Clinical Review

Adults with Congenital Heart Disease: The Critical Transition from Pediatric to Adult Care



The ACC/AHA appropriately recommend that the transition process for CHD patients begin at 12 years of age, with a goal of discussing future expectations of the child’s education, employment, and independent living.41 As part of this process, it is important that the practitioner educate the child and the family of the need for lifelong surveillance. The exact timing of the transition process is heavily influenced by a number of factors, including the degree of dependence of the child on their guardians, the severity of the congenital heart lesion, and the anticipated short- and long-term prognosis. However, regardless of these circumstances a reasonable age of transition into adult services should be established early on so that an expectation remains in place and the family is adequately prepared.

The challenge of learning how to navigate the adult health care system is as daunting for the transitioning patient as the medical consequences of their disease process. It is critical for patients to have easy access to social workers and case managers, ideally in the setting of a medical home, to connect them to community resources as needed. It is incredibly important that patients consider vocational options and training along with planning their insurance and/or disability qualifications as they move into adulthood. Establishing guardianship is also an important consideration for young adults with CHD who have remained dependent on their guardians.

Towards this end, the AHA/ACC has developed a curriculum that outlines the core principles that should be addressed before the patient moves to the ACHD clinic.27 The transition program should be flexible to accommodate for the patient’s degree of development, and the transfer should not occur before the adolescent has demonstrated the ability to independently manage their own health care to the greatest possible extent.

The ideal transition occurs through the auspices of a medical home that can coordinate the multiple subspecialists involved in the patient’s care. However, what often occurs is that a patient transitions from the pediatric cardiologist’s care before transitioning from pediatric to adult primary care. Prior to transition, the pediatric cardiologist should identify a cardiac destination at an ACHD center. This must be done in conjunction with the pediatrician, who will help identify an internist to take over the patient’s primary care and continue the coordination via the medical home. Information regarding the patient’s complete medical history, medication lists, exercise prescriptions, dietary restrictions, anesthetic issues, functional status, diagnostic studies, and comorbidities should be compiled in a health summary.40 To aid the process of transitioning, the ACC has developed several tools that may be used during the transition process, including self-knowledge assessments and medical summary templates.42

The Primary Care Provider’s Role and the Medical Home

Ensuring adequate care during the transition period requires close coordination between the patient’s various subspecialists. It is vital to avoid multiple subspecialists providing care without knowledge of each other’s treatments, as the treatment course for each ACHD patient is dependent on their unique history of prior therapies.27 The role of the primary care physician in establishing a “medical home” in this setting, as defined by the American Academy of Pediatrics Policy Statement, is exceedingly important.43 In this structure, the primary care physician maintains an easily accessible, centralized, and comprehensive record of the patient’s entire medical history, including surgical and medical treatments of both cardiac and noncardiac issues. Establishing the medical home framework is crucial, as it has been shown to lead to better outcomes in transitioning youth with special health care needs.44

With the establishment of this centralized care, the primary care physician must be able to negotiate the various medications prescribed by subspecialists and monitor for drug levels, adverse effects, and drug-drug interactions. ACHD patients also need regular monitoring and care aside from the care related to their chronic disease. Medical issues of particular importance to the ACHD patient include vaccinations, cholesterol and hypertension screening, cancer screening, and nutritional counseling. The primary care physician is responsible for addressing both the cardiac and noncardiac needs of the patient, ensuring that the patient truly receives comprehensive care. Thorough knowledge of a patient’s unique medical/surgical history will enable the primary care physician to adequately triage and appropriately refer for the development of a new symptom in an ACHD patient. On the other end of the spectrum, the patient’s subspecialists must maintain accurate and up to date information regarding their patient and transmit this to the patient’s medical home.

ACHD Centers

ACHD centers are an important part of any ACHD patient’s clinical team. Regardless of the complexity of the heart defect, there is tremendous value in the education and anticipatory guidance ACHD centers provide for their patients. The providers at these centers are often board-certified ACHD physicians who will work within a multidisciplinary team that includes mid-level practitioners, electrophysiology physicians, high-risk obstetrics/gynecology physicians, pulmonologists, and hepatologists. Each center differs in terms of their on-site interventional capacity and experience. However, the ACHD provider community is highly capable in directing patients who require interventions to centers of excellence, where there is proven quality in congenital surgical and interventional outcomes. ACHD centers often serve as the portals of reentry into care and are critical for providing and coordinating the complex care of each patient. Regular follow-up at these centers will ensure that patients receive adequate management of complications as they arise and preventive care against acquired heart disease.

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