Family members with 70-year age difference share connection of the heart
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A great-grandmother and granddaughter share a special connection. Nine-year-old Emma Thill and 79-year-old Susan Engstrom have the same heart valve.
“I tell her that she’s the princess that wears the crown in her heart,” Engstrom said. “Because it does look like the crown […] I’m the old queen and you’re the princess.”
The pair sit side by side on a park bench in Wyoming, Minn., near where the Thill family lives.
Emma describes their connection as “exciting and cool.”
Both have endured years of heart problems.
Emma was diagnosed with Tetralogy of Fallot when she was just 10 days old. It’s a rare heart condition that includes a combination of heart defects. Doctors found a narrow pulmonary valve and three holes in her heart.
“The biggest one would need to get patched and they were hoping they could wait until 6 months old when she was a little bigger,” Kristina Thrill, Emma’s mother, said.
When Emma was about 8 weeks old, however, they noticed she was turning purple when she cried. Doctors determined she was having tet spells. According to M Health Fairview, these spells are caused by a sudden drop in blood flow to the lungs.
Emma was rushed to the hospital and underwent open-heart surgery a week later at 9 weeks of age.
“They cracked her chest,” Kristina said. “She was in surgery for three and a half hours and they put a patch on her hole and were able to fix it basically. The other thing they were watching was her pulmonary valve. […] They had told us right away she would need a valve replacement. They were hoping that we would be able to wait until puberty, teenage years, get a little bigger.”
She was monitored closely through annual appointments. Last year, her April appointment was postponed due to the pandemic. Meanwhile, Emma’s family noticed a change in her behavior.
“We had noticed over the summer she would get more tired and winded,” Kristina said. “She just wasn’t herself, we knew something was going on.”
When they finally had an appointment in September, doctors decided it was time to replace the valve.
Thill describes feeling dread and panic when they received the news.
“I was expecting it but then I wasn’t,” she said. “We weren’t ready for it.”
Dr. Varun Aggarwal explained Emma’s valve wasn’t working properly.
“The blood was going forward but it was also coming back, which is not supposed to happen,” Dr. Aggarwal said, using a 3D model of Emma’s heart to explain the condition. “This is the right lower chamber, as you can see it’s much bigger as compared to the left lower chamber. They should be of the same size.”
According to Dr. Aggarwal, children can typically tolerate the condition for 10 to 20 years.
“Then, the right lower chamber becomes bigger and bigger and if you don’t treat this in time, it can create long term problems for them,” he said. “When you start getting symptoms, that tells us we need to do something.”
The surgery was scheduled for December 2020 but then pushed back to January due to the pandemic. It was delayed a second time when Emma’s pre-surgery COVID test came back positive, despite the family’s decision to isolate during the holidays. Her mother said Emma didn’t have symptoms.
Finally, in February she was able to get her new valve.
“When I woke up, I was nervous and then we went to the doctor,” Emma said. “Then the doctor told me what we were going to do and I was pretty excited.”
Dr. Aggarwal performed a transcatheter pulmonary valve replacement (TPVR) procedure.
“The valve comes trimmed on a balloon so it can go through a small catheter in the groin and, once we are in position, we inflate it,” Dr. Aggarwal said. “Because it’s made of metal, it attaches to the tissue itself so it does not fly out or embolize.”
The valve was approved by the U.S. Food and Drug Administration in August 2020, according to M Health Fairview. The procedure is a less-invasive option.
“The only other way to get it inserted is open-heart surgery and that entails a big operation,” Dr. Aggarwal said. “It has a long recovery time and since these children need future heart operations, every operation means you get more scarring, fibrotic tissue.”
Emma is one of the youngest patients to receive a transcatheter valve replacement.
“It makes it more complex because the tissues are smaller and the vessels are small,” said Dr. Aggarwal. “She did fantastic.”
Just four months later, her great-grandmother had a valve replaced the same way.
“It’s cool, isn’t it?” Engstrom said.
She was diagnosed with cardiomyopathy in 2007, which resulted in a pacemaker in 2012.
“My heart forgets to beat,” said Engstrom. “Then, this year, the doctor said the aortic valve has gotten weaker and it needs to be replaced.”
Doctors determined Engstrom had aortic stenosis, a condition that restricts blood flow through the aorta, which carries blood to the rest of the body.
In June, she had a transcatheter aortic valve replacement (TAVR).
“For me, the thing that I noticed was my legs were lighter, they weren’t as heavy,” Engstrom said. “So when Emma says her legs didn’t hurt, that’s what I noticed.”
The FDA approved the TAVR procedure for high-risk patients in 2012 before it was later expanded to all patients. M Health Fairview has conducted at least 1,000 of these procedures.
The TPVR, which Emma had, became an option more recently.
According to Dr. Aggarwal, Emma’s valve will last about five to 15 years. They plan to use the same procedure to replace it. Due to her age, Engstrom likely won’t need a replacement.
Both are now taking advantage of a more active summer.
“We go on the trampoline a lot and sometimes in the pool,” Emma said.
Engstrom added, “Getting ready for downhill skiing.”
Kristina said she enjoys watching the special connection the pair has.
"It’s been amazing," Thill said. "Just the connection they have now is really fun to see."
They will also be participating in the 10th annual Our Community Has Heart Walk, which is hosted by the nonprofit Lasting Imprint. The organization helps support families of those with a congenital heart defect. To visit Emma’s page, click here.