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Journal of the American Academy of Physician Assistants:
doi: 10.1097/01.JAA.0000451416.29847.8c
Review Article

Congenital heart disease: Transitioning from pediatric to adult cardiology care

Kasinskas, Kaitlyn MHS, PA-C; Colomb-Lippa, Dawn MHS, PA-C

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Author Information

Kaitlyn Kasinskas is a recent graduate of the Quinnipiac University PA program in Hamden, Conn. Dawn Colomb-Lippa is a professor in the PA program at Quinnipiac University. The authors have disclosed no potential conflicts of interest, financial or otherwise.

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ABSTRACT: Medical and surgical advances are prolonging the lives of children with congenital heart disease (CHD), who are now living into adulthood. Physician assistants are well-positioned to play an integral role in supporting the transition of patients with CHD from pediatric to adult medical care, thereby reducing patients' risk of adverse outcomes.

Congenital defects associated with congenital heart disease (CHD) are the most common types of birth defects in the United States. The CDC reports that CHD is present in about 40,000 live births each year.1 About 25% of these children will need invasive surgery in the first year of life.2 In the past, CHD was primarily recognized as a disease of childhood, but new medical and surgical advances mean that children are now living into adulthood.3 The CDC estimates that nearly 1 million adults in the United States have CHD.1 Physician assistants (PAs) in primary care can expect to encounter adult patients with CHD much more frequently.

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Research has demonstrated that many patients with CHD experience lapses in cardiac care when they become adults. This lapse in appropriate cardiac follow-up can be directly related to adverse outcomes, including arrhythmias, heart failure, valvular disease, endocarditis, pulmonary hypertension, and stroke.4 PAs are well-positioned to play an integral role in supporting the transition of patients with CHD from pediatric to adult care, as well as identifying patients with CHD who have experienced lapses in care and addressing their needs. PAs in primary care will need to guide, educate, and refer adult patients with CHD so that they can receive the medical care and information that they will need to manage their chronic condition.

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The American Heart Association (AHA) has published a statement describing the best practices in managing the transition from pediatric to adult care in patients with CHD.5 Citing recent literature that demonstrates gaps in transitional care and education, the AHA statement reviews aspects of a good transition program and suggests timelines for its accomplishment. The statement also describes the main elements of the transition plan and defines stages of transition.5

These stages are pretransition, transition, and the actual transfer of care.

* Pretransition starts when the patient is young. The concept of a lifelong need for cardiology follow-up is introduced and reinforced with the child and parents. This information can be reiterated by any of the healthcare providers who care for the child.

* Transition occurs when the adolescent is deemed emotionally mature enough to begin participating in his or her own care. The education component continues throughout this stage. Also in this phase, the child is integrated more fully into the healthcare decision-making process.

* Transfer, the final stage, occurs when the young adult acquires a new healthcare provider. The AHA recommends that pediatric cardiologists have a definitive policy on transfer, including a timeline, to ensure that the transfer ultimately takes place. The AHA document encourages a flexible policy that allows for emotional maturity and intellectual variability in each patient.5

The AHA's recommendations about transition address the patient and family. The patient's emotional maturity should be considered when planning the timing of the transition, and the discussions should begin to focus more on the patient's involvement than solely on the parents' involvement in healthcare decisions. Quality-of-life discussions should be initiated between the healthcare provider and patient based on the patient's age and developmental maturity. Themes may include vocational and educational issues, genetics, contraception and pregnancy, benefits and risks of surgeries, and advance directives. The healthcare provider should talk to the parents about transition and encourage a partnership with the parents in this endeavor. The healthcare provider needs to acknowledge that parents may fear letting their child manage his or her own healthcare.5

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When planning for transition, the patient should have a medical home that is managed by the primary care provider. The primary care provider should have comprehensive medical records on the patient, prepared by the pediatric cardiologist, and including the transition plan.

The adult cardiologist should continue to update the medical records in the medical home whenever the patient is seen. The patient should understand that regular follow-up is essential and will continue indefinitely.5

The AHA statement also acknowledges that counseling is very important in caring for patients with CHD. Patients with CHD, like those with other chronic conditions, may need counseling intermittently throughout life.

* Genetic evaluation and counseling should be performed when appropriate, such as when the patient begins to consider marriage or childbearing. Prenatal counseling and diagnostic testing are also important in women with CHD, so they can make informed decisions about contraception and pregnancy.

* Formal exercise testing should be done when necessary to guide the provider in creating an exercise prescription. Providers also should review the benefits of exercise with patients. The exercise prescription should include recommended modes of exercise, types of exercise that should be avoided, and frequency of exercise. For safety purposes, patients should be taught to monitor their pulse and exercise within appropriate heart rate limits.5

* Health insurance issues should be reviewed with the patient, including the importance of maintaining coverage. Adequate health insurance is integral to allowing the adult patient to continue follow-up care. Patients also should talk to their providers about advance directives and end-of-life care. These subjects are important because some patients, as they enter adulthood, may perceive that they are cured and have no need of follow-up care. Patients must understand that CHD is a chronic condition and can be associated with medical complications.5

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In 2008, the American College of Cardiology in conjunction with the American Heart Association (ACC/AHA) released guidelines outlining management of adults with CHD. These guidelines include a description of transition of care from the pediatric to adult setting and strategies for the transition. The guidelines recommend that education on transition begin when patients are age 12 years and continue through the teen years. Topics that should be addressed with the patient include a description of the heart defect and subsequent surgical procedures, medications, risk modification, exercise, health promotion, and the need for cardiac follow-up. The ACC/AHA guidelines recommend the use of advanced practice nurses as well as PAs to optimize the transition from pediatric to adult care; advanced practice nurses and PAs can screen, educate, and counsel patients and families.6

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A recent study investigated the incidence of lapses of cardiac care in adult patients with CHD, and sought to identify patients at risk for lapses of care and the reasons for those lapses. Twelve adult CHD centers in the United States participated in the study and recruited 922 subjects with mild, moderate, and severe CHD. Lapses in cardiology care were reported in 42% of the subject population. A significant number of subjects experienced their first gap in care at age 19 years. Some of the common reasons for gaps in care included financial concerns and loss of insurance, feeling well and not experiencing symptoms, “losing track of time,” and decreased parental involvement in medical appointments. Other reasons cited by study participants were a perception that the heart problem was resolved, and the need “for a break” from addressing heart problems.7

Another study examined barriers to the transition of care as perceived by pediatric cardiologists. The most common barrier that was identified by the pediatric cardiologists was the emotional attachment of the patient or parents to the pediatric cardiologist. Another barrier was the pediatric cardiologists' perception of a lack of qualified cardiology providers knowledgeable about CHD in adults.8 Another obstacle to continued care is the lack of education for patients, parents, and healthcare providers about transitioning care.9

PAs in primary care have a critical role in identifying patients with CHD who have not had regular care and are at risk for complications of CHD. PAs can provide basic prevention strategies that are important for all patients, but particularly essential for patients with CHD, including the need for regular exercise, weight control, avoidance of smoking, and management of other cardiac risk factors. PAs must know how to advise patients with CHD about the importance of having health insurance and how to obtain it.10 PAs also can direct patients to other resources for information. The AHA, for example, has developed web resources for adults with CHD, including information on insurance, genetic counseling, and pregnancy.10

PAs also can make referrals to cardiologists who are well-versed in taking care of adults with CHD. The local branch of the AHA, the pediatric facility where the patient received treatment as a child, and the local medical center will have information on cardiologists who specialize in the treatment of adults with CHD.

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New medical and surgical interventions mean that children with CHD are living longer, thereby creating a population of adults with CHD who need ongoing cardiac care. Many barriers can prevent a smooth transition from pediatric to adult cardiac care. Recognizing the need for medical transition programs for pediatric patients with CHD, the AHA has published a statement outlining best practices for addressing this challenge. PAs in primary care must be aware of these guidelines and can play an important role in the success of the transition process, guiding patients through the transition so that they may be best served within the medical system. PAs in other subspecialties are well-positioned to recognize patients who are at risk for lapses in cardiac care, and can provide education and information on resources as well as referral for appropriate follow-up care.

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1. Centers for Disease Control and Prevention. Congenital heart defects. Data and statistics. Accessed April 5, 2013.

2. American Heart Association and American Stroke Association. Statistical fact sheet: congenital cardiovascular defects. Accessed September 20, 2013.

3. Marelli AJ, Mackie AS, Ionescu-Ittu R, et al. Congenital heart disease in the general population: changing prevalence in age distribution. Circulation. 2007;115(2):163–172.

4. Yeung E, Kay J, Roosevelt GE, et al. Lapse of care as a predictor for morbidity in adults with congenital heart disease. Int J Cardiol. 2008;125(1):62–65.

5. Sable C, Foster E, Uzark K, et al. Best practices in managing transition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues: a scientific statement from the American Heart Association. Circulation. 2011;123(13):1454–1485.

6. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. J Am Coll Cardiol. 2008;52(23):e143–e263.

7. Gurvitz M, Valente AM, Broberg C, et al. Prevalence and predictors of gaps in care among adult congenital heart disease patients: HEART-ACHD (the health, education, and access research trial). J Am Coll Cardiol. 2013;61(21):2180–2184.

8. Fernandes SM, Khairy P, Fishman L, et al. Referral patterns and perceived barriers to adult congenital heart disease care: results of a survey of US pediatric cardiologists. J Am Coll Cardiol. 2012;60(23):2411–2418.

9. Clarizia NA, Chahal N, Manlhiot C, et al. Transition to adult health care for adolescents and young adults with congenital heart disease: perspectives of the patient, parent and health care provider. Can J Cardiol. 2009;25(9):e317-e322.


cardiology; pediatric; continuity of care; congenital heart disease; transition; adult

© 2014 American Academy of Physician Assistants.


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