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Adult Congenital Heart Disease (Drs Krasuski&Pettersson 2 14 13)

Thursday, February 14, 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 and Dr. Pettersson answer your questions about adult congenital heart disease.

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Patent Foramen Ovale (PFO)

kevinmlamb: I'm a 54-year-old male with a PFO that was diagnosed when I was 41. Symptoms are regular palpitations, sometimes breath-taking, but usually mild. I'm a long-distance runner. Two questions: can my running exacerbate my symptoms? And what are non-pharm treatment options, if one is necessary?

Dr__Krasuski: We recently performed a study at Cleveland Clinic where we ambulated patients referred for assessment of patent foramen ovale (PFO) and measured oxygen saturation. We found that on climbing stairs approx 1/3 of patients dropped their oxygen sat. This suggests that there may be some affects on exercise in patients with PFO.

Interestingly the patients who underwent device closure of the PFO had significant improvement in their oxygen levels on repeat testing and also experienced an improvement in how they felt. This is preliminary data and further studies need to be done to prove the benefits of such a procedure. In general, device closure of PFO is still very controversial and there are no clear consensus recommendations on how to proceed in most patients with PFO and other pathology.

Dr__Pettersson: If you are an athlete like you are, perhaps you should have an oxygen saturation test while exercising.

SoCal: What are the indications for closure of a PFO?

Dr__Krasuski: The only group where there is clear consensus at this time for closure is when a patient experiences a recurrent stroke on appropriate medical therapy, where there is no alternative explanation for the stroke.

SoCal: How does the presence of Factor V Leiden influence the decision to close a PFO?

Dr__Krasuski: The product labeling for most closure devices states that devices should not be implanted in the presence of a hypercoagulable state. For Factor V Leiden, it is common to have the mutation present on one gene (heterozygous), this is not felt to be a true hypercoagulable state. If the patient is homozygous (2 mutant alleles)then a device should not be implanted due to the risk for device thrombosis.

SoCal: What implications does situs inversus introduce for PFO closure?

Dr__Pettersson: No difference.

Ventricular Septal Defect (VSD)

crnvl: Hello, My adult daughter had a gaping VSD @ birth, with banding of the pulmonary artery @ 3 months. She had 2 other surgeries to expand the pulmonary stenosis. She still has pulmonary stenosis near the bifurcation of the artery. Have you successfully performed stenting of the pulmonary artery in this area of the artery an if not, is there an alternative? I realize that every case is different. Thank you.

Dr__Pettersson: Our interpretation is that your daughter has had closure of VSD and band removed. To answer your question whether residual artery of pulmonary artery can be addressed with catheter and stenting, we need to see the location of the stenosis. It probably can be treated by catheter. Alternatively, if the narrowing is severe enough and causes load on right ventricle, surgical patching of the narrowing is possible.

Dr. Krasuski: We typically perform stenting procedures if there are suggestive symptoms or if the muscle of the right ventricle is thickened suggesting a large pressure load. If only one vessel is narrowed, then we can perform perfusion scanning of the lungs to assess the significance of the narrowing.

Dr__Pettersson: Usually the blood flow should be roughly equal to each lung.

MeeraR: I need to know what options are available for closure of subpulmonic VSD. apart from the traditional open heart surgery.

Dr__Krasuski: No. There are several types of ventricular septal defects, the most common being the perimembranous defects and the muscular VSDs. A subpulmonic or subaortic VSD is also referred to a supracristal VSD makes up about 5% of VSDs. Due to the proximity to the aortic and pulmonary valves to the defect, there are no nonsurgical repair options (such as use of closure devices in the catheterization lab) available for such defects. As such, surgical repair is the only option. These defects often are associated with leaking of the aortic valve and the valve may require repair or replacement at the time of surgery as well.


Sue-F: At what size would a child’s ascending aorta be considered an aneurysm? 4cm? 4 year old boy Annulus 1.8 cm Root 2.5 cm Sinotubular Junction 2.55 cm (Max norm 1.99) Ascending Aorta 2.86 cm (Max norm 2.01) Aortic Isthmus is 1.01 cm. Thank you.

Dr__Pettersson: The question doesn't have a single clear answer. We have to know the underlying reason for why the aorta is enlarged. There are syndromes with weakened tissue - there are valve defects - and there are cardiac anomalies where the actual flow is increased which causes disproportionate growth of the aorta. The importance of the size itself relates to tissue quality and the size of the patient. Physically the stress in the aorta wall increases with the diameter. In adults the usual cutoff number for advising surgery is about 5 cm. In children all these other aspects have to be taken into consideration.

waleslar1: I am 59 year old, male, with an aortic aneurysm at the root at 4.9 cm. I have no chest pains or symptoms. My aortic valve has some leaking, but is not stenotic. I am also "Bicuspid", which brings me to my question : What is the evidence that being bicuspid will hasten the chance for dissection and rupture? I know it's not as bad as having marfans syndrome. And is bicuspid really a connective tissue indication? Any further details would help me evaluate my risk. I know there is some conflicting data out there. Thank you.

Dr__Pettersson: Patients with bicuspid aortic valve very often have this association or this combination of aortic valve regurgitation or stenosis and ascending aorta enlargement. The risk of dissection, although increased, is not as high as in the patient group you referred to that has marfan syndrome. Doctors are still discussing and arguing whether the association is related to weakening of the connective tissue or not.

The indication to do surgery is related to size of the aorta and the degree of dysfunction of the aortic valve. At 4.9 cm the size of the aorta is approaching the size when this alone would be an indication to do surgery.

Pulmonary Atresia

MattMo: Where is the most advanced facility for an adult with Pulmonary Atresia to be seen? Have there been any advances in the treatment of this condition?

Dr__Krasuski: We have a population of patients that we follow who have had surgical corrections for pulmonary atresia. In some instances, we see patients who did not undergo repair and have now developed complications such as advanced pulmonary hypertension. Repair of these patients is not possible - it is also not possible to repair atresia if the pulmonary vasculature beyond the atresia has not developed adequately.

Shones Complex

Shones: I m 32 years old and I was born with Shones Complex. 4 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 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?

Dr__Krasuski: Sounds like you had a difficult recent clinical course. Sometimes despite the best of intentions and preparation, mistakes can occur. I have seen a patient with Shones complex and a very similar course before my time at Cleveland Clinic. The left circumflex runs in the location of the mitral annulus in all patients and oversews can occasionally occur. These can be very difficult to acutely recognize, as damage to the heart from a left circumflex artery occlusion can be electrically silent - they may not be easily seen on an electrocardiogram. Once the damage occurs in such cases, reopening the vessel (particularly after 12 hours) may not help to recover the heart muscle that was damaged. In terms of stents, they actually do last forever (unless they are dissolvable - a brand new technology that is not yet commercially available), as they become part of the vessel in which they are implanted. They can occlude, however, though without knowing the specifics from your case, it is not clear how this could impact you. In terms of your perivalve leak, this is not an uncommon finding with heart valve insertion, particularly when the annulus of the valve is calcified. It may have nothing to do with the vessel oversew. If the leak is small, it is unlikely to cause much in the way of problems. Sometimes it can lead to what we call hemolysis - breaking up of blood cells - which leads to anemia. In such cases we can try to occlude the leak with a catheter procedure. If the leak is large, occasionally the valve needs to be replaced. In terms of the effect of your current anatomy on future surgery, it is a well known fact that reoperation is more challenging and complicated in general than first surgeries. With adequate preparation, however, it is very likely that surgery can be safely and effectively performed.

Dr__Pettersson: One important deciding factor for the risk is the actual damage the heart has taken from the ligation of the circumflex. If the residual function is good - the risk of another operation should not be much increased.

Coronary Fistula

Lori-J: I am 41 and just diagnosed with a Cardiac anomaly. I have a left anterior descending arterial fistula to the Pulmonary artery. My Cardiologist said he will be contacting Cleveland Clinic. He already contacted Cincinnati Children's and said no one can tell him how they would treat this. I am trying to reach out myself and speak to someone who may guide me in the right direction. I have hypertension and before now couldn't relate my symptoms to anything, but now wondering if intermittent chest pain, pressure, and shortness of breath could be related. Please let me know if your group has any experience with this. Thanks so much!

Dr__Krasuski: We have seen many patients with coronary artery fistulas (communications from the coronary artery to other parts of the circulation). This can take blood away from the heart muscle and send it to where it should not go. In general there are 2 main indications to fix these - the presence of coronary ischemia (documentation that there is "stealing" of the blood away from the distal artery by the fistula) or the presence of a significant "shunt" (extra blood going to the lung circulation and causing problems for the circulatory system) leading to heart enlargement. We often find small fistulas that have no bearing on the patient (they are small and insignificant) and that the chest pains and shortness of breath is unrelated. In such cases repair of the fistula may serve no benefit, while exposing the patient to some unnecessary risk and complications. If the fistula is large and causing problems, then either surgical closure or catheter-based closure is indicated. If the fistula is proximal in the vessel, the latter can be performed. It is a procedure done without surgery and only requires an overnight stay.

ljackson17: I submit a question about the LAD fistula to the Pulmonary artery, but wanted to add a few things. Is closure always recommended? If not, what would be the recommended protocol for following. Stress ECG was normal. Stress Echo report small region of stress induced wall motion abnormality. Study suggests myocardial ischemia. This was not a nuclear scan. Should that be done. I have chest discomfort, and SOB at times, but did not during the stress test. What determines if intervention is needed. If it is not repaired. Are there any activity limitations. Risk of sudden death or heart damage. Why wait to fix when problems arise? Is the vessel stable? I am taking Metoprolol and Indapamide for Hypertension. Are those drugs appropriate with this condition? Thanks so much for your input!!

Dr__Krasuski: For fistula cases, we really need to review the images. In some cases the fistula is quite large and it is obvious that it could be causing problems - in other cases - the fistula is small and likely insignificant. This really changes what we do.

Dr__Pettersson: The combination of symptoms and evidence of ischemia by stress test could be an indication for catheter based or surgical treatment.

JillEllen: Is there another way of fixing stenosis of the pulmonary artery, at the bifurcation if stenting is unsuccessful?

Dr__Pettersson: There is a surgical approach as mentioned before.

Valve Disease

Complications: On Oct. 14, 2012 I had mitral valve repair done. I had many complications from this surgery, blood clot in my right eye, A-Fib, fluid in my right lung, & very high heart rate. My doctor has me on Metoprolol 12.5 twice daily, & Xarelto, & Aspirin 81mg.He said I may have to stay on Xarelto forever; this really concerns me about the antidote. May I have your opinion on taking Xarelto for the rest of my life? Sincerely-Lavina.

Dr__Pettersson: Xarelto is a new drug - a thrombin inhibitor - used as an alternative to coumadin. It is equally effective but at this point there is no good antidote which gives you a higher risk of bleeding in an emergency situation. Coumadin is potentially reversible with blood products, fresh frozen plasma or vitamin K. The risks and benefits of the two drugs are yet to be finally decided. This discussion about drugs is independent of the underlying indication for you to be on a blood thinner.

Dr__Krasuski: It is a natural tendency for doctors to want to prescribe the newest medications even before the data is overwhelmingly in favor of its use. In the case of coumadin, patients often complain about the need to have their blood checked repeatedly and how this impacts on their quality of life. Despite this, coumadin is still the accepted gold standard of blood thinning for most indications, particularly for patients with mechanical valves. There have recently been reports of physicians prescribing these newer medications for patients with mechanical valves - this should strongly be discouraged; As studies for these uses have yet to be completed or published. Regarding the lack of an antidote, there are active investigations ongoing in this area and it would surprise me if an antidote were not available in the near future.

Lydia-B: Dr Pettersson, Hello, I am asking for your help. I am a 49 yo female with MVR, that needs replacement of a St. Jude valve, chronic afib, and other issues with a strong positive outlook on life. I've spoken to you two years ago after sending you my documents and at that time you thought I was not ready for surgery. Now, two years later I am hoping you give me the ok that you will take my case. I have a lot of symptoms and a lot of issues so I am sending you all of my past and recent documents with the help of my doctor some time this week. I know it's a long shot to have my afib cured but with some research I wonder if you would recommend the CS Cox Maze method. Are there Drs that perform this method at the CC? I'm so looking forward to having you review my case and getting back to me at your earliest convenience. –Lydia.

Dr__Pettersson: In the absence of a good memory of your case and its details, I can only agree to carefully review your case again if I find it doable and indicated, I would be happy to do your surgery. Combining a Cox Maze procedure with other valve procedures is very common and is done on a daily basis at Cleveland Clinic.

Dr__Pettersson: Adding a maze procedure adds 15 - 20 minutes to the procedure and not much to risk.

Dancemoms3: My 10-year-old daughter had her pulmonary valve replaced at Johns Hopkins last Nov. Her surgeon said she could have it replaced without OHS next time. Do you think there is a possibility with technology that she may never have to have another OHS? Also, when she has it replaced non-invasively I want her to have it done where they have the most experience and are the best at this procedure. Would you say that is Cleveland Clinic? Thank you so much for your service and time.

Dr__Krasuski: It all depends on whether there are any other additional congenital heart lesions or whether she develops problems such as leaking of the tricuspid valve in the future. It also depends on the size of the valve that was inserted. Catheter-based valve insertion of valves - particularly the aortic and pulmonic valves - has really taken off over the last few years and there are at least 2 such valves available for use in the pulmonic position based upon the anatomy of the pulmonary outflow. There are several centers that are experienced in implanting such valve including Cleveland Clinic. My terrific pediatric colleagues and I work very closely on such procedures and we are very proud of our excellent outcomes. There are some very exciting animal data that suggest that a pulmonic can be inserted with another valve and within another valve and so-on with very limited reduction in the size of the opening. This would suggest that such procedures can be repeated, though its important to note that there are multiple assumptions of such a model and it does not account for the calcification of such a valve over time, which can certainly impact how big an opening we can create in the cath lab.

bav_patient: I am 44 y.o. male, with bicuspid aortic valve, and both AR and AS. Until recently, my echo results were OK, with end systolic diameter 32 mm, ejection fraction 65%, and aortic diameter 32 mm. A year ago, I started swimming 4 times a week, covering 1500 meters in one hour. My fitness level improved, with max oxygen consumption increased from 35 to 55 ml/kg/min. But my recent echo showed one odd result, with max gradient jumped from 35 to 72, even though I have no symptoms, and feel OK. My cardiologist is not concerned about the gradient change, which he thinks is biased in my case. He argues that the gradient depends on the stroke volume, which may be higher for two reasons: first, my AR, and second, increased stroke volume due to my recent aerobic training. Just in case, he measured my BNP level, which changed little, and stays at 12 pg/mL. So my cardiologists suggested that I ignore the odd result, and continue with my swimming routine as before. Would you agree with his opinion?

Dr__Pettersson: Yes. But you need to undergo repeated echos to follow the development of the gradient. I would also advise you to start exercising slowing with proper warm up before you go to maximum effort. If you get any kind of symptoms - dizziness, lightheadedness - talk to your cardiologist.

grant1: Dr. Pettersson, you are known to be an expert in the Ross Procedure. However, Cleveland Clinic is not performing many Ross Procedures these days. Can you please explain your position on the Ross Procedure and why Cleveland Clinic is not performing many of them any longer. I know of a surgeon at Indiana University Medical Center and also Mt. Sinai Medical Center who are still performing them on a regular basis. Please give your thoughts.

Dr__Pettersson: I still believe the Ross procedure is a good procedure for children and young adults. Particularly, women in child bearing age. The reason I am not doing more Ross procedures is the fact that it is a slightly bigger operation and the fact that many referring physicians are biased against the procedure. When a patient is asking for it and has the right indication, I happily do it. I have to admit that today I do more reversed Ross procedures than primary procedures. This fact that the Ross procedure can be reversed actually in my mind lowers my threshold to do primary procedures - not the opposite.

Dr__Krasuski: For those unfamiliar with the Ross procedure, this is a procedure where the pulmonary valve is harvested and implanted into the aortic position and then a cadaveric valve is implanted into the pulmonary valve. The idea is that the live valve being placed in the aortic position can continue to grow and provide a good valve for life.

bettyL: I have congenital TR which is now severe 3+. I am wondering if there are non-surgical treatments for this.

Dr__Krasuski: Sometimes if the right ventricle has dilated as a consequence of the regurgitant valve aggressive medical therapy such as liberal use of diuretics can reduce the chamber sizes and improve regurgitation. In terms of catheter based technologies, there is nothing currently available but the field is rapidly advancing.

MAY-B: I had my heart fixed when I was 2 years old. I now need my mitral and tricuspid valve fixed for leaky heart valves. I am worried about this next surgery. What are the special risks related to congenital heart problems when young with someone who is now 45 years old and needing heart surgery? Is this something that can be done minimally invasive?

Dr__Krasuski: It is very common for patents with congenital heart disease that was repaired during childhood to require further surgical procedures as adults. It is important for such patients to undergo a very thorough evaluation prior to undergoing surgery, which includes assessment for underlying lung disease, exclusion of coronary artery disease by heart catheterization, and a thorough assessment of the cardiac anatomy by either ct scanning or MRI. Repeat surgery is always a little more complicated than the initial surgery due to presence of scar tissue and other problems. My colleague Dr. Pettersson is world renown for his abilities to operate successfully in such patients.

Transposition of the Great Vessels (TGA)

cg13: My son has repaired TGA, I have seen different suggestions regarding monitoring of the coronary arteries, some suggest angiograms and some suggest just routine echo. What is the best way to monitor this in a family that has a genetic history of high cholesterol and fatal heart attacks below the age of 45? Thank you.

Dr__Pettersson: There are two issues: the technical issues related to the repositioning of the coronary arteries from one great artery to another - that could be assoc with some proximal narrowing of the coronary arteries - that is related to the TGA. The second is development of coronary artery disease related to your family history.

Dr__Krasuski: The issue of coronary arteries related to TGA is completely dependent upon the primary anatomy and the surgical technique used for repair. The old repair for TGA was a Mustard or Senning procedure which switched the venous return to the atria. Coronary disease was not an issue for these patients. The Jantene procedure or arterial switch requires transfer of the coronary arteries and thus, can cause problems with the coronary buttons.

At this point in time, for the latter group of patients, I recommend stress testing using echocardiography every 2 - 3 years or as symptoms develop. I do not have a strong feeling about routine coronary angiography other than to make sure it is carefully performed to avoid complications. In terms of modifying the risk for atherosclerotic coronary disease. we recommend avoiding smoking, eating a healthy diet, and treating blood pressure and cholesterol aggressively.

Fontan Procedure

MattMo: How common is it to develop atrial fib after a fontan procedure? Is there anything besides taking medicine to control/get rid of atrial fib?

Dr__Pettersson: It is relatively common. Ablation is possible either catheter based or surgical. catheter based ablation in this situation is more difficult. This difficulty depends on what procedure was previously done. If you have a true fontan procedure, conversion to a more modern total caval pulmonary connection without including the atrium (extra cardiac tunnel) may be indicated.

Dr__Krasuski: Atrial fibrillation can cause symptoms for the reasons of either loss of the atrial component of ventricular filling or the rapid rate that is often associated with fibrillation. Either of these can be managed medically, catheter based or surgically. Patients with fontan are very dependent upon atrial kick and tend to get quite symptomatic with atrial fibrillation, thus we manage them more aggressively.

Irregular Heart Rhythm

Kellie-H: Need to find out what a right ventricular conductive delay is? My child's neurologist thinks she may have this but says there is nothing he can do for it, pretty much. I can't find any information on it anywhere. Something is going on with my daughter's brain and nothing is showing up on any tests. It's almost as if the "professionals" think I am making this up. I am fed up with the "medical professionals" not caring anymore. Please help me help my child. Thank You.

Dr__Krasuski: If what you are referring to is an incomplete right bundle branch block on your daughter's EKG, this can be a non-specific finding or it could suggest the presence of a congenital heart defect such as an atrial septal defect. Detection of such a lesion requires an echocardiogram.

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.

Reviewed: 02/13

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