The end-of-life experience for adults with congenital heart disease: Resource utilization, functional decline, and circumstances surrounding death

Abstract Background Advance care planning is essential for patient-centered care at the end of life, yet little is known about the end-of-life experience for adults with congenital heart disease. Methods /Results: In order to characterize the end-of-life experience for this population, a retrospective case series was performed including 86 patients followed at a single referral center, of whom 61 (71%) died at the study institution. Cyanotic heart disease was common (n = 33, (39%)). Median age at death was 41 years (range 20 - 89). The majority had living parents and 19 (35%) identified a parent as their medical proxy. Hospitalizations and office visits both tended to increase in frequency during the last year of life, but 44% of patients experienced no decline in NYHA functional class during that time. The most common causes of death were circulatory failure (36%) and arrhythmia (22%). Invasive procedures were common in the last year of life. 59 (69%) deaths occurred in an intensive care unit and 34 (40%) occurred after operation or transcatheter intervention. The provision of advance care planning was infrequent. Conclusions These data provide a framework for providers to use when counseling their patients about end-of-life issues. They also reinforce the need for early advance care planning for adults with congenital heart disease, especially for those with complex anatomy and those undergoing surgery or transcatheter intervention.