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Novel program gives congenital heart disease patients new options

Aug. 23, 2018, 9:20 AM

Patient Michael Sharpe, second from left, with members of the Advanced Congenital Cardiac Therapy team, including, from left, Jonathan Menachem, MD, director, Ben Frischhertz, MD, Rachel Fowler, MSN, APRN, and Emily Sandhaus, RN. (photo by Anne Rayner)

Experts at the Vanderbilt Heart and Vascular Institute are forging new ground in the development of a first-of-its-kind program aimed at adults with congenital heart disease (ACHD).

The novel program in Advanced Congenital Cardiac Therapies (ACCT) allows for patients to be evaluated for heart transplantations and ventricular assist devices (VAD) — a foreign concept to Michael Sharpe, 39, who received a new lease on life in May.

Diagnosed at 9 months old with tetralogy of Fallot, Sharpe had resigned himself to the lifelong condition caused by the combination of four heart defects present at birth. He had already undergone the routine surgeries and procedures to improve the oxygen-poor blood flow out of his heart to the rest of his body.

“I did not know that transplant was an option for congenital heart patients,” said Sharpe. “My parents were with me when the doctors mentioned it and they were shocked too.

“This has allowed me the incredible option to watch my son grow up. Evan is my motivator. When they told me that I was in heart failure, the thought of not seeing him grow up hit me the most.

“I know people don’t live very long in heart failure and I kept thinking, ‘I’m getting maybe two to five more years.’ But now? I have 20, 30-plus years. That’s the absolute best part.”

Sharpe is one of three patients with ACHD transplanted at Vanderbilt within the last year. An additional three patients are currently listed for heart transplant and another three have undergone VAD implants.

More than 30 patients have been evaluated for advanced therapies since the launch of the program, according to Jonathan Menachem, MD, assistant professor of Medicine and Pediatrics at Vanderbilt.

The program’s referral volume continues to grow each week, said Menachem, director of ACCT at Vanderbilt.

“To my knowledge, we are the only place in the country that has dedicated resources to develop a program and collaborative network focused on providing advanced heart failure therapies to ACHD patients,” said Menachem. “This is possible at a place like Vanderbilt because of the strengths of the multitude of specialists required to care for complex patients.”

Patient Michael Sharpe, left, and his father, Larry Sharpe, pose for a photo prior to Michael’s monthly visit to check the viability of his heart after his heart transplant in May. (photo by Anne Rayner)

Menachem has spent time at national conferences presenting evidence on the topic of providing dedicated programming for ACHD patients as well as collaborating and networking with medical teams locally, regionally and nationally.

“Adult congenital heart is the fastest growing segment in cardiology, as more than 85 percent of children born with congenital heart defects survive to adulthood,” said Ben Frischhertz, MD, assistant professor of Medicine. “Furthermore, heart failure is the No. 1 cause of death in ACHD, and yet these patients are less likely to undergo transplant or have a VAD implanted.”

Menachem hopes that as the field moves forward, more centers will offer this service.

“But it is going to take a bit for health care to catch up, especially as more and more congenital heart patients make it into adulthood. We have more survivors and not enough providers,” said Menachem.

Menachem said Vanderbilt, as a high-volume heart transplant center, is poised to be a leader in the area. His team recently published data in the Journal of Heart and Lung Transplantation demonstrating that ACHD patients benefit from undergoing transplantation at high-volume centers.

“And, in ACHD patients who survive the first year post transplant, their outcomes are at least equivalent if not better than non-congenital patients who undergo transplantation,” said Menachem. “This is a vast improvement from years ago when most ACHD patients were considered too high risk for transplant.”

Part of ACCT’s goal is to introduce the idea of heart transplantation and VAD earlier in a patient’s treatment plan. In the past, patients presented too late and were too sick to be considered for transplant.

Under the new design, patients will be plugged in sooner and medical teams will be better prepared about the appropriate timing for an advanced therapy.

Sharpe was 38 before he heard about the prospect of transplantation and counts himself among the lucky ones.

In late 2017, he was off shore working as a paramedic on an oil platform when he went into heart failure. Once stabilized, he returned to Vanderbilt to see Frischhertz, who connected him with Menachem.

“Congenital heart patients decline rapidly,” said Sharpe. “It’s like jumping off the cliff instead of going downhill when we start having symptoms.”

Now on the mend, Sharpe can tell a stark difference since receiving his new heart.

During his rehabilitation session, it’s not his heart telling him to stop or slow down, it’s fatigue in his legs.

“My son keeps telling me, ‘Daddy you got a new heart and you might be able to keep up with me, but I’m still going to beat you when we race.’”

Sharpe said he is looking forward to the challenge.

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