Congenital Heart Disease
What is Congenital Heart Disease?
Congenital heart defects are problems with the heart's structure that are present at birth. These defects can involve the interior walls of the heart, valves inside the heart, or the arteries and veins that carry blood to the heart or out to the body. Congenital heart defects change the normal flow of blood through the heart.
There are many different types of congenital heart defects. They range from simple defects with no symptoms to complex defects with severe, life-threatening symptoms. Most of these defects are simple conditions that are easily fixed or need no treatment, but certain defects require major operations that make major changes to the heart's anatomy. Congenital heart defects may produce symptoms at birth, during childhood, and sometimes not until adulthood.
Treatment is based on the severity of the congenital heart disease. Some mild heart defects do not require any treatment. Others can be treated with medications, procedures, or surgery.
How are Cardiac Arrhythmias Related to Congenital and Pediatric Heart Disease?
Patients with congenital heart disease may develop arrhythmias that are directly related to the abnormal anatomy of their heart that they have had since birth. Other patients acquire them as a result of the natural aging process (which may result in cardiac defects, changes in the way the blood circulates, thickening of the heart valves) and some patients develop arrhythmias that are specifically related to previous surgery used to repair their abnormal anatomy, a result, for instance, of scarring or postoperative abnormalities in the way the blood circulates.
In other cases, pediatric patients with normal cardiac anatomy can suffer from arrhythmias.
What Are the Risks for Patients Born with Arrhythmias or with Congenital Heart Disease who Develop Arrhythmias?
Arrhythmia is the main cause of additional medical problems, including death, in patients born with congenital heart disease who grow into adulthood, leading to a high rate of hospitalization in this population. Arrhythmias that are not harmful in the general population can be more dangerous in these patients.
Some children have ventricular arrhythmias associated with a risk of sudden death, cardiac arrest, fainting, or heart failure. For these children, catheter ablation is a preferred treatment option and can be performed even in the first months of life or during the earlier childhood.
What Are the Treatment Options for Patients Born with Congenital Heart Disease who Develop Arrhythmias?
The improved outcomes and increased availability of surgery for congenital heart disease over the last three decades have created a small but steadily increasing subset of patients with unique needs: children and adults with complex arrhythmias in the setting of structural cardiac abnormalities. Some doctors refer to this patient group as "Grown Up Congenital Heart" patients or GUCH.
Drugs
As the number of these patients increase, more research is being done into the efficacy of drugs to treat heart rhythm problems in patients born with congenital heart disease, particularly in light of how difficult it is to plan lifelong drug therapy for young patients. Concerns about the possible long-term side effects of most of these drugs can be a limiting factor, and many of these drugs lose their effectiveness over time.
Catheter ablation
Catheter ablation is an approach that was limited in the past by technical challenges. Historically, poor understanding of the peculiar anatomy in patients born with structural heart disease as well as the very small hearts of pediatric patients made for extremely difficult catheter manipulation. Also, tools capable of intracardiac diagnosis and therapy in anatomically complex and/or small hearts remained scarce for some time.
Sophisticated tools are now available that can navigate the unusual anatomy of these patients, enabling a higher rate of efficacy than what is possible via manual catheter navigation techniques, with a great degree of safety and less exposure to x-ray radiation. These catheter ablation tools enable procedures whose duration and arrhythmia recurrence rates were comparable to those found in previous reports of similar procedures performed in adults with structurally normal hearts.
