End of Life Care for Infants and Children with Single Ventricle Anatomy

Introduction: Care of children with single ventricle anatomy has advanced, leading to improved survival, however, mortality remains high. By understanding the end-of-life trajectory for these children, we aim to identify ways to optimize their care. Methods: This retrospective review was conducted for children with complex congenital heart disease following a single-ventricle surgical pathway who underwent at least second-stage palliative surgery [N=20 (< 18 years)], between 2010 and 2023 at a single institution. Descriptive statistics are presented as median (range) for continuous variables and as frequencies and percentages for categorical variables. Results: Median age at death was 3.1 years (4 months–16.7 years). Eighteen deaths (90%) occurred in the intensive care unit (ICU). During the final year of life, children spent a median of 122 days (8–254) in hospital. Death occurred following withdrawal of life-sustaining therapies in 13 (65%) children, non-escalation in 6 (30%) children, and failed resuscitation in 1 (5%) child. Palliative care was consulted for 13 children (65%), a median of 68 days (2–470) before death. In the last week of life, children experienced a median of five symptoms (3–12), most commonly pain (95%), anxiety/agitation (95%), respiratory distress (65%), and edema (65%). Conclusions: Children with single-ventricle anatomy experience a high symptom burden, with most dying in ICU following withdrawal of life-sustaining therapies. While palliative care was frequently utilized, its timing varied. Understanding these patterns can improve symptom management, optimize palliative care integration, and enhance support for children and families.