Lize-Mari Du Toit, Jennifer Conway, Lindsay M. Ryerson, and Hayley Turnbull. End-of-Life Care for Infants and Children with Single Ventricle Anatomy. Pediatric Cardiology. 2026. doi:10.1007/s00246-026-04242-y.
Pubmed: https://pubmed.ncbi.nlm.nih.gov/41989586/
Take-Home Points
- This retrospective study examined 20 children with single ventricle (SV) congenital heart disease who had completed at least Stage II palliation (Glenn) and subsequently died between 2010 and 2023.
- Median age at death was 3.1 years, and children spent a median of 122 days hospitalized during their final year of life.
- Most deaths occurred in the ICU (90%), and 65% died following withdrawal of life-sustaining therapies.
- Symptom burden was substantial, with a median of five symptoms in the final week of life, most commonly pain (95%), anxiety/agitation (95%), respiratory distress (65%), and edema (65%).
- Pediatric palliative care (PPC) was consulted for 65% of patients, but referral timing was highly variable, occurring a median of 68 days before death.
- Many children continued to receive highly intensive therapies very near death, including inotropes, ECMO/VAD support, surgery, and cardiopulmonary resuscitation.
Commentary from Dr. Varun Aggarwal (Wilmington, DE, USA), editor-in-chief of Congenital Heart Disease Journal Watch:
Modern management of single-ventricle heart disease represents one of congenital cardiology’s greatest achievements. Through staged palliation, many children born with lesions that were once uniformly fatal now survive into adolescence and adulthood. Yet despite these advances, mortality remains substantial, and relatively little has been written about one inevitable aspect of the single-ventricle journey: how these children die and what their final months and days look like.
The study by Du Toit and colleagues addresses an area often overlooked within the congenital heart literature. While most single-ventricle studies focus on surgical outcomes, Fontan physiology, transplant-free survival, or quality of life, this investigation examines the end-of-life experience itself.
The findings are sobering.
The median age at death was only 3.1 years. Even after surviving initial palliative surgeries and progressing along the single-ventricle pathway, these children experienced an enormous burden of illness. During the final year of life, the median hospitalization exceeded four months. Many children underwent repeated admissions, numerous operations, invasive procedures, and prolonged ICU stays.
For clinicians caring for single-ventricle patients, these findings reinforce an uncomfortable reality: survival statistics alone do not fully capture disease burden.
Perhaps the most striking observation is where death occurred. Ninety percent of deaths took place in the ICU. This mirrors previous reports from pediatric heart failure and advanced congenital heart disease populations, suggesting that death in children with severe cardiac disease remains highly medicalized and technologically intensive.
The pathway toward death was equally noteworthy.
Nearly two-thirds of children died following withdrawal of life-sustaining therapies, while another 30% died following decisions not to escalate treatment further. Only one patient died despite attempted full resuscitation. These patterns suggest that, in most cases, death followed a process of recognizing limits of therapy and transitioning toward comfort-focused care rather than sudden catastrophic deterioration.
Yet the timing of those decisions varied considerably.
Many goals-of-care transitions occurred very close to death, with the median interval between first code-status modification and death only four days. Similarly, end-of-life discussions frequently occurred late in the disease trajectory. These findings raise important questions regarding whether opportunities exist for earlier advance care planning in this population.
The symptom burden documented in this study deserves particular attention.
Pain and agitation affected 95% of patients, while respiratory distress and edema were common. These findings highlight that children dying with advanced single-ventricle physiology often experience multiple concurrent physical symptoms requiring active management. Importantly, this burden extends beyond hemodynamics alone and reinforces the need for expertise in symptom-directed supportive care.
The study also provides insight into just how medically complex these children become.
Nearly all patients carried significant chronic non-cardiac comorbidities. Many required long-term nutritional support, respiratory support, or both. Developmental delay or intellectual disability was present in 70% of patients. During terminal admissions, major complications including infection, thrombosis, chylothorax, neurologic injury, and multiorgan dysfunction were common.
For families, the cumulative burden of these complications may be as impactful as the underlying cardiac anatomy itself.
One particularly important aspect of this paper is its evaluation of pediatric palliative care integration.
Palliative care was involved in approximately two-thirds of cases, which is encouraging compared with older studies. However, consultation often occurred relatively late and was frequently triggered by transplant evaluation, refractory symptoms, or recognition that no further surgical options remained.
This observation reflects a broader challenge within pediatric cardiology.
Despite growing evidence supporting early palliative care involvement, many clinicians and families continue to associate palliative care with end-of-life care alone. Increasingly, however, the field recognizes palliative care as a parallel support system focused on symptom management, communication, coping, decision-making, and quality of life throughout the disease course.
The single-ventricle population may be especially suited for earlier palliative care engagement.
Their clinical course is characterized by repeated surgeries, uncertain prognoses, recurrent hospitalizations, and the constant possibility of significant complications. Establishing palliative care relationships early may allow families to develop trust and coping strategies long before difficult decisions arise.
The findings regarding mechanical circulatory support are also important.
Nearly half of patients received ECMO or ventricular assist support during the final week of life, and many remained on support for prolonged periods. Several underwent major surgery while receiving mechanical support and eventually died despite these efforts. These cases highlight the increasingly complex ethical and clinical questions surrounding advanced therapies in children who carry limited long-term survival prospects.
For pediatric cardiologists and intensivists, the study serves as a reminder that technological capability should not replace careful discussion regarding goals, burdens, and likely outcomes.
Notably, the authors describe several patients whose goals of care transitioned much earlier than others. These children frequently shared common themes: failing Fontan physiology, ineligibility for transplantation, progressive pulmonary vein disease, or severe chronic comorbidities. Such characteristics may serve as practical triggers for earlier discussions regarding advance care planning and palliative support.
As survivorship improves, the congenital heart community increasingly focuses on quality of life, not simply quantity of life. Understanding how children with single-ventricle heart disease experience the final phase of illness is an important component of that effort. This study provides valuable data that can inform both clinical practice and family counseling.
Ultimately, the message is not that aggressive treatment is inappropriate. Rather, it is that palliative care and disease-directed care should coexist much earlier and more consistently throughout the single-ventricle journey.
Clinical Perspective
Children with single-ventricle heart disease who die after staged palliation experience prolonged hospitalization, substantial symptom burden, and intensive medical intervention near the end of life. Most deaths occur in the ICU following withdrawal or limitation of life-sustaining therapies. Earlier integration of pediatric palliative care, structured advance care planning, and proactive symptom management may improve quality of life for both patients and families throughout the disease trajectory.
