Early intervention, as needed, improves outcomes for babies born with heart conditions.
Originally posted: U.S. News & World Report 6/26/2018
A LOT HAPPENS BEFORE A baby ever comes crying into the world.
Along with its head, shoulders, knees and toes, intricate organ development takes place that allows a baby to take its first breath, and blood to circulate throughout the child’s body. “When the fetus is still in utero – the heart begins to form within the first couple weeks of gestation, and is pretty close to fully formed – at least all the basic components are in place – by about 49 days, although everything continues to undergo maturation and some sort of final regulation to get everything in the right position,” says Dr. Jeanne James, chief of cardiology and medical director at Herma Heart Institute at Children’s Hospital of Wisconsin.
Most times, when it comes to the heart – as with other organs and body parts – everything turns out well, which is no small wonder given how much needs to happen in nine months. However, in some cases kids are born with congenital (present at birth) heart conditions – including structural defects. James notes that the heart starts out as a single tube. “Think of it as tube sock with a hole in the bottom. … The tube has to fold correctly and form valves correctly to go from a tube like that to a heart with four chambers,” she says of the complex muscular organ. “Honestly, from my standpoint as a cardiologist, I think it’s almost amazing that anybody’s heart turns out normal because of the amount of complicated things that have to happen very early in development for a normal heart to be the result. And in fact heart defects occur in about close to 1 in 100 patients – it is the most common birth defect.”
Many of these babies are essentially born with a hole in their heart – which can be as dramatic and dire as it sounds if not detected and treated. Yet, experts say early intervention isn’t always required, either, as organ development continues after birth. There are a variety of congenital heart conditions kids may face, and while early diagnosis (sometimes while the child is still in utero) is key, it doesn’t necessarily precipitate medical intervention in the first weeks of a child’s life. “Not all of them are conditions that need treatment in the neonatal period or even during childhood. Some of these are conditions that children grow out of,” says Dr. Stephanie Fuller, a cardiothoracic surgeon at Children’s Hospital of Philadelphia. “For example an atrial septal defect – a small hole in the heart between the top chambers of the heart … that’s something that basically a child can kind of grow out of, or it becomes relatively insignificant to them as they reach adulthood.”
In many other cases, though, treatment is needed – and surgery or another procedure to close a hole in the child’s heart, for example, is required. One congenital heart condition some kids face is called tetrology of Fallot. This involves a combination of issues, including a hole between the bottom chambers of the heart – or a ventricular septal defect – and an obstruction of the flow of blood between the heart’s right ventricle and pulmonary artery, which carries blood to the lungs – or pulmonary stenosis. As a result, oxygen-poor blue blood goes out to the body. “Those children come to our attention because they turn blue,” says Dr. James Tweddell, director of cardiothoracic surgery and executive co-director of The Heart Institute at Cincinnati Children’s Hospital Medical Center. Babies take on a bluish tinge, with the degree of blueness based upon how obstructed that blood flow is. “Like most congenital heart disease, it’s a spectrum. So babies with severe obstruction will need surgery during the newborn period,” Tweddell says. “The way we repair that is we relieve the obstruction between the right ventricle and the pulmonary artery, and we patch the hole between the pumping chambers.”
Though complete repair of the defect is usually done early in a child’s life, in some infants a shunt, or a small tube, is sewn between the aorta – the body’s main artery, which distributes oxygenated blood – and the pulmonary artery to ensure adequate blood flow to the lungs, according to the American Heart Association. When a full repair is done later, the shunt is closed.
In other cases, isolated issues like a hole between the chambers of the heart – such as a secundum atrial septal defect, the most common type of atrial septal defect – can be repaired with a less invasive procedure rather than requiring open-heart surgery. In a catheterization, or cath, lab, a clinician passes a catheter, or a thin tube, into the patient’s body to the child’s heart. “Rather than having their chest opened, they’ll be just accessed through a blood vessel usually in the groin, and they’ll pipe [in] a little expandable device,” Tweddell explains. That will be opened up to close the hole between the atria, the upper chambers of the heart that receive, or collect, incoming blood. The device, in this case, is called an atrial septal defect occluder. “The most commonly used one is one called an Amplatzer device; and they’re very ingenious devices,” he says. “They look kind of like a finely woven basket [shaped] like two Frisbees connected by a tuna fish can in the middle; and you can scrunch them all down and sort of blow them up so one disc is on one side of the defect and the other disc ends up being on the other side – and that’s how they close holes.”
Understandably parents of children diagnosed with any kind of congenital heart disease are typically overwhelmed. For that matter, they’re panicked, Fuller says. “Realistically, I think it’s a very challenging position to be a parent or a grandparent, mainly because you’re inundated with a lot of new information. A lot of it is very technical information,” she says. Where doctors have spent years getting a grasp on the complexities of various heart defects and options to treat them, parents often get a crash course at a critical time. “You’re all the sudden forced to learn about this within hours or even days of when your child might need a lifesaving operation,” Fuller says.
For that reason, a big part of what pediatric cardiologists do is bridge the information gap by walking parents through what’s going on. That said, experts advise parents to do their homework as well – checking reputable online sources, from the AHA to respected medical centers of excellence – and have an open, ongoing dialogue with treatment providers about care needs and options over the short and long term. That goes for everything from risks and benefits to timing of treatment, as well as lifestyle considerations for the child – like if their activity needs to be restricted at any time or in any way because of a heart condition. “My advice [to] parents would be to ask a lot of questions,” James says.
Fortunately, where time and understanding are of the essence to make informed treatment decisions, diagnoses are sometimes made in utero – depending, for instance, on the nature and severity of a heart defect. In other cases, through careful monitoring of infants’ health, the problem is identified around the time of birth or otherwise well in advance of when an operation is imminently needed – or in cases where surgery or another procedure isn’t needed at all.
What’s more, the prognosis for kids with heart defects has improved significantly such that a major focus now, experts say, isn’t simply saving lives but quality of life for patients into adulthood.
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“What I like to tell parents is that we have come such a long way in pediatric cardiology as a field from back in the 1960s when we really had nothing to offer a child born with a heart defect to now, in the 21st century really, most children live with their heart defect,” James says. Kids may need to take medication or require other ongoing care and, even with surgery or other treatment, some have activity restrictions that limit things like participation in sports, among other ways a heart condition may affect their daily lives. Still, frequently heart defects don’t have a significant impact over the long term. “They can have surgery that can either be a curative surgery,” James says, “or palliative surgery, which doesn’t necessarily completely correct the flow of the blood but allows the heart to function well enough that the child can go to school and play soccer and be on the swim team and have a normal, healthy, fulfilling life.”
