The first clue came with a murmur.
At a mere week old, Joe LaRocca was diagnosed with an extraordinarily rare heart defect. Both ventricles were reversed.
Fortunately, with this particular defect, the arteries are reversed too, essentially “correcting” the abnormality. That’s where it gets its name — congenitally corrected transposition of the great arteries (CCTGA).
In a normal heart, the demanding duty of pumping oxygenated blood into the body is handled by the left ventricle, and the right ventricle pumps blood a short distance into the lungs. But Joe’s heart was far from normal.
“The right ventricle is not meant to do the harder work,” says Dr. Elizabeth Blume, Heart Failure and Heart Transplant Program medical director at Boston Children’s Hospital. “Since the ventricles are reversed in patients with CCTGA, over time, this muscle tires out.”
For the past three decades, the team at Boston Children’s Heart Center has medically and surgically managed Joe’s condition. And at 33, his heart is still ticking.
Tackling congenitally corrected transposition of the great arteries
Joe remained relatively healthy until he was 13, at which time he underwent a double-switch repair, a complex operation where surgeons reverse the arteries and make a tunnel between the heart’s upper chambers in order to redirect blood flow. This allows the left ventricle to then do the work it was meant to do, pumping blood to the body.
“As a young teen, I didn’t understand the magnitude of what was happening,” says Joe. “I didn’t know it would take so long to recover, but once I did, I felt fine all the way through college.”
Then, a year following his graduation, Joe began to feel unusual fatigue. Although he managed to complete a graduate degree in film from Boston University, it became apparent that something was seriously wrong.
“I was thinking, ‘Maybe I am depressed, maybe I don’t eat right.’ I didn’t want to believe my heart was dying in my body.”
Even standing for an hour was taxing. He felt nauseated and utterly exhausted.
“I went to Boston Children’s and was given a stress test. To get a reading, you peddle for eight minutes. After one minute, they said, ‘Stop.’”
The test was too much activity for Joe’s failing heart. He was admitted to Boston Children’s and his primary cardiologist immediately consulted the Advanced Cardiac Therapies (ACT) team to talk heart transplant. That was the first time he met Dr. Blume.
“It was the most surreal meeting ever. I’m sure she said many other things, but all I heard were the dangers of rejection, how a transplant lowers white-blood-cell counts and the link between lymphoma and immunosuppressant medications. It was like she dropped an atomic bomb. I am still recovering.“
Like many adolescents and young adults, this was the first time Joe had faced the reality of his situation. After the ACT team took over Joe’s care, he was started on multiple medications and was placed on the heart transplant list.
It’s like a cancer patient in remission. I fully expect what happened to happen again.
Despite being on the list, Dr. Blume’s team continued to work with medications and pacing therapies to improve Joe’s heart function. At the same time, Joe focused on exercise, nutrition, cardiac rehabilitation and medication compliance with the support of his care team and family.
Living with an uncertain future
With a new pacemaker and a new round of medications, during the next year, Joe’s heart improved enough to be taken off the transplant list.
It was a relief, but, as Joe explains, the brain rewires after being under stress for so long.
“It’s like a cancer patient in remission,” he says. “I fully expect what happened to happen again. It makes you recede, shuts you off from the world, from all your friends. That’s why I got Odie.”
Odie is Joe’s adopted coonhound. She’s old, blind in one eye, and her back foot is somewhat disfigured, as a result of a farm accident during her youth.
“It is just too obvious of a metaphor,” he says. “She had a bad thing happen to her and she doesn’t care. It’s cheesy. We should be on Oprah.”
But despite the cliché, Odie has helped Joe find himself again. Every day, he takes her to the dog park near his home in Somerville, Massachusetts. It’s his way of finding common ground with other people, and it keeps him moving. He’s also following his bliss, teaching filmmaking and film history at Boston College.
“My life is great right now. I do think a transplant is in my future, but the longer we wait the better. If I can make it another five or six years, maybe they can grow me a heart in a jar, or maybe Apple will have an iHeart.”
Learn more about the Boston Children’s Heart Transplant Program.