Stories of Hope and Recovery

I. Uphill Battle

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. It is called “Running Down the Hill.”

But for Clemson freshman Steven Mets, everywhere on campus seemed to be uphill. 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 at 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. Steven’s heart health gradually deteriorated during high school in his hometown of Aiken, S.C despite many heart operations. The signs were a subtle but persistent increase in respiratory issues and fatigue.

Steven, an excellent student, chose Clemson to be close to home yet far enough away to be alone. 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 little energy to schoolwork, 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.

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 that Steven consider a procedure called a transcatheter aortic valve replacement (TAVR) rather than getting a heart transplant. A TAVR replaces a patient’s aortic heart valve without open-heart surgery. 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.

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 plugged up and 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 a stroke. “This was a bit scary to us… you really don't want to be the experiment,” said Steven’s mother, Mindy Mets.

Steven and his parents eventually agreed that TAVR was the best option despite these concerns. The procedure was scheduled for December after Steven finished his fall exams.

In October, however, Steven, now a sophomore, got sick. It was 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 undergo the TAVR. But then they began to worry that Steven would die if they did not do it.

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.

Steven's parents were terrified when he was taken to the catheterization (cath) lab for the procedure on that day in early November.

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

Mindy and Mike Mets’ second child was born at 8 pounds 2 ½ ounces on his due date in December. 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 did not.

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 was not 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 do reconstructive surgery to 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 deciding, however, a cardiothoracic surgeon took a closer look at Steven’s heart using a heart catheter. After a lengthy 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.¹

Mike and Mindy researched the disease, and what little they found offered a grim conclusion: Shone’s babies do not 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 that he could go home with his family.

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 ages 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 before each operation or procedure, which posed its own 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 decided 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 difficulty 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 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 rather 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.”

Steven became highly 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, which 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 went on almost every camping trip, trying to stay in the background as much as possible.

Steven played many sports, such as T-ball, basketball, baseball, swimming, bowling, and even noncontact karate. They let him ride a bike but always ensured he wore a helmet and protective gear. But “normal” was not 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 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.

Steven was sent to see Dr. Wendy Book, director of the Adult Congenital Heart Center at Emory University in Atlanta in January. Dr. Book determined that Steven’s weak heart would benefit from a resynchronization therapy called a pacemaker-defibrillator. This implant helped Steven’s heart pace more efficiently and could also 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.

A 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. She said outcomes are good after the transplant, but survival is 50% after 15 years.

The new pacemaker-defibrillator made Steven feel significantly better.

“He was still 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 worsened 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 would not stay open despite several attempts.

Dr. Book thought what appeared to be an infection could be part of Steven’s congenital abnormality. Despite the challenges, she urged Dr. Babaliaros to go ahead with the TAVR as soon as possible. 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 high—risk procedure, given Steven’s condition. But Dr. Babaliaros had trained with the best. He worked with Alain Cribier, MD, FACC, FESC, an interventional cardiologist in Rouen, France, who pioneered both TAVR and aortic valvuloplasty, the balloon procedure Dr. Babaliaros had unsuccessfully tried to open up Steven’s aortic valve.²

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 could 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 (extracorporeal membrane oxygenation)? Dr. Babaliaros wondered.

ECMO, or extracorporeal membrane oxygenation, is a heart-lung bypass machine typically used in babies or small children, where its outcomes are much better than when used in adults. Many risks are 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.

“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 did not tolerate the anesthesia very well because he was so sick going into it.

“And I said, yes, you should because he can survive and get through this.”

So when Dr. Babaliaros and his team could 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 was not 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 would not regain consciousness until a week or so after the procedure. With a full red beard and bedraggled appearance, Steven did not look too good, Mindy said. His immediate concern was he missed registering for classes at Clemson. The unwelcome news was that, yes, he had missed registration, and he probably would not be registering for classes again anytime soon. The good news was that Steven’s brain was working.

“That’s when I knew Steven was still in there somewhere,” Mindy said.

For as bad as Steven looked, he felt worse. He could not lift his head and even had trouble lifting his fingers. He had a challenging 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 exhausted. His muscles had atrophied. His organs were damaged. His vocal cords were damaged. He could not 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 could not. 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 20^th birthday in December. 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 is 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 would not 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 the next year he was ready to return to Clemson and its hills.

VI. Can Technology Keep Up With Steven?

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 is even given up his moped.

After returning to campus in the summer, 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 in his computer sciences honors program. The group develops video games and other applications, such as training simulations for implanting medical devices and 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 nonprofit 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 reprogramming. 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 novel 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 and older patients. It shows those with advanced diseases can benefit from valve replacement. It shows the promise of different approaches, such as going through the carotid artery, which has become much more common. And it shows the importance of heart specialists collaborating—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 anymore.”

¹ Data and Statistics on Congenital Heart Defects | CDC
² Percutaneous Transcatheter Implantation of an Aortic Valve Prosthesis for Calcific Aortic Stenosis | Circulation (ahajournals.org)