Director of Adult Congenital Heart Disease Services, Staff Cardiologist
Robert and Suzanne Tomsich Department of Cardiovascular Medicine
Specialties: adult congenital heart disease, cardiac catheterization, echocardiography, pediatric and congenital heart surgery, valvuloplasty
Hello, I’m Dr. Richard Krasuski, Director of Adult Congenital Heart Disease Services at Cleveland Clinic’s Miller Family Heart and Vascular Institute. Today I’d like to talk about my specialty, congenital heart disease in adults.
Congenital Heart Disease
A congenital disease is one that a person is born with; so congenital heart disease refers to a heart problem that is present at birth. Sometimes the problem is first discovered in childhood; in other cases, the defect or disease isn’t found until adulthood. Like the problems themselves, treatments for congenital heart defects vary, and relief depends on the patient and their particular circumstances.
Adults with congenital heart disease fall into two major groups. The first group includes those who are newly diagnosed. They have never had symptoms before, or may have had a few minor symptoms that they’ve attributed to other problems. This group includes people with atrial-septal defects, a hole between the upper chambers of the heart, a relatively simple problem. It also includes people who have other, sometimes more serious, malformations.
The second group includes people who were diagnosed and treated during childhood. This is a large group because our ability to diagnose children with heart defects has improved tremendously in recent years.
When people from this group come to us as adults, they face some unique challenges. Oftentimes, they think that the treatment they had as children cured their heart problem. Unfortunately, this is often not true. What happened was that their symptoms were alleviated, but the defect may still be there. They often develop other problems over time, including heart rhythm disturbances, heart chamber enlargement or heart failure.
Adult congenital heart disease includes a wide variety of disorders and affects a wide spectrum of people. It can be very simple, such as an atrial-septal defect, or it can be complicated, for example hypoplastic left heart which is an under-development of the pumping chamber of the left side of the heart. Folks with hypoplastic left heart generally undergo a repair during childhood, but know that they will need to have a heart transplant someday.
Adults come to us at any age, either for newly diagnosed problems, or defects they’ve known about since they were kids.
When do adult congenital heart defects need to be treated or repaired?
A lot of times our patients ask when will their adult congenital heart defect needs to be treated or repaired. My answer always is that it depends on the defect and on the person. We ask our patients, what are they able to do? Do they have symptoms that occur with exertion – like shortness of breath, palpitations, rhythm disturbances, or chest pain? Are there symptoms that limit their activity? Obviously, the more symptoms there are, the more we are inclined to do some sort of intervention.
Sometimes we see people who have no symptoms but who have evidence that the problem is starting to take its toll on the heart. Maybe the chambers are enlarged, or the heart valves are not functioning properly.
Then there are patients with major complications, like sudden heart rhythm disturbances. A patient who may not have had symptoms before is starting to have episodes where they are nearly passing out. It could be that the heart compensated for the defect for a long time, but now it’s reached a point where the complication has pushed them over the edge, so to speak.
In some cases, medications can be used to treat the complications of congenital heart disease. If someone has developed high lung pressures, for instance, we may be able to prescribe medications to lower the pressures.
In general, I tell patients that the longer you can manage with your own anatomy the better off you may be. Yet surgical techniques for treating adult congenital heart disease have improved dramatically in the last few decades.
We now offer many percutaneous, or non-surgical, treatments that can be done in the catheterization lab. They include inserting devices to plug holes or defects, closing off abnormal blood vessels, and creating new openings between chambers. We’ve learned to prop open blood vessels by implanting stents and stretch valves using balloons.
We can combine regular surgery with catheter procedures, fixing one problem with a catheter technique, and another with surgery. This way, we can lower the risks of surgery.
We can address electrical issues, or heart rhythm disturbances, that can develop because of the abnormality itself or from earlier interventions. Sometimes treating structural problems improves the problem. But, some people require other types of therapy, such as ablation - zapping the precise spot known to trigger an abnormal rhythm. Or we can implant devices – like a defibrillator or pacemaker -- to maintain a normal heart beat.
Some of the problems of congenital heart disease can be overcome through exercise. It is true that some patients’ risk for developing a problem may go up slightly during an exercise program. However, the result of exercise is that their risk for the rest of the day is decreased. So, the net effect of exercise is positive and well worth it.
I advise that all congenital heart disease patients lead a healthy lifestyle -- maintain a healthy weight, consume nutritious foods, exercise and don’t smoke.
Are your children susceptible to congenital heart disease if you have it?
Another very important question is whether a patient’s children are susceptible to these problems. And, yes there is clearly a higher risk of heart defects among offspring of parents with congenital heart disease. The risk in the general population is a little less than 1%. However, if you have congenital heart disease, your children have a risk of roughly 2.7% of having a heart defect.
It’s important that mothers with congenital heart disease recognize that they may face some serious risks during pregnancy, as would their fetus. Women should discuss their health history carefully with their obstetrician, even before becoming pregnant.
My suggestion to adults with congenital heart disease is to go to a center that offers expert diagnosis, and the entire gamut of procedures -- surgery, catheter-based techniques and the capability of managing electrical issues -- plus rehabilitation. That’s what makes Cleveland Clinic unique. Here you can choose from among the best people in the world in all of those areas, who work together on behalf of each patient.
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