First partial heart transplant in New England helps 4-year-old boy: Jack was in the right hands – The Boston Globe

The Mangans hurriedly piled Jack and his 1-year-old brother, Declan, in their SUV and drove to Childrens, with Amy Mangans parents following in another car. At about 10 p.m. around 11 hours after his mother received the call doctors began operating on Jack.

Over 4 hours, the surgeons painstakingly replaced two key parts of Jacks heart and circulatory system with living tissues from the donor: his aortic valve and a three-quarter-inch segment of his aorta, the main artery in the body.

It was the first so-called partial heart transplant in New England, a potentially life-saving cutting-edge operation designed specifically for children, primarily for valve defects. For years, doctors have used mechanical valves or valves from cadavers, cows, or pigs to replace diseased valves in youngsters. Jacks operation has a distinct advantage over those procedures. Because he received living human tissue, his new valve will grow with the rest of his body, which doctors hope will eliminate the need for risky follow-up operations to insert bigger valves.

The worlds first partial heart transplant took place in the spring of 2022 on a 17-day-old boy at Duke Health in North Carolina. At least 15 other children in the United States have undergone partial heart transplants since then, including nine more at Duke, according to Dr. Joseph Turek, chief of pediatric cardiac surgery there, who pioneered the procedure.

Seven weeks after Jacks transplant, the rambunctious blond boy appears to be fully recovered. During a Zoom call in late June, he happily held up several of his 200 monster trucks at his house in Freehold, N.J., including a fire truck with oversized wheels.

I want to be a fireman, he said.

Jack pulled up his red New York Fire Department T-shirt to proudly show a 6-inch surgical scar running down his chest. He repeated the mantra his parents had taught him before the transplant: Im brave. Im strong. Im incredible.

This was Jacks sixth heart operation at Childrens. His parents are optimistic it will be his last, given how well the new valve which opens and closes like a door to let blood flow to the aorta is functioning. He has more energy than he did before the surgery and has no restrictions on activities.

The prognosis is excellent, said Amy Mangan, a dental hygienist, smiling. Theres nothing holding him back.

In a way, the path to bring the innovative operation to Childrens began more than three decades ago with a chance encounter in a college dormitory in Evanston, Ill.

Dr. Sitaram Emani, the cardiac surgeon who led the team that operated on Jack, met Turek, now at Duke, when they were freshmen living on the same hallway at Northwestern University. They later became surgeons in training in overlapping residencies at Duke and stayed in touch.

After Turek performed the first partial heart transplant, Emani spoke with him at least a dozen times and became excited about the possibility of doing the procedure at Childrens, said Emani. Childrens had been considering doing partial heart transplants for years, he said, and decided to try it after Tureks success.

We wouldnt want to do an operation that we think doesnt have a good outcome, Emani said. Dukes track record emboldened us to do this and to really take a chance.

The operation is ideal for children who have a defective valve but whose heart muscle is otherwise healthy. Patients with failing hearts are better suited for full transplants. Childrens performs 15 to 20 of those a year, Emani said, but its crazy to do that if the only problem is a valve; recipients of full transplants must take anti-rejection drugs for the rest of their lives, which pose potentially serious side effects. Patients who undergo partial transplants also receive those drugs, but doctors hope to lower the dosages over time.

The problem with Jacks heart was identified in an echocardiogram 22 weeks into Amy Mangans pregnancy. Doctors diagnosed aortic stenosis, a congenital heart defect that occurs when a fetuss aortic valve doesnt form properly, reducing the flow of oxygen-rich blood out of the heart. The narrowing puts stress on the left ventricle the lower left-sided pumping chamber of the heart and can lead to a more severe condition called hypoplastic left heart syndrome.

Physicians referred Mangan to Childrens Hospital. At 23 weeks and 28 weeks into Mangans pregnancy, doctors performed procedures to widen the fetuss aortic valve with an inflated balloon, according to Dr. Matan Setton, the pediatric cardiologist at Childrens who has helped oversee Jacks care. The baby was born in February 2020 and had additional surgeries when he was 11 days old and 10 months old.

Setton moved to Columbia University Irving Medical Center in New York City soon after Jacks birth and continued to handle his case. Although Jack generally appeared to be healthy he played soccer and loved to run Setton grew concerned the defective valve was making it hard for the left ventricle to relax after it pumped blood into the aorta.

Its not that [Jacks condition] was immediately life-threatening, but we were worried about the long-term health of the left ventricle and what it would mean for him as an adult, said Setton.

In 2023, after the first partial transplant at Duke, surgeons at Columbia performed similar operations on two children. When Setton returned to work at Boston Childrens last October, he suggested Jack as a candidate for the new procedure to Emani.

Jack was placed on a waiting list for a donated aortic valve in March. The list is maintained by New England Donor Services, the nonprofit that coordinates organ donation in the region. Childrens contacted the group in May when the donor was declared brain dead.

Alex Glazier, head of the organization, said she could not disclose anything about the donor because of confidentiality rules, but she called partial transplants a major innovation.

It solves a problem for pediatric heart patients because current grafts dont grow with the child, which leads to additional surgeries, she said.

An hour after receiving the phone call at 11:30 a.m. telling them to come to Boston, the Mangans left their house in Freehold and got on the road. Christopher Mangan drove.

We, of course, hit traffic, said Jacks father, who works for a Wall Street broker-dealer. I was trying to get us there in one piece. Amy Mangans parents followed them because they planned to help take care of Declan, Jacks younger brother, during the operation.

Amy Mangan spent the drive praying for Jack, his doctors, and the family of the donor, she said. She also repeatedly told Jack that he couldnt have tortilla chips, despite his protests, because he wasnt allowed to eat anything before the operation.

The family arrived in Boston around 6 p.m. Emani led two operations over a 12-hour period first to remove the deceased donors heart, which was preserved on ice in a cooler, and then to transplant the aortic valve and part of the artery into Jack. A total of 16 medical staffers participated in both procedures, with the second operation ending around 3 a.m., Emani said.

Emani knew the transplant had worked, he said, when he removed a clamp from Jacks aorta. The cane-shaped artery bulged with blood that flowed easily from the left ventricle, and no blood leaked back into the heart.

That was the aha moment, Emani said.

Jack slept for more than a day after the operation because he was sedated and had a breathing tube down his throat. After medical staffers removed the tube, he awakened.

He promptly told his parents he wanted exactly what he had been asking for in the car ride to Boston: tortilla chips.

Medical staffers and his mother laughed.

Well, Amy Mangan said, Jacks back.

Now, Jack returns to Childrens every two weeks for follow-up appointments. He was back last week and is doing fine.

We feel incredibly honored to have this level of care, his mother said. I just knew Jack was in the right hands.

Jonathan Saltzman can be reached at jonathan.saltzman@globe.com.

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First partial heart transplant in New England helps 4-year-old boy: Jack was in the right hands - The Boston Globe