a ventricular septal defect. Since your son required surgery as a young boy I am assuming that he had D transposition. We used to perform atrial switches (Mustard or Senning) for this anatomy but are now doing arterial switches. Presumably he had the latter surgery. We will closely monitor the aorta in such patients to insure that it does not enlarge. If his aorta is fine and his ventricle function is normal, there is no reason he should not be able to participate in aerobic exercise and non-competitive sports. Ideally he should avoid sports with heavy contact such as football or hockey because of its potential impact on the aorta. The Bethesda guidelines are quite conservative in what they permit patients with adult congenital heart disease to do. I have to admit that I am less stringent in my restrictions. I believe the benefits of exercise to outweigh the risks in the majority of patients with congenital heart disease.

Tetralogy of Fallot

andrewatwal: What sort of precautions should be taken for a 24 year old male with Tetralogy of Fallot? had full repair at a few months old, plus PVR (pig or cow valve) this past December. Always have felt great, kept in shape and feel even better now following pulmonary valve replacement.

Richard_Krasuski,_MD: It sounds like you have done beautifully. It is important to emphasize that patients with congenital heart disease can also develop acquired heart disease including coronary artery disease. One of the things we stress is preventative measures to avoid the development of high blood pressure, high cholesterol, or diabetes. We strongly counsel our patients not to smoke as well. Regular exercise as I have emphasized repeatedly is very important. This can help keep your weight down. Regular follow up with a congenital heart disease expert is important particularly to follow your pulmonic valve as well as other areas that can become problematic years after tetralogy repair. These include: the aortic valve and the left ventricle.

Inter-atrial Septal Aneurysm

WVgal: I have an interarterial septal aneurysm and PFO has had dizziness and headaches. My cardiologist told me there is nothing to do and they don't usually close the PFO - but I am worried about my symptoms. I read that there may be an increased risk for stroke with this - and it worries me.

Richard_Krasuski,_MD: What you are describing is an inter-atrial-septal aneurysm. This means that there is a little extra tissue in the atrial septum. Often the septum is mobile and bounces back and forth between the right atrium and the left atrium. It is often associated with a patent foramen ovale (PFO). This combination of lesions appears to occur more frequently in young patients with otherwise unexplained stroke. That being said this combination of lesions is common in the general population - probably up to 10 percent. So having this, doesn't necessarily mean that your risk of stroke is increased. There is some link between PFO and migraine headache with aura. There are studies currently ongoing to assess the impact of closure of the PFO using a catheter implanted device. You may qualify for such a study providing that your headaches are migraines with aura and are felt to be resistant to medical therapy.

Ebstein Anomaly

Gardnerbw: How many Ebstein Anomaly surgeries do you do at Cleveland Clinic? What is the success rate? Can a person live a "normal" life once the surgery is done and successful?

Richard_Krasuski,_MD: We probably do about 10-15 repairs a year at Cleveland Clinic. About 80% of the time we are successful at salvaging the valve and in the other 20% the valve needs to be replaced. I have quite a few patients that I have followed after repair and they are all leading fruitful lives with excellent quality of life. Patients after repair should continue, however, to be closely followed by adult congenital heart disease specialist.

BobbyS: Hello, I am a 45 years old. I have had ASD repair and have pulmonary stenosis and hypertension. My doctor told me my right side is weak and causing failure, that I have Ebstein's anomaly with a very leaky tricuspid valve and a moderate mitral valve with atrial fibrillation too. Is this something that can be repaired? Will I need a heart transplant?

Richard_Krasuski,_MD: It sounds like you have a number of valvular heart lesions and it would be difficult to make recommendations without looking at your imaging studies. Ebstein anomaly is associated with an atrial septal defect or patent foramen ovale about 85% of the time. It is rarer to see left sided lesions (aortic or mitral disease) in Ebstein patients. We have certainly performed such complex surgery in patients like yourself with outstanding results, but, there are many steps that are necessary to insure that you are a good candidate for such a surgery and that you will do well afterwards.

Shones Complex

shones: I am 32 years old and I was born with Shones Complex. Four years ago I had open-heart surgery to repair my calcified Mitral valve. However, my surgeon was not willing to repair the valve opting to replace with a bovine valve instead. This surgeon did not order an Angiography prior to surgery, Thus, he sutured shut my Circumflex artery during surgery. Immediately after taken off of the heart/lung machine I suffered an acute infraction. The cause was not discovered until 5 days later. At that point I was administered a heart catheterization and 2 stents (they don’t last forever) were inserted at the point of the circumflex occlusion. Now my bovine valve has developed a small leak near the location of the erroneous placed suture. What should be the current course of action in regards to this leak? How serious is this? My most important question is when my bovine valve needs to be replaced will past medical errors prevent me from having a successful Mitral valve replacement?

Richard_Krasuski,_MD: It sounds like you have had a very challenging recent clinical course. The circumflex artery runs in the atrioventricular groove – very close to the mitral valve. Your complication is not an unusual one. I previously followed a young lady (before I came to Cleveland Clinic) with Shones who had a very similar course of events. The small mitral leak should be followed with echocardiography, but from what you describe, this should not give you problems in the near future. Reoperations are always more challenging in congenital heart surgery than the initial operation. Our success rate with reoperations here, however, is on par or better that initial surgeries in other places because of the talent and experience of our fabulous surgeons. A lot of the impact of your prior problems will be mitigated by the state of your left ventricle. It therefore depends on how much damage your heart sustained from the heart attack that you suffered. If the ejection fraction is under 40%, this is a risk factor for perioperative risk. I still think that you can have a successful valve replacement when the time comes. Interestingly some of our surgeons have been working on a catheter-based technique (non-surgical) for mitral valve replacement. Though this is far from being ready for primetime, it appears well on its way toward human studies soon.

Biventricular Aortic Valve (BAV)

kr43110: I have slight aortic stenosis, murmur, with a BAV. Is this something that will eventually lead to a valve replacement? I was told I have leakage both ways. Should I have my kids tested?

Richard_Krasuski,_MD: A good rule for bicuspid aortic valves is that the major indication for repair or replacement in early adulthood is leaking (regurgitation), while for older patients (I define this age by older than me – over 45) is usually narrowing (stenosis). If your leak is mild it is unlikely that this will lead to a need for surgery (unless you were unfortunate enough to develop an infection – endocarditis – which is fortunately very rare). It is more likely, however, that the stenosis will progress over time and that will be a need for replacement. I would not be surprised if we are replacing the majority of such valves in the future through catheter-based techniques (so call TAVR). Another thing to follow would be the size of your aorta. You should have echocardiography on a regular basis to follow your valve and aorta.

waleslar1: About two years ago I found out that I am bicuspid and have an aortic aneurysm at about 4.8 to 4.9 just above the root. Last year's CT scan showed no change/stable. I'll be 60 in November. My next CT scan is due now, and I'll probably have that done next month. What exactly might or might not cause this thing to grow past the 5.0 - 5.5 size where OHS will be scheduled? I'm on a beta blocker and ARB. Larry.

Richard_Krasuski,_MD: Aortic enlargement is a common problem found in patients with bicuspid aortic valve. In general, we do not operate for such a lesion until the aorta is at least 5 cm in size. Some physicians believe that the risk for tearing or rupturing of the aorta does not begin to increase significantly until 5.5 cm. We will operate for aortas under 5 cm if there other lesions that need to be repaired. There are certain risk factors for progression of aorta enlargement, the most important of which is probably blood pressure. If the blood pressure can be kept under good control (less than 140 mm) this reduces the risk of aortic enlargement. It looks like you are on the best regimen of medications to prevent such enlargement. A lot of what we know about slowing the progression of aortic enlargement with BAV comes from knowledge obtained in Marfan Syndrome. A recent study showed particular benefit to the use of losartan in patients with enlarged aortas and Marfans. We believe this benefit extends to all of the ARBs (angiotensin receptor blocking drugs).

jerry56: I am a 45 year old male with BAV, which resulted in AS and AR. But echo exams show that my aortic dimensions are not enlarged (end systolic dimension 32 mm, aortic diameter 31 mm), and ejection fraction is typically between 65-70%. At present I swim 3-4 times a week, usually covering 1,500 meters in about 45-50 minutes, and feel great afterwards. Do you think that this amount of exercise is safe for heart condition, or I should aim at a smaller amount of exercise?

Richard_Krasuski,_MD: I believe that it is fine to exercise as you are. Aerobic exercise has been shown to be safe and potentially beneficial in almost every type of congenital heart disease. It improves quality of life and possibly impacts improved survival as well. The one activity I would caution you about would be lifting weights. We do not recommend high resistance weight training for patients with leaking aortic valves or with aortic enlargement.

LAM62: Is lifting weights and other isometric exercises ok after a BAV repair? I know that you shouldn't lift heavy weights when you have stenosis, but what about after repair?

Richard_Krasuski,_MD: My general rule about weight lifting in patients with aortic valve disease or aortic disease is that you should avoid heavy lifting. This is particularly important if you have significant leaking of your aortic valve or if your ascending aortic size is enlarged. A good rule to remember is if you cannot perform 20 repetitions with a particular weight easily, then it is probably too much weight for you to be lifting. For my patients that insist on lifting weights I monitor them a little more closely for changes in aortic size.

nanc107: I am 59 year old and was born with bicuspid aortic valve. I have severe AR and moderate-severe AS. At what point do you replace the valve? My cardiologist keeps telling me it must be based on symptoms and that I by doing the surgery, I am trading one problem for another. Do you agree and when do you decide to replace valve?

Richard_Krasuski,_MD: The traditional text book teaching for aortic valve disease is to wait until symptoms develop before replacing the valve. One of my fabulous colleagues in cardiothoracic surgery - Dr. Mihaljevic - recently published a paper suggesting that earlier replacement of aortic valve before symptoms develop has some benefits. In your case, this is a little more complicated since you have both narrowing and leaking. For leaking the left ventricle can enlarge and even become dysfunctional before symptoms develop. As such, we watch such patients much more carefully and often use ventricular dimensions (by echo or MRI) to determine the timing of valve replacement.

Probably the reason why your cardiologist has been delaying valve replacement surgery is that the choice of what type of valve to replace it with is controversial. A mechanical valve requires the lifelong use of a blood thinner (coumadin) while a bioprosthesis (tissue valve) degenerates with time and has a half life of about 10 - 15 years. As patients are now living well into their 80s, it is possible that you would need another intervention for this in the future if a tissue valve was used. TAVR, transcatheter implantation of an aortic valve, appears to be feasible within tissue valves so it is possible that surgery would not be necessary for your next intervention.

Reviewed: 09/13

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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