More than one million adults in this country are living with congenital heart disease or CHD. These patients survived the repair of various types of structural abnormalities in their hearts while infants or children. Many are not aware however, that seeing a doctor who specializes in the repercussions of those defects as they age, is critically important. The American Heart Association and the American College of Cardiology issued guidelines in 2008 for adults with CHD and recommended they be seen by physicians familiar with their condition.
Sansum Clinic’s
Dr. Taylor Holve is the only cardiologist in the surrounding area who specializes in adult congenital heart disease. His combined residency at the University of Southern California in pediatrics and internal medicine focused his training on childhood chronic illnesses and their progression. At Kaiser Foundation Hospital in San Francisco, he pursued a fellowship in adult CHD, a specialty just recently recognized and certified by medical schools. Only a few hundred doctors nationwide practice this small but growing niche.
“These patients are often left without any place to go because there has been no medical home for them, so to speak,” says Dr. Holve. “A lot are just not being seen, a lot of them just fall through the cracks unless they really feel poorly. There are patients who will feel fine, but have really severe things on their way to making them feel a lot worse.”
The loss in follow-up care often occurs in early adolescence, when these unique patients are too old to see a pediatrician, but have no symptoms requiring them to see a cardiologist. They mistakenly believe that the surgery they had as children was a fix for life. In some cases that’s true, but many have unnoticed complications that only develop in adulthood. Sometimes it takes years for problems like leakages or arrhythmias (abnormal heart rhythms) to arise. While arrhythmias may not be an issue for healthy people, they could be life-threatening for someone with CHD. Adults with CHD may also perceive that their overall quality of life cannot be improved.
“In reality, that’s a rarity,” explains Dr. Holve. “If you examine them carefully and do the appropriate evaluation, you can find a lot of things that can be done. If they have problems and they are caught, most of these patients can have fully productive lives.”
Because of technological advances and great success in treating congenital heart defects in utero and after birth, there are now more adults than children living with CHD. The transition of care for this growing population of survivors is a work in progress though, with too many dropping off the medical radar. Dr. Holve prefers to enter the process during the teen years.
“Ideally, we want to see the patient at the same time they are still seeing their pediatric cardiologist. This helps to get them comfortable going to a new place and to start to recognize complications that are more familiar to an adult congenital heart disease doctor,” recommends Dr. Holve. “Sometimes cardiologists that only see adults don’t have this specialized training, so people get undertreated or poorly treated. A regular cardiologist might see one or two of these patients in their career, but if you see a lot of them, you know a little more about what they need. Each individual patient, even with the same diagnosis, has completely different anatomy and considerations to deal with.”
The initial tests to evaluate adults with CHD are non-invasive, starting with echocardiography to look at the status of the repair that was done and to check the ventricular function. A cardiac MRI can access the anatomy of the heart more closely, and provides an accurate picture of the chamber sizes and blood flow.
Researchers are still investigating the causes of congenital heart disease. But genes passed down from a parent are one possible trigger. Some heart defects may be inherited, meaning that a parent with the defect has a 50% chance, with each pregnancy, to have a child with the same abnormality, according to Stanford Children’s Health. Screenings for those who are pregnant or wish to become pregnant is essential, as well as evaluating siblings. “We are now starting to amass databases that allow us to understand the repercussions of congenital heart disease. As we do this, we can implement to get better outcomes,“ adds Dr. Holve.
Treating these patients the same as those with acquired heart disease cannot be an option, stresses Dr. Holve. It’s his medical passion to come alongside those with adult CHD who may have already endured a stressful childhood experience, and offer reassurance. His hope is that they receive care from a physician with the necessary expertise to understand their complex physiology and the insight to decipher their special needs.
Visit www.achaheart.org for more information on adults with congenital heart disease.