In 1940, children born with severe congenital heart disease (CHD) had less than a 10% chance of living to age 18. Over the years advanced new treatments, including surgeries, were developed. Survival rates improved: Children born in 1960 with CHD had less than 20% survival rate. In 1980, the survival rate had improved to 80%. After 2000, the survival rate goes up to 90 to 95%. These children surviving and thriving into adulthood meant that a new medical subspecialty was born: adult congenital heart disease (ACHD). This subspecialty, recognized by American Board of Internal Medicine in 2015, is still emerging. There are fewer than 300 board-certified ACHD specialists in the U.S.; but more are being trained to be board-eligible every year. There are only a few in Kentucky with this training, including Walter L. Sobczyk, M.D. and Melissa L. Perrotta, M.D., both with Norton Children’s Heart Institute, affiliated with the UofL School of Medicine.
There are approximately 1.5 million ACHD patients living in the U.S. Some 20,000 congenital heart disease patients graduate to adult care per year. With the current state of pediatric heart surgery and cardiac health care, survival rates can only get better and better, according to Dr. Sobczyk.
“Early diagnosis with fetal cardiology means better care and better early survival, leading to a growing population,” Dr. Sobczyk said. “The population of adults with ACHD has been larger than that of children with CHD since 2005.”
According to Dr. Sobczyk, the medical subspecialty of adult congenital heart disease began close to 20 years ago. As more and more congenital heart disease patients lived longer and began experiencing issues associated with adult cardiology, fewer and fewer pediatric cardiologists felt comfortable treating these ongoing, long-term adult heart conditions. — They didn’t have experience with patients living that long. The Adult Congenital Heart Association, an organization formed in 1998, made up of patients, families and clinicians dedicated to the needs of this patient community helped bring forth standards that created a new medical subspecialty.
Why adult congenital heart disease care?
While surgeries and treatments in childhood can “correct” a heart condition, they are never truly fixed or cured of the condition. There are complications that develop that can put ACHD patients at risk as they age. That’s why adults with ACHD need to be monitored regularly by specialists.
According to Dr. Sobczyk, people with ACHD can be at risk for:
- Heart rhythm issues (arrhythmia)
- Vascular lesions
- Residual shunts or holes in the heart
- Abnormal blood flow patterns
- Valvular disease
- Heart failure
Their congenital heart condition also can affect and be affected by conditions that happen as adults age:
- High blood pressure (hypertension)
- Coronary disease
- Smoking/chronic obstructive pulmonary disease (COPD)
- Sleep apnea
- Gastrointestinal disease
- Kidney disease
ACHD specialists have an understanding and expertise in managing these complications in hearts that may have had multiple surgeries in childhood and as an adult, along with the effects of natural aging, lifestyle and other factors.
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Growing up and growing old with adult congenital heart disease
At Norton Children’s Heart Institute, patients as young as 12 are encouraged to take ownership of their condition. As with many congenital or chronic childhood conditions, managing the transition from childhood to adulthood is important. It requires young adults to:
- Understand their condition.
- Take medications as instructed.
- Keep appointments with doctors — making sure to keep appointments to monitor and stay ahead of any complications.
- Be honest with their doctors — honesty can help ACHD specialists understand a patient’s current health and what could happen down the road.
- Expect health changes as they age.
Taking ownership of a condition also involves living a heart-healthy life — and factoring in your condition at big life milestones, such as starting a family.
“The ultimate goal is to not just live long, but to live well,” Dr. Sobczyk said.