The unique lifetime care needs of adults with congenital heart disease—particularly young adults who are making the transition out of pediatric cardiology care—are the focus of new practice guidelines released jointly by the American College of Cardiology and the American Heart Association.
The first of their kind in the United States, the adult congenital heart disease (CHD) guidelines are designed to help cardiologists make routine clinical decisions for the ever-increasing number of patients who, thanks to advances in the treatment of CHD, are now surviving into adulthood but are doing so with complex cardiac anatomy and physiology related to the repaired heart defects, according to Dr. Carole A. Warnes and her colleagues on the American College of Cardiology/American Heart Association guidelines writing committee. “In particular, there are a substantial number of young adults with single-ventricle physiology, systemic right ventricles, or complex intracardiac baffles who are now entering adult life,” the authors wrote in the executive summary of the guidelines (J. Am. Coll. Cardiol. 2008;52:1–121; Circulation 2008;118:2395–451).
The infrastructure of most pediatric cardiology centers supports the multiple unique needs of children with CHD through, for example, comprehensive case management by advanced practice nurses and social workers, but the adult health care system is not similarly structured, the authors noted. Additionally, “young adults have many psychological, social, and financial issues that present barriers to proactive health management,” they wrote. In an effort to minimize “the compound effects of a complex and unfamiliar disease with an unprepared patient and healthcare system,” the practice guidelines outline the most important diagnostic and management strategies and indicate when to refer patients to a highly specialized center.
Improving the delivery of care and ensuring access to care figure prominently in the recommendations. The guidelines include these recommendations:
▸ Care of adult CHD patients should be coordinated by regional centers of excellence.
▸ Adult CHD patients should carry a complete “medical passport” containing their medical histories and contact information for the regional centers of excellence.
▸ Designated health care guardians should be included in the medical decision-making process for patients whose care is complicated by additional special needs.
▸ Patients should have a primary care physician and a local cardiovascular specialist, each of whom has copies of current clinical records on file.
▸ Patients should establish a relationship with a regional adult CHD center to ensure the availability of care when needed.
The guidelines specify that, in the absence of specific training or experience in adult CHD, primary caregivers and cardiologists of patients with CHD should work in collaboration with trained specialists, and patients should have access to specialized follow-up care. For example, the guidelines state that patients with low-risk, simple CHD should have at least one follow-up at a regional adult CHD center, while more frequent follow-up (every 12–24 months) is advised for “adults with complex and moderate CHD.”
Additionally, certain clinical scenarios warrant consultation with, treatment at, or transfer to a regional adult CHD center. Such scenarios include hospital admission for urgent or acute care in most cases, the performance of diagnostic or interventional procedures, surgical procedures requiring general anesthesia or conscious sedation, urgent or acute care of cardiac problems, and urgent or acute care of noncardiac problems in high-risk patients.
The guidelines also address the psychosocial needs of adult CHD patients with the recommendation that the comprehensive care of these patients should incorporate individual and family psychosocial screening, counseling, and education regarding the possible social, emotional, and vocational impact of CHD.
Because CHD patients are at increased risk for infectious endocarditis, it is important that patients and their families be educated about the signs and symptoms of infectious complications, as well as how to prevent them, according to the authors. Specifically, the guidelines recommend antibiotic prophylaxis in high-risk CHD patients “before dental procedures that involved manipulation of the gingival tissue or the periapical region of teeth or perforation of the oral mucosa.” However, antibiotic prophylaxis against infectious endocarditis “is not recommended for nondental procedures [such as esophagogastroduodenoscopy or colonoscopy] in the absence of active infection,” the guidelines state.
Pregnancy and contraception require special consideration in women with CHD. With respect to contraception, oral estrogen-containing drugs are not recommended for patients at risk of thromboembolism, including those with pulmonary arterial hypertension or cyanosis related to an intracardiac shunt, according to the guidelines. Patients are advised to consult with an adult CHD expert to determine a labor and delivery management plan prior to becoming pregnant.
In addition to the general recommendations for the care of adult CHD patients, the guidelines also include comprehensive information on the clinical features, diagnosis, treatment, and preventive strategies related to specific lesions.