Young hearts at risk
Frincella Rivera holds her newborn daughter, London, who has a cardiac defect and will require surgery within the next 6 months.
Frincella Rivera holds her newborn daughter, London, who has a cardiac defect and will require surgery within the next 6 months.
Frincella Rivera holds her newborn daughter, London, who has a cardiac defect and will require surgery within the next 6 months.
A day after she entered this world, London Rivera took a helicopter ride. She has since been diagnosed with Down syndrome. And, within her first six to 12 months of life, she will have undergone surgery to correct a congenital heart defect that, left untreated, could threaten her development, if not her life.
Suffice it to say, the first days and weeks for the McClellandtown infant have been tumultuous for her and her family.
“The heart problem really shocked me,” says London’s mother, 27-year-old Frincellar Jackson. “But it’s making me be a better mother. I’m very cautious with her, and I’m spending so much time with her.”
London’s situation isn’t uncommon, and heart disorders aren’t reserved only for older adults. About 1 percent of all children in the United States–about 40,000 births per year–are born with a congenital heart defect, the most common type of birth defect in the U.S., according to the U.S. Centers for Disease Control and Prevention (CDC).
“That’s actually a pretty significant number,” says Dr. Leif Lovig, a pediatric cardiologist with Children’s Hospital of Pittsburgh. “At this point, there is a fairly large demand for pediatric cardiology.”
Given the prevalence of childhood cardiac conditions like London’s, parents of newborns, toddlers or teens need to be on the lookout for warning signs and seek prompt medical attention should any arise, Lovig advises.
A tough start
London Rivera was born Jan. 17 at Uniontown Hospital. The next day, tests revealed that her oxygen saturation, a measure of the amount of oxygen carried by her blood, was low. The pediatrician evaluating the newborn also detected a heart murmur, an abnormal sound made during a heartbeat.
London was immediately flown to Children’s Hospital, where specialists diagnosed her with a complete atrioventricular septal defect (AVSD), a common problem for children with Down syndrome, Lovig says.
A normal heart has four chambers: the left and right atria (upper chambers) and left and right ventricles (lower, pumping chambers). A wall of tissue, or septum, separates the right and left chambers, and blood flows from the atria to the ventricles through the tricuspid valve on the right side of the heart and the mitral valve on the left side.
A complete AVSD occurs when, during development, the septa do not grow all the way into the middle of the heart, creating a large opening where the atrial septum and ventricular septum would normally connect. Blood that normally flows from the atria to the ventricles instead flows through a common valve in the middle of the heart (during development, the common valve fails to separate into the tricuspid and mitral valves).
As a result, extra blood flows to the lungs, causing breathing problems. AVSDs also cause symptoms similar to those of heart failure in older adults, particularly fluid accumulation and swelling.
Eventually, London will require open-heart surgery to correct the AVSD, most likely within the next six months, says Lovig, who is overseeing London’s cardiac care until her surgery. Doctors typically delay surgery for an AVSD until a baby develops symptoms, to allow as much time as possible for a newborn to grow.
“Children often become symptomatic from this problem because they start having more difficulty breathing,” Lovig explains. “When they have difficulty breathing, it makes it more difficult for them to feed. When they’re breathing hard, they’re actually burning more calories, so it makes it more difficult for them to gain weight. They can also have swelling in other parts of their body, including their intestines, which makes it harder to absorb calories, as well. With all of these things working together, you can get into a situation pretty quickly where a baby can be pretty malnourished. All of those things together are what push us toward surgery.”
The surgeon will repair both septal defects in London’s heart and also rebuild her defective valves. It’s a complex operation, as is any cardiac surgery, but London’s prognosis is good, Lovig says.
“Generally, a repair of a complete AVSD is a single operation, and generally in an uncomplicated case, patients do well with it and ideally don’t need any further interventions in the future,” he says. “The long-term outcome from this is usually very good, provided that everything is optimized prior to the surgery, the surgery goes well and her course afterward is uncomplicated. We expect all of those things.”
Young hearts at risk
London’s condition is one of many congenital heart problems in the world of pediatric cardiology. On the spectrum of severity, her AVSD is moderately severe. With the most severe congenital heart defects, babies develop symptoms immediately after birth.
In other cases, these defects go unnoticed until adulthood. Such was the case with Pittsburgh Penguins defenseman Kris Letang, who several weeks ago suffered a stroke. Letang was found to have a hole in his heart known as a patent foramen ovale, which normally closes at birth. The hole can allow a blood clot to pass from one side of the heart to the other, enter the bloodstream, travel to the brain and cause a stroke. (One study estimated that, in 2002, 650,000 to 1.3 million adults were living with a congenital heart defect, according to the CDC.)
Many newborn children have remnants of their fetal circulation, which allows them to function and remain symptom-free for days after they’re born. However, as these remnants disappear in the first week, the baby’s circulation changes significantly, and newborns who rely on those fetal structures can become quite sick once they’re gone, Lovig explains.
So, Uniontown Hospital and many other medical centers are now performing testing on newborns before they leave the nursery, in an effort to identify severe congenital heart defects that may not be apparent on a physical exam. This same testing, known as pulse oximetry, identified London’s condition, Lovig says.
Otherwise, newborns should undergo scheduled physical examination with their pediatricians, especially during the first year of life. Those exams, Lovig says, should include listening to the baby’s heart, checking pulses and reviewing any potential cardiac symptoms (see chart, “Symptoms of Concern,” for examples).
Lovig, who once a month provides pediatric cardiology services at Laurel Pediatrics, in Uniontown, says parents also need to remain vigilant for signs of heart problems as their children get older, especially if they occur during physical activity.
“There are many symptoms that warrant a referral to a cardiologist,” he says. “The things that are most worrisome to me are symptoms with exertion or exercise. A child who has chest pain or palpitations or becomes dizzy or faints with exercise certainly warrants further evaluation. Some, but not all, of these patients with exertional symptoms may have cardiac defects or heart-rhythm problems that would predispose them to sudden cardiac death. That’s really what we’re trying to avoid. We’re trying to find those children who are at risk of sudden cardiac death to modify their exercise and activities and minimize that risk before an event actually happens.
“As parents, if your child is complaining about these exertional symptoms, it’s really important to take those seriously,” Lovig adds. “Kids have lots of bumps and bruises, and it’s easy to discount some of those things. But, as parents and pediatricians, it’s important to take those cardiac symptoms seriously and evaluate them.”
Ounce of prevention
In the overwhelming majority of cases, congenital heart defects are unavoidable, yet many mothers feel a sense of guilt, as if they could have done something different to prevent these disorders, Lovig says.
“When I found out that London had Down syndrome and the heart problem, I was blaming myself. I thought maybe I did something wrong during the pregnancy,” Jackson says. “I had stopped taking my prenatal vitamins after six months because they were making me sick. When they told us she had Down syndrome and the heart problems, the first thing I thought about was the prenatal vitamins, but they said that had nothing to do with it. It just happens.”
However, other cardiac problems in children are preventable. The growing prevalence of childhood obesity is leading to an increasing risk of cardiovascular problems–such as atherosclerosis, the accumulation of artery-clogging fatty plaques that can lead to heart attacks and strokes–as children grow up. Studies have found atherosclerotic changes in the blood vessels of children as young as age 10, Lovig notes.
Parents need to encourage their children to exercise and remain physically active, he says. And for kids who are overweight, parents need to modify their diet, minimizing or eliminating foods and beverages high in fat, sugar and calories and focusing instead on whole grains, lean meats, fruits and vegetables.
“Maybe in the last 10 to 15 years, we’ve really started to realize that it is very important to establish these good practices and try to stop the process of atherosclerosis from happening in these young kids,” Lovig says. “Lack of physical activity and poor diet not only contribute to childhood obesity and early atherosclerotic changes, but they also create bad habits early. The habits you have as an adult are formed as a child.”
London’s outlook
Jackson and her fiancé, Johnny Rivera, 49, who works as a home health aide, are taking preventive measures of a different sort with their daughter. For example, “We can’t pass her around too much for people to hold her,” Jackson says. “If anyone’s sick, we have to keep them away from her. We have to be very cautious.”
As she awaits her surgery, London must take medication to prevent fluid buildup in her lungs and other tissues. She sees Lovig each month, has periodic appointments with her pediatrician and with a Down syndrome clinic, and receives weekly visits from a home-care nurse. “Every day is doctors, appointments, doctors calling, the nurse coming to the house,” Jackson says. “There are going to be therapists coming to the house pretty soon. I’m trying to prepare myself for that.”
For now, Jackson is a stay-at-home mother as she takes care of London, but she plans to return to work at Lady Luck Casino.
Right now, she says, London exhibits few signs of her condition, other than some fussiness. She rests in her mother’s arms, attentive, her full head of hair belying her age of little more than a month.
“They say she’s developing really well so far,” Jackson says. “She has a good chance. With daily activities when she gets older, she’ll be doing things that normal kids do.”