Advance Care Planning Challenges in Adult Congenital Heart Disease

Due to advances in congenital heart disease management, there are now more adults than children living with congenital heart disease in the United States. This population of over 1.4 million people is expected to continue to grow rapidly as a result of improving survival. However, patients with adult congenital heart disease continue to face increased rates of morbidity, hospitalizations, and medical interventions compared to the general population, in addition to elevated mortality. Therefore, comprehensive advance care planning is integral to caring for this patient population. Yet despite recommendations for early advance care planning in adult congenital heart disease, evidence suggests that it is rarely performed prior to patients’ end-of-life. As demonstrated in this case, there are several challenges to advance care planning in this population which may account for this finding. Difficulty with accurate prognostication combined with the relatively young age of these patients has been reported to contribute to clinician hesitancy in initiating advance care planning prior to the onset of serious illness. Patients may have difficulty grasping their shortened life expectancy and may not feel ready to discuss serious illness care ahead of its onset. Furthermore, comorbid poor mental health and other psychosocial challenges are frequently prevalent in this population, further complicating matters. Therefore, patients with adult congenital heart disease may need more directed support with the advance care planning process, and early involvement with specialist Palliative Care can be invaluable. Further research specific to this population is needed to create a framework for successful palliative care delivery.