There are few things as frightening for a parent as finding out a child is sick. However, this is the situation for about one in 100 children born each year with a congenital heart defect. Many of these defects will require a lifetime relationship with a cardiologist, but, fortunately, medicine is developing a better understanding of how to help these children grow into healthy adults.

Understanding Congenital Heart Defects

Although “congenital” means present from birth, some congenital heart defects are not detected until significantly after birth. “Most (congenital heart defects) are not life threatening or clinically important,” says Dr. Michael Ralston, pediatric cardiologist with Dayton Children’s Hospital. He explains some of the types of congenital heart defects that children and parents might encounter.

The most common congenital heart defect to be diagnosed soon after birth is a ventricular septal defect. This is a hole in the heart between the two lower chambers, or ventricals, that can lead to already oxygenated blood circulating back to the lungs instead of throughout the body, putting strain on the lungs. This defect is typically not diagnosed until after birth when doctors recognize a characteristic noise when they listen to the heart.

Ralston says that the defect can be small and close on its own, or it can be larger and require surgery, either immediately or after waiting a time to see if it spontaneously closes.

However, the overall most common congenital heart defect is a bicuspid aortic valve, a condition in which the aortic valve has two leaflets instead of three. This can mean that the valve is less successful at preventing blood from flowing back into the heart after it is pumped into the aorta. Often, babies and children will have no symptoms and the condition is not recognized until young adulthood or after. At that point the person may need surgery or they may be eligible for less invasive treatment via cardiac catheterization.

These are just two of the most common congenital heart conditions; there are many more that are much more rare. However, Ralston points out that the common theme is how far medicine has come in the past three decades. Some conditions, like hypoplastic left heart syndrome, used to be fatal; today, the five-year survival rate for that condition is more than 90%. And, as technology and medicine continue to evolve, there is increasing reason for optimism.

Transition to Adulthood

When people think of congenital heart defect they think first of the team of pediatricians, neonatologists and pediatric cardiac surgeons that help care for the child in the early days and years. However, according to Dr. Mukul Chandra of Premier Cardiovascular institute, treatments during childhood are typically not the last time these children will need the attention of a cardiologist. “Patients are seldom cured of their condition,” Chandra says. He explains that a heart with a congenital defect can rarely be corrected in a way that makes the heart structurally, mechanically and electrically the same as a heart born healthy.

For that reason, Chandra views some of the treatments as palliation rather than cure. “(Congenital heart defect) comes back,” he says, explaining that additional challenges may arise as the person and their heart grown into adulthood. This maturation process may mean that the person’s heart is carrying an increased load due to size of the person or the organ and repairs done when the child was small may need revised to handle the demands an adult body puts on the heart.

To monitor this need children born with congenital heart defect need to transition to an adult congenital heart defect specialist. “It’s very different going from neonate to adult,” Chandra says. “One person cannot handle the entire spectrum.”

An adult congenital heart defect specialist can coordinate the monitoring of other systemic problems that may go along with congenital heart defect and those who are surgeons can perform the latest procedures to help the adult live a healthy life.

This follow-up is particularly important for adult congenital heart defect patients because those with congenital heart defect tend to have somewhat shorter lifespans than those born with normal hearts. According to the American Cardiology Association/American Heart Association, those diagnosed as a child with congenital heart defect should have their heart checked at least once by an adult congenital heart defect center and those born with a more complex congenital heart defect should do so every two years.

Experts recommend looking for an experienced and cohesive team to help care for the adult congenital heart defect patient. Chandra applauds the team approach at Premier Cardiovascular Institute where he finds the contributions made by nurses, technicians and all members of the care team that work in partnership with surgeons.

Likewise, Ralston is proud of Dayton Children’s Hospital’s state-of-the-art congenital heart defect management program. This program includes relationships with experienced adult congenital heart defect surgeons from Columbus and Cincinnati who see many of these patients and have the experience to deal with sometimes complex cases.

Other important members of the team for someone with congenital heart defect are the friends and family members who support them. Parents, grandparents, aunts, uncles and friends can all be critical parts of the support network that help the child grow into a healthy adult with the best chance of staying that way.

The Adult Congenital Heart Association (achaheart.org) offers many resources that may help, including fact sheets about congenital heart defect conditions and a directory of physicians who specialize in adult congenital heart defect.

The past three decades have brought many improvements in the way children and adults with congenital heart defect are treated and how these defects are understood. The next 30 years hold the promise of more progress to come.