Evolution of a Heart
Since the 1980's, children born with structural heart defects have been extremely fortunate: for the first time, most of their defects could be successfully repaired, and they were able to enjoy normal lives. As a result of this success, the first generation of adults with congenital heart disease is thriving — a brand new population that simply did not exist in large numbers until today.
That they survived was clearly a tremendous success. But what would happen to their hearts as those children matured was largely not known, says Emile Bacha, MD, Director, Congenital and Pediatric Cardiac Surgery at NewYork-Presbyterian Hospital/Columbia University Medical Center. Without any precedent to guide them, surgeons believed that once a defect was repaired, the patient would be fine for the remainder of his or her life.
As it turns out, repairs for each of the 30 or so congenital heart defects have consequences of their own in the long term, explains Marlon Rosenbaum, MD, Director of the Schneeweiss Adult Congenital Heart Center at NewYork-Presbyterian/Columbia.
Today Dr. Bacha and Dr. Rosenbaum see many patients who underwent heart surgery during the 1970's or 1980's. According to Dr. Bacha, "During surgery for Tetralogy of Fallot, for instance, the surgeons would cut across the pulmonary valve and allow some regurgitation (leakage from the valve) to remain. That slow leakage can be well tolerated for a long time; children did well for many years after surgery. They did not know that creating that leak would slowly cause the right ventricle to increase in size, but by the time patients reached their mid-20's or 30's, ventricular enlargement would begin to present a serious problem."
Since consequences of early heart repairs often develop slowly and without symptoms, patients are often unaware that a problem may exist. Dr. Rosenbaum and Dr. Bacha emphasize that anyone who has had open heart surgery as a child should be evaluated by a specialist in adult congenital heart disease (ACHD), and at the very minimum have an annual echocardiogram. "If an adult had the Fontan operation for a single ventricle defect as a child, it is important to check for problems now," says Dr. Rosenbaum. Twenty to thirty years after undergoing the Fontan operation, patients may develop severe enlargement of the right atrium, rhythm problems, blood clots, and reduced cardiac output. Because the pressure in the atrium can become so high, some patients also develop liver problems, problems with the veins in their legs, enlarged spleen, and other issues.
As Dr. Bacha explains, "Thousands of people have had open heart surgery and think they are fine, but they are typically sedentary. They often say, "I feel fine, but I don't exercise because I get short of breath or cramps." So they feel okay, but they are living sedentary lives. When we see such patients, we often find they have a leaky heart valve, or a hole in the heart that was left behind. Once that problem is corrected, patients are thrilled — they can go up stairs without becoming winded, and they stop feeling short of breath. Yet until they come in for evaluation, they just think it is normal to feel that way."
Evaluation by a specialist in adult congenital heart disease can also be very important for women with ACHD who wish to become pregnant. According to Dr. Rosenbaum, a careful evaluation can help patients to know whether pregnancy will be safe for them, or it may guide them about how to prepare for pregnancy by addressing any significant issues. "This is a very different approach from that of general cardiologists, who would typically tell patients to simply not get pregnant."
Treating ACHD requires multiple modes of treatment. For some patients, that could mean treatment with a catheterbased procedure; for others it could mean medications, electrophysiology therapy, surgery, or heart transplantation. For others, a combination of several approaches may be required to treat more than one issue, such as a valve problem and an abnormal rhythm. The Schneeweiss Adult Congenital Heart Center is one of only a few centers in the nation that provides multidisciplinary expertise in every subspecialty for these complex problems.
"In order to preserve heart function and decrease arrhythmias, we are very proactive about evaluating in the absence of symptoms," says Dr. Rosenbaum. This approach differs from that of regular cardiologists, he explains, who usually evaluate and treat patients based on their symptoms. "For adults with congenital heart disease, we don't wait for symptoms to appear, because by then it may be too late."
"Our goal is to achieve significant longevity for adults with congenital heart disease," he continues. "Whereas pediatric cardiologists had the goal of allowing children to survive to adulthood, now we have our sights set on helping those adults achieve normal lifespans."
Learn more about treatments for adult congenital heart disease at congenitalheart.hs.columbia.edu or by calling 212.305.6936.