Mayo Clinic's approach

Multidisciplinary aortic valve disease teamwork at Mayo Clinic Multidisciplinary aortic valve disease teamwork at Mayo Clinic

At Mayo Clinic, a multidisciplinary team works together to provide expert care to people with aortic valve disease.

Collaboration

At Mayo Clinic, cardiovascular surgeons work together as a team with cardiologists and other health care providers to give coordinated, comprehensive care to people who have aortic valve repair or aortic valve replacement.

Mayo Clinic care providers collaborate to treat you as a whole person. If you have other health concerns in addition to aortic valve disease, care providers can often evaluate and treat other conditions during your visit.

Valve problems in children with heart disease: What patients and families should know

Joseph A. Dearani, M.D., a cardiovascular surgeon at Mayo Clinic, discusses common issues in treating children with heart valve problems, including the timing of medical intervention and treatment options.

My name is Dr. Joseph Dearani, and I am the Chair of Cardiovascular Surgery at the Mayo Clinic, with an area of expertise in pediatric and congenital heart disease. So today, I'd like to talk about valve problems in children with heart disease. What would be important for patients and families to know? We'll focus on the leaky valve, or the regurgitate valve, as the physicians call it. Mitral, tricuspid, and aortic valves are the classic valves that could have a leaky problem that would require treatment. Any of them could consist of a variety of diagnoses that range from an actual structural abnormality of the valve, such as Ebstein's anomaly of the tricuspid valve, or congenital, mitral, or tricuspid regurgitation, mitral or tricuspid valves, as they relate to the atrial ventricular septal defect or atrial ventricular canal defect.

Pulmonary atresia with intact ventricular septum can have tricuspid valve problems. And there can also be the bicuspid aortic valve which an individual may be born with. And that value may be vulnerable to narrowing or leakage. Finally, there actually can be iatrogenic injury of a valve during the repair of another lesion. For example, injury to the tricuspid valve during the course of closure of a ventricular septal defect.

There can be valve problems related to dilation of the annulus, which may be a secondary problem to an abnormality with the ventricle. And in the current era, now, there are many congenital lesions that eventually require placement of a pacemaker or an internal cardio defibrillator lead. And these can also create regurgitation. So a very wide range of lesions.

Now the important thing from a patient or family standpoint, and from a physician's standpoint, is the timing of surgery. And this can be actually quite difficult, particularly in children, because many of these leaky valve lesions have minimal symptoms. Sometimes symptoms may not even be present. And it's important to know that there could be quite advanced disease present even in the absence of symptoms.

So what would symptoms include? The classic symptoms with leaky valves would be shortness of breath, fatigue, or in a very young child it may be failure to thrive, that is, poor weight gain. Symptoms may be very subtle, not keeping up with peers, more naps in the afternoon, going to bed earlier in the evening, something that only a parent might notice. Or patients may be completely asymptomatic and actually be doing everything quite well.

In any situation, we do an echocardiogram. And an echocardiogram really helps provide information that also helps with timing of the operation. Importantly, it will give us information about whether the ventricle is dilated or not, and whether the ventricle has any dysfunction or not. The presence of either of those two things would clearly prompt the need for intervention.

When ventricular function gets dilated, and then the atria, the receiving chambers get dilated, then arrhythmias may become present. And the onset or progression of arrhythmias also can precipitate the need for operation. Finally, if the surgeon is quite confident with their ability to repair the valve, even earlier operation may be considered in an effort to avoid abnormality subsequently developing with the ventricle.

Other imaging studies besides Echo, which includes two-dimensional and three-dimensional Echo, is really ideal to determine anatomy of the valve. It really is a roadmap for the surgeon to determine whether or not they can repair the valve. This often is complemented with either magnetic resonance imaging, or CT imaging, which gives important function about the ventricle, particularly the right ventricle, how large it is, and what the function of it is.

Now there are many techniques of repair. And very importantly, the success of repair has a high correlation with surgeon experience. You should specifically ask your surgeon how many he or she has done. There are a variety of repair techniques that get tailored toward the specific abnormality. Techniques might include mobilization of one or more leaflets. It might include augmenting, that is, increasing the size of a leaflet. It may involve placement of artificial cords, that is, artificial strings to take the place of some that may be absent or broken. And some include reducing the size of the annulus, sort of like tightening a belt around your waist. Sometimes this is done with suture alone. Sometimes this is done with artificial rings or bands.

As mentioned earlier, arrhythmia commonly may coexist. And if arrhythmias are present, then an arrhythmia procedure, often referred to as the Maze procedure, should also be added at the time of valve repair.

Now there can be some curveballs with all of these problems, particularly when patients are referred late for surgery. And there are other strategies that should be in the armamentarium of the surgeon when ventricular function is below normal. Some of these may involve re-routing the blood. Re-routing the plumbing, so to speak, of blood going back to the heart, so that the ventricle that a struggling has less of a workload to deal with. The most common re-routing procedure would be the bi-directional Glenn shunt, where blood going back to the heart is diverted directly to the lungs in an effort to relieve the burden of the ventricle that is struggling.

And then, of course, there's medical therapy. Medical therapy in the perioperative period, but also, importantly, medical therapy in the long-term that would be driven and navigated by the cardiologist.

I can't emphasize the importance of a team approach, a multi-disciplinary approach, that is usually run by the surgeon, cardiologist, and anesthesiologist, but a wide variety of other allied health care professionals, individuals in radiology and imaging that all play a part in the care of these children.

The risk of surgery is largely determined by ventricular function and, in general terms, it is low when ventricular function is normal. An important piece of information for patients and families to know is that, when you are fixing a leaky valve the immediate response to ventricular function is actually to go down. That is to say, ventricular function gets worse initially, and then generally gets better with time. Hopefully, it returns to normal. But this is in a large part due to what the function of the ventricle is before surgery. Again, emphasizing the importance of proper timing of operation. Late results, that is survival, are also largely determined by ventricular function. Common questions are, how long will I live? Or how long will my child live? And will there be a need for other operations?

Generally speaking, survival is optimized when valves can be repaired as opposed to being replaced. But also, there is going to be a high probability for subsequent repair procedures down the road, depending upon how many have been done previous, and what the nature of the specific problem is. So in general, valve repair is preferred. Late survival is beneficial. There's low incidence of infection. And everything is optimized when ventricular function is preserved. Valve replacement may eventually be necessary. And there should be reasonable and sensible attempts at repair in the beginning and on repeated occasions before resorting to replacement.

We, of course, in our practice, have extensive experience with repair of all valves, mitral, tricuspid, and aortic valves. We emphasize the team approach, surgeon experience, and the importance of lifelong oversight cannot be overemphasized by the cardiologist who knows what to look for, knows how to monitor it, and knows when to refer for surgery. If anyone is interested in trying to obtain a consultation with either a surgeon or a cardiologist with your child who has been told that they have a valve problem that needs intervention, please feel free to let us know. We would be happy to review any information and provide recommendations accordingly. Thanks for listening.

Technology

People who come to Mayo Clinic for aortic valve disease treatment have access to state-of-the-art diagnostic and treatment facilities.

Mayo Clinic providers offer the latest aortic valve disease treatment options, including several minimally invasive options that involve smaller incisions and, possibly, a quicker recovery. These options include:

Discussing minimally invasive heart surgery at Mayo Clinic Discussing minimally invasive heart surgery at Mayo Clinic

Cardiovascular surgeons, cardiologists and radiologists at Mayo Clinic offer the latest in minimally invasive heart surgery options.

Research and innovation

Mayo Clinic's care providers and researchers are established pioneers in the field of heart surgery. They're involved in many international and national studies to improve the technology and techniques used in aortic valve repair and aortic valve replacement.

Researchers study many areas related to heart valve diseases. Focus areas include the use of new blood-thinning medications for people with a mechanical valve replacement and genetics research in people with aortic valve disease.

As a patient at Mayo Clinic, you may have access to heart-related clinical trials as a part of your treatment.

Expertise and rankings

Heart valve disease team meeting at Mayo Clinic Heart valve disease team meeting at Mayo Clinic

At Mayo Clinic, a multidisciplinary team of heart specialists works together to provide the best available care to people with aortic valve disease.

Treatment expertise

Mayo Clinic cardiologists and cardiac surgeons have extensive experience in aortic valve repair and aortic valve replacement.

Mayo Clinic surgeons perform more than 500 aortic valve repairs and aortic valve replacements each year.

Mayo doctors and other care providers in the Valvular Heart Disease Clinic specialize in diagnosing and treating people with aortic valve disease and other heart valve diseases. Cardiologists evaluate people with aortic valve disease and determine whether aortic valve repair, aortic valve replacement or another treatment option is most appropriate.

Pediatric cardiologists and pediatric cardiac surgeons at Mayo Clinic's campus in Minnesota have experience treating children with aortic valve disease and other heart conditions.

Aortic valve repair in children

Joseph A. Dearani, M.D., a cardiovascular surgeon at Mayo Clinic, discusses aortic valve repair in children and tailored treatment options available at Mayo Clinic.

My name is Joseph Dearani and I am the Chair of Cardiovascular Surgery in Mayo Clinic in Rochester, Minnesota and my area of expertise is in pediatric and congenital heart disease.

Today we're going to talk about the treatment of aortic valve disease in children, which presents a challenging dilemma. The difficulty has to do with the benefits of early surgery to protect the heart from pressure and volume overload from either aortic stenosis or aortic regurgitation versus the lack of a sub-optimal valve substitute in children and the limited durability provided by the various aortic valve repair techniques.

Causes of aortic valve disease in infants and children include either changes in the aortic valve leaflets or changes in the geometry of the aortic root. In general, the repair technique is tailored to each individual anatomy encountered. The indications for surgery, or in some cases balloon valvuloplasty with aortic stenosis is more vague in children and includes a peak-to-peak pressure gradient that exceeds 40 to 50 mmHg, the presence of left ventricular hypertrophy or strain on the electrocardiogram, or evidence of left ventricular dysfunction with exercise testing.

Importantly, symptoms are often a late finding with aortic stenosis and intervention is usually advised on the basis of abnormal diagnostic and imaging studies and should be advised before obvious symptoms become present. Indications for surgery with aortic regurgitation include severe diastolic flow reversal in the descending thoracic aorta that results in progressive volume loading on the left ventricle, presence of an increase left ventricular end-diastolic volume that exceeds three standard deviations on appropriate afterload reduction therapy, the presence of reduced systolic function with exercise or just general decreased exercise tolerance.

Pre-operative imaging, particularly two dimensional and the more recent three dimensional echocardiogram, can provide very valuable information about the aortic root anatomy and valve structures, the mechanism of the valve dysfunction, and it really helps guide the surgeon with strategies for valve repair. As mentioned earlier, aortic valve repair — the technique applied is generally tailored to the individual anatomy encountered. It is important to know that aortic valve repair is viewed as palliative, often temporizing for the eventual valve replacement, which in children is usually the Ross procedure, or the autograft procedure. Concern about autograft root dilatation over time has resulted in the approach of one or more valve repairs being performed in order to delay the ultimate or inevitable autograft operation. Tissue-engineered valve research has been underway for many years now and provides promise for the future.

The most common congenital heart defect, interestingly, is the bicuspid aortic valve. Infants and children with congenital aortic stenosis are often treated with balloon valvuloplasty at first. Surgical valvotomy is ultimately required in about a third of these patients within the subsequent five years. In older children, dilatation of the ascending aorta can result in aortic regurgitation, and this is much more common in the conotruncal anomalies — such as tetralogy of Fallot, pulmonary atresia with ventricular septal defect, truncus arteriosus, double outlet right ventricle, and Marfan and some of the other syndromes.

In general, valve repair techniques are preferred and the valvuloplasty technique of the aortic valve is often the most difficult of all valve repairs present. Many valve repair techniques have been described and include suture repair of leaflet tears or fenestration, commissurotomy, re-suspension of prolapsing leaflets, thinning of thickened leaflets, free margin placation, leaflet resection, and leaflet free edge shortening or leaflet extension maneuvers.

Importantly, repair techniques often require a combination of the above described approaches to obtain a valve that doesn't leak and is not obstructive and the outcome is directly proportional to the experience of the surgeon. The risk of surgery is generally low, particularly beyond the neonatal and infancy periods. Unfortunately, a third to half of patients will require re-intervention within five to 10 years. Mayo has a surgical history exceeding 50 years and an extensive track record of surgery in thousands of patients with aortic valve lesions, both stenosis and regurgitation, in all age brackets — from the newborn, through childhood, and on up into adulthood. Thank you very much for listening to this topic on aortic valve disease in children.

Cardiovascular surgery experience

Mayo Clinic is an established pioneer in the field of cardiac surgery, and our cardiac surgeons perform more than 4,500 cardiac surgeries each year.

Mayo Clinic cardiovascular and cardiothoracic surgeons at Mayo Clinic's campuses in Arizona, Florida and Minnesota use the most advanced techniques, including minimally invasive heart surgery and robot-assisted heart surgery. Our skilled surgeons can perform complex, specialized procedures unavailable elsewhere.

Nationally recognized expertise

Mayo Clinic campuses are nationally recognized for expertise in cardiology and cardiovascular surgery:

  • Mayo Clinic in Rochester, Minnesota, Mayo Clinic in Phoenix/Scottsdale, Arizona, and Mayo Clinic in Jacksonville, Florida, are ranked among the Best Hospitals for heart and heart surgery by U.S. News & World Report.
  • Mayo Clinic Children's Center in Rochester is ranked the No. 1 hospital in Minnesota, and the five-state region of Iowa, Minnesota, North Dakota, South Dakota and Wisconsin, according to U.S. News & World Report's 2022–2023 "Best Children's Hospitals" rankings.
  • Mayo Clinic in Rochester, Minn., and Mayo Clinic in Phoenix/Scottsdale, Ariz., are ranked as high performing for aortic valve replacement and transcatheter aortic valve replacement (TAVR) procedures by U.S. News & World Report. "High performing" is a designation given to the top 16% of TAVR programs across the nation (600+ programs). U.S News & World Report ranked hospitals for this procedure for the first time in 2022-2023.

With Mayo Clinic's emphasis on collaborative care, specialists at each of the campuses — Minnesota, Arizona and Florida — interact very closely with colleagues at the other campuses and the Mayo Clinic Health System.

Learn more about Mayo Clinic's cardiovascular surgery and cardiovascular medicine departments' expertise and rankings.

Locations, travel and lodging

Mayo Clinic has major campuses in Phoenix and Scottsdale, Arizona; Jacksonville, Florida; and Rochester, Minnesota. The Mayo Clinic Health System has dozens of locations in several states.

For more information on visiting Mayo Clinic, choose your location below:

Costs and insurance

Mayo Clinic works with hundreds of insurance companies and is an in-network provider for millions of people.

In most cases, Mayo Clinic doesn't require a physician referral. Some insurers require referrals, or may have additional requirements for certain medical care. All appointments are prioritized on the basis of medical need.

Learn more about appointments at Mayo Clinic.

Please contact your insurance company to verify medical coverage and to obtain any needed authorization prior to your visit. Often, your insurer's customer service number is printed on the back of your insurance card.

More information about billing and insurance:

Mayo Clinic in Arizona, Florida and Minnesota

Mayo Clinic Health System

Clinical trials

Explore Mayo Clinic studies of tests and procedures to help prevent, detect, treat or manage conditions.

May 24, 2022
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Aortic valve repair and aortic valve replacement