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

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. 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. 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%). 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.