As a cardiac patient at Boston Children’s for the past 29 years, Lauren Hoey had learned to thrive in spite of her condition, but she never knew that she would be learning these coping skills in order to teach her daughter, Kaylee, the same skills and attitude.
When Lauren was just 18 hours old, she underwent her first heart surgery for truncus arteriosus, a rare congenital heart disease (CHD) that means only a single vessel arises from the heart. Normally, there are two separate vessels coming out of the heart. In 1983—the year Lauren was born—she stayed at Boston Children’s for more than one month. Most children who’ve had surgery for this condition recover and grow normally, although they are at risk for future arrhythmias, leaky valves and other heart complications. Also, like Lauren, these patients may require additional procedures before they reach adulthood.
Over the years, Lauren had three more cardiac surgeries and back surgery for scoliosis, and was fitted for two hearing aids. Twenty-five percent of babies born with congenital heart defects develop scoliosis. Yet, she didn’t allow these challenges to hold her back—succeeding as a competitive jazz and ballet dancer. She was an active member of the dance team during all four years at Westfield State College in Massachusetts.
“While I knew I couldn’t run up and down a soccer field, I found other sports in which I could participate and still enjoy,” Lauren says. “From the time I was a child, I had to be acutely aware of what I was capable of doing, but I didn’t allow the limitations to hold me back.”
Soon after getting married in 2008, Lauren began discussing the possibility of starting a family with Mary Mullen, MD, PhD, who recommended an additional heart surgery with Frank Pigula, MD, a cardiac surgeon at Boston Children’s, to revise her initial repairs. “I likely would have needed another surgery at some point, so the prospect of pregnancy brought the possibility to the forefront,” Lauren says.
All went well with the surgery, and she became pregnant in 2012. The cardiologists and obstetricians within the Adult Congenital Heart Program at Boston Children’s specialize in treating adult female patients who wish to get pregnant and throughout their pregnancies.
Statistically, as a mother with CHD, her chance of having a child with heart disease was slim—between 3 and 5 percent. Also, truncus arteriosus is particularly rare, affecting less than one out of every 10,000 babies. The specialists here recommend that all expectant mothers with CHD undergo a fetal cardiac echocardiogram between 18 and 20 weeks of gestation.
Unfortunately, Lauren’s 20-week ultrasound performed by Beryl Benacerraf, MD, who performed the ultrasound on her mother in 1983 diagnosing her with truncus arteriosus, revealed that her baby also had the condition—which is incredibly rare. For the third trimester of the pregnancy, Lauren began her twice-weekly hospital visits with Mullen, who works with patients with adult congenital heart disease at Boston Children’s.
“Because of Lauren’s personal experience with the disease, she understood the severity of the diagnosis and also the potential for successful surgery,” Mullen points out.
Kaylee Hoey was born on October 23, 2012, and Lauren’s heart tolerated the birth well. She and her husband Bob had already chosen to entrust their daughter’s surgery to Pigula since he had successfully taken care of Lauren. The three-day-old Kaylee underwent a six-hour open heart surgery, and the family prepared for a month-long recovery process. “In general, Kaylee experienced a much shorter and less complicated procedure than her mother did during her infant surgery,” explains Mullen. Also, due to the progress in surgery over the past 30 years, Kaylee was safe at home in time for her three-week birthday.
Much to Lauren’s excitement, Mullen agreed to follow Kaylee as well, treating both mother and daughter—29 years apart. “It was a privilege for Dr. Pigula and me to care for both Lauren and her daughter,” Mullen says.
“We anticipate this type of familial situation occurring with more frequency in the future, as the surgical outcomes with pediatric cardiology continue to improve, resulting in more patients living long enough to become mothers with congenital heart disease,” says Mullen. “Kaylee’s prognosis and quality of life should be quite good, especially due to her mother’s positive outlook. We will continue to track the progress of both mother and daughter in the coming years.”
“I knew that I wanted to raise my daughter in the same way that I was raised. We have to know our limitations, but not be held back by them,” Lauren says.
Because of the extreme rarity of both mother and daughter having the same condition, Lauren and Bob agreed to be enrolled in an ongoing genetic study at Boston Children’s, which is conducting research and continuing to uncover advance to help future generations of congenital heart disease patients.