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Adult Congenital Heart Disease (Drs. Krasuski and Pettersson 8/30/12)

Thursday, August 30, 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. Cardiologist Richard Krasuski, MD, and cardiac surgeon Gosta Pettersson, MD, PhD answer your questions about adult congenital heart disease.

More Information

Aortic Aneurysm

mrosenberg: I am a 51 year old extremely active male (bicycle racer) in perfect health except for a 4.8 cm aneurysm in my aortic root. The valve is in good shape and no other problems apparent. Two questions. How much aerobic exercise can I do? I have gotten several different recommendations, but have been taking it pretty easy (for me anyway). Am I a candidate for surgery now? I would like to get back to my old ways of hard exercise.

Dr__Krasuski: A lot of our recommendations for surgical intervention depend on the underlying cause of the enlargement. If you have Marfan syndrome, a connective tissue problem that can affect many different organs, we tend to repair these sooner as an aorta above 4.5 cm and even above 4 cm can be at risk for tearing or rupturing.

If enlargement is due to a bicuspid valve, we traditionally wait for it to reach 5 cm unless there is additional surgery that is required, in which case 4.5 cm is significant enough to warrant surgery.

For someone with an aorta your size, we would strongly recommend that you avoid weightlifting, though aerobic exercise is still good. Walking, jogging and light running are perfectly appropriate activities, which are considered to be low risk. We would also recommend avoiding any contact sports or activities which requiring rapid starting and stopping. In some cases the push for avid athletes to return to sporting activity has influenced our decision to offer earlier surgical intervention. Without more clinical information or the ability to view your images, specific recommendations are not possible.

mrosenberg: Thanks for the feedback. I have actually already forwarded all of my records this week, after contacting the department of aortic surgery. It would be great if these can be reviewed and we can continue the discussion. Look forward to hearing from you.

Aortic Coarctation

dengrl: How is a Pseudocoarctation different than a coarctation?

Dr__Krasuski: A pseudocoarctation is a kinking of buckling of the aorta which is believed to be due to overgrowth and over elongation of the aortic arch. In such cases there is simply not enough space for the aorta and it bends. The obstruction that it creates is usually minimal. It can, however, dilate in portions and cause problems later in life.

A coarctation, however, is a discrete narrowing of the aorta that leads to obstruction. This obstruction can cause problems, mainly elevation in the blood pressure. As such a coarctation is something that needs to be aggressively treated (either with surgery or stenting when significant obstruction is present, while a pseudocoarctation is often conservatively managed through close follow-up.

Aorta and Truncus

CrystalSatin: My cardiologist says that they don't know when is an appropriate time to repair an enlarged aorta in Truncus Patients. At what point would you suggest a repair? If its moderately enlarged, but patients is severely symptomatic, would that be cause for repair?

Dr__Pettersson: There is increasing amount of information suggesting that the risk associated with aortic dilatation in patients with different congenital defects involving the aortic root is lower than for other patients with aneurysms of the aorta.

Subaortic Stenosis

CrystalSatin: How is subaortic stenosis visualized and what techniques are used in an adult truncus patient with a mechanical aortic valve? How do you find calcification deposits or growth on a mechanical valve? Would that be difficult to see on an echo and explain afterload cardiac symptoms?

Dr__Krasuski: In a patient with a mechanical valve there can be shadowing which limits the assessment of such lesions. Usually with some combination of MRI, TEE, or CT scanning we can usually get a sense of the degree of narrowing.

Dr__Pettersson: Occasionally catheterization including transeptal puncture and measuring pressures on both side of valve and membrane can be helpful.

Bicuspid Aortic Valve

scott_99: Hello, I am an active 23-year-old and have been diagnosed with a congenital, mildly calcified Bicuspid valve with significant regurgitation. I have spoken with two cardiologists and one has suggested quickly undergoing AVR, the other suggesting waiting and watching for a while. Is this a condition that needs to be dealt with promptly? I've also been told that I have Left Ventricular enlargement (LVIDs of 3.8 cm)...So my question is 1. Is this a birth defect that can worsen significantly? 2. At what point do I need AVR in my case, considering the two different opinions? 3. Are there benefits to just getting it done and out of the way instead of waiting? THANK YOU!

Dr__Krasuski: In general we recommend intervening on a leaky aortic valve when there is evidence that the left ventricle is enlarging over time or the squeezing function is being negatively impacted. Another thing that impacts our decision is if other heart surgery is required as well (doing it as part of an aorta replacement surgery or repair of other defects) or if the patient has symptoms which we feel are related to the leak. More recently we also take into account what the chances of repairing the valve are as well.

Dr. Pettersson has had tremendous success in repairing leaky, bicuspid valves and the ability to avoid a replacement is important here, particularly for a young woman of childbearing age. If a valve can't be repaired the usual valve choices are a prosthesis (which will deteriorate over time and usually is good for 10-15 years) or a mechanical valve which requires taking a blood thinner such as warfarin long term. Neither is perfect and the choices need to be carefully discussed with the patient before the surgery.

JonKT: I am a 30 year old male with a bicuspid aortic valve and murmur. at this point the doctor is keeping an eye on it. Is it possible to go through life without needing surgery?

Dr__Pettersson: Every bicuspid aortic valve is associated with a murmur. This is true whether the valve is well functioning, leaky or stenotic. About 50% of patients with well functioning bicuspid valves will never require surgery.

Dr__Krasuski: In general, patients with leaky valves will require repair or replacement earlier in life. While, narrowing or stenotic valves in an adult usually present with problems in the fifth and sixth decades of life.

Aortic Valve Surgery – Ross Procedure

ShanaK: Can you talk about the Ross Procedure for patients?

Dr__Pettersson: The Ross Procedure is an operation for young patients including children with aortic valve disease who need aortic valve replacement. We would consider using this operation in patients up to the age of 35 to 45.

The rationale for the operation is that we use the best valve that the patient has (if they have a normal pulmonic valve) in the most important position - the aortic valve. The pulmonic valve is used to replace the aortic valve and the pulmonic valve is replaced in its original position with either a human cadaver valve or any other valve conduit.

The complications and problems with the Ross procedure are that it is a slightly more complicated and riskier procedure than a simple replacement of the aortic valve with a regular prosthesis. The pulmonic valve does not always stand up to the systemic blood pressure which is higher than the pressure in the lung circulation. The valve dilates and becomes leaky in about 10% of the patients in spite of different surgical maneuvers to prevent this from happening.

The second problem is the replacement valve on the right side which eventually degenerates and (may) need replacement. May stands for the fact that the pulmonic valve is not as important as the aortic valve and even if the pulmonic valve degenerates - the patient may not need it replaced.

We still believe that the Ross operation is a good surgery for well selected patients. It provides a normally functioning valve which does not require a blood thinner. In case of failure, we have reversed the Ross operation putting the failed valve in the aortic position back in the pulmonic position, where it may still function satisfactorily because the pressure is lower.

In growing children, there is really no good alternative to the Ross operation since tissue prosthesis fail quickly in children; size may not allow implantation of a good sized valve; and no artificial valve grows.

Valve Surgery and Endocarditis Prevention

laura2: Cleveland Clinic "ask a cardiovascular nurse" told me to ask you this question in this forum because you know about endocarditis. I was taking probiotics for digestive issues and then I read that some of the specific probiotic bacteria I was taking were associated with endocarditis. I had a mitral valve repair at CC a year ago. Should I be concerned about endocarditis risk, and if so, what should I do about it? Thanks.

Dr__Krasuski: Fortunately most of us have a very good protective system against infection consisting of our skin and our mucous membranes. If there is damage to these, bacteria still often does not reach the blood stream and so systemic infection remains rare. To have a gut organism reach the bloodstream usually requires some other problem such as polyps, cancer or erosion. The latest endocarditis prevention guidelines recommend antibiotics for only certain patients who are undergoing dental procedures and prevention prior to gastrointestinal procedures is no longer recommended. My interpretation of this is that the risk of infection through the intestine is extremely small. I don't think taking a probiotic is therefore dangerous.

Dr__Pettersson: Compared to a diseased valve or a prosthetic valve, a repaired mitral valve has a lower risk of becoming infected.

laura2: I don't have any of those other problems. I do have some kind of "ring" that they implanted, and carry an endocarditis risk card. So, can I continue the probiotics?

Dr__Pettersson: Most repairs of the mitral valve includes a ring support of the annulus. The risk of the repaired valve getting infected is still lower than having a prosthetic valve or a diseased valve which has not been operated on.

Mitral Valve Surgery

MWindt: Is there any existing technology for minimally invasive mitral valve repair, rather than opt for an elective 2nd open heart surgery?

Dr__Pettersson: Usually not. Re-repairing a mitral valve is more complex procedure than doing a primary repair. It is a two step procedure, taking down the original repair; understanding the pathology as modified by previous surgery; and re-repairing the valve. Most of the time, this has to be done by a conventional operation since there is scar tissue making a minimally invasive procedure less safe and need for best possible exposure.


adorsey: I was diagnosed in late 2006 (after being misdiagnosed for many months) with a left atrial myxoma. It was robotically removed - I was told it was "unlikely" hereditary but go for echos almost annually. Is this a type of congenital heart disease?

Dr__Krasuski: Tumors generally occur when there is a genetic problem in an individual cell in the body, which then replicates quickly forming a mass. Most of these genetic flaws occur spontaneously later in life and are not felt to be inherited. It is possible, however, that having certain flaws genetically can set the stage for such problems. These flaws can be inherited. Myxoma is one of the tumors that can have an inherited pattern and is seen as part of several genetic syndromes including Carney complex, LAMB syndrome and NAME syndrome. There are a number of problems in each of these syndromes with a number of different organ systems in your body being affected. If you are otherwise healthy, it seems unlikely that you have one of these syndromes. IF there is any question, however, seeing a geneticist can be helpful.

Patent Ductus Arteriosus (PDA)

Chloe40: I am a 40 year old female just diagnosed with PDA - can that be closed with a non surgery procedure?

Dr__Krasuski: Catheter closure of Patent Ductus Arteriosus (PDA) is the gold standard of management. For small PDAs we typically use coils to close them. For larger PDAs the Amplatzer ductal occluder has been shown to be quite successful. The procedure takes 1 - 2 hours and the patient is typically discharged on the following day.

Deciding which patent ductuses should be closed is a little bit more complicated. In general if the left ventricle or left atrium are dilated, then the shunt is likely significant. Alternatively, if pulmonary hypertension is present and it is mild, closure is also likely to be beneficial. Some people believe that all ductuses should be closed to reduce the risk of infection (endocarditis) but this would be considered controversial.

Patent Foramen Ovale (PFO)

GlennaC: I am a healthy forty five year old woman who had a minor stroke last year. after the million dollar work up I was diagnosed with PFO. They placed me on aspirin 325mg qd and pravachol to lower my cholesterol although it was only 200. They also told me I had a floppy septum. After taking the aspirin for nearly a year I am full of bruises but if it prevents another stroke I am not complaining. My question is do I need 325mg? should I consider having the PFO closed? How does the floppy septum play a part? Obviously the thought of another stroke frightens me and I would like to do all I can to prevent it. In this situation diet and exercise will not help so I feel like it is out of my control. Thank You Glenna

Dr__Krasuski: This is an area of extreme controversy at this time. There is epidemiological data that suggests that PFO closure may be better than medical therapy but the only randomized study called CLOSURE1 showed no difference between medical therapy and closure in patients with a first stroke and PFO. The floppy septum that you referred to is often referred to as an atrial septal aneurysm. Data from some studies suggests that PFOs in the presence of atrial septal aneurysm have a slightly higher risk for recurrent stroke than PFO without septal aneurysm.

Dr__Pettersson: In the operating room surgeons occasionally see small clots in the folds of the atrial septum being as substrate for the possibility of embolus and stroke. In the end, it is real issue but very uncommon.

Dr__Krasuski: For medical therapy we know from the CLOSURE study that there was no difference in events between aspirin and Coumadin and we also know from studies in patients with atrial fibrillation that 325 mg of ASA has a protective affect. The data with a baby ASA is less clear. So - continue 325 mg of ASA. Should there be any recurring neurologic events; most cardiologists would recommend closure of the defect as this would be suggestive of a failure of medical therapy.

AlyseM: I am 24 Years old and have been diagnosed with Factor V Leiden & a PFO. My sister at the age of 19 had a stroke because of a clot (due to the FV Leiden) that traveled thru the hole in her and then to her brain. Her hole was about the size of a quarter. She had an Occluder implanted to close the PFO and she is on warfarin for the rest of her life. My maternal grandfather had a pulmonary embolism in his late 30s and DVT in his 50s, one of his brothers died at 28 because of a blood clot, and some of this other family members have had blood clotting issues. I have had a miscarriage due to a blood clot in the Umbilical cord and I was lucky enough to have 1 successful pregnancy before I was diagnosed. I would like to have my PFO closed to dramatically reduce my risk of stroke and to avoid all the other complications. I see the struggles my sister has due to her stroke and I would like to be proactive and not reactive. Do you think I’m a candidate for a robotic assisted PFO closure?

Dr__Pettersson: I do think you are a candidate for closure of your PFO with catheter. Robotic closure is not necessary.

Dr__Krasuski: As much controversy as there is in closing PFO in a patient with a stroke, it is far more controversial in closing a PFO without history of stroke. Your circumstances certainly merit more discussion and weighing of the risks and benefits before making such a decision. It is important to note that any procedure catheter or surgical - can have complications.

DeborahFLA: When do they decide to do PFO closure by intervention, robotic surgery or surgery?

Dr__Krasuski: As mentioned previously this is a controversial topic. Most of us would agree that if a patient is on medical treatment (aspirin or Coumadin) and has a second neurologic event, an intervention should be performed. Ideally if a patient has a first neurologic event we should enroll them in one of the clinical studies examining the role of closure. In general, if closure of the PFO is decided upon, and no other heart lesions need to be repaired, catheter closure is preferred. If other surgery is necessary, then robotic or traditionally less invasive surgical techniques should be used if possible.

Intracardiac shunt

NJ24719: My mom was told she has an intracardiac shunt. What does that mean?

Dr__Krasuski: An intracardiac shunt is a communication from one side of the heart to the other. Examples of this include atrial septal defects; ventricular septal defects; and patent foramen ovale.

Scimitar Syndrome

Marla: My 34 year old daughter was diagnosed with Scimitar Syndrome 6 months ago. Per her heart cath, her shunting is 2.3. During the heart cath there was a coil and two plugs placed on an anomalous artery originating at her aorta and attached to her right lung. I have two questions.
1. My daughter went to the ER twice complaining of chest pain and difficulty breathing. Her chest pain, including pressure and jaw pain, have been constant over the last 6 months, even before her diagnosis. The docs have been telling her that pain is not a normal symptom of SS and they cannot help her with it. In addition, she has had constant pain in her back on the right side, under her ribs which also began before her diagnosis. This is the same area where the coil and plugs were placed during the heart cath three weeks ago. Are you familiar with other patients who have complained of pain with SS? Can you explain what may be causing her pain?
2. How many SS patients does your clinic typically treat in a year and how many of those are adults? Of these patients, approximately how many surgeries do you perform?

Dr__Pettersson: Scimitar Syndrome means that patient has a common return vein from the whole right lung to the lower cava vein immediately above the diaphragm. This means that the blood from the right lung is going back to the right atrium and is only recirculated in the lung circulation instead of draining to the left atrium which would be normal. A shunt of 2.3 means that the lung recirculation is 2.3 times systemic circulation - overworking the right side of the heart.

The second issue - is an extra artery from the aorta to the right lung. That is an abnormal artery and could be the only or most important blood supply to one part of the lung which would then be called a sequestered lung. This sequestered lung is also not contributing to additional blood oxygenation and gas exchange. There is an association between scimitar and sequestered lung although most of the time, the scimitar is an isolated abnormality.

Dr__Krasuski: Left unrepaired, the large shunt can result in heart failure, arrhythmias, and pulmonary hypertension. The latter condition can occasionally result in chest pain. It is possible that this could explain your daughter's chest pain. In general, the situation you describe should be surgically repaired to prevent these complications. Ideally you should be evaluated be an experienced center.

mbeeson: How many patients do you treat each year with Scimitar Syndrome? Of those, how many are adults? How many SS surgeries do you perform each year on adults and children?

Dr__Krasuski: Scimitar syndrome is a rare condition that some institutions see one in a decade, but in our experience we see 2 or 3 a year and perform surgery on a couple.

Dr__Pettersson: Patients need to be further evaluated for surgery. Additional information including the size and location of the vessels is important in determining the risks and benefits of surgery.

Adult Congenital Heart Disease and Pregnancy

RachelK: I had transposition corrected when I was 9 years old. Now I am 32 years old and thinking of starting a family. 1) Do you recommend genetic counseling first - can my children have this and 2) is my heart strong enough to have a baby. I feel fine.

Dr__Pettersson: There are two types of transposition - one where only the arteries are transposed and one where both the arteries and the ventricles are transposed resulting in "congenitally corrected transposition". The age of correction and wording of your question doesn't allow any conclusion to what you actually had and what procedure you actually had. That needs to be sorted out before answering question #2.

Dr__Krasuski: Any patient with moderate or severely complicated congenital heart disease should see a congenital heart specialist and possibly a high risk obstetrical before conceiving. We often involve geneticist as they can provide better data on the risk to the fetus.

In general - the risk of a child being born with congenital heart disease is 1%. If one of the parents has a congenital heart lesion that risk increases to about 3 percent. For some conditions, the risk is greater.

The assessment of a congenital heart patient who wants to get pregnant involves collection of various information. In your case, we would need to know the type of surgery you had; the status of the repair; and how well the pumping chamber to your body (systemic ventricle) is holding up. It is possible that the stress of pregnancy can lead to further deterioration of this chamber and therefore you should be closely followed throughout your pregnancy. In the last three months we have had 7 patients with complex congenital heart disease successfully give birth in our new special delivery unit. This is very exciting!

Adult Congenital Heart Disease and Atrial Fibrillation

sharon23: Hi. I have had pulmonary atresia since birth. I have had several surgeries and have atrial fibrillation requiring multiple cardioversions and medications. Can this be cured?

Dr__Pettersson: We need information about how well your pulmonary atresia and other defects have been repaired and how well your ventricles and valves are functioning and the dimensions of the chambers of your heart to decide how likely we are able to treat and possibly cure your atrial fibrillation. The surgical procedure to treat atrial fibrillation is a MAZE procedure which actually divides the atrial wall into maze- like corridors which do not allow re-entry circuits to form.

Dr__Krasuski: For this operation to be successful, the atria should not be too enlarged nor should the atrial fibrillation be to long standing. This procedure is less likely to be successful if you have other residual cardiac problems. We recently examined the success of catheter ablation of atrial fibrillation in patients with congenital heart disease. Though the results are slightly less successful than in patients without congenital heart lesions, it appears that the procedure is still beneficial. We have a lot of options to treat atrial fibrillation including surgical; catheter ablation; and new antiarrhythmic agents. It is an exciting time in the management of this condition.

Reviewed: 09/12

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