Adult Congenital Heart Disease

As innovations have dramatically improved the prognosis of children with congenital heart disease, surviving adults now present unique problems as a result of both their anatomy and complications from their pediatric repairs.

It took vision in 1987 to recognize the need for a congenital heart specialist for the adult population. While the number of adult survivors of significant congenital heart problems was small, the development of successful pediatric cardiac surgery was beginning to vastly improve the prognosis for these patients. To Marlon Rosenbaum, MD, a specialist in both adult and pediatric heart disease, it seemed just a matter of time.

Flow is seen (in red) across the ASD.Following device insertion, the ASD has been closed, eliminating blood flow across the atrial septum.

"Adult congenital heart disease did not exist as a subspecialty during the 1980s, but children were now surviving into adolescence and adulthood. We were beginning to see problems that had never been seen before," recounts Dr. Rosenbaum, Director of the Schneeweiss Adult Congenital Heart Center at Columbia and one of the few specialists in the United States who devotes full time to this field.

The unique needs of this patient population now demand the expertise of specialists who can place congenital defects and their repairs in the context of an adult patient. "The whole spectrum of pediatric cardiology is superimposed on the problems encountered by the adult cardiologist," comments Dr. Rosenbaum. "Traditionally, pediatric cardiologists followed their adult patients because there were very few adult cardiologists familiar with their anatomy and management. Today, adult congenital heart specialists can bridge the gap between pediatric and adult cardiology, and are often best suited to manage difficult adult-related problems such as complex arrhythmias and chronic heart failure." These patients present with a broad range of problems, which may include the need to revise a childhood repair, the development of new lesions superimposed on an early repair, newly recognized congenital heart defects, complex arrhythmias, and pregnancy issues.

Dr. Rosenbaum, who coauthored Congenital Heart Disease in the Adult with Welton Gersony, MD in 2002, says that treatment of difficult cases may require a complex plan involving congenital heart surgeons, interventional cardiologists, electrophysiologists, and other specialists. "For example, our patients may have both hemodynamic problems and complex arrhythmias, and require both reoperation and sophisticated arrhythmia mapping with radiofrequency ablation." At the same time, "the emergence of catheter-based interventions has an important place in the treatment of congenital heart defects, and in some cases, may be used in lieu of an operation," says Dr. Rebecca Hahn, Associate Professor of Medicine at the Columbia University Medical Center and Director of Interventional Echocardiography. Dr. Hahn is a specialist in treating heart disease in pregnancy and adults with congenital heart disease.

According to Dr. Rosenbaum, "The reality is that we do not know exactly what problems these patients will face as they reach their 50s and 60s. Even for those of us who work in this area day to day, the field continues to evolve." Dr. Rosenbaum and Dr. Hahn concur that the lessons learned during follow-up of adults with congenital heart disease can provide feedback that is crucial for the pediatric cardiologist and surgeon. "For example," states Dr. Rosenbaum, "late problems with the early surgical repair forD-transposition of the great arteries and single ventricle led to a change in the type of surgery performed for these lesions. This is a very dynamic field likely to pose new challenges not only as the initial survivors age, but as we are confronted by new problems from the subsequent innovations in pediatric repairs."

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Schneeweiss Adult Congenital Heart Center