EXCLUSIVE: Teen’s heart stopped 400 times in a week. Doctors were stunned to find out the cause

When Amy Simpson was 11, she started experiencing dizzy spells and stomach pain. At the time, she was going through a lot of changes and thought stress caused her symptoms. Still, her sister encouraged her to see a doctor.

“My sister brought it to my attention, like, 'Hey, it’s not normal that this is happening. We should really get you checked out,'” Amy, 17, from Brownsville, Texas, tells TODAY.com. “It was nerve-wracking.”Doctors found tumors in Amy’s stomach and eventually diagnosed her with Carney-Stratakis syndrome. It's a rare, inherited condition that causes tumors in the stomach, neck, head and torso, according to the National Cancer Institute. For many patients, the tumors are harmless, though they are sometimes cancerous.

400 cardiac arrests in one week

While Amy’s tumors were not cancerous, she had them removed surgically and did fine until, at age 14, when she started having chest pain and fainting.

Doctors performed a scan and found something stunning: there was a large tumor crushing her coronary artery, which supplies blood to the heart muscle. Surgery to remove it carried a high risk because of Amy's age and the tumor's placement, and many doctors balked at doing it.

The teen wondered if she might die.

“I felt very defeated,” she recalls. “It was like, ‘Oh man, here we go again. I thought I was done with this.’”

At the time, Amy was living in Colorado, but she moved to Texas for care. At first, she took a medication that doctors hoped would shrink the tumors. But it only made her feel worse.

“It didn’t really work,” she says. “Taking that medicine put me in a whole loop of dizzy spells and passing out almost every day.”

Amy’s health declined. “I started having really bad chest pains,” she says. “My heart started doing really weird things.”

Doctors had her wear a heart monitor for a week. During that time, she experienced 400 episodes where her heart stopped anywhere from five to 25 seconds, Dr. Avichal Aggarwal, pediatric cardiologist with Children’s Memorial Hermann Hospital and one of Amy’s doctors, tells TODAY.com.

“Anything over three seconds is dangerous,” Aggarwal explains. "She was having chest pain. She would feel it, but she wasn’t exactly sure what was going on.”

Soon after the monitor results were analyzed, Amy was transported to Children’s Memorial Hermann Hospital in Houston, where doctors tried to come up with a plan.

Aggarwal and his colleague Dr. Jorge Salazar believed surgery was Amy's only option. But others worried about how dangerous it was.

“Dr. Salazar and Dr. Aggarwal kept pushing for the surgery, asking for it,” Amy says. “They had their work cut out for them.”

In May 2023, she underwent an open-heart procedure to have the tumor removed.

“When I woke up, it was so crazy how much I felt like a new person,” she says. “Right away, I could tell the difference.”

Removing a tumor emmeshed in the heart

Patients with Carney-Stratakis syndrome “can have tumors almost anywhere in the body, but mostly in the (gastrointestinal) tract, chest, head and neck,” says Aggarwal.

“She had this huge tumor in the chest that was compressing some key components of the heart, causing other symptoms, which made her such a huge high-risk patient,” he explains.

The biggest danger with Carney-Stratakis syndrom normally occurs when tumors compress any vital structure in the body. That’s exactly what Amy experienced.

“She was having these episodes of chest pain, dizziness and passing out,” Aggarwal says, adding that the tumor compressed the left main coronary artery “to almost 10-15% of its normal size.” That restriction caused the episodes where her heart stopped.

“The coronary arteries are like the gas lines to the heart,” Salazar, a pediatric cardiothoracic surgeon at Children’s Memorial Hermann Hospital, tells TODAY.com. “When the left main coronary artery is pinched or kinked or obstructed, it’s like having a heart attack. … She was having multiple episodes, really death spells, every day.”

While the pair knew they surgery was risky, they thought of their own teen daughters and how they'd do anything to save their lives. They believed Amy deserved that, too.

“She’d been turned down by a lot of other surgeons,” Salazar says. “We told the family that without surgery she was definitely going to die soon. … We were just very determined (to help).”

In the six-hour open heart surgery, Salazar hoped to free the tumor from her coronary artery while preserving the structure of the heart and arteries.

“We exposed the heart and saw the tumor. It was pretty jaw-dropping to see how vascular the tumor was,” he says. “The tumor had basically caused all these blood vessels to grow into it and feed it.”

Because the tumor was so intertwined with the blood vessels, Salazar knew he needed to take a different approach to removing the tumor.

“We cut out part of her aorta, part of her pulmonary artery,” he says. “We found the part of the tumor that was pinching the left main artery, and we shaved it off.”

The tumor had thinned out part of the artery in one spot, and when they removed it, it left a tiny hole. After removing everything, Salazar repaired the hole and reconstructed her aorta and pulmonary artery like he would in patients with congenital heart defects.

“At the end of the surgery, she not only had a normal heart, but also had a coronary artery that was normal,” he says. “She’s had just an amazingly fast recovery. We’ve done multiple scans and visits for her after surgery, and she literally has a normal heart. Now all those death spells went away.”

‘I felt really free’

After spending about two weeks in the hospital, Amy went home.

“I pretty much bounced back,” she says. “Mentally I was very, very drained. But physically I felt really great, and it felt amazing … to feel that sense of normalcy.”

Over last summer, she spent long days at the pool and time with friends being active.

“I remember racing with my friends because running all of a sudden felt so great,” she says. “I felt really free.”

Sadly, Amy has another tumor growing in her neck and compressing nerves. “I can feel it,” she says. “My vision is (blurrier) in my left eye because of it.”

The doctors she visited thinks if they remove it, she will lose feeling in her face. “I wouldn’t be able to talk,” she says. “I wouldn’t be able to sing and singing is my life. … I would probably have a feeding tube.”

A doctor predicted that she has five to 10 years before the tumor becomes a serious issue, possibly life-threatening. She's currently back on the medication to try to shrink the tumor.

While it feels overwhelming to have another mass, she is trying to enjoy life as much as she can. Amy graduated from high school early and is pursuing music at college in Utah next year.

Undergoing so many surgeries at such a young age has inspired Amy to embrace life and chase her dreams, like one day performing on Broadway. She hopes her story encourages people to advocate for their health, even when it seems dire.

“There are doctors that can do the impossible,” Amy says. “I should say my story and share my piece and help out how I can.”

This article was originally published on TODAY.com