When they thought they were out of options, Nancy O'Brien brought her adult daughter, Jessica, to Mayo Clinic to look into palliative or comfort care after Jessica went into heart failure. However, Mayo Clinic doctors were able to find an innovative solution that reset Jessica's clinical course and helped her get her life back.

Watch: Hope beyond heart failure

Journalists: Broadcast-quality video (2:55) is in the downloads at the end of this post. Please "Courtesy: Mayo Clinic News Network." Read the script.

Jessica has a way of making everyone she meets just feel a little better. But in November 2022, it was Jessica who needed help feeling better. The usually active, gold medal-winning Special Olympics athlete started experiencing symptoms of heart failure.

"We knew something was wrong, but we didn't know what," says Nancy.

Jessica was hospitalized with rapid onset of heart failure in upstate New York, where she and Nancy live. Like many people with Down syndrome, Jessica was born with a congenital heart defect and received treatment at Mayo Clinic when she was 7 years old. But now, as a 42-year-old, it was unclear why she had developed sudden heart failure. So the O'Briens once again turned to Mayo Clinic.

"If any place knew how to handle that type of long-term care and the deterioration, we knew they would do it the right way," says Nancy.

Doctors at Mayo Clinic were able to identify a hole that had developed between Jessica's aorta and the right side of her heart.

"They had been told, really, there was nothing that could be done," explains Dr. Luke Burchill.

Dr. Burchill, an adult congenital heart disease specialist, says the uncertainty of what Jessica's future would hold may have been as big of a burden as her symptoms themselves.

"I think they really arrived with the understanding that there wasn't really an option. And perhaps we were needing to shift into a different phase of care — looking at palliative care," he says.

The hole in Jessica's heart could be corrected with open-heart surgery, but that is high risk. A multidisciplinary care team brought various experts to the table to develop a plan for Jessica that didn't involve open-heart surgery.

"Mechanical problems need mechanical solutions. In this case, it was finding a device that could be placed across the hole in her heart and plugging the hole. And, therefore, changing the clinical course of her heart failure," says Dr. Burchill.

To the O'Briens, hearing that was like "a gift from heaven."

"When we discussed that there might be another option, I think that they were in disbelief. You know, they had really done 180 degrees from being told there's nothing we can do. It's just a case of keeping her comfortable versus, actually, we have a solution. And that solution might reset the clinical course," says Dr. Burchill.

Within a week of arriving, Jessica underwent the procedure to plug the hole in her heart, and after a few days of recovery, she was able to return home.

"He helped me get my strength back," says Jessica.

Dr. Burchill is leading the development of new heart failure care pathways for adults, like Jessica, with congenital heart disease.

"Jessica is exactly the kind of patient that this new care pathway is designed to help. So it has really given me a lot of energy to keep going with building this pathway," says Dr. Burchill.

The post Hope beyond heart failure: Innovative treatment allows woman to change the course of her heart failure appeared first on Mayo Clinic News Network.

Could cells taken from a small patch of skin become heart muscle and repair a rare congenital heart defect? It's a scientific question that Timothy Nelson, M.D., Ph.D., and collaborators hope to answer for those born with an underdeveloped left heart chamber — a rare, complex condition known as hypoplastic left heart syndrome (HLHS).

Discovering the first cell-based therapy to rebuild heart tissue for HLHS has been a decade-long research passion for Dr. Nelson, who is the director of the Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome at Mayo Clinic.

"The problem is that the majority of complex single ventricle patients born with congenital heart defects, like HLHS, are going to eventually develop heart failure. Unfortunately, heart transplantation is less than ideal for this patient population, that can include children with complex comorbidities, even if there wasn’t a severe shortage of suitable organs," says Dr. Nelson. "Mayo Clinic research plays an important role in advancing innovative discoveries for rare and complex heart conditions to expand options."

Remuscularizing the heart

Approximately 10,000 people in the U.S. are living with HLHS — a condition that severely weakens the heart. Surgery to reposition arteries enables a single ventricle to pump blood to the lungs and the rest of the body, but that restores only a portion of circulatory function. While advancements are being made, few people with HLHS live past the age of 30.

"HLHS is essentially like taking your lawn mower engine and putting it into your SUV and driving to work at 70 miles an hour every day," says Dr. Nelson. "Our research is trying to understand if we can convert defective hearts from a 5-horsepower engine to a 50-horsepower engine. We probably will never make it into the 100-horsepower engine that a normal heart is, but our goal is to make the heart bigger and stronger."

Current technologies in clinical studies, such as cord blood and bone marrow, which Dr. Nelson calls "fertilizer," can be injected into the heart muscle during planned surgeries and may be useful to preserve and protect the heart muscle. Ultimately, this has limited therapeutic value after the onset of heart failure.

"Fertilizer approaches may stimulate the heart to grow the best it can. That doesn't get us to the finish line most of the time because the fertilizer by itself just isn't able to grow it big enough and strong enough," says Dr. Nelson.

In search of new options, Dr. Nelson's research team is investigating whether a bioengineered heart product could regenerate the patient's own heart tissue and repair the underdeveloped part of the heart — essentially remuscularizing the heart.

From patch of skin to a heart cell

Research into heart tissue regeneration begins with a biopsy of the patient's skin the size of a pencil eraser. Cells are extracted and then reprogrammed to become induced pluripotent stem cells, which is like turning back the clock in time to when cells were first forming in the womb. At that state, the cells are dividing and capable of becoming any type of cell in the body. In this case, they are then trained to become heart cells, known as cardiomyocytes. They become beating, contracting heart muscle cells.

It is a nine-month process from cell extraction to the scale up of the hundreds of millions of cells that would be needed to test whether they could form new heart tissue.

"We have reprogrammed these cells in the lab and tested them in multiple preclinical models where we observed them engrafting into the heart muscle of the recipient, then they grow, divide and expand," says Dr. Nelson. "We are now interested in learning whether these cardiomyocytes could regenerate heart tissue in humans."

Dr. Nelson's decade-long circuitous research path has led to an important nexus. The Food and Drug Administration will permit the team to study the safety of induced pluripotent engineered heart cells in a phase 1 trial at Mayo Clinic for adults with congenital heart defects.

The HLHS Consortium, comprised of multiple cardiovascular hospitals across North America, was founded at Mayo Clinic and provides a team approach to conduct large-scale studies for complex congenital heart disease patients. The HLHS Consortium works together to advance scientific knowledge and practice for rare congenital heart disease.

This important work — to repair, replace or restore diseased cells, tissue or organs — is associated with regenerative medicine. Mayo Clinic's Center for Regenerative Biotherapeutics supports HLHS research through distributing and expanding the network of scientists and researchers utilizing these tools at the Regenerative Medicine Biotrust.

This HLHS research milestone at Mayo is made possible, in part, by collaboration with Mayo Clinic Ventures to license the technology to a nonprofit organization that will be sponsoring the clinical trial. The nonprofit organization, HeartWorks, and its staff of 20 have worked with Mayo Clinic’s program for HLHS to co-develop technology that will be tested. The research is also supported by the generosity of the Todd and Karen Wanek Family.

Depending on the outcome of the phase 1 safety study, it could take years before engineered heart tissue is approved for use in daily clinical care for congenital heart disease.

About Dr. Nelson

Dr. Nelson graduated with degrees in biochemistry and molecular biology from the University of Wisconsin Eau Claire and Platteville. He completed the Medical Scientist Training Program at the Medical College of Wisconsin, followed by a research fellowship and residency at Mayo Clinic. He has additional training in cardiovasology through the National Institutes of Health.

Research by Dr. Nelson:

Single-Cell RNA-Sequencing and Optical Electrophysiology of Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes Reveal Discordance Between Cardiac Subtype-Associated Gene Expression Patterns and Electrophysiological Phenotypes

Stem Cell Therapy and Congenital Heart Disease

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DEAR MAYO CLINIC: My daughter was born with congenital heart disease and had an operation as a child. She is now 18 years old and went off to college last month. I am concerned about her now being in a new state and having an urgent medical care need. Are there precautions I can take now to help her?

ANSWER: Your concerns are completely understandable. It is hard to see our children grow, become independent and leave the nest. It is even more nerve-wracking if they have a health issue. It is wonderful that you are thinking about your daughter and how to ensure she remains healthy, even though she is not at home.

Congenital heart disease means there are one or more problems with the heart's structure that exist since birth. Nearly 1% of children born in the U.S. annually are diagnosed with a congenital heart defect, which can change the way blood flows through the heart.

There are several forms of congenital heart disease. Some forms are simple — such as ventricular septal defects, where there is a hole in the wall, the septum, of the heart's lower chambers which allows blood to pass from the left to the right side of the heart. Others are highly complex — such as a single ventricle, where the heart is lacking one of the two lower pumping chambers.

People with congenital heart issues require lifelong treatment, which may include regular checkups, medications or surgery. Unfortunately, only 10%-40% of adults with congenital heart disease are being seen by a specialized team. At Mayo Clinic, for instance, the Adult Congenital Heart Disease Clinic is comprised of staff who specialize and are trained in all matters of heart disease, heart surgery, and other advanced imaging techniques and therapies to care for and treat these conditions.

It is important as your daughter begins to transition towards adulthood that she has a good understanding of her condition. Talk with her about the importance of having lifelong care and how her condition may change as she ages. Thanks to innovations in care, more than 2.4 million people are estimated to be living with a congenital heart defect in the U.S. Bringing up this concept during adolescence will help set the foundation for a healthy adult life.

You may want to consider setting up an appointment for both of you to speak with her current health care professional to review the status of her condition, medications, surgical history, necessary screenings and areas of concern that may cause challenges while she is away at school. Empower your daughter to ask questions as well. This will allow your daughter to have an up-to-date understanding in case she has any issues. It also will be important to keep a copy of her medical information at school and on her phone.

Here are a few additional tips to help your daughter transition in the best possible way:

1. College can be a stressful time for many young adults. During these moments, they can become sedentary and get exposed to alcohol, drugs, nicotine and vaping. Openly discussing these issues with your daughter — and her congenital heart team — is important to help her make appropriate choices, and it can counterbalance the overwhelming weight of peer pressure.
2. Encourage her to exercise. Bear in mind that incorporating regular exercise as a stress-relief strategy will pay back not only throughout college, but the rest of her life. Of course, exercise is a valuable tool for not only stress, but maintaining heart health. Ensure you speak with her health care team about any restrictions or limitations based on her congenital condition.
3. Discuss family planning. It may seem overwhelming to think about this now as your daughter is still a teenager, but it is important to open a dialogue with her congenital heart team to develop an optimal family planning strategy for the future.
4. Become familiar with the existing resources that could be helpful to you and her, including the Adult Congenital Heart Association Clinic Directory, a free resource that includes self-identified heart clinics in the U.S. Identifying an alternative care resource near her college campus will be useful.

Planning ahead and supporting your daughter through this transition into caring for her congenital heart condition as an adult will benefit her tremendously. — Dr. Katia Bravo Jaimes, Cardiovascular Medicine, Mayo Clinic, Jacksonville, Florida

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Related Articles 

• Mayo Clinic Q&A podcast: Innovative procedures for kids with congenital heart disease published 2/18/22
• Mayo Clinic Q and A: Congenital heart disease revision surgery published 8/24/21
• Mayo Clinic Q&A podcast: The silent defects of congenital heart disease need lifelong surveillance published 3/22/21

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A congenital heart defect means that a child was born with a problem in the structure of his or her heart.

Some congenital heart defects in children are simple and don't need treatment. Others are more complex and may require several surgeries performed over a period of several years.

Improvements in imaging, monitoring and surgical techniques have improved outcomes for pediatric heart surgery patients.

A prenatal diagnosis is scary for parents, but support and care from the cardiology team continue through the child's life and on into adulthood. And support from others, including patient organizations, also helps.

"It can feel like you're alone, especially if the diagnosis is new," says Dr. Elizabeth Stephens, a pediatric cardiovascular surgeon at Mayo Clinic. "It can feel very daunting. But there are many families out there who are not just dealing with congenital heart disease, they're thriving with it. These kids are incredibly resilient."

On the Mayo Clinic Q&A podcast, Dr. Stephens joins Ask the Mayo Mom host Dr. Angela Mattke for a discussion on congenital heart defects and new treatment options.

Watch: Mayo Clinic Children’s Center pediatric cardiovascular surgeon Dr. Elizabeth Stephens adds an extra touch of care with artwork on her patients’ bandages.

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DEAR MAYO CLINIC: I am a 29-year-old active military service member without any significant health issues. Yet during my most recent routine physical examination, it was discovered that I have a heart murmur. I was referred for an echocardiogram and evaluation with a cardiologist who said I have a bicuspid aortic valve. What are the short- and long-term consequences of having a bicuspid aortic valve? At what point should a bicuspid aortic valve be treated, and what is the treatment?

ANSWER: The aortic valve sits at the base of heart, and it separates the heart's main pumping chamber (the left ventricle) and the main artery (the aorta), which carries the oxygen-rich blood to your body. It functions as a one-way valve, opening and closing with each heartbeat, which keeps the blood flowing in the correct direction. It usually consists of three flaps of tissue — what are called valve cusps. This makes the aortic valve a tricuspid valve.

Approximately 1%–2% of the population are born with an aortic valve that only has two cusps. This is called a bicuspid aortic valve. This condition is the most common heart valve defect present at birth.

Most people with a bicuspid heart valve do not have any signs and symptoms of valve disease until they are an adult, but it occasionally can cause symptoms in childhood. It most commonly is diagnosed as an incidental heart murmur during physical examination in otherwise healthy adults or discovered while a patient is having medical tests for another health condition.

A new diagnosis of a bicuspid aortic valve usually does not require immediate intervention or have significant health effects in the short- or mid-term. It does require lifelong surveillance because it may cause heart problems in the long term. Over half of patients will need aortic valve repair or replacement within 25 years after bicuspid aortic valve diagnosis.

Most commonly, the bicuspid aortic valve results in early narrowing of the aortic valve, called aortic stenosis. This causes reduced blood flow from the heart, which creates excessive work on the heart. Sometimes the opposite can happen, and the valve does not close tightly enough, allowing backward flow of the blood into the heart and causing leakiness, or aortic regurgitation.

A narrow or leaky valve can lead to heart failure over time, causing symptoms such as chest pain, shortness of breath and difficulty exercising. Some people with bicuspid aortic valve also may develop enlargement of the aorta. The enlargement of the aorta above a certain size can lead to a rupture or tear of the wall of the aorta, which is life-threatening if not recognized or treated in a timely manner. As you can see, a bicuspid aortic valve can affect not just the valve, but also the aorta, with a wide spectrum of disease manifestations.

Most bicuspid aortic valve cases occur by chance, but approximately 10% of the cases tend to occur more often in family members. While screening of first-degree relatives is routine after the initial diagnosis of bicuspid aortic valve, routine genetic testing is not recommended.

The initial test, as in your case, is an echocardiogram. This test uses sound waves to image the heart chambers, its valves, blood flow through the valves and the size of the aorta. It is an excellent initial screening tool. An echocardiogram can reliably diagnose a leaky or narrow valve or the enlargement of the aorta. Should there be evidence of aortic enlargement, your health care provider may order a CT for more precise measurement.

Treatment of the bicuspid aortic valve depends on the extent of the valve's disease, the patient's symptoms and the size of the aorta. Open-heart surgery may be needed if the narrowing or leakiness is severe or the patient is experiencing symptoms.

The most commonly performed procedure is aortic valve replacement, with either an artificial mechanical valve or a biological tissue valve. In certain cases of a leaky valve, repair of the aortic valve may be more feasible than replacement, which is more advantageous, especially in younger patients.

During open-heart surgery, the surgeon may remove the enlarged section of aorta, if necessary, and replace it with a synthetic tube, or graft. While there are no medications for bicuspid aortic valve, your health care provider may prescribe medications to treat associated heart problems, like high blood pressure. Patients with bicuspid aortic valve are at increased risk for developing infection of the lining of the heart and the valves, so recommendations for proper, regular dental care can lower this risk.

In summary, once the diagnosis of bicuspid aortic valve is established, you will require lifelong care and surveillance by a cardiologist using an echocardiogram to monitor your valve and aorta. Since the bicuspid aortic valve can be passed down in families, your health care provider also will recommend a screening echocardiogram for your parents, siblings and children ― your first-degree-relatives. — Dr. Gabor Bagameri, Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota

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Related Articles 

• Mayo Clinic Q and A: Mechanical or tissue heart valve replacements published 2/26/21
• Mayo Clinic Q and A: Most aortic aneurysms are small, develop slowly published 2/7/20
• Sharing Mayo Clinic: Surviving an aortic tear and living to navigate its aftermath published 2/2/20

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DEAR MAYO CLINIC: I consider myself to be in good health. I work out several times a week, but recently I began experiencing episodes of shortness of breath after going up and down the stairs in my home. After running on the treadmill a few weeks ago, I got dizzy and fainted. I went to my doctor who told me that I have a bicuspid aortic valve. Can you share more about what this is and if it can be fixed? Also, I have children. Are they at risk for this condition?

ANSWER: It can be a shock to receive a diagnosis that you have a heart condition. The good news is that you should be able to live a healthy and active lifestyle with the right care.

As a reminder, the heart has four major valves. The two valves on the left side of the heart are the aortic valve and the mitral valve, and the two valves on the right side are the pulmonary valve and the tricuspid valve. The aortic valve is the main "door" out of the heart. Blood flows through the aortic valve to exit the heart, and supplies oxygen and nutrients to the rest of the body.

The normal aortic valve has three leaflets, also known as cusps.  Some people can be born with one, two or even four cusps of their aortic valve. The most common of these abnormalities is an aortic valve with two cusps — thus, a bicuspid aortic valve.

A bicuspid aortic valve is a common cardiovascular condition, affecting about 1% of the general population. Bicuspid aortic valves are more common in men, but also affect women. A bicuspid aortic valve is a congenital condition, meaning that people are born with two rather than the normal three cusps on their aortic valve. Although bicuspid aortic valves can occur sporadically without any inheritance pattern, the condition also can run in families. Many people can live with a bicuspid aortic valve for their entire life, but there are those who may need to have their valve surgically replaced or repaired.

When people are born with a bicuspid aortic valve, the bicuspid valve typically functions well throughout childhood and early adulthood. When people reach middle age, bicuspid aortic valves can begin to degenerate. Degeneration is normal for aortic valves as people age, but occurs at a younger age in bicuspid aortic valves compared to normal aortic valves.

Degeneration occurs in two forms: narrowing, also known as stenosis; or leaking, also known as regurgitation. People do not feel any symptoms of bicuspid aortic valves until the narrowing or leaking becomes severe enough to affect heart function. At that point, people with bicuspid aortic valves may notice shortness of breath, difficulty exercising, lightheadedness or chest pain. This sounds like what happened in your situation.

If heart function becomes significantly impaired, people can develop heart failure — the symptoms of which include fluid retention, weight gain, swelling in the legs, substantial breathing difficulty and, potentially, even syncope, which means passing out.

Health care providers usually diagnose bicuspid aortic valves with an ultrasound of the heart called an echocardiogram. CT scan and MRI also can detect bicuspid aortic valves. Bicuspid aortic valves often make characteristic sounds when health care providers listen to hearts.

In addition to early valve degeneration, people with bicuspid aortic valves carry a risk for enlargement, or aneurysm development, of the ascending aorta, which is the main blood vessel that carries blood out of the heart. People with bicuspid aortic valves rarely can have narrowing, or coarctation, of the aorta. Echocardiogram, CT scan and MRI can detect aneurysms and coarctations of the aorta. Your health care provider may want to monitor you with scans at different intervals.

Bicuspid aortic valves are more prone to infection than normal aortic valves. Infection of a heart valve is called infective endocarditis. It can have devastating consequences. Infective endocarditis can occur from bacteria that are a normal part of the human mouth. People with bicuspid aortic valves in addition to dental abscesses or other mouth infections carry a higher risk of infective endocarditis. It is critically important that people with bicuspid aortic valves undergo regular dental cleanings and maintain excellent oral hygiene.

People with bicuspid aortic valves need to have examinations from their health care provider and tests to monitor the valve and aorta on a regular basis. Echocardiograms are the most common tests to monitor people with bicuspid aortic valves, but CT scans and MRIs also can serve that purpose. The frequency of monitoring depends on the degree of valve stenosis or regurgitation, ascending aorta enlargement, and a person’s family history. Tests may be necessary as frequently as every six months to as rarely as every five years. Because bicuspid aortic valves can run in families, all first-degree relatives (i.e. children, siblings and parents) of people with bicuspid aortic valves should have an echocardiogram to check for a bicuspid aortic valve and an ascending aortic aneurysm.

There are no medications to treat a bicuspid aortic valve. The only treatment is surgery to repair or replace the aortic valve if the stenosis or regurgitation becomes bad enough, or if the ascending aorta becomes too large.

Not all patients with bicuspid aortic valves will need heart surgery. Studies suggest that up to 75% of people with bicuspid aortic valves will require intervention at some point in their lives. If people with bicuspid aortic valves have regular monitoring and prompt treatment, their lifespans are similar to the general population.

People diagnosed with a bicuspid aortic valve should understand that they will require regular monitoring and may eventually require valve replacement or repair. They should otherwise live an active, healthy and normal lifestyle. ― Dr. Michael Cullen, Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota

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Congential heart defects are the most common birth defect in the U.S., affecting approximately 1 in every 110 (about 40,000) babies each year. Some congenital heart defects in children are simple and don't need treatment. Other congenital heart defects are more complex and may require several surgeries performed over a period of several years.

This special edition of the Mayo Clinic Q&A podcast features an #AskMayoMom episode, which is hosted by Dr. Angela Mattke, a pediatrician at Mayo Clinic Children's Center. Joining Dr. Mattke to discuss cardiovascular surgery is Dr. Elizabeth Stephens, a Mayo Clinic pediatric cardiovascular surgeon.

The post Mayo Clinic Q&A podcast: What to expect when children with congenital heart disease need surgery appeared first on Mayo Clinic News Network.

To listen, click the link below.

Congential heart defects

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Woody Hust, an athlete and outdoorsman, is living his dream in the Rocky Mountains. It's a picture-perfect existence made even more special because he's achieved his goals despite having a congenital heart defect. Thanks to the lifelong, personalized care Woody's received from his Mayo Clinic cardiologist, the sky continues to be the limit for him.

For nearly as long as he's walked, Woody Hust has skied. Fitted into his first pair of skis at just 17 months by his ski instructor parents, he grew up on the slopes. By his teen years, his talents and love of the sport had propelled him to competitive racing, where he qualified for the Junior Olympics when he was 15.

"It's probably my biggest passion," says Woody, who's now 25. "I don't know what I would do without skiing. When I'm skiing, I'm just in my happy place."

Woody's devotion to the sport and savvy on the slopes are all the more remarkable because he was born with a congenital heart defect. The condition, called critical aortic valve stenosis, has required him to undergo five open heart surgeries — three of them at Mayo Clinic. 

While many factors contribute to Woody's athleticism, the encouragement of his parents and the supportive care offered by his Mayo Clinic pediatric cardiologist Allison Cabalka, M.D. — who became Woody's doctor when he was in utero — rank high on the list of reasons for his continued success.

"I'm lucky," says Woody, who lives in Whitefish, Montana, and works as a ski instructor and a forester for the state of Montana. "I've come a long way, and I've gotten to do everything I've wanted to do, and I credit that to Mayo Clinic. I feel fortunate to be able to get the care that I've gotten at Mayo."

As is the case with some people who have congenital heart defects, Woody lives with activity restrictions to protect his heart health. In Woody's case, those restrictions have been carefully developed through years of testing his limits.

"Woody has always been an avid outdoorsman and athlete with downhill skiing, and now living in Montana with outdoor activities and working in the forest industry," Dr. Cabalka says. "I think he's coped very well with his limitations. He might be a little superhuman."

Treating a life-threatening defect

Woody's history of defying the odds started just after birth, says his mom, Heidi, explaining that when Woody's heart defect was identified at 6 months' gestation, Dr. Cabalka — who at the time worked at a medical center in Minneapolis — became his cardiologist. "His aortic valve was a lump, and he had critical valve stenosis," Heidi says.

Just four hours after he was born, Woody underwent his first open-heart surgery. During the valve repair, his misshapen and narrowed aortic valve, which is the main pump to the body and normally has three cusps to regulate blood flow, was opened up and reformed into a bicuspid valve.

For six weeks after surgery, Woody remained connected to breathing support in a neonatal ICU in the Twin Cities, and he struggled to heal. More than once, doctors told his parents their son would not survive the night. During this time, members of Woody's surgical team urged the family to allow them to give Woody an artificial heart valve, emphasizing that without a new valve, he wouldn't thrive.

But Dr. Cabalka was firm in her belief that Woody would fare better if left to grow and develop without an artificial valve. "So we let him sit and grow. Dr. Cabalka was adamant that was the best solution," Heidi says. "And he was fine. He pulled out his own breathing tubes at 6 weeks old and started eating. It was amazing. He was so strong. He's a really tough kid."

Overcoming significant hurdles

Woody's resilience was tested time and time again. As a kindergartener, he required his second open-heart surgery, which was performed at a hospital in Michigan. Known as a Ross-Konno procedure, the surgery entailed replacing Woody's abnormal aortic valve with his own pulmonary valve. In place of the pulmonary valve, a human donor valve was placed.

"We were so grateful we followed Allison (Cabalka) down to Mayo because we definitely got the cutting-edge care that was right for Woody."

Heidi Hust

When Woody was 15, Dr. Cabalka, who by then was working at Mayo Clinic in Rochester, recommended a third surgery. This operation, which was performed by Mayo Clinic cardiovascular surgeon Joseph Dearani, M.D., replaced the valve and aortic root, which is the portion of the aorta closest to the heart. In addition, the pulmonary valve that had been implanted a decade earlier was replaced with a bovine valve.

Nine months after surgery, however, the bovine valve became infected with a rare, hard-to-identify microbacteria. The infection required a month-long hospitalization and a fourth surgery to replace the damaged valve, giving Woody a new pulmonary valve.

"We were so grateful we followed Allison (Cabalka) down to Mayo because we definitely got the cutting-edge care that was right for Woody," Heidi says "They looked at what Woody needed and when he needed it. We had so much confidence in Dr. Dearani when we met him. We were just really scared, and he helped."

For Woody, managing his hospitalizations and recovering from surgery has always been closely linked to skiing. "Whenever I was having a hard time in the hospital, I'd visualize myself skiing and imagine I was in my favorite spot," he says. "I kind of use it as my benchmark after surgery: How quickly can I ski again?"

For Woody's parents, encouraging their son to pursue his goals while guarding his health was a balancing act. "Raising him was difficult because he didn't outwardly show these signs," Heidi says. "We always raised him that he should just try anything, but sometimes it's a modification. While we understood the risk, we let him try things once."

Changing course

Following back-to-back open-heart surgeries as a teen, Woody realized that he would be unable to pursue a career as a competitive skier, but that didn't stop his dreams of earning a living on the slopes. Following high school, he moved to Montana and took a position requiring familiarity with the nature in forested, mountainous regions.

Woody's job is physically demanding, often entailing 5- to 6-mile hikes to job sites. During his off hours, he routinely practices backwoods skiing, which involves skiing in areas not outfitted with lifts, boundaries or safety patrols. Despite the physicality his job and hobbies require, Woody embraces his active lifestyle.

"Sometimes I have to be a little careful, but I am open with my friends about it and tell them what is going on," Woody says. "People are open and accepting of it, and I'm still able to do everything I want to do."

In fall 2019, however, the demands of the work began weighing on him. "I could feel myself slowing down," Woody says. "I'd get home after a hard day of hiking, and I just had to go sit on the couch and recover for the next two days."

"After the operation, I felt like a different person, in a really good way."

Woody Hust

At his next appointment with Dr. Cabalka, the decision was made to refer him to Dr. Dearani for his fifth open-heart surgery. "His aortic valve worked really well for many years, and then eventually started to leak," Dr. Cabalka says. "The leakage caused blood to back up into Woody's heart and necessitated another surgery where Woody received an artificial, mechanical aortic valve."

"After the operation, I felt like a different person, in a really good way," Woody says. "I have more energy. My heart rate is not as high. My heart doesn't pump when I'm doing those activities, and all around I feel a lot better."

The best part for Woody, who was back to skiing six weeks to the day after his surgery, was how the operation enhanced his performance on the slopes. "It's definitely improved my ability to ski. It's a big positive to have gotten that surgery."

Forging close relationships

With the mechanical valve keeping his blood flowing smoothly, Woody should not need another open-heart surgery for a long time, Dr. Cabalka says. "We hope these valves will be really durable for most of his life. Barring infection or other issues, they could last many, many years."

When the time comes for Woody to receive a new pulmonary valve, the hope is that the valve can be replaced with a transcatheter valve, which would use a catheter inserted through the femoral artery, rather than open-heart surgery, to place the valve, Dr. Cabalka says.

To ensure that his heart valves and his heart function remain normal, Woody will continue visiting Dr. Cabalka annually for checkups. The visits are something he looks forward to. "It's been cool to go on this journey, from when I had that first surgery at Mayo at 15 to now, to be able to go back, just to say hi," Woody says. "I feel so cared about and valued there. I know a lot of other people with congenital heart defects who go elsewhere, and they don't have the same stories."

Visits with Woody and his parents are happy occasions for Dr. Cabalka. "We do have the medical visit, but then we spend the rest of the time chatting about their family and my family," she says. "It's always nice to have the personal relationship. It's this rewarding part of the doctor-patient relationship in Pediatric Cardiology that we really appreciate. As someone who's been able to care for Woody through his entire life so far, it really is a privilege to be able to see this young man grow up to the amazing person that he is."

HELPFUL LINKS

• Read more about congenital heart defects.
• Check out Mayo's Center for Congenital Heart Disease.
• Learn more about Pediatric Cardiology at Mayo Clinic.
• Visit the Mayo Clinic Children's Center.
• Request an appointment.

The post Embracing His Heart’s Passion, Despite Congenital Heart Defect appeared first on Mayo Clinic News Network.

On the next Mayo Clinic Radio program, Dr. Joseph Dearani, chair of Cardiovascular Surgery at Mayo Clinic, will discuss diagnosis and treatment of congenital heart defects. Also on the program, Dr. Tatnai Burnett, a gynecologist at Mayo Clinic, will discuss endometriosis. And Dr. Jonathan Fillmore, an oral and maxillofacial surgeon at Mayo Clinic, will explain treatment for temporomandibular joint disorders.

Here's you're Mayo Clinic Radio podcast.

The post Congenital heart defects / endometriosis / temporomandibular joint disorders: Mayo Clinic Radio appeared first on Mayo Clinic News Network.