Editor's Corner | Because of the many successes with corrective surgery for complex congenital heart disease that have occurred over the past 50+ years, we now have a growing population of adults with surgically repaired congenital heart disease. Names like Blalock, Taussig, Glenn, Fontan, Rastelli, Senning, and Mustard become familiar to families and patients who usually are aware of their disorder, and the correction that was done to improve or redirect blood flow through the heart and great vessels.

While a full correction of the defect is sometimes possible, as in the arterial switch operation for transposition of the great vessels, in most cases the surgery results in significant anatomic alterations to the heart, leading to modified hemodynamics and loading of the ventricular chambers. These changes have both acute and long-term consequences. Exercise tolerance may be reduced in patents with a Fontan conduit, arterial desaturation may be present when a right-to-left shunt persists, and ventricular failure may follow a lifetime of systemic loading of the right ventricle in an atrial switch operation for transposition.

To be complete, we should also consider atrial and ventricular septal defects, congenital aortic and pulmonary stenosis, aortic coarctation, bicuspid aortic valve, anomalous coronary artery, patent ductus, channelopathies, accessory bundles, and many other less significant inherited cardiac defects. But the challenge we are facing at present is the adults with complex congenital heart disease who have had one or more surgical procedures to restore function and blood flow through the heart. These patients seek a cardiologist to follow them throughout their lives because of the complexity of their disorders, and they need a cardiologist with additional expertise who can provide proper care and advice.

Even in adulthood, these patients often come for their office visit with a parent who has accompanied them to the doctor’s office from early in life. Their health records are complex and must be preserved to document the surgery or surgeries that were performed years ago, as well as their hemodynamics, oxygen saturations, and complications over time. I have been in the habit of making a complete copy of the records and giving it to the patient to take with them wherever they go. Many of my patients moved for jobs or educational opportunities, and nothing is worse than encountering a patient with corrected congenital heart disease without their medical records. As a clinician, it is a wonderful experience to discuss with a surgeon a patient that he or she operated on in childhood and then 20 years later hear the details of the surgery recited as though it was done yesterday. However, that is an uncommon luxury.

Given the increasing number of these patients, it is inevitable that many of them in their teens—or even later—will ask about participating in sports and other activities certainly not endorsed by current guidelines. With experience and a growing body of data, we are learning that these conservative approaches prohibiting participation in anything other than very low-intensity sports are not appropriate. Some patients can participate in sports if they are properly corrected, others can be physically active within the bounds of their cardiovascular capabilities. Can a patient with a fully repaired tetralogy of Fallot participate in competitive sports? Some do. Nearly all have some degree of tricuspid regurgitation that does not interfere with activity; others have mitral regurgitation that requires some surveillance; while still others are prone to arrhythmias and need periodic monitoring. However, these situations can be managed enough to allow participation in sports.

Management of these patients can be challenging. Patients with a Fontan conduit, for example, are very sensitive to fluid-volume changes. I make a habit of discussing fluid management with the anesthesiologists and surgeon when a Fontan patient is going for a surgical procedure. We expect most patients to adjust to the fluids we provide during procedures, but the Fontan patient can develop significant venous congestion if too much fluid is administered. They also don’t tolerate hypovolemia as the venous pressure is all that drives blood through the lungs.

These patients come to us for care and for advice. Advice about sports, marriage and pregnancy, travelling to high altitudes, going to college, doing their jobs, and leisure activities like swimming or even scuba diving. Physical exam, echo, CT scan, MRI, pulse oximetry, CBC, and other lab studies all play a role in providing these patients care.

Most important, however, is to connect with a recognized congenital heart disease center to share the care of these patients and to have the in-depth expertise needed to provide top-quality care. This relationship should be established for all of your complex congenital patients. As in most other areas of cardiology, team care is the best way to go. It is a great pleasure to see these patients succeed in sports, become pregnant and have a child, graduate college and go on for advanced degrees, and to assimilate into their communities without being stigmatized with a heart problem. As adult cardiologists, we all can participate in the care of these patients, but we need the support of experts and must connect with a center of excellence for adult congenital heart disease to provide the best possible team-based, patient-centered care.

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Alfred A. Bove, MD, PhD, is professor emeritus of medicine at Temple University School of Medicine in Philadelphia, and former president of the ACC.

Keywords: CardioSource WorldNews, ACC Publications

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