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Formal Referral and Access to Insurance Improves Continuity of Care for Teens with Congenital Heart Disease

Pamela G Burg, MD, FAAP, FACC, ABIM
Jeremy Nicolarsen, MD, FACC
Katrina Schneider, PA-C
Providence Adult and Teen Congenital Heart Program (PATCH) – Spokane 

Nearly 1% of infants in the United States, approximately 40,000 per year, are born with Congenital Heart Disease (CHD). Of these, 25% are considered to be critical and require surgical/cath based intervention in the 1st year of life. Congenital Heart Disease is the most common birth defect in live born infants.  With advances in CHD screening, congenital heart disease care, and surgical and catheter based interventions, the infant with even the most complex congenital heart defect has an excellent chance for survival to adulthood. Between 1979 and 1993, about 67% of infants with critical CHDs survived to one year. In contrast, between 1994 and 2005, about 83% of infants with critical CHDs survived to one year. (1) The predicted survival to age 16 beyond the 1st year of life is 96%. (2) In 2010 the estimated number of Adult Congenital Heart Disease (ACHD) patients exceeded the number of children. With an estimated 1.4% mortality rate per year, the ACHD population is growing and centers for specialized care of the ACHD patient have been developed.

Additionally, the American College of Cardiology and the American Heart Association published “2008 Guidelines for Management of the Adult with Congenital Heart Disease.” The goal is for primary prevention and avoidance of emergency admission secondary to sequelae from neglected CHD issues.

We have two nationally recognized accredited centers for Adult Congenital Heart Disease Care in the state of Washington: Providence Adult and Teen Congenital Heart Program in Spokane and the University of Washington program in Seattle.  Even so, the successful transfer of care from pediatric cardiology care givers to an ACHA accredited program is low. A recent study in Oregon reported 46% of CHD pediatric patients lost to follow up, 21% continued care with their pediatric cardiologist, and only 34% were successfully transferred to the only ACHA program in the state.

There are multiple barriers to the transfer of care. Often teens with CHD may not have an intense need for intervention, so making an appointment with a new medical team is the last thing on their mind. Late adolescence is a time of transition, including relocation for school or employment.  Teens are transitioning away from a parent to independent self-care. This is also a time of transition out of the pediatric health care practice to adult-based medicine. (3) Additionally, there is often the misconception by families or patient that they were “fixed,” though surgical and catheter-based interventions are constantly evolving, and what may have been considered the state-of-the-art therapy in the 1990s or early 2000s may actually be only a palliation resulting in residua. Lastly, especially in the case of complex CHD, there is often a hesitancy to transfer away from a pediatric cardiologist with whom they may have an established relationship. Factors for successful transfer of care included complexity of diagnosis, insurance, and formal referral from the pediatric provider to the ACHD program. (3)

Like other individuals with chronic illness, patients with CHD have benefitted from progress in the field and are surviving to adulthood. The ACHD patient requires lifelong specialized care. Secondary emergency admission and intervention can be prevented by primary intervention at centers with specialized ACHD multidisciplinary care programs, but attrition is high. Access to insurance and formal referral to an ACHA (Adult Congenital Heart Association) accredited program can make a difference.

1 Centers for Disease Control and Prevention;

2 “Survival with Congenital Heart Disease and Need for follow up in Adult Life” Wren, C; Heart 2001;85:438–443

3 “Challenges of intra-institutional transfer of care from paediatric to adult congenital cardiology: need for retention as well as transition. Bohun,C et al; Cardiology in the Young (2016), 26, 327-333