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Adult Congenital Heart Disease (Drs. Krasuski and Pettersson 2/16/11)

Wednesday, February 16, 2011 - Noon

Gosta Pettersson, MD, PhD
Vice Chairman,
Department of
Thoracic and Cardiovascular Surgery
Miller Family Heart
& Vascular Institute

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

Description

It is estimated that approximately one million adults 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.


Cleveland_Clinic_Host: Thank you for joining us Dr. Krasuski and Dr. Pettersson, let's begin with the questions.

Dr_Richard_Krasuski: Thanks for having us.

Dr_Gosta_Pettersson: Let's begin with the questions.


Adult Congenital Heart Disease—General Questions

E_65584: Adult Congenital Heart Disease - I've been fortunate to be in good health and have NOT been evaluated for/diagnosed with congenital heart disease. But several members of my family have had problems (heart valve replacements or repairs, high blood pressure). What kind of symptoms should I be reporting to my doctor?

Dr_Richard_Krasuski: The incidence of congenital heart disease increases from 0.8% in the general population to about 3% in those folks who have family members with congenital heart lesions. It is hard to tell from your information whether any of these problems in your family members were actually congenital (present from birth) or acquired. In general heart disease can present with a number of different symptoms which include chest pains (usually with exertion), shortness of breath (again, usually with exertion), palpitations (feeling that your heart is beating abnormally), shortness of breath when you lie down, abnormal swelling in the ankles and increasing fatigue. Many of these problems can occur to a smaller scale in folks without heart problems, but the more of these that you have the greater the chance that something is wrong.

Janet: I have a congenital heart condition and I am a current patient of CCF. I was wondering how many babies are still born with heart conditions. So many new treatments and wonderful things have happened since I was born ... my hope was that there would be far less babies than ever going through this. (I was born in 1960 ... thankfully just after the physicians at CCF created the catheterization.) (My physicians were Drs. Sones, Shirey/Groves ... Drs. Moodie/Gill in 1981 and now Dr. Prieto)

Dr_Richard_Krasuski: The number of births affected by congenital heart disease is just under 1%. This number does not appear to have changed much as it is probably driven by genetics. I agree with you, that the advances that have occurred are just amazing.

Megan: Is surgery is the only treatment for adult congenital heart disease?

Dr_Gosta_Pettersson: Like for all other diseases of the heart, most conditions can be treated medically and an increasing number of traditional surgical problems can be treated less invasively. These less invasive procedures often mean compromises. Improving or solving one problem leaves a less important problem behind. Today, all patients are carefully discussed between the cardiologist and the surgeon and we try to decide the best treatment for that individual patient.

The decision making for these patients is often difficult and complicated and requires a lot of experience. Patients with more complex congenital heart disease have often undergone one or several operations in childhood before presenting with new problems as adults. So therefore it is important to go to an experienced center with these problems.


Septal Defects: ASD, VSD, PFO

dbec: I had OHS to repair multiple ASDs. It is at all possible that a surgical repair could leak or tear and I would require further surgery in the future?

Dr_Gosta_Pettersson: Possible, but very unlikely.

concerned_mom: Dr Krasuski did a PFO/ASD closure on an 18 year old patient that began going blind due to a blood clot, and now, less than 3 years later, she is married, and now pregnant.... there had been mention that her pregnancy may be high risk, needing shots through the pregnancy in her abdomen... can you please talk about pregnancy and congenital heart disease?

Dr_Richard_Krasuski: Great to hear that your daughter is doing so well. I always recommend that any patient with a history of congenital heart disease be re-evaluated by a cardiologist when she gets pregnant. The decision about whether to do heparin injections is challenging and should only be done with a hematologist or vascular medicine specialist being involved. Because we closed your daughter's heart defect, her work-up for a hypercoagulable state (clotting disorder) was likely to have been negative (I always check this to avoid the risk of a clot forming on the device) and her overall clotting risk is likely to be low. You are correct, however, that the risk increases during pregnancy. There are a lot of changes that occur to the body during pregnancy—the blood volume increases, the vessels get softer, there is anemia, etc. It is just an incredible process and certainly one in which a cardiologist should be involved in if there is a history of heart disease.

heartmatters: I had surgery at age 54 to repair partial AV septal defect w/ mod-severe tricuspid regurg and mild mitral regurg. There was a patch closure of ostium primum and secundum; mitral cleft closure and tricuspid valvuloplasty. All is well. I have only RBBB, LAFB, and first degree AV block remaining. Are there any particular areas I should be aware of in terms of aging and what to watch for, given this?

Dr_Richard_Krasuski: The combination of electrical abnormalities noted on your EKG often referred to "trifascicular" it places you at some risk for development for complete heart block. This would require a pacemaker. You need to have regular follow-up with your physician and be closely monitored. If you feel lightheaded or have excessive fatigue you should contact your physician sooner than planned.

kfs: I was just diagnosed at the age of 57 with a Mobile Interatrial septum and a PFO with a large right to left shunt. I am scheduled to have a consult at the CCF main campus followed by a TEE but not until April 29th.. My concern is because both my grandmother and mother died of strokes I have a great deal of anxiety in the waiting. In the interim, I will try to get into to see a cardiologist just to answer some questions.. My test results stated that my left ventricular diastolic function is consistent with abnormal relaxation(stage 1); is that referring to heart failure stage 1? Should I be taking a baby asp 81 mg daily until April 29th?

Dr_Richard_Krasuski: A foramen ovale is a normal heart structure that is present to allow blood to cross from the right to left structure of the heart therefore bypassing the lung before you were born. At around the time of birth this structure normally closes. In about 25% of people this does not close and results in what we call a patent foramen ovale (pfo).

In a very small number of people there can be problems associated with the pfo. These can include stroke, migraine headache, and a condition we refer to as platypnea-orthodeoxia which refers to low oxygen blood going from the right side of the heart and mixing with the blood that is higher in oxygen on the left side. If you have never had these problems we would refer to this as an incidental pfo. Generally I recommend taking a baby aspirin to prevent stroke although there is no evidence that it actually reduces stroke, but at least it makes sense.

With your family history of stroke, a baby aspirin makes complete sense in any case. I would not consider this a medical emergency and I believe the risk without having any of these problems is quite low.

Dr_Gosta_Pettersson: The fact that it is a mobile septum doesn’t really change these risks or indication for treatment.

KateY: I had a closure device recently to fix my ASD. How long does that last. Are there any restrictions in activity? Should I take antibiotics before procedures?

Dr_Richard_Krasuski: There are two different devices which have been approved for device closure for ASDs, the Helex device and the Amplatzer septal occluder. These are implanted in the cardiac catheterization labs and patients are typically discharged on the following day. We usually recommend that patients refrain from lifting over 20lbs for 3 days after the procedure, but otherwise there should be no physical restrictions. The guidelines recommend 6mos of antibiotics during dental procedures, but I recommend this be performed a full year after the procedure. The device itself is designed as scaffolding for the body to grow tissue on and should last forever.

Dr_Gosta_Pettersson: We typically recommend the use of aspirin and clopidogrel for 6mos after the device implantation to prevent blood clots from forming on the device while tissue overgrows the device.

kfs: In addition, I would like to ask, if my thoracic echo would be definitive in gauging the size of the PFO and the exact location on terms of which of the secundums? Also the saline contrast was done to confirm the PFO.. would this echo delineated whether the PFO had a residual flap, or if it was permanently opened allowing the deoxygenated blood in the right atria to mix at will whenever it wanted to shunt over to the left atria carrying with it the potential to stroke?

Dr_Richard_Krasuski: For clarification purposes, a pfo is an abnormality in the atrium septum. It is best to think of these septal abnormalities in tow different flavors: an atrial septal defect is a true hole in the septum which allows blood to pass from the left to the right atrium. This results in complications which include right heart enlargement, pulmonary hypertension and increases the risk for the development for rhythm disturbances like atrial fibrillation.

A patent foramen ovale is a flap of tissue that works like a little trap door allowing blood to cross from the right to the left side. As such, it should not cause enlargement of the right heart or the other problems listed above. The best way to tell between these two lesions is to perform a special echo which is performed from the esophagus: trans-esophageal echo.


Heart Valve Disease

Sherry: Are leaky valves considered a congenital heart defect and is there anything that can be done aside from surgery? Is it something that should be corrected? If so, how long to wait before having it fixed?

Dr_Richard_Krasuski: The heart has four valves which are designed to facilitate blood flowing in one direction through the lungs and then back out to the body. The two problems that can occur with heart valves is abnormal narrowing that prevents blood flow from passing normally or leaking which sends blood back in the opposite direction and makes the heart work harder. In general, patients with narrowed valves will present with problems which include shortness of breath with activity, chest pains and fatigue.

These symptoms usually tell us that something serious is going on and that an intervention needs to be performed. The situation is much more challenging in patients with leaky valves. If we wait for symptoms to develop permanent damage to the heart can occur. Ideally, we like to intervene before this damage occurs. We can follow this with the use of sonograms of the heart that we call echocardiograms.

Dr_Gosta_Pettersson: Valve surgery is most often performed on the left sided valves of the heart: the mitral valve and the aortic valve. The right side of the heart works with much lower pressure and these valves are less often affected. Replacement of the aortic and mitral valve surgically are very common procedures which are all performed with low-operative risks. Stenotic valves usually have to be replaced while there is a good chance of repairing a leaky valve. The possibility of repair has encouraged earlier surgery of leaky valves - this is particularly true for the mitral valve.

Dr_Richard_Krasuski: In many cases valvular heart disease is in fact congenital - the best examples are mitral valve prolapse and bicuspid aortic valve.

Dr_Gosta_Pettersson: In spite of being congenital many of these patients will not require any operations before they are adult or even late in life.

son13: I am 46 years old with BAVD. I've been treated for high blood pressure since my early 30's and have had several med adjustments due to the BP going high or low over the years. Is the BP problem likely related to the BAVD or should my cardiologist (and other specialists) continue to look for a cause?

Dr_Gosta_Pettersson: BAVD stands for bicuspid aortic valve disease. I would like to know how well the bicuspid valve is functioning: if it is leaky or stenotic. Patients with BAVD and hypertension have systolic hypertension related to a very leaky valve. The leak means that with each heart beat a large amount of blood is thrown out into the aorta. BAVD is also associated with the tendency for the aorta to enlarge and it is also associated with coarctation of the aorta and the patient with BAVD should be screened for these conditions. The screening usually includes an echo and CT scan.  The most serious aortic complication from bicuspid aortic valve disease is the dissection of the aorta.


Tetralogy of Fallot

Kuwait: Dear, My brother had an Operation in 1984 in USA when he was 3 years old with the following details: Preoperative Diagnosis: Tetralogy of Fallot Postoperative Diagnosis: Same Name of Operation: Total correction of tetralogy of Fallot And we brought him again for a check up in 1986 and the latest one in 1991After the operation, he was still having narrow pulmonary annulus, in 1991 they tried to solve it but it did not work because of his small age and the final decision was: Another operation is required but when he grows up enough after completing 25 years. Now he is 30 years old we are planning to have this check up in the United States and many people recommended Cleveland Clinic so what do you advice or recommend for us to start the treatment? by the way we are from Kuwait. Thanks and we appreciate your Support

Dr_Richard_Krasuski: We are glad to see him in the Adult Congenital Clinic. He will need an echocardiogram and a cardiac MRI for assessment and then I can see him afterwards.


Modified Fontan Procedure

Kallshan: I am 35 years old female- I have had 6 surgeries to fix my congenital heart defect. My last one was a Modified Fontan Procedure. I am wondering what the rest of my life will be like. Will I keep having surgeries? What should the follow up be like - just wondering if the care I am getting at home is the correct follow up?

Dr_Gosta_Pettersson: This is a good example of what kind of difficult problems physicians deal with adult congenital patients. Your specific question doesn't have a good answer as it requires a detailed documentation and cardiac anatomy. If you are doing well at this time there should be good possibility that you can stay that way. It is important for you to have regular contact with an experienced cardiologist understanding the specifics of Fontan.

Dr_Richard_Krasuski: Some of the problems which you could potentially experience include heart rhythm abnormalities, liver damage and potential weakening of the single pumping chamber in your heart. Some of these problems can be managed with medication or catheter (heart catheterization lab procedures) and others require more surgery. In a small number of patients with fontan anatomy, a heart transplantation is eventually required. At this point there is no easy way to predict which patients these are before the problems occur. So I would encourage you to be followed closely.


Transposition of the Great Vessels

jillrn: How many adults in their 20's & 30's do you operate on with Complex Transposition of the Great Vessels a year?

Dr_Gosta_Pettersson: There are two types of transpositions. One that we usually refer to the aorta and pulmonary artery are truly switched and take off from the wrong ventricles. The treatment of this condition today is arterial switch.

Dr_Richard_Krasuski: This surgery has replaced the atrial switch procedure. Which routed blood from the body to the left atrium and blood from the lungs to the right atrium. In this situation the patient is left with a right ventricle that is pumping blood to the body because the morphological right ventricle is not designed to pump in a high pressure system, in many cases the muscle weakens and its valve eventually begins to leak.

Dr_Gosta_Pettersson: There is another condition which is called congenitally corrected transposition. In this condition both the arteries and the ventricles are switched so the left sided ventricle is a morphological right ventricle giving off the aorta. These patients also face the same possible complications as the patients with regular transposition after the atrial switch. This means weakening of the right ventricular muscle and valve leakage. On an annual basis we treat 4-5 patients with one or the other type of surgery.

Dr_Richard_Krasuski: The most responsive to surgery are cases where the valve leak is severe, but the heart muscle function appears preserved. In these cases we may prevent further complications down the road and improve how patients feel. Unfortunately most patients get to us too late too be able to offer a surgical intervention and in some cases they may require a heart transplant as a consequence. Some medications which are traditionally used for heart failure may also benefit these patients and we typically start these after we asses the patient.


Arrhythmia

DanRobGolf: Will my WPW progress into something more? 61 yrs old with WPW always.

Dr_Gosta_Pettersson: "Wolf Parkinson White"(WPW) means that you have an extra conduction pathway from the atria to the ventricle this can result in very rapid heart for two reasons. One reason the atria has a very high rate that is conducted abnormally fast to ventricles of the heart, the second mechanism is that such a pathway can allow reverse conduction and allow development of re-entry circuit which can maintain rapid heart rate.

Dr_Richard_Krasuski: If you have been stable up to the age of 61 this problem is unlikely to get worse.


Mitral Valve Surgery

roullac: I had mitral valve repair (median sternotomy) 17 months ago; my mitral valve was successfully repaired and there is no regurgitation. During my last echo doppler though which was in December, my cardiologist said that, looking at the echo doppler, it appears that there is Paradoxal Movement of IVS. He feels that this is post surgery related and there is a possibility of blood collection behind the posterior leaflets. What would be your view on this? I am very anxious and worried at the moment as I believed that everything would be o.k and would have been able to get on with my life. What do you think would have caused this? Do I need to have any treatment for this, and if so what kind of treatment should I expect to have? Thank you

Dr_Richard_Krasuski: I wouldn't think that this would be causing abnormal blood collection or impacting heart function in anyway.

Dr_Gosta_Pettersson: Sounds very much like normal post-operative finding and there is no specific treatment for this.

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

Reviewed: 02/11


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