32ND BETHESDA CONFERENCE REPORT
Care of the adult with congenital heart disease: introduction
Gary D. Webb, MD, FACC, Conference Co-Chair and
Roberta G. Williams, MD, FACC, Conference Co-Chair
Consider the changes in the practice of cardiology in the lifetime of a 40-year-old patient with tetralogy of Fallot. In the 1960s it was common for adult and pediatric cardiologists to practice together in a medical school setting, with the exception of a few freestanding children’s hospitals. Patients were discussed at a joint conference. Without the demands of highly technical procedures, there was a greater commonality of knowledge and skills than we find today. Infant surgery was practically non-existent, and surgery on young children was usually confined to palliative procedures. Repair of lesions such as tetralogy of Fallot or atrial septal defect was generally delayed until early adolescence. Patients who survived until adult life were few.
The rapid technological developments that have occurred in the three past decades have enabled patients with more complex congenital cardiac defects to survive into adult life. Now, for the first time, the number of adults with congenital heart disease (CHD) equals the number of children with the disorder. The range of abnormalities and the complexities of postoperative anatomy are now well beyond the educational and experiential background of the adult cardiologist. Pediatric cardiologists, who often follow their patients into adult life, are at a disadvantage when confronted by superimposed acquired adult diseases. Both groups of subspecialists are drawn deeper into the complexities of their own fields and have vanishingly few opportunities to interact in an ongoing professional forum. A few adult congenital heart disease (ACHD) programs have served as referral centers for adult patients who require surgical or catheter intervention, but most patients do not have their cases reviewed in these centers. There is no organized effort at monitoring clinical outcomes for these patients, and the frequency with which these patients appear late at tertiary centers with avoidable complications indicates that health care delivery to this population falls far short of that for adults with acquired heart disease or children with CHD.
The transition from childhood to adulthood is particularly difficult for patients with chronic disease. Some are multiply-handicapped with cognitive and physical disabilities. The less disabled do not qualify for public insurance and are at risk for the development of secondary disability as a result of inadequate continuity of care. Adolescence is a time of particular anxiety about conformity, social success, and uncertainty about the future. Denial is a frequently used defense, coupled with a sense of immortality and a desire for risk taking. Relocation for educational or occupational reasons is common and often results in the transfer of inadequate information to the new provider. When one is distracted by the demands of job and family, it is easier to put aside routine health care, particularly when one is asymptomatic. Considering the human and financial resources that have been expended in nurturing these individuals to adult age, it is important to improve the system of care for adults with CHD in order for them to maintain the functional status that has been so hard won.
The American College of Cardiology is the professional organization where adult cardiovascular specialists, pediatric cardiologists, and cardiothoracic surgeons join together in the interests of care delivery, professional education, and advocacy. The authors are grateful for the opportunity to bring together these formidable resources in the format of the Bethesda Conference. The conference was organized into five sections that represent: 1) the present number of adults with CHD, 2) the special needs of these patients, 3) the workforce needed to deliver ACHD health care, 4) recommendations for a system of health care delivery that would produce the best clinical outcomes with the most efficient use of resources, and 5) the steps needed to ensure access to adequate health care for these patients. The answers reduce to three requirements: 1) education of a modest number of ACHD specialists who can lead highly specialized teams and serve as a referral and continuing educational resource for the greater number of providers with training in either adult or pediatric cardiology; 2) integration of highly specialized centers with community-based providers and with each other in order to provide a high level of care for all patients and to provide an informational base for continual improvement in care; and 3) unfettered access to an appropriately trained provider for all adults with CHD. These actions would require a modest outlay of resources, but the status quo will result in greater long-term expenditures for terminal care and loss of productivity for this ever-growing population. The product of this conference is a comprehensive analysis of the problem and a recommendation for corrective steps. The realization of the vision articulated in the following sections will require the passion and constancy of focus that have been exhibited by so many of the participants in this conference.
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