I. Up Hill Battle
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Clemson University has a tradition for home football games in which its players run, leap and skip down a grassy hill into a packed Memorial Stadium as orange balloons and cheers fill the air.1 It’s called “Running Down the Hill.”
But for Clemson freshman Steven Mets, everywhere on campus seemed to be up hill. The hills of Clemson, S.C. – 30 miles from the Blue Ridge Mountains – forced him to take his moped or plan the flattest possible walking route. Even so, he sometimes would arrive to class gasping for air as he collapsed into his seat.
Steven had a weak heart due to a rare congenital heart condition called Shone’s complex. Despite a number of heart operations, Steven’s heart health had gradually deteriorated during his high school years in his hometown of Aiken, S.C. The signs were a subtle but persistent increase in respiratory issues and fatigue.
An excellent student, Steven chose Clemson to be close to home yet far enough away to be on his own. In addition, many of his friends were going there and it had a good computer sciences program, an area of interest for him.
Steven’s heart got worse in the fall of his freshman year, leaving him increasingly out of breath and tired. He devoted what little energy he had to school work, making it difficult for him to make social connections or join student organizations. It began to look like he would need a heart transplant soon.
In January 2012, Steven went to a specialist in adult congenital issues at Emory University Hospital in Atlanta who prescribed a pacemaker-defibrillator and drugs to help him feel better. She also recommended rather than getting a heart transplant, Steven consider a recently approved procedure called a transcatheter aortic valve replacement (TAVR). In a TAVR, a patient’s aortic heart valve is replaced without open-heart surgery.2 Instead of opening the chest, an interventional cardiologist would use a flexible tube called a catheter to access and replace the faulty valve with a new valve.3
But there were concerns about whether the TAVR procedure would be right for Steven. Steven’s pediatric cardiologists had often warned that adult heart treatments are rarely appropriate for young patients with congenital issues. TAVR was performed mostly on older adults with aortic valves that were plugged up and were too sick to have open heart surgery – not on patients like Steven. Due to Steven’s anatomy, the TAVR would have to be done through the carotid artery in his neck, a rarely used access point due to fears of causing stroke.
“This was a bit scary to us … you really don't want to be the experiment,” said Steven’s mother, Mindy Mets.
Despite these concerns, Steven and his parents eventually agreed TAVR was the best option. The procedure was scheduled for December 2012, right after Steven finished his fall exams.
In October, however, Steven, now a sophomore, got sick. It seemed to be just a common cold – until he started shaking.
Steven had pneumonia. He was soon admitted to Emory University Hospital in Atlanta. His heart began to fail. So did his kidneys and liver. He was put on a ventilator to help him breathe. He lost consciousness.
At first, his doctors said Steven was too sick to have the TAVR done. But then they began to worry if they didn’t do it, Steven would die.
What had been a planned procedure now became a rare emergency TAVR.
Steven’s older brother, Joey, and his wife, flew to Atlanta from Boston to be there. A Catholic priest blessed him.
As he was taken to the catheterization (cath) lab for the procedure on that day in early November 2012, Steven’s parents were terrified.
And that was before the interventional cardiologist came out to explain to them why he had to stop the procedure.
II. Growing up with Shone’s Complex
|Steven was born with CHD and ultimately diagnosed with Shone’s Complex. On Christmas Day 1992, doctors said they could help him.
Mindy and Mike Mets’ second child was born at 8 pounds 2 ½ ounces on his due date, Dec. 22, 1992. Steven had a good Apgar score — his breathing, heart rate, muscle tone, reflexes and skin color all looked fine.
He looked like a healthy, robust baby. Until he didn’t.
Just a day or so old, Steven began to breathe rapidly. His legs turned blue. He was sent to Augusta, Georgia for a pediatric cardiology evaluation.
When Mindy got to the hospital, she thought Steven looked out of place among the tiny preemie babies in the neonatal intensive care unit (NICU). She wasn’t prepared for what came next.
Steven’s doctors said there were three options. They could put him on the list for a rare and risky infant heart transplant, or they could do reconstructive surgery that would gain Steven only a few months of life.
“Or we could take him home and love him while he's still here,” Mindy said.
Before a decision was made, however, a cardiothoracic surgeon took a closer look at Steven’s heart using a heart catheter. After a long examination on Christmas Day, he met with Steven’s parents.
“I think we can help him,” he said.
Steven was ultimately diagnosed with a rare condition called Shone’s complex (also known as Shone’s syndrome) in which the structures, such as the mitral and aortic valves, on the left side of his heart are narrow, underdeveloped or misshaped, making it difficult for blood to be pumped out of the left ventricle of his heart to the rest of his body. Birth defects of the heart happen in nearly one percent, or about 8 out of every 1,000 newborn babies; Shone’s complex occurs in less than one percent of all congenital cardiac anomalies.4 5
Mike and Mindy researched the disease and what little they found offered a grim conclusion: Shone’s babies don’t live long.
On the day after Christmas, 4-day-old Steven had surgery to remove a blockage in his aorta and correct a few other issues in his heart. It was successful enough so that he could go home with his family.
|During Steven’s childhood he would undergo multiple operations and procedures, including three open heart surgeries.
It would be the first of many operations and procedures Steven needed to stay alive. When he was nearly four years old, Steven had open heart surgery to replace his defective mitral valve with a mechanical valve, requiring him to take blood thinners. Given that blood thinners can cause uncontrolled bleeding, this was a particular challenge for Steven – after all, little boys are prone to getting bruises and scrapes.
He would have to have two more open heart surgeries at the ages of 9 and 14 to replace mechanical mitral valves that became too small as Steven grew. Before each operation or procedure, Steven would have to be taken off blood thinners, which posed its own set of risks.
“It was never in and out of the hospital,” Mindy said. “It was always a few days before and a few days after, and many of those procedures have resulted in some pretty significant complications as well.”
His parents made the decision early on that even though he had a weak heart, they would try to make Steven’s life as normal as possible. Research showed that “heart kids” who are over-protected tend to have a hard time functioning independently or finding a job as an adult, Mindy said.
Giving Steven a “normal life” was scary. A scraped knee or a bruised elbow could result in a serious bleeding. Any respiratory illness or infection could be deadly.
When Steven started kindergarten, his parents alerted his teachers and school administrators of his condition and that he was on blood thinners. They tried to warn but not alarm. After a while, they got pretty good at it.
“We did not want them to isolate him or not let him participate in things and make him feel like he's being punished,” Mike said. “It’s a fine line.”
|Although Steven could not participate in contact sports, he became very active in Boy Scouts, eventually achieving the highest rank, Eagle Scout.
Steven became very active in Boy Scouts, eventually achieving Eagle Scout, the highest rank. He loved summer camp at Camp Barstow along the banks of Lake Murray in Saluda County, S.C. and at the Wild Turkey Federation preserve near Edgefield, S.C. But trips to the woods were risky for a boy on blood thinners, and this worried his Boy Scout leaders.
“There's nothing quite like the look on a Scout leader's face when you tell them your child has a heart issue and is on blood thinners," Mindy said.
His father ended up going on almost every camping trip, trying to stay in the background as much as possible.
Steven played a lot of sports, such as T-ball, basketball, baseball, swimming, bowling and even non-contact karate. They let him ride a bike, but always made sure he wore a helmet and protective gear. But “normal” wasn’t always possible. Steven’s weak heart made it hard to keep up with other kids on the field. Contact sports were out. So were activities like climbing trees and water skiing.
Fortunately, like the rest of his family, Steven was better with books than with balls.
“His college scholarships were all academic,” Mindy said.
Steven’s health increasingly became an issue in high school. During his first year at Clemson, discussions turned to the possibility of a heart transplant.
In January 2012, Steven was sent to see Dr. Wendy Book, director of Adult Congenital Heart Center at Emory University in Atlanta. Dr. Book determined that Steven’s inefficient heart would benefit from a resynchronization therapy called a pacemaker-defibrillator. This implant helped Steven’s heart pace more efficiently and it also could shock his heart back to life if it went dangerously out of rhythm (this would happen twice). She also put him on medicine to treat his heart failure.
Dr. Book hoped these measures would help Steven become healthy enough to stave off a heart transplant and, instead, get a new aortic valve through TAVR.
“It was important to delay transplant as long as possible,” Dr. Book said.
Heart transplant is a risky surgery that requires patients to take drugs for the rest of their lives that suppress their immune systems so their bodies will not reject the “foreign” heart organ. Outcomes are good after transplant, but survival is just 50 percent after 15 years, she said.
The new pacemaker-defibrillator made Steven feel significantly better.
“He was still very, very sick, but that gave us a window to consider replacing the aortic valve instead of going straight to transplant,” Dr. Book said.
The TAVR was scheduled. All went according to plan – until Steven got pneumonia.
III. Trying to Keep Steven Alive
Not long after Steven was wheeled to the cath lab for the TAVR, Vasilis Babaliaros, MD, FSCAI, Steven’s interventional cardiologist at Emory, came out again to meet with the Mets family in a little room away from the waiting room.
“If you're in the hospital waiting room and they take you into the little room, you know you're in trouble,” Mindy said.
Dr. Babaliaros said it appeared Steven had an infection around his aortic valve. He had to stop the procedure.
That evening, Steven’s condition grew worse as he went from stable heart failure into acute respiratory distress, as his lungs filled with fluid due to his failing heart. Dr. Babaliaros tried to open Steven’s aortic valve through a balloon procedure, but the valve was “gummy” and wouldn’t stay open despite several attempts.
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|Watch this animation to learn more about a new, less invasive procedure for replacing the aortic valve called transcatheter aortic valve implantation or TAVR. (Animation provided courtesy of Medtronic.)
Dr. Book thought what appeared to be an infection could be part of Steven’s congenital abnormality. She urged Dr. Babaliaros to go ahead with the TAVR as soon as possible, despite the challenges. Steven was too sick for the heart transplant list. Without TAVR he would die – possibly within 24 hours.
The following morning, Dr. Babaliaros and his team were determined to complete the TAVR “regardless of what state we find things in.”
It was a novel, challenging and, given Steven’s condition, high-risk procedure. But Dr. Babaliaros had trained with the best. In 2004, he worked with Alain Cribier, M.D., FACC, FESC, an interventional cardiologist in Rouen, France, who pioneered both TAVR in 2002, as well as aortic valvuloplasty, the balloon procedure Dr. Babaliaros had unsuccessfully tried to open up Steven’s aortic valve.6
Dr. Babaliaros threaded a catheter smaller than his finger through an incision in Steven’s neck into his carotid artery.
He discovered Steven’s aorta was kinked. There was no way to come straight at the valve.
“We were very worried we would miss the valve with the implant,” Dr. Babaliaros said.
It took several tries before Dr. Babaliaros was able to position the catheter to get at Steven’s valve. He then carefully guided a bioprosthetic valve crimped inside the frame of a stent to the aortic valve and opened it up like an umbrella.
No sooner than they placed the valve, Steven went into cardiac arrest. They did cardiopulmonary resuscitation (CPR) on him to get his heart beating again. But soon it stopped again. So they had to do CPR again. And again.
Even when they got Steven’s heart beating, his heart and lungs had trouble oxygenating his blood. Should they put Steven on ECMO? Dr. Babaliaros wondered.
ECMO, or extracorporeal membrane oxygenation, is a heart-lung bypass machine that is typically only used in babies or small children, where its outcomes are much better than when used in adults. There are many risks associated with mechanical support of the heart, including severe infections, bleeding, blood clots and stroke. Dr. Babaliaros worried putting Steven on ECMO would simply prolong his life without giving him much benefit.
On the other hand, Steven was a special patient to Dr. Babaliaros, mostly due to his youth and lack of life experience. The day before his first TAVR attempt, he asked Steven what movie he would like to see that night. He ended up spending hours searching before he found a copy of “The Blues Brothers.”
Steven was too young to die, too young to give up on.
“The next call I got was, ‘CPR is in progress, should we put him on ECMO?’ ” Dr. Book said. “The valve had been successfully deployed and everything looked good. But Steven didn't tolerate the anesthesia very well, because he was so sick going into it.
“And I said, yes, you should, because he can survive this and get through this.”
So when Dr. Babaliaros and his team were able to get Steven back one more time, they quickly put him on ECMO and stabilized him.
But had there been brain damage? And could Steven stay alive on his own?
IV. “I missed registration!”
Dr. Babaliaros explained to the Mets family that while the procedure went well, Steven had gone into cardiac arrest and had to be put on a heart-lung machine. ECMO would allow Steven’s heart to rest and, hopefully, recover. But a lot of things could go wrong, Dr. Babaliaros warned them.
“Recovering from ECMO was probably Steven’s biggest challenge,” Dr. Book said.
It wasn’t his only challenge. He had been clinically dead – so he had to recover from that. His heart, liver and kidneys had all failed, so they had to recover, too.
But Steven had youth on his side.
It took four to five days to get Steven off ECMO, and another three or four days to get him off the ventilator. But given there were no guarantees he would get off either machine, this was a major accomplishment.
Steven wouldn’t regain consciousness until a week or so after the procedure. With a full red beard and bedraggled appearance, Steven didn’t look too good, Mindy said. His immediate concern was he missed registering for classes at Clemson. The bad news was that yes, he had missed registration, and he probably would not be registering for classes again anytime soon. The good news was, Steven’s brain was definitely working.
“That’s when I knew Steven was still in there somewhere,” Mindy said.
For as bad as Steven looked, he felt worse. He couldn’t lift his head and even had trouble lifting his fingers. He had a hard time focusing on the television. He had bad dreams. Steven did not want to be alone, so his family never left his side.
Recovery, if it came, would be a long ordeal.
V. Steven’s Long, Slow Recovery
Steven had gone through a major heart procedure – while having pneumonia. He had been on a ventilator and a heart-lung bypass machine. He had been clinically dead.
Now the 19-year-old found himself lying in a hospital bed just trying somehow to get his strength back. He was totally exhausted. His muscles had atrophied. His organs were damaged. His vocal cords were damaged. He couldn’t swallow without aspirating.
“If you've ever been in a hospital, about the only excitement in your day is when you get to eat or drink,” Mindy said. “That was completely taken away from him. The thirst and dryness in his mouth left him in distress.”
Ever so slowly with the help of his parents and the medical professionals at Emory University Hospital, Steven got better. It was several weeks before Steven was strong enough to start speech, physical and occupational therapies.
“I had to relearn to swallow, which is harder than you would think,” Steven said.
When they first tried to help him stand, he couldn’t. It took Steven weeks before he could get from his bed to the door of his room – and then only with a walker and help from others. It took more weeks before he was able to walk around the hospital floor with a walker by himself.
His goal was to go home before his 20th birthday on Dec. 22, 2012. Steven was highly motivated to get better, and that motivation plus faith enabled him to celebrate his birthday at home.
After seven grueling weeks in the hospital, the hope was Steven would recover faster and more comfortably in his own home. With the help of home health aides and nurses as well as speech, physical and occupational therapists, that’s what he did.
Once free of the walker, he still needed a brace on his left foot due to nerve damage from being connected to the heart-lung machine. He needed prolonged physical therapy before he was able to regain balance, strength and endurance in that foot. One exercise was to stand up from sitting in a chair without using his arms.
“I still can’t feel my toes, but I have full use of my ankle – I didn’t know if I would get that back,” Steven said.
He started taking online classes at Clemson so he wouldn’t be too far behind his classmates.
Finally, six months after the procedure, Steven began to feel like himself again.
“I can't even describe how much more energy I had and how much better I felt after having gotten the TAVR valve,” Steven said.
By fall of 2013, he was ready to return to Clemson and its hills.
VI. Can Technology keep up with Steven?
|Steven has pushed beyond limits, maintaining a 4.0 grade average in college. He plans to pursue a career in computer science after graduation.
Reflecting back, Steven believes he would not be alive today without medical technology and his doctors and specialists.
When his heart stopped on the cath lab table, “a more reasonable doctor would have probably just called it.” Instead, his doctors said “there's one more thing we can try.” Also, if he had needed the TAVR just a year earlier, he would have been out of luck. “That’s God’s grace,” he said.
Steven is more than just alive. He has rarely felt better in his life.
Before he had his TAVR, he remembers how difficult it was to get around the hilly Clemson campus.
“I'd be walking with friends and I'd have to ask them to slow down when we got to that point, and it was just embarrassing,” Steven said.
Now few of Clemson’s hills slow him down. He’s even given up his moped.
After returning to campus in the summer of 2013, Steven became active in campus activities. He joined Tiger Vision, the student-run cable network, as a technical engineer, and is now its web administrator.
The Clemson senior is also part of the Virtual Environment Group, a research initiative part of his computer sciences honors program. The group develops video games and other applications, such as training simulation for implanting medical devices and for robotic surgery. Lately, Steven has been working on a class project using a head-mounted computer display to help diagnose and, eventually, treat people with balance disorders.
“We put people in various situations – such as at the edge of a virtual cliff – to see how that affects their balance,” Steven said. “Doctors can use the data from this simulation to help diagnose the balance disorder.”
Steven (who has a 4.0 grade average) has a job related to virtual reality training lined up with Dynetics, a company in Huntsville, Alabama, whose clients include the U.S. Department of Defense and other government agencies, commercial customers, and non-profit agencies. He will move to Huntsville, a seven-hour drive from Aiken, shortly after he graduates.
Steven still has a weak heart that requires medication. He continues to have heart rhythm issues for which his pacemaker-defibrillator occasionally needs to be reprogrammed. He is at risk for other potential complications of his Shone’s complex. Eventually, he will almost certainly need a transplant, Dr. Book said.
“They have known since I was born I would eventually need a new heart,” Steven said. “The question is, how long can we push it off? My hope is medical technology keeps improving fast enough so that can be a long time.”
With continued innovation, collaboration and growth in treating structural heart disease and heart failure, including new devices and new approaches, Dr. Babaliaros believes Steven’s hope could be realized.
“Cases like Steven's will help realize that hope for him and others,” Dr. Babaliaros said. His case shows TAVR can be used on younger patients as well as older patients. It shows those with advanced disease can benefit from valve replacement. It shows the promise of different approaches, such as going through the carotid artery – which has since become much more common.7 8 9 “And it shows the importance of heart specialists collaborating together – a key to Steven’s survival,” Dr. Babaliaros said.
“Steven shows us what's possible when you push the limits of medicine and innovation,” Dr. Book said.
Steven sometimes asks, “Why me?” But he cannot imagine life without his condition.
“I am who I am because of this condition – I wouldn’t be the same person without it,” Steven said. “I wouldn’t ask to have never had it – I would just ask I not have it any more.”
Steven graduates from Clemson Dec. 17, 2015. He plans to walk in the ceremony. Compared to where he was three years ago, that will be like Running Down the Hill.
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SecondsCount hosts the Unbroken Hearts program to raise awareness of cardiovascular health and wellness, including prevention, management and treatment of cardiovascular disease. The program is an effort by SCAI, the Society for Cardiovascular Angiography and Interventions, with support from Abbott Vascular and Abiomed. We gratefully acknowledge these grants while taking sole responsibility for all content developed and disseminated through this effort.
1 Clemson University. Traditions. Running down “The Hill.” Accessed Nov. 4, 2015. http://www.clemson.edu/about/traditions.html
2 SecondsCount.org. Transcatheter Aortic Valve Replacement (TAVR or TAVI). Jan. 23, 2015. http://secondscount.org/treatments/treatments-detail-2/transcatheter-aortic-valve-replacement-tavr-2#.Vh1MJ-xViko
3 American Heart Association. What is TAVR? Content last reviewed March 3, 2014. http://www.heart.org/HEARTORG/Conditions/More/HeartValveProblemsandDisease/What-is-TAVR_UCM_450827_Article.jsp#.Vjjt_tKrS70
4 Centers for Disease Control and Prevention. Congenital Heart Defects: Data and Statistics. Page last reviewed: July 9, 2014. Page last updated: July 9, 2014 http://www.cdc.gov/ncbddd/heartdefects/data.html
5 Children’s Hospitals and Clinics of Minnesota. Shone’s Syndrome. The Children’s Heart Clinic. 2012. http://www.childrensheartclinic.org/DiagnosisIllustrations/Shone_s.pdf
6 Cribier A, Eltchaninoff H, Bash A, Borenstein N, Tron C, Bauer F, Derumeaux G, Anselme F, Laborde F, Leon MB. Percutaneous Transcatheter Implantation of an Aortic Valve Prosthesis for Calcific Aortic Stenosis: First Human Case Description. Circulation (2002) 106: 3006-3008. http://circ.ahajournals.org/content/106/24/3006.full
7 Petronio AS, De Carlo M, Bedogni F, et al. 2-year results of CoreValve implantation through the subclavian access: a propensity-matched comparison with the femoral access. J Am Coll Cardiol. 2012;60:502-507.
8 Guyton RA, Block PC, Thourani VH, Lerakis S, Babaliaros V. Carotid artery access for transcatheter aortic valve replacement. Catheterization and Cardiovascular Interventions (2013) 82; 4: E583–E586.
9 Mylotte D, Francois Obadia J, Sudre A, Modine T, Teiger E. 5TCT-700 Transcarotid Transcatheter Aortic Valve Replacement: Feasibility and Safety. J Am Coll Cardiol. (2015) 66: B285.