Wednesday, June 9th, 2010 - Noon
Richard Krasuski, MD
Director of Adult Congenital Heart Disease Services and a staff
Staff cardiologist in the Section of Clinical Cardiology
Miller Family Heart & Vascular Institute
Tomsich Family Department of Cardiovascular Medicine
Congenital heart disease is a type of defect in one or more structures of the heart or blood vessels. Congenital heart defects may be diagnosed before birth, right after birth, during childhood or not until adulthood. It is possible to have a defect and no symptoms at all. About 500,000 adults in the United States have grown into adulthood with congenital heart disease. This number increases by about 20,000 each year. Most adults with congenital heart disease should be monitored by a heart specialist and take precautions to prevent endocarditis (an infection of the heart) throughout their life. Richard Krasuski, MD answers your questions about diagnosis and treatment of adult congenital heart disease.
Cleveland_Clinic_Host: Welcome to our Online Health Chat with Dr. Richard Krasuski. We are thrilled to have them here today for this chat. Thank you for being here Dr. Krasuski. Let’s begin with the questions.
Dr__Richard_Krasuski: Thank you for having me.
Ventricular Septal Defect (VSD)
JackR: Do you have any suggestions for the treatment of a large VSD? When we closed the shunt, the hemodynamic will change a lot. Should we open a palliated window in the atrial septum? Some doctors from the other country recommended this procedure? What are your suggestions for the treatment of a large VSD?
Dr__Richard_Krasuski: The condition that you refer to is likely the development of pulmonary hypertension from a large vs. If this becomes advanced we refer to this as eisenmenger's syndrome. When this develops it is generally considered a contraindication to surgery. If the pulmonary htn is not yet advanced, or it can be demonstrated that it is at least partially reversible, then the defect may be able to be closed.
In some cases, there may be shunting in both directions across a VSD and it is therefore working as a "pop-off" valve. In such cases, the idea of creating a small atrial septal defect may provide for a pop-off valve once the VSD is closed. Though we discuss this frequently, we rarely do this.
In general, you need a very careful hemodynamic evaluation with some type of vasodilator challenge to know whether the defect is fixable. If the pulmonary pressures are only mildly elevated, or the lung vessels are responsive, then surgery should be possible. In such cases, creating an ASD is generally not necessary.
Atrial Septal Defect (ASD)
LonaJ: My question regards the safety of using an occluder with metal in it to close an ASD in an adult patient who has a nickel allergy. Are there currently any occluders that you use that do not contain any nickel or metal? Thank you.
Dr__Richard_Krasuski: Nickel allergies are quite common in the general population. Despite this fact, and the fact that I have occluded well over 300 defects, I have only had one patient with what we felt was a systemic reaction to nickel. In this patient, we treated with an antihistamine for a period of time before the symptoms completely resolved. That being said, I have had several patients turn to a surgical repair because of fears of nickel allergy.
Nickel allergy is generally tested by placing nickel on the skin. The cells that react against the nickel are present in the skin but whether these cells play any role in allergic reactions in the blood stream is unclear.
There are 2 occluders which have been approved for closure of ASD and include the amplatzer septal occluder and the helex device. Both of these devices contain nickel though the helex contains a far smaller amount. The starflex device is another device that has been utilized for ASD closure and has an amount of nickel that is less than the amplatzer and slightly more than the helex. Thus - there are no current approved percutaneous options if one is trying to avoid subjecting the patient to nickel. Of interest there is a study published a number of years ago that measured nickel levels in patients getting amplatzer devices. While it appears that nickel is present for the first few months, the levels decrease and are almost undetectable at the end of 6 months. This is probably related to a layer of tissue covering the device or what we call endothelialization.
jillrn: What determines if closure of ASD should be done interventionally or surgically?
Dr__Richard_Krasuski: Percutaneous closure is currently only indicated for patients with secundum atrial septal defects. Thus patients with primum defects or sinus venosus defects are not candidates. Exclusions for device closure include the presence of an anomalous pulmonary vein, or the lack of adequate septal rims to be able to properly hold the device. There is also a limitation based on the size of the defect, though holes up to 4 cm in diameter have been successfully closed. In my experience if there is concurrent heart disease, such as coronary disease that is significant or if atrial fibrillation has occurred, a surgical repair may be the best treatment option. In general percutaneous closure has become the gold standard of repair for most secundum septal defects.
janetK: Are these devices successful as far as long-term outcomes?
Dr__Richard_Krasuski: There are a number of studies which have followed patients several years after device occlusion with results comparable to operative repair. Since these devices have only been around for less than 20 years, long term follow up (greater than 10 years) is not available for a large number of patients.
Patent Foramen Ovale (PFO)
Tigerhawk: 52 year old male experienced a TIA - which led to the discovery of a small PFO with no other obvious health issues - what is the best way to manage this condition ( current instructions are 325mg aspirin daily) and when should closure be considered.? Would sending records be advisable for a second opinion?
Dr__Richard_Krasuski: This is probably the most controversial topic that I face on an almost daily basis. Patent foramen ovale (PFO) is an extremely common lesion in the general population, occurring in 1 out of 4 people. In young patients, experiencing a stroke, PFO is present in over 50 percent. Thus there clearly is an association between PFO and stroke but causation is less clear.
On the big picture level, one has to presume that a blood clot developed and crossed the PFO to reach the brain and cause a stroke. Thus the therapeutic options in such patients include medications for the prevention of blood clots such as aspirin, theinopyridines (such as plavix), or anticoagulatants (such as coumadin). Another option is closure of the PFO with either a percutaneous device (a procedure done through the skin in the cath lab) or an operative repair. Finally there is the option of randomization into a clinical study in which case you may end up with either medical treatment or catheter closure.
Which strategy is most optimal is not currently known. A trial called the CLOSURE I study will be presented at the American Heart Assoc sessions in November which should help us answer this question. This study compared the implantation of a starflex device to medical therapy in patients with a first neurological event. I would certainly encourage you to seek further opinion about your therapeutic options.
terry: I have a PFO and had a DVT a couple months ago. Could the clot in my leg be related to the PFO in my heart? I am wondering if I should come to the Clinic for a second opinion as my doctor said not to worry about this. How do you make an appointment, Does my doctor need to refer me - how quick can I be seen?
Dr__Richard_Krasuski: I would have to agree with your physician that the PFO is unlikely to be related to the DVT. As mentioned above, PFO is a very common lesion. The presence of a PFO however does increase your risk for an arterial embolism when a venous thrombus develops. Thus adequate anticoagulation with coumadin is essential in your case. We are certainly glad to help in any way that we can and would be delighted to see you if you like. You do not need a physician referral unless your insurance requires you to have one. Please call 866-289-6911 if you would like to speak to a nurse or for more information to come to Cleveland Clinic.
Endocardial Cushion Defect
jillrn: What is endocardial cushion defect?
Dr__Richard_Krasuski: The endocardial cushion is the embryonic structure which helps to form the valves and the layer of tissue which separates the heart into four chambers. When an abnormality occurs during development, you can end up with an endocardial cushion defect. Other terms for this defect are AV Canal defects or primun atrial septal defect. Depending on where the defect is you can have an ASD, a VSD, or both. Cleft mitral and tricuspid valves are also commonly seen. It is quite common to have this defect repaired during childhood and then to return with problems related to the mitral valve later on.
In general we approach such patients similar to the way we approach patients with isolated regurgitation of the mitral valve. If there is evidence of heart chamber enlargement, the development of pulmonary hypertension, or the presence of rhythm disturbances, surgery should be performed in expeditious fashion.
jillrn: My daughter was born with endocardial cushion defect. which included ASD, VSD and a deformed mitral valve. By the time she had surgery at age two; the VSD had closed on its own. The ASD was patched and the mitral valve was "stitched". Now 25 years later, she has a moderate to severe mitral valve leak. Her heart strength is "4" out of 5. She is asymptomatic. Do you think it would be worthwhile at this point to have the echo results sent to Cleveland Clinic for evaluation of the decision to not do surgery at this point? Or is it generally agreed upon that for young, asymptomatic adults, holding off surgery as long as possible is the standard of practice.
Dr__Richard_Krasuski: It is hard to comment on your daughter's case without knowing about these additional factors. In generally, if the leaking is severe, even if it is asymptomatic, we recommend operative repair of the cleft. If your treating physician is less familiar with this type of anatomy, we would certainly recommend getting a second opinion.
Valve Disease: Pulmonary Valve
MichelleW: As an infant (10 days old) I had surgery to close my PDA, PFO, and remove my stenotic pulmonary valve. I am now 34 and would like to know what criteria would indicate I should have my valve replaced. How do you determine the right time? Is it possible I will never have to have it replaced. Can I do anything to prevent the need for valve replacement? Would pregnancy be safe without a valve? I follow with a cardiologist, and have sought additional opinions, and get very mixed messages.
Dr__Richard_Krasuski: Much of what we know about implantation of pulmonic valves in patients with congenital heart disease comes from data derived from patients with tetralogy of Fallot. Whether this also applies to patients with pulmonic valve stenosis treated with valvotomy and resulting in severe regurgitation is less clear. The indications for valve implantation include a progressive decline in exercise tolerance, a progressive increase in size or function of the right ventricle as assessed by cardiac MRI or echocardiography and electrocardiographic findings suggestive of electrical problems. In general this is a tricky decision and it can vary from patient to patient. In general I like to get a cardiac MRI and follow this over time looking at the right ventricular size and function - if it enlarges or worsens I am likely to send the patient to valve implantation. Of note we are in the early stages of implanting valves through catheters and if this proves to be successful we may find ourselves recommending valve implantation at an earlier stage.
Medically, nothing has been shown to reduce the leaking or the progression of the heart dysfunction. We just completed a study looking at the effects of pulmonary vasoactive medications that we have not yet published. It is too early to tell whether this may be a strategy to consider in the future.
Pregnancy is generally safe in such circumstances, though I would normally like to get a better feel for the size and function of the right ventricle and any associated problems before I recommend it. You should definitely seek consultation with a congenital heart expert and a high risk obstetrician before getting pregnant, however.
Valve Disease: Mitral Valve
jillrn: Also, I read that for mitral valve re-repair, one has to find a surgeon that has experience with the particular deformity of the valve. Lastly, is there any chance she would be a candidate for the robotic assisted, mini-thoracotomy method or is that reserved for more straight forward cases ?
Dr__Richard_Krasuski: The experience of the surgeon is absolutely critical in such cases as the surgical technique is far from simple. Depending on the material used during the initial procedure, the original repair may need to be updated and this can necessitate a much more technically challenging procedure. Ideally it is best to repair rather than replace such valves and surgical experience clearly drives this possibility. In general, such cases exceed the limits of robotic repair.
roullac: Dr Krasuski, I had successful Mitral Valve repair - sternotomy - at your center due to Severe Mitral regurgitation and persistent left superior vena cava. The operative findings were: Myxomatous mitral valve disease with a prolapse of the P2 segment of the posterior leaflet and a partial calcification of the base of the posterior leaflet as well as the posterior aspect of the mitral annulus. I was an asymptomatic patient and otherwise healthy (apart from cholesterol). First I would like to ask whether I have inherited this disease from my father who had surgery for endocarditis, age 68? I would also like to know whether I can now have the same life expectancy as anyone else (a healthy person). What is the likelihood of me needing to have another surgery to replace my valve given the fact that there seems to be some calcification (as described above).
Dr__Richard_Krasuski: First of all mitral valve prolapse (MVP) is an extremely common problem affecting the general population with a prevalence of 1 -2 %. There clearly are kindreds in whom mitral prolapse appears more commonly, so there is undoubtably a genetic influence. It is certainly possible that your father also had MVP and this was the cause of his endocarditis.
You had what sounds like a very successful operative repair and there is a failure rate, however, that has been estimated at approx. 1% per year for the first 15 - 20 years, even at the best medical centers in the world. It is certainly possible that you may need further surgery in the future. In general, it appears, that patients with successful repairs can lead normal active lives with lifespans that are probably no different from the general population.
Arrhythmia – Abnormal Heart Rhythms
Lynnh: At what point would an ablation be necessary for a heart patient? I have a pacemaker/defibrillator and keep going "into A-Fib".
Dr__Richard_Krasuski: In general, ablation has been reserved for patients with atrial fibrillation who have failed multiple anti-arrhythmics. It is interesting to note however, that multiple studies are currently examining the role of ablation therapy much earlier in the disease process. In fact the NIH is currently beginning a trial called CABANA which will compare early ablation vs standard therapy.
We have examined the outcomes of ablation in the congenital heart disease population and found results quite similar to other patients with structural heart disease. This, it is reasonable to assume that we will be moving toward the use of ablation much earlier in the presentation of patients with atrial fibrillation. It is important to recognize that even though the results of ablation are quite favorable, they come with some risk including a slight risk of stroke and bleeding complications.
Lynnh: How would a recent (1 year) stroke effect having an ablation done for a heart patient in and out of A-Fib?
Dr__Richard_Krasuski: The ablation procedure requires the use of blood thinners (heparin) to prevent clotting complications during the procedure. If the stroke is recent, the use of heparin may increase the chance of converting a "bland" stroke into a hemorrhagic stroke. In general, such risk is mainly limited to the first month after the event and anticoagulation is generally safe afterward. I would recommend consulting with your electrophysiologist for further details.
All patients undergoing atrial fib ablation at the Cleveland Clinic undergo a transesophageal echocardiogram (TEE) to insure that there is no blood clot present in the heart. The utility of this procedure would be even higher in a patient who has experienced a recent stroke as the likelihood of finding such a clot would be higher.
RosemaryJ: my grandson had a repair of aortic coarctation when he was 2. he is now 19 and has aortic valve bicuspid leak and high blood pressure - on a medication for this. I am wondering what he should be doing in regards to activity and wondering what type of diagnostic testing and treatment options he has available
Dr__Richard_Krasuski: The development of high blood pressure is quite common in patients after coarctation repair even when successful. I still recommend MRI scanning of the aorta to insure the absence of re-coarctation in every such patient. Re-coarctation occurs in at least 15 percent of patients and can be easily treated with either balloon dilatation or stenting. This treatment has become the gold standard for re-coarctation.
Bicuspid valve occurs in up to 80 % of patients with coarctation. It can be complicated by either narrowing (stenosis) or leaking (regurgitation). Treating blood pressure is absolutely essential in patients with regurgitation. Some studies have suggested that treatment of blood pressure with medications that relax the peripheral vasculature may delay the time to surgical valve repair or replacement. Thus, aggressive treatment of blood pressure in your grandson is essential.
Visit with Adult Congenital Cardiologist
ChelW: The closest Adult congenital cardiologist is 3 hrs away. It has been suggested that I consider following either annually with a pediatric cardiologist or follow with a local adult cardiologist annually and travel to an adult congenital specialist every 5 years. Do you have an opinion on this? I felt as though I should have someone closer than 3hrs who is familiar with me in case I require local care urgent/emergently.
Dr__Richard_Krasuski: This is a very common scenario that I face with patients. I always recommend that they have a physician (cardiologist) that is close to their homes and that we work in concert with one another. Depending on the level of complexity of the patient, I see patients either once a year, every other year, or every 3 years. I frequently request that the echocardiograms or MRIs be sent to my office for my review in the interim however.
Transposition of the Arteries
starrynight12: I have transposition of the arteries congenital but just found out about it at 30 years old. What is the normal treatment for this? Do you have surgical correction?
Dr__Richard_Krasuski: There are two types of transposition of the great vessels. There is the d-type and l-type.
In d-transposition, the atrial are connected normally to their respective ventricles - thus the right atrium is connected to the right ventricle and the left atrium is connected to the left ventricle. Unfortunately the right ventricle is attached to the aorta instead of the pulmonary artery and the left ventricle is attached to the pulmonary artery instead of the aorta. In such cases, a communication needs to be present at the atrial, ventricular or great artery level (a shunt) to allow survival through infancy. These patients generally require surgery during childhood.
The second type of transposition, the l-type, also known as congenitally corrected transposition, has slightly different anatomy. In this case, the right atrium connects to the left ventricle and then connects to the pulmonary artery. The left atrium connects to the right ventricle and then the aorta. Thus, your venous blood goes to the lungs and your oxygenated blood goes normally to the body. Unfortunately, the pumping chamber to the body is a morphologic right ventricle. It is therefore not properly designed to pump blood against systemic resistance (high blood pressure). As a result, this ventricle may eventually begin to fail and the valve which comes with the ventricle may start to leak. This same process can occur with d-transposition patients who have undergone a Mustard or Senning procedure. In such patients, we treat with medications to try to make the heart stronger and prevent complications. In some cases, the leaking valve can be repaired or replaced and improve how the patient feels. Eventually patients with this type of anatomy often require heart transplantation. It certainly sounds as though you are doing quite well clinically and are far away from needing something so drastic.
Dr__Richard_Krasuski: Thank you for having me today.
Lynnh: Thank you.
Dr__Richard_Krasuski: It is our pleasure.
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