The moment Kirsten Dorsey realized she would deliver her second child and have open-heart surgery on the same day, her heart raced and her breath quickened.
But then, a sense of calm overtook her as she lay in her hospital bed in Connecticut. After weeks of uncertainty about her own health and her baby boy’s well-being, Dorsey knew she was exactly where she needed to be.
“Let’s do this!” she said, waving goodbye to her fiancé, Zac Connors, and putting on a brave face as medical staff wheeled her into the operating room.
Seventeen days earlier, Dorsey – then 29 and already the mom of a 1-year-old daughter – had nervously sat in a cardiologist’s office awaiting the results of an echocardiogram, an ultrasound that shows how blood flows through the heart, and an electrocardiogram, which measures the heart’s electrical activity.
She’d never had heart problems before, or any health problems, really. But in recent years, she learned about a family history of them.
The revelations began when her grandfather died of a heart attack that was traced to Marfan syndrome, an inherited disorder that impacts the body’s connective tissues. People with the disorder can have problems with their aorta.
Only then did her step-grandmother give her a detailed family history outlining the family battle with Marfan syndrome. Because the condition is genetic, Dorsey obviously was at risk – and her offspring. And, she happened to learn all this while pregnant with her first child.
She’d already had an anatomy scan and all went well, and Dorsey’s pregnancy was healthy. No one suggested additional testing.
During her second pregnancy, Dorsey told the maternal-fetal medicine doctor performing the anatomy scan about her family history. Concerned, the doctor referred her to a cardiologist for testing.
Now, in the cardiologist’s office, the doctor asked her a nerve-wracking question before delivering the results: “Is there anyone you can call?”
Dorsey phoned Connors at work. Together, they learned that she had a balloon-like bulge in her aorta called an aortic aneurysm. Hers was 4.6 centimeters wide – just shy of the threshold of 5 cm or more that typically leads to surgery.
Medication could help slow the growth. Could it be slowed enough for the remaining 11 weeks of her pregnancy?
Evaluating all her options, Dorsey saw a series of specialists. There were two problems to navigate: The health and safety of the baby, and her own health and safety.
They made a game plan to deliver the baby early via cesarean section and to keep close watch on the size of her aneurysm.
Adding to the complexity of Dorsey’s situation is that the doctor suspected her aortic aneurysm traced to undiagnosed Marfan syndrome.
Pregnant women with Marfan are at higher risk of complications – and the risk goes up as they get closer to giving birth. She was advised against giving birth naturally because hormones that help during labor can soften and dilate the aorta; also, pushing strains the aorta.
Dorsey was disappointed she wouldn’t get to deliver her baby naturally like she did with her first, Olivia. The upside was that she now knew the exact day her baby would be born: 26 days later.
Less than a week into that stretch, Dorsey underwent a follow-up echo. Everyone figured the test would confirm that the medicine was keeping the aneurysm from growing very much.
Instead, results showed that Dorsey’s aneurysm was a full centimeter larger – and that she had a tear in her aorta, known as an aortic dissection.
Luckily, it was considered “type B,” which is less dangerous than type A and can sometimes be treated with medicine. However, combined with the larger aneurysm, Dorsey was in danger. And considering her pregnancy, that meant the baby was at risk, too.
“You need to get to the emergency room immediately,” her cardiologist said.
“What’s immediately?” Dorsey asked in a shaky voice.
“Five minutes ago.”
Through tears, Dorsey called Connors at work, then her sister to watch Olivia. Before leaving, she embraced her daughter, holding on tight.
While it was urgent to get Dorsey and her baby under constant observation, the care team needed time to craft a plan.
That’s when they settled on back-to-back surgeries.
First came the emergency C-section. Baby Brian was born at 31 weeks, weighing 3 pounds, 12 ounces. As he was whisked to the neonatal intensive care unit, doctors tied Dorsey’s fallopian tubes so she couldn’t conceive any more children.
Then Dorsey’s surgical team repaired her aortic aneurysm. The cardiac surgeon, Dr. Sheelagh M. Pousatis, also replaced Dorsey’s aortic valve with a mechanical one because they discovered it was leaking.
Two days later, Dorsey met her baby boy. “I love you so much,” she told Brian, holding his tiny hand. “I’m so sorry for how chaotic your arrival was.”
When Dorsey was strong enough to recover at home in Pomfret Center, Connecticut, Brian stayed in the NICU in Hartford – an hour away.
For the next month, he gained weight and got stronger. Dorsey and Connors were only able to visit every few days. Nurses kept them in the loop with regular updates and photos.
The nurses helped the baby bond with his parents by having them wear fabric hearts; the cloths soaked up their scents, then the nurses placed them in Brian’s crib. On Valentine’s Day, they made a plaque with an imprint of 3-week-old Brian’s feet.
Finally, at a little over a month old, Brian came home.
Eight months later, Dorsey is back to her administrative job at a construction management company and is exercising again. She takes medication to keep her blood pressure and heart rate steady. She can’t lift anything over 50 pounds.
She also got the results of genetic testing. It confirmed she has Marfan syndrome.
Her doctors are monitoring her dissection, which they didn’t address, opting to use medication for now. If the tear gets worse, she may need a series of surgeries to repair it.
“I’m not completely in the clear, and life still looks a little different,” Dorsey said. “But I’m home with my fiancé, our two children and our dog.
My strength is returning more and more every day. I’m so grateful for the medical teams and research and science that helped provide me with the best possible care and outcome.”
Dorsey and Connors haven’t done genetic testing yet to see if Olivia and Brian carry the Marfan gene, but they get regular checkups. In the meantime, Dorsey is happy to have found the support of a group of other people living with Marfan syndrome.
“Marfan affects everybody differently,” she said, “and there’s a lot to learn on my part.”
Both Dorsey and Connors are adamant that people need to ask their parents and grandparents about health conditions and be informed. “Check your family history, and then follow through,” Connors said.
The Dorsey-Connors family also call Brian their miracle baby, and not merely for what he endured.
“If I hadn’t been pregnant with Brian,” Dorsey said, “I’m not sure when I would have found out about my heart.”
Written by Deborah Lynn Blumberg.
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