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As a result of extremely successful diagnostic and treatment strategies developed and employed over the past 40 years, the number of adults with congenital heart defects in the U.S. has reached ∼800,000. Of these, half are complex enough to require ongoing follow-up and treatment by health care professionals with expertise in the care of these patients. The health care system in the U.S. has developed neither a plan nor the required systems and facilities to care for these patients. Consequently, for the present and foreseeable future, most cardiologists who treat adults with congenital heart disease (CHD) have had informal training and considerable experience in the care of these patients. Over the next 10 years, more specifically trained adult congenital heart disease (ACHD) cardiologists should be trained in this subspecialty.
The American College of Cardiology (ACC) convened this 32nd Bethesda Conference to study the needs of these patients and to invite expert participants to recommend changes that will improve these patients’ care and access to the health care system.
The Conference report consists of an introduction and five Task Force reports. These documents largely focus on the coming decade. A series of recommendations are made, as summarized here.
Organization of care
• The care of adults with CHD should be coordinated by regional ACHD centers.
• One regional ACHD center should be created to serve a population of 5 million to 10 million people. Approximately 30 to 50 such centers should be developed or strengthened across the entire U.S.
• Adults with moderate and complex CHD (defined in the Task Force 1 report) will require regular evaluations at a regional ACHD center and will benefit from maintaining contact with a primary care physician.
• The ACC should recommend to the National Heart, Lung and Blood Institute (NHLBI) and/or the Agency for Health Care Research and Quality (AHRQ), the formation of adult congenital cardiac centers for documenting and improving outcomes, education, and research.
• Each pediatric cardiology program should identify the ACHD center where their patients will be transferred.
• Every adult cardiology and adult cardiac surgical center, as well as every cardiologist, should have a referral relationship with a regional ACHD center.
• All emergency care facilities should have an affiliation with a regional ACHD center.
• Physicians without specific training and expertise in ACHD should manage adults with moderate and complex CHD only in collaboration with physicians with advanced training and experience in the care of adults with CHD.
• An ACHD cardiologist should evaluate all adults with moderate and complex CHD at least once. The initial ACHD evaluation allows stratification of these patients according to risk and anticipated management difficulty.
• Patients with moderate or complex CHD usually require hospital admission or transfer to a regional ACHD center for urgent or acute care.
• Most cardiac catheterization and electrophysiology procedures for adults with moderate and complex CHD should be performed in regional ACHD centers with appropriate experience in CHD and in laboratories with appropriate personnel and equipment. After consultation with staff in regional ACHD centers, it may be appropriate for local centers to perform these procedures.
• Cardiovascular surgical procedures in adults with moderate and complex CHD should generally be performed in regional ACHD centers with specific experience in the surgical care of these patients.
• Each regional ACHD center should participate in a medical and surgical database aimed at defining and improving outcomes in adults with CHD.
• Appropriate clinical records for each patient should be kept in the regional ACHD center; the primary care provider and patient should also keep such records.
Workforce description and educational requirements
• A joint task force of the American Board of Internal Medicine and the American Board of Pediatrics, facilitated by the ACC, should be formed to determine the specific pathways and years of training required for Level 3 ACHD subspecialist cardiologists.
• Level 3 training programs should collaborate to maximize learning opportunities for the ACHD cardiologists-in-training and provide continuing education for trainees, graduates, and ACHD practitioners.
• A network of centers of research and education in ACHD should be created and funded through the NHLBI.
• Research fellowships in ACHD should be created so that these fellows can dedicate 75% to 100% of their time in protected research over a two- to three-year period.
• Training programs for other key staff (e.g., nurses, physician assistants, psychologists, social workers, other nonphysician personnel) on ACHD teams should be established.
• The ACC should lobby Congress for an educational loan repayment program for ACHD specialists to lessen the financial constraint of the prolonged educational process leading to an academic career.
Access to care
The ACC should:
• Work with the American Heart Association (AHA) to develop a strategic plan for an organized advocacy group, which includes health care professionals and patients and their families in the context of a public relations campaign.
• Collaborate with the AHA to develop educational materials to guide adolescent and adult patients in the transition to independence, including the need for health (and perhaps life) insurance, barriers that may exist in obtaining coverage, and strategies to obtain optimal coverage.
• Sponsor a multicenter study with economic forecasting to develop a better understanding of the true economic impact (e.g., payments, future income potential) of CHD in the adult.
• Include, in formal and regular discussions with insurance companies and other public and private payers and purchasers, information on the special problems encountered and expertise necessary in the care of adolescents and adults with CHD.
• Reduce barriers to multidisciplinary services by developing innovative reimbursement methods. Pilot programs established between one or more ACHD centers and major payers (public and private) should be encouraged.
• Work at the chapter level with state legislators to specify CHD in a demonstration project of the Work Incentives Improvement Act.
• Recommend that physicians discuss individual patient coverage concerns with insurance company medical directors.
• Advocate health care coverage for all. As an incremental step, all adults with CHD should be covered, thus removing a significant barrier to access.
• Develop additional educational materials to help adolescent and adult patients as they approach the job market, focusing on their legal rights (e.g., health should not be discussed during an interview), tips for success, and where to go for job training and vocational counseling.
• Recommend, at the patient’s request, that individual physicians work directly with patients, their schools, and their employers or potential employers to optimize opportunities.
Special needs of adult patients with CHD
Each patient’s transition to adult life should include:
• A structured plan to help patients transition from pediatric to adult CHD care.
• Individual patient education regarding their diagnosis and specific health behaviors
• A “health care passport.” Important historic information, including comprehensive diagnostic data, procedures, operations, and medications, should be kept by patients as a summary of past and present important health issues. The ACC should support the development of a health care passport, which would be useful for all patients with CHD and their health care providers.
• A continuum of vocational assessment, beginning in late childhood and proceeding through the adolescent and young adult years.
• Transfer of information to the patient and family, within a transition program, at a rate commensurate with the prevailing psychosocial development and circumstances of the patient.
Recommendations regarding noncardiac surgery
• Noncardiac operations on patients with moderate and complex CHD should be performed at a regional ACHD center, with the consultation of an anesthesiologist with experience in CHD. This applies particularly when more complex surgery is indicated, or when patients have adverse risk factors, including poor functional class, pulmonary hypertension, CHF, and cyanosis.
Recommendations regarding reproductive issues
• Contraceptive counseling must be available, when appropriate, to all patients with CHD.
• A multidisciplinary team at a regional ACHD center is needed for pregnancy care and delivery, as well as the management of indeterminate, intermediate-, or high-risk patients.
• The recurrence risk of CHD is highly variable and should be discussed prospectively with all patients. Genetic counseling should be made available through regional ACHD centers.
Recommendations regarding exercise and rehabilitation
• Guidance for athletic participation for patients with CHD can be found in the published recommendations of the 26th Bethesda Conference report, which represents the best consensus data available.
• The efficacy and safety of exercise rehabilitation programs for adults with CHD have not been studied, and research in this area should be supported.
Recommendations regarding psychosocial issues
• The emotional health of adults with CHD should be a priority in the overall care of this patient population. Appropriate screening and referral sources for treatment should be available at all regional ACHD centers.
• Tools for screening for psychosocial problems in this population should be developed and tested.
• Data should be developed to assess the effectiveness of regular follow-up care on the long-term physical and psychosocial health of adults with CHD.
• Professionals and facilities for the treatment of psychological disorders are scarce, and creative solutions for counseling patients in groups or those who live a far distance away should be developed.
The participants in this Bethesda Conference on adults with CHD have compiled these recommendations, along with supporting information, to construct a road map for future actions. Action will be needed from governments, health insurance organizations, health care institutions, clinical and academic units, and health care providers.
Ongoing efforts made by the ACC, as well as the present and future leaders, in the care of these patients will be required to help realize this vision over the next decade. We urge readers to acknowledge the serious problems in current health care delivery to these patients in the U.S. and commit themselves to doing what they can to become a part of the solution, which requires the collaboration of many individuals and organizations across this country.
- American College of Cardiology