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Fri, Nov 22 1:33pm · CV Grand Rounds - The Fontan Operation: Unanticipated Consequences in Congenital Heart Disease

2019-11-15 CV Grand Rounds Fontan

The Fontan operation is a procedure applied to patients with a single ventricle that results in venous blood from the body being directed into the lungs.  It is common for a palliative procedure (shunt or pulmonary artery band) to have been required prior to the Fontan procedure.  The procedure is typically performed at approximately 3 years of age.

In the following video, Dr. Alexander Egbe, delineates the hemodynamics of the single ventricle, reviews the basics of the Fontan operation as well as explains the unanticipated consequences of the operation. He lists the following consequences:

  • Atrial arrhythmia, which in the event of a pulmonary embolism the absence of subpulmonary ventricle can have severe consequences and explains the research on DC Cardioversions as a form of acute arrhythmia management as well as other strategies, citing their effectiveness.
  • Thromboembolisms, explaining their difficulty to treat, due to balancing a tendency to clot and a tendency to bleed while answering multiple questions that arise from trying to balance those risk factors.
  • Lastly, liver disease due to low cardiac output while having high central venous pressure.

 

https://www.youtube.com/watch?v=ipiuOknvfhg

 

HELPFUL LINKS

Tue, Jul 30 1:34pm · The Mayo Adult Congenital Heart Disease Biobank in Congenital Heart Disease

MACHD Registry

Mayo Clinic is one of the pioneering centers for congenital cardiac surgery, and the Mayo Adult Congenital Heart Disease Clinic has cared for more than 10,000 patients since its inception more than 3 decades ago.  We leveraged the robust outpatient practice to create the Mayo Adult Congenital Heart Disease (MACHD) Biobank.  The purpose of this project is the collection, analysis, and cryopreservation of biospecimens from patients with congenital heart disease.  This purpose fits into the overarching mission of the MACHD research program which is ‘to improve the lives of people with congenital heart disease through in-depth understanding of the disease pathophysiology’.

The Biobank is part of a multi-faceted research infrastructure, and is designed to integrate seamlessly with the other existing databases

To learn more about the current research of the Congenital Heart Disease clinic, read this interview with Dr. Alexander Egbe.

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Fri, May 24 2:38pm · CHD May Research Update in Congenital Heart Disease

shutterstock_786243178

In mid-March, Dr. Alexander Egbe, along with Dr. William Miranda and Dr. Carole Warnes, went to and presented at the American College of Cardiology Conference. After the conference, Dr. Egbe took some time to discuss what was presented at the conference, the importance of the conference, as well as an overall update of congenital heart disease research since discussing the importance of research about CHD at the end of January.

When we last spoke, we discussed the importance of research for congenital heart disease. Since then you’ve attended one of the three major heart medical conferences. What was the name of this conference? Where and when did it take place?

The conference is the American College of Cardiology Conference, ACC, and it is the largest conference held in the United States.  The last one was in New Orleans and was held from March 15- 17. We had about 12,000 physicians and scientists in attendance.

 

What is the goal of conferences like ACC? Is it simply to get everyone together and share research across institutions?

 Yeah, so the importance of that conference is that the high-impact research that’s been done in the last 12 months is shared, and then people come to review and discuss it. That becomes a great environment to cross-pollinate ideas, about what to be done the next year.

The other thing is, it takes a while for research to make it into the mainstream where people actually know about it and use it. This conference has become a way to allow problems to be compared to something that is new. That will decrease the literacy period between the time the study’s done to the time it takes to be used in real life.

 

These conferences then are a way that the Congenital Heart Disease department can reach that goal of changing the way congenital heart disease is treated not only nationally but globally as well?

Correct. So congenital heart disease is a very small segment of this conference. This all of cardiology so you can’t attend everything. When I’m there, I kind of restrict myself to the congenital heart disease programs which generally run from morning till evening those three days.

 

You presented multiple research papers at this conference correct?

Correct we actually participated very actively this year just like every other year as we presented seven different studies.

The first study was about the management of atrial arrhythmias in patients with tetralogy of Fallot. Actually, all seven studies are about tetralogy of Fallot. Just to give you an overview, tetralogy of Fallot is the most common cyanotic heart disease in children. These children grew and became adults so it became the most common cyanotic heart disease in adults and is the most common cause of sudden death and heart failure. So trying to understand how to manage it and prolong the life of the patients is important. So what we’ve done in the last year is focus all our research attention in a 12-month period on this single diagnosis.

One of the things we worked on is arrhythmias which is atrial fibrillation and atrial flora, because these two arrhythmias are part of the reason why the survival of this population is low. The median survival is about 55 years, compared 75-80 years in the general population. What we found out in our study is contrary to popular belief that atrial fibrillation is not very common in this population, the study at Mayo showed that atrial fibrillation is the most common atrial arrhythmia in people with tetralogy of Fallot, and is the major driver for heart failure and for death. We found that if you send patients for rhythm control through ablation, the survival is better.

The second study that we did, was trying to come up with risk model, that would help us predict the patients who are at risk for death. It’s almost like having a crystal ball to try and predict the future, instead of waiting for it to happen. Looking forward with this mathematical formula can help us know who’s at risk. The goal then would be to do something about it, hopefully, to modify their risk. We came out with a comprehensive risk model, and this risk model has everything in it. The patient’s age, demographics, their heart function on echocardiogram, MRI, cardiac catheterization, all the information. So we came up with a risk core that allows you to type in a patient’s information and it will spit out their risk of death in ten years, and it will give you the things that are driving the risk. So then you’ll see somebody has valve obstruction or has very poor function of one of the pumps, the right ventricle for example, so that will allow you to say this is the problem that will likely make the patient die within the next ten years. You can do something about valve obstruction by replacing it. You can do something about the ventricular function by trying to treat everything that you know can affect ventricular function.

The third study that we did, was that we looked at coronary artery disease, in patients with tetralogy of Fallot. Because we traditionally think about congenital heart disease of children and young people, we tend not to worry about their acquired heart disease. Coronary artery disease is the most common reason people have heart failure in the elderly, we found out that in the patients we see here at Mayo, who are at risk of heart failure in their early 50s, a number of them had coronary artery disease. The big finding here was, the type of coronary artery disease that somebody else would survive and be okay, with these patients, they would have heart failure and death with that same coronary artery disease. We don’t know why, but what we think is happening is that these group have a lot of people who have had surgeries. So their pump function, that is their ventricular function on the left side and right side is weak, that any of them superimposed in salt will make it irregular and lead to a bad outcome. Based on the result of the study what we are now doing, is creating an aggressive plan for coronary artery disease, and counseling the patients on what they can do like, weight control, exercise, don’t smoke, give cholesterol medications when indicated. Then the second thing we can do is prevent them from having coronary artery disease, because once they have it, the outcome is not very good.

The next study, the fourth, is about endocarditis. Endocarditis is an infection, inside the heart. Generally patients with tetralogy of Fallot have the surgery in childhood, and by the time they get to adulthood one of their valves, one of the pulmonary valves, tend to leak a whole lot, and the treatment is to replace the valve. Once you replace that valve, and put an artificial valve into the heart, that increases the chance of infection more than ten-folds. What we found out from our series is that having the artificial valve will put you at risk, which is already known, but what is new from this study is that, even with the modern-day medicine and care at Mayo Clinic, about one in three of the patients that have the infection, when they go for surgery they will die. It’s a very scary thing.  On the side of the patient, what this research means for them is being more meticulous with dental hygiene, regularly visit the dentist and they should get antibiotics before they have any dental procedure done. What it means to us as clinicians, based on this very sovereign result, is that we now have very high index of suspicion, in case a patient is complaining of having a high fever or doesn’t feel well, you assume it’s endocarditis until you can prove otherwise. Because if you overlook it, there is a one in three chance that the patient will die.

The fifth study, is about management of heart failure in patients with tetralogy of Fallot. That study ties in with the first study about atrial arrhythmias. What we did is we looked over a 28- year period, and we looked at everyone with tetralogy of Fallot that was admitted for heart failure to Saint Marys hospital. We found that about 15 percent of the patients, were admitted for atleast one episode because of heart failure. Interestingly, the main reason why they came in was atrial fibrillation. That brings us back to being aggressive managing atrial fibrillation by recommending an ablation that will control atrial fibrillation that will prevent heart failure and death.

The sixth study that we looked at was about outcomes of pregnancy in patients with this diagnosis. Normally during pregnancy, the blood volume in the patient increases by about 50 percent. This is well tolerated in someone without heart disease, but in someone with heart disease it can be a problem. What we try to do, is when we see the patient before they get pregnant is try to predict, is how their heart is going to react to this volume stress during pregnancy. What happens is that even a valve disease of ventricular dysfunction, that is not severe when you are not pregnant, may become severe when you are, and at that time it becomes late because they are already pregnant, and your solution of surgery is not their because you’d probably lose the baby. We’ve all seen bad things happen, so with some foresight, being careful and trying to fix whatever is needed in the heart before allowing the patient to get pregnant. What we found out is that, for pulmonary regurgitation, which is leakage of the right sided valve, even if you don’t do any procedure, they tend to do well. So this is different than what we know from disease where the problem is on the right side, where they tend to get into heart failure they tend to have bad outcomes in pregnancy. If you have someone with tetralogy of Fallot that wants to get pregnant, and all they have is pulmonary valve regurgitation, no matter how sever it is, they tend to do well without any problems. That will save you from having to do an elective procedure, that they don’t need at that time, allow them to get pregnant and wait until they meet the guideline to do something.

The last study we looked at was using ECG, which a cheap test that is used routinely during clinic visits, using it to predict the future of the patient. So again, it’s trying to see what’s the risk of having a cardiac arrest or the risk of having heart related death or transplant. Normally, we look at ECG and it gives us the rate and whether they have an arrhythmia, but we came up with something called QRS fragmentation, based on the ECG, it takes less than a minute to do. That can actually give you very useful information to decide what to do for the patient. So why did this study get a lot of traction at the conference is because ECG is the cheapest that we do during our visits, it can be done even in a remote place, you don’t need any special equipment just look at the paper, calculate the fragmentation and that’s all you need.

In a nutshell that’s the major studies that we did in the last few months that we showcased at the ACC.

 

Outside of the ACC conference we just discussed, are there other developments between now and when we last spoke that you would like to discuss?

 Yeah, so in addition to the things that we’re doing, that we’re publishing, we’re running a number of prospective studies, those are the studies that we’re doing in real-time, as they are happening. We have a number of studies in the echo lab, where we are using exercise stress to identify subclinical diseases. With people that have normal studies at rest, we are trying to apply exercise stress to see if it will unmask any of their problems. We also have a pilot study taking place in the cardiac catheterization laboratory where we are trying to use exercise as well. The rationale for this is that when the patients come to us, they tell us that they are short of breath when they exercise, or I get chest pain when I do something, So we try to replicate their symptoms at home and hopefully help us to determine what to do with the patient. What is coming in the future is that we’ll be doing a clinical trial using a new drug called entresto, that is used for heart failure and we are gonna try it for the first time in congenital heart disease patients. That trial is going to end at some time near the end of the year.

 

Thank you for educating us on the latest research on congenital heart disease. When and where will the next conference be?

Of the major cardiac conferences, the next major cardiac conference will be the ESC, the European Society of Cardiology Conference, which is going to be in Paris August, and again we’ll be presenting a number of studies there. And the AHA, the American Heart Association conference is going to be in November.

For more information on these studies, you can find them on the US National Library of Medicine National Institute of Health’s PubMed website.

HELPFUL LINKS

 

Tue, Feb 12 1:41pm · The Importance of Congenital Heart Disease Research in Congenital Heart Disease

Dear Judy,
Hope you are well and I am sorry to hear about the multiple procedures that Amy has gone through. It will be easier to answer your questions on the phone instead of email. Please call my secretary.

Have a great day.

Wed, Jan 30 9:46am · The Importance of Congenital Heart Disease Research in Congenital Heart Disease

2018-01-30 CHD Research

“It is a great thing to make scientific discoveries of rare value, but it is even greater to be willing to share these discoveries and to encourage other workers in the same field of scientific research.”

-Dr. Will Mayo

Medical research is an integral component of the advancement and effectiveness of medicine. In this Q and A with Mayo Clinic cardiologist Alexander Egbe M.B.B.S., research within the field of Congenital Heart Disease is discussed, including current research studies, important innovations both now and in the future, as well as the goals of this research to not only impact Mayo’s patients, but patients across the country and world.

 

Q: What are you currently researching in Congenital Heart Disease?

A: I’m working on a number of things. Of all the 15-key diagnosis in congenital heart disease, my research focus is on three main disease types. One is tetralogy of Fallot, which a right sided disease, the second one is about coarctation of the aorta, which is a left-sided disease, and the final group is patients who have a complex single ventricle that have had Fontan operations.

Q: What does the research of these three groups, those with tetralogy of Fallot, coarctation of the aorta, and patients with complex single ventricle that have had Fontan operations, entail?

A: Each group has different things. For example, with tetralogy of Fallot. Patients who have tetralogy of Fallot have their surgical repair sometime in infancy. By the time they come to us as adults, they usually have pulmonary valve regurgitation, which is leakage of the pulmonary valve, and leads to enlargement of the right side of the heart. This is a trigger that leads to end-stage heart failure or sudden cardiac death. It is because of this, that the average survival of patients with tetralogy of Fallot is 55 years, that’s compared to 75-80 years in the general population. My research target, is to find ways to prevent end-stage heart failure, and sudden cardiac death which are the two reasons why they die in their 50’s instead of their 70’s. To do that would be to focus on two things, one, best way to identify and treat pulmonary hypertension, so we don’t do the procedure too early or too late. Secondly, identify the best way to find people at risk for sudden cardiac death, and do something about it because that’s one thing that doesn’t give you a second chance. So identifying those who are at risk, and doing an ablation or putting ICD’s (implantable cardioverter-defibrillator) in them. Everything I’m doing is like a multi-stage process of actually finding people at risk. We call it risk stratification, finding out at what point is the benefit of doing something outweigh the risk of the procedure. That’s what we are trying to figure out. This will take years to actually make clinical impact.

Q: What is a major goal of congenital heart disease studies?

Once you find out something new, it takes awhile to get assimilated as standard of practice across the country. That’s what will affect the population outcome. Our goal is not to just improve the lives of patients who come to Mayo Clinic, but improve the life of every person with tetralogy of Fallot for example, in North America and in the world. To do that, it will be contingent on people imbibing what we find out from here. So for instance, from our studies we’ve shown that doing pulmonary valve replacement which is a surgical procedure that you use to treat pulmonary valve leakage, that you use to treat patients with tetralogy of Fallot, we suspect that the current guidelines about when to do the procedure is too conservative, that people are undergoing this procedure too early and they probably could afford to wait. If you do it too early, these valves don’t last forever they last around 12-13 years and the valves with fail. Then you need to go for a second procedure which is usually far more technically difficult than the first one, and if you look at the average age of a person that goes for pulmonary valve replacement, they are around 30 years old. So then you need to put in a second valve when they are 42. If you put in a valve when they are 42, then they go in for a more risky procedure in their early 40’s and then the valve will fail in 12 years and they’ll be in their 50’s. By the time you do this procedure a third or fourth time the risk becomes prohibitive that you can’t even do it. So you factor in the calculated risk of the procedure and if you can not afford to wait it’s better off for the patient.

The second problem is if you put in the valves they are at risk for prosthetic valve endocarditis which has high mortality rates. So if you can buy some time before putting in the first valve, you’re certainly at risk for infection. What we have found out here so far in our practice, is doing the procedure later might benefit the patient and that is influencing out clinical practice. As we publish these studies, we are hoping that people will read it and will apply it to their own clinical practice and over time as more people are doing studies that confirm what we have already found, it might result in changes to the guidelines. So to answer your question, what we are doing is influencing our practice, already, which over time, in the space of two or three years we’ll be seeing the affect on outcomes in our patient, but more importantly the end goal is to influence practice in the whole country and change outcome for patients in the whole country.

Q: So eventually your end goal is to not only change and improve the care across the country, but across the world as well? 

A: Yes, exactly.

Q: Great. As you work on these research projects, what are the positives and negatives of the current state of CHD research? What are some problems you encounter?

A: If you look at congenital heart disease research as a whole, it is still very much undeveloped compared to other areas of cardiology just because it is a young specialty compared to coronary heart disease and heart failure and everything else, that’s one problem. Two, is that congenital heart disease is not as common as other heart diseases so research has been hampered by small patient size. That’s a problem across the board everywhere. Mayo Clinic has an advantage, and that is it is one of the first centers to do congenital heart surgery in the world so they’ve had 50 years of experience doing this. With this, we have 50 years worth of clinical data floating around somewhere. Going back to when I came to Mayo Clinic in 2014, the old model of just figuring out how I’m going to look for the patients to do the research was what was done everywhere else, that’s what’s been done here. So the innovation I brought to the table when I came here was establishing something we call “MACHD,” Mayo adult congenital heart disease database. This is a comprehensive database that contains the data of every patient seen here from January 1, 1990. That’s already 28 years worth of data. So that’s everybody that’s been scene, every piece of clinical data they have, is logged in a database with a unique ID. By doing that what we found out was that it dropped the time used to do research, by 80 percent, by a factor of four. A good way to measure that is that prior to 2014, the average publication from our congenital heart disease practice was about four or five papers a year. In 2016 we published about 13 papers, 2017 we published 18, and last year, which is 2018 we published 22. We spend more on this this year, and at full production we should be at more than 30 papers every year. What we need is data, data will influence our practice.

Just as the MACHD database was one of the biggest innovations that improved productivity, the other big innovation that came in late last year, into this year, was establishing prospective registries. So that’s a registry in the cath (catheter) lab and the echo (echocardiogram) lab where we are not just waiting for things to happen, where we can collect the data to place in the MACHD database, but we are actually influencing things to happen. We are enrolling patients in prospective studies. To do that we need money, and we have several lines of funding now, from multiple grants including the NIH and some external which hasn’t happened before, this is the first time we have had this funding, and now this money is being used to fund the non-invasive perspective studies in the echo-lab, and the invasive prospective studies in the cath-lab, so we now have an exercise echo-program.

What we are hoping will happen by this year, is we would like to establish a biobank. In North America, there is only one center that has a biobank, that’s the Harvard program and in Europe there is only one in Amsterdam, so this would be a way to store blood samples from patients indefinitely. This would allow us to run multiple biomarkers that would help us figure out who is at risk, and who should we treat differently, to try to individualize patient treatment. More biomarkers are being invented every other year, so if you already have the blood samples saved, and they invent a new biomarker in ten years, all you have to do is go back and test the sample and make risk stratification. It is something that has been done in other areas of cardiology, and in congenital heart disease we think it will be a game changer going forward.

Q; Are there other potential innovations that you see coming down the road, or is biomarking the big one right now?

A: Yeah, so patients die from heart failure, which is a plumbing problem, or sudden death which is an electrical problem. So when they come into the clinic we do an ECG which tells us what the heart is doing within the ten seconds it take to do that. Sometimes we do a whole time monitor, if we are worried and that records the heart rate for about 24 hours. So there is a very nice heart monitor that can record the heart rate for 30 days called the bodyguardian. It’s used in clinical practice here at Mayo. We have access to funding, so that sometime in the summer or fall, we can establish a reading laboratory where we can get a baseline of our patients who are normally at risk for arrhythmias by doing this 30 day monitor on as many patients as possible. The goal is to identify patients who have brief, asymptomatic arrhythmias, so they are not even aware the have it. The question is, why is that important? It’s been shown, in the non-congenital population, that having brief atrial fibrillation for a few minutes that you’re not aware of, still increases your risk for stroke. Because you don’t know it’s happening, it doesn’t save you from a stroke. How much is that a problem in our population? We don’t know, and you won’t know until you look.

Q: At Mayo, how many congenital heart disease research studies are happening right now?

A: I spearhead most of the research, so about 90 percent of the studies that are actually going on, are spearheaded by me. Last year, of all the publications that happened, I’m directly involved in, about 95 percent of them. So ongoing, the way to look at it is, are they retrospective studies, studies that involve the MACHD database where are going to look at data that is already collected. Those we are doing so quickly that we are averaging about two a month with a minimum of 24 a year. We crossed the 24-barrier last year and we have that as a goal moving forward, so that’s one thing. The prospective studies, where you are collecting data as you are going along, those ones take years to complete one study. Right now, we have eight that are perspective and ongoing. One is funded by the NIH, others are funded by a number of funding bodies, from Mayo and outside Mayo. So we have all these things going on and there is a clinical trial that we’re doing that has been funded by the Mayo Clinic, where we’re looking at people with Fontan physiology to figure out a way to manage liver disease. This is the first randomized trial to look at the effects of it on liver disease, so that trial will be ending in September of this year, so we should have the result by the end of the year.

There is also a second funded studied by a benefactor grant from outside Mayo, that is looking at patients with tetralogy of Fallot and their exercise capacity, so the first trial I mentioned, that looked at liver disease, runs about two years, ending in September, this one has also run about two years and will be ending in December.

We also have a new study that is being funded by the Mayo Clinic Foundation for Research. And that one will be looking at exercise testing in the cardiac cath-lab, with patients with coarctation of the aorta and tetralogy of Fallot. That funding was approved last month, we have yet to enroll our first patient but sometime in February or March that will start.

Altogether we have eight prospective studies, and would like to start more of them.

Q: Any final thoughts?

A: To wrap everything together, there is a need to use what we already have, which is a lot of data. So having that MACHD database was a major innovation that moved things from 5-6 studies a year to close to 30 studies a year within a short period. Perspective studies are good because it gives you a different type of data, and that kind of sets us to be a site to get a lot of funding from the government and from other sources in the future for clinical trials. Then the biobank, that I talked about, is going to be the next generation things, that is going to come at some time in the future, that if we set it up, we will be the lead site in the country, and set up in a model that other people can copy.

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Sep 30, 2018 · Dr. Alexander Egbe presents at European Society of Cardiology in Congenital Heart Disease

2018-09-30-EgbeESC

Mayo Clinic cardiologist Dr. Alexander Egbe recently presented at the European Society of Cardiology congress. The congress was held in Munich Germany from August 25-29, 2018. This is the largest meeting of cardiovascular physicians and scientists in the world. Dr. Egbe, presented data about novel application of Doppler echocardiography for noninvasive assessment of right ventricular to pulmonary artery coupling in patients with tetralogy of Fallot.  The three research studies from the Mayo Clinic that were presented at this congress showed that this novel application of echocardiography can help identify patients at risk of adverse events, and potential guide timing of interventions to prevent such adverse events.

To view more information on the studies click the links below:

Right ventricular and pulmonary vascular function indices for risk stratification of patients with pulmonary regurgitation

The impact of pulmonary vascular resistance on fontan hemodynamics and outcomes

Apr 25, 2018 · The Role of Anticoagulation in Fontan Patients in Congenital Heart Disease

Anticoagulation in Fontan Patients

Mayo Clinic congenital cardiologist, Alexander Egbe, M.B.B.S., discusses the role of anticoagulation in Fontan patients.

Thromboembolism, or blood clots, can affect all systems in the body causing stroke, pulmonary embolism, and heart attacks. In Fontan patients, blood clots are typically found in the heart, Fontan connection, or in the calf, also known as deep vein thrombosis or DVT.

Fontan patients are at a higher risk for blood clots due to sluggish blood flow, arrhythmias, hereditary clotting disorders, or cirrhosis. Decisions on medical therapy with warfarin or aspirin should be determined on a case-by-case basis.