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.”
Happy Valentine’s Day. Appropriately enough, this heart-centric holiday falls right in the middle of National Heart Awareness Month, and we couldn’t think of a better way to celebrate than giving our readers a brief update about Parker Bolton, a Children’s Hospital Boston patient born with a congenital heart defect (CHD), called hypoplastic left heart syndrome (HLHS), and the face of Thrive’s Our Patients’ Stories section. (That’s Parker, smiling broadly over there on the left-hand side of this page.)
Parker first came to Children’s for open-heart surgery two and half years ago. At the time his health was questionable and his mother Casey knew very little about HLHS; it’s amazing how much can change in two and a half years.
Since his first surgery performed by Sitaram Emani, MD, of Children’s Cardiovascular Program, Parker has been growing consistently and his mom Casey has gone from knowing nothing about HLHS to becoming a heart advocate in her home state of New Hampshire. In fact, thanks to a letter writing campaign headed by Casey, New Hampshire governor John Lynch proclaimed February 14, Congenital Heat Defect Awareness day in his state, two years in a row.
Yesterday we shared a patient story, written by a mother whose baby was born with a congenital heart defect (CHD) that wasn’t recognized until hours after birth. Thankfully, once her condition was discovered, baby Lindsay was rushed to Children’s Hospital Boston in time for open-heart surgery. It’s a story with a happy ending, but Lindsay’s first week of life was very touch and go, which caused her parents almost unbearable amounts of stress and worry.
But had Lindsay’s heart defect been recognized prior to birth, much of that anxiety might have been avoided. When a heart condition is diagnosed prenatally, caregivers can proactively provide additional testing, delivery planning and counseling that benefits the baby’s health and parents’ piece of mind.
In some cases, it’s even possible to perform fetal therapy on the child before he or she is even born. Children’s Fetal Cardiology Program can try to repair heart valves the size of a pen point in a fetal heart no bigger than a grape; all while the child is still in the mother’s womb.
Fetal or in-utero therapy is a very complex process that brings together cardiologists, obstetricians, anesthesiologists and nurses in a team effort to perform these procedures. In the following video, Wayne Tworetzky, MD, director of Children Fetal Cardiovascular Program discusses how difficult these procedures can be, not just because of the small size of fetal hearts, but also because of complications that can arise from performing invasive surgery on a fetus when the mother carrying the baby is perfectly healthy.
In-utero heart surgery is an amazing feat of modern medicine, but before doctors ever get to that point a problem needs to be recognized. Ultrasounds are essential in monitoring fetus development in the womb, but they’re not perfect.
“Virtually all pregnant women who receive pre-natal care will have a fetal ultrasound during the course of their pregnancy, but that doesn’t mean the ultrasound catches everything,” says Tworetzky. “Congenital heart defects such as hypoplastic left heart syndrome or transposition of the great arteries, and others, can be detected by ultrasound, but research shows that about half the time, they’re overlooked.”
When heart defects go undetected it can produce a host of health problems when the child is born, many of which can have long-lasting effects. But when heart conditions are caught early, caregivers can provide parents with counseling on treatment options and a planned delivery with appropriate pediatric and obstetric staff present.
“With planned and specially managed care before and after delivery of a child with a CHD, caregivers can treat the infant’s heart right from the start,” Tworetzky says. “They can also minimize the risks for future problems.
During a fetal ultrasound, Tworetzky suggests parents ask the technician and the doctor the following questions concerning the fetal heart:
Do you see four chambers in the baby’s heart? (Desired answer is yes)
Are there two upper chambers (left and right atria) with valves controlling blood flow into the heart? (Desired answer is yes)
Are there two lower chambers (left and right ventricles) with valves controlling blood flow out to the body (aortic) and lungs (pulmonary)? (Desired answer is yes)
Do the two valves and vessels (aorta and pulmonary arteries) exit the heart in a crossing fashion? (Desired answer is yes)
Are there any large holes between the lower chambers of the heart? (Desired answer is no)
Is the baby’s heart normal? (Desired answer is yes)
If the answers to the above are as desired, then almost all serious heart defects, and > 90% of all heart defects, can be ruled out.
If the ultrasound shows the possibility of a heart defect, parents should immediately make an appointment with a pediatric cardiologist, who will have access to the most advanced and accurate diagnostic tests. “If further testing show signs of a heart defect your caregivers will help you plan appropriately for your baby’s birth,” Tworetzky says. “This way you’ve given clinicians as much time as possible to make the birth, and early treatment, as smooth as possible.”
One treatment option—and it sounds like something out of science fiction—is repairing CHDs on hearts no bigger than a grape while the baby is still in the mother’s womb.
As you might imagine, it’s an incredibly complex and delicate procedure that brings together doctors, surgeons and nurses. In the video below, Tworetzky discusses how difficult these procedures can be, not just because of the small size of fetal hearts, but also because of complications that can arise from performing invasive surgery on a fetus when the mother carrying the baby is perfectly healthy.
And in this recent interview with NPR, Tworetzky talks about fetal cardiology and we hear from the parents of a Children’s patient who had surgery while she was still in her mother’s womb.
Prior to the late 1930s, if your baby was born with a congenital heart defect (CHD), chances are doctors suggested you save for a funeral instead of college funds or wedding dowries. Morbid but practical advice; at the time, less than 20 percent of pediatric CHD patients lived to see adulthood. But thanks to advancing technology and the brilliant minds and hard work of many dedicated medical professionals, this type of bleak worldview is no longer applicable to most babies born with a CHD. Current data shows that over 90 percent of children born with one of these heart conditions now survive to adulthood.
This incredible success has led to an unexpected new medical specialty: caring for adult survivors of CHDs who experience later-life complications from their original conditions and treatments.