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

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Ryerson, Hayley Turnbull This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8524691/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 16 Apr, 2026 Read the published version in Pediatric Cardiology → Version 1 posted 9 You are reading this latest preprint version Abstract 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. Pediatric Palliative Care Pediatric Cardiology End-of-life Care Single-ventricle anatomy Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Introduction/Background There have been significant advances in the medical and surgical care of children with congenital and acquired heart disease leading to improved survival. However, cardiac disease remains a leading cause of non-accidental death in the pediatric population. The burden of disease is significant with most deaths occurring in hospital accompanied by costly and highly invasive interventions near the end of life [ 1 ]. Although single ventricle congenital heart defects account for only 8–10% of all congenital heart disease (CHD), children with single ventricle (SV) anatomy face high morbidity and mortality during childhood and adolescence [ 2 – 4 ]. Surgical management of these conditions generally involves a series of three palliative surgeries with the goal of using the single ventricle to pump systemic blood flow and transitioning to passive pulmonary blood flow. The Fontan procedure is the final palliative surgery; many patients with a Fontan circulation are now living into adulthood [ 5 ]. For children who receive a prenatal diagnosis of a congenital heart defect with SV anatomy, approximately 70% will survive to complete their Fontan palliation which is typically done around two to four years of age [ 5 – 6 ]. Death can occur at any age and at any stage of the surgical palliation, with the highest mortality rate (10–20%) being in early infancy around the first palliative surgery [ 7 – 9 ]. In children who have completed their third palliative surgery, recent studies have found that the estimated 20-year survival ranges from 60–85% [ 10 – 13 ]. During their life, children with SV anatomy have significant morbidity and often face multiple complications, many of which have a known impact on life expectancy [ 10 , 12 , 14 ]. Possible complications following third stage palliative surgery include arrhythmias, ventricular systolic dysfunction, protein losing enteropathy (PLE), hepatic dysfunction, renal dysfunction, thromboembolism, and plastic bronchitis [ 5 , 15 – 17 ]. In patients with Fontan circulation who develop protein losing enteropathy at any time after their Fontan surgery, the 20-year survival drops to 19% [ 13 ]. Frequent complications and hospitalizations can negatively impact overall quality of life for these children [ 5 , 18 ]. Pediatric Palliative Care (PPC) is a philosophy of care that supports children living as well as possible, for as long as possible. PPC is being increasingly recognized as an important member of the care team for children with complex CHD. Palliative care integration has been shown to improve symptom burden and end-of-life experiences for children with complex CHD and their families [ 19 – 23 ]. Although PPC teams are frequently consulted for psychosocial support and goals of care discussions, their involvement for symptom management and advanced care planning is often delayed [ 23 ]. Early and consistent involvement of PPC is crucial to ensure that care aligns with the patient’s and family’s goals and preferences [ 20 – 21 ]. Palliative care for children with single ventricle anatomy has been increasingly recognized as essential, yet it continues to be underutilized [ 19 , 24 – 25 ]. Although the research around the quality of life of patients with SV anatomy is improving, there continues to be a substantial gap in the literature around what the end-of-life trajectory looks like for these children, and how to be support children with SV anatomy and their families through end-of-life and into bereavement. Mery et al. recently published a life-long journey map for patients with SV congenital heart disease and their families, in which the end-of -life, was notably absent despite death being universally part of the journey [ 26 ]. Several studies have shown that most children with advanced heart disease die in intensive care following the withdrawal of life-sustaining treatments, which reflects an aggressive approach to care near death [ 1 , 10 , 19 ]. The clinical trajectory and symptom burden towards the end of life for children with SV anatomy has not previously been described. This study aims to address these gaps by examining the clinical course, symptom experiences, and healthcare utilization of children with single ventricle anatomy towards the end of life. Methods Patient Population and Site: A retrospective chart review was conducted of 20 children who received care at the Stollery Children’s Hospital in Edmonton, Alberta, Canada. The study included children (< 18 years) with complex CHD following a single-ventricle surgical pathway, who underwent at least their second stage palliative surgery (Glenn/cavopulmonary connection) and died in hospital between 2010 and 2023. Children were identified by a data analyst through an electronic database using surgical and disposition codes. Charts of identified children were reviewed by members of the research team to ensure children met inclusion criteria. Children were excluded if they died out of hospital or if they received a heart transplant. Stollery Children’s Hospital is a 236-bed quaternary hospital facility that provides inpatient and outpatient care for infants, children and youth up to age 18 years. The site has a comprehensive cardiac program that includes advanced therapies such as ECMO, VAD, and transplant and a pediatric palliative care (PPC) program that functions as a consult service. The PPC program has no access to pediatric hospice beds. Since 2015, a consult to pediatric palliative care has been included in the standard heart transplant assessment for all children undergoing this assessment. Data Extraction and Analysis: Data from electronic and paper medical records was collected from the terminal admission (the admission where the child died), any additional hospital admissions in the last year of life, as well as from the second and third stage palliative surgery admissions. Detailed chart review included review of operative records, discharge summaries, consult notes, daily progress notes written by physicians, nursing assessments and notes from allied health care team members (registered respiratory therapists, occupational therapists, physical therapists, registered social workers, spiritual care providers and certified child life specialists). Physician’s orders and medications administration records from the last week of life were reviewed for information about medication use. All available investigations, autopsy reports, procedure notes were reviewed to capture a high level of detail of the child’s care and course in each hospital admission. Variables were chosen and recorded to document the burden of disease, treatments, health care utilization and the end-of-life trajectory. Extracted variables included patient demographics, cardiac diagnosis, reasons for admission, length of stay, procedures (arterial or venous line placement, intubation, chest tube placement, etc.), cardiac catheterizations and location of inpatient care. Operative procedures were recorded as definitive surgeries if they took place in an operating room and provided disease directed therapy. The number and type of surgeries were extracted from operative reports and were categorized as cardiac or non-cardiac. Surgeries directed at treatment of the congenital heart disease were categorized as cardiac. Non-cardiac surgeries included surgeries of other organ systems as well as surgeries related to complications of previous cardiac surgery (diaphragmatic plication, thoracic duct ligation, pacemaker insertion, thoracotomy and decortication and sternal wound debridement). To provide information on the medical complexity of these children we recorded chronic (defined as lasting > three months) non-cardiac comorbid conditions that were active (defined as requiring medical treatment, surgical treatment, and/or active follow up by a member of the child’s health care team) in the last year of their life. The chronic comorbid conditions, excluding the child’s congenital heart disease, were captured using a previously published system-based categories based on ICD-9 diagnoses [ 27 ] with one modification: mental conditions (ICD codes 290–319) were split into two groups: 1. Mental health conditions (ICD codes 290–314) and 2. Developmental delay/intellectual impairment (ICD codes 315–319 and 783.4). The two conditions that were present in the cohort in the circulatory system diseases category were pulmonary hypertension and cerebrovascular disease. Children were defined as having growth impairment if their weight for age, length for age or body-mass-index (BMI) z-score was less than − 2. Additionally, to demonstrate the use of medical technology, life sustaining therapies and resuscitation, we documented chronic (> three months) use of medical technology (enteral/parental nutrition and respiratory support of any kind) along with the use of cardiopulmonary resuscitation (CPR), and mechanical circulatory support (MCS). Mechanical circulatory support was defined as any of extracorporeal cardiopulmonary resuscitation (ECPR), extracorporeal membrane oxygenation (ECMO) as well as included short and long-term ventricular assist devices (VADs). To further delineate the burden of disease and intervention, acute comorbid conditions that were active (required medical and/or surgical treatment) were recorded for each admission. Acute comorbid conditions were defined as medical conditions that were newly diagnosed and treated during the admission, or, conditions previously diagnosed requiring new and/or escalating treatment during the admission. Definitions for the acute comorbid conditions were informed by the consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Health Disease 2008 [ 28 ]. The primary reason for admission was recorded from the discharge summary and these were categorized. Admission for cardiac investigations was defined as an admission for cardiac catheterization, cardiac MRI or cardiac transplant work-up. Admission for congenital heart disease-related comorbidities was defined as admissions for protein losing enteropathy, chylothorax, heart failure, ascites/edema secondary to congenital heart disease, thrombosis, plastic bronchitis and Fontan circulatory failure. Other categories were admission for cardiac surgery (surgery directed at treatment of the congenital heart disease) and infection. In order to document symptoms at the end of life, validated scores were used where available [FLACC Behavioral Pain Assessment Scale (Faces, Legs, Activity, Cry, Consolability scale), Wong-Baker FACES Pain Rating Scale, Numeric Pain Rating Scale (NPRS), Cornell Assessment of Pediatric Delirium (CAPD), Agitated Behavior Scale (ABS)]. Additional information about the prevalence of symptoms in the last week of life was gathered from written documentation from any member of the health care team and through medication administration records. End-of-life discussions, as defined by Lee et al. [ 29 ], and code status was documented as well as changes to the code status. Additional details on resuscitation, use of life sustaining treatments, PPC consultation and involvement, and psychosocial/spiritual supports were documented. The mode of death (categorized as withdrawal of life sustaining therapies, non-escalation of therapies, failed resuscitation, or death by neurological criteria as previously defined by Trowbridge et. al) and cause of death were recorded [ 30 ]. All data were entered into a secure REDCap database hosted on a University of Alberta server. Identifiable information was maintained on a separate, encrypted master list accessible only to the research team, with de-identified data stored on password-protected external devices. Ethics approval was obtained from the University of Alberta Ethics Board (Pro00092382) and the Ethic Board provided a waiver for informed consent. Descriptive statistics are presented as median (range) for continuous variables and as frequencies and percentages for categorical variables. Results Patient Characteristics A total of 20 children with SV anatomy were included in the study cohort. The median age at death was 3.1 years (range 4 months–16.7 years). Patient characteristics are summarized in Table 1. All 20 patients (100%) completed second-stage palliative surgery (Glenn procedure), and 11 (55%) completed third-stage palliative surgery (Fontan procedure). In their last year of life, children had a median of two admissions to hospital (1-6), spending a median of 122 days hospitalized (8-254) and experiencing multiple surgeries. The timeline of patient deaths within the stages of their surgical palliation is provided in Figure 1. Table 1 Patient characteristics Characteristic Number (%) or median (range) Age at death, median (range) 3.1 years (4 months -16.7 years) Sex, N (%) Male Female 6 (30) 14 (70) Location of residence, N (%) Alberta Out of province 14 (70) 6 (30) Distance between home and surgical center (km), N (%) 500 5 (25) 10 (50) 5 (25) Size of home community, N (%) Rural/small (pop. 30,000) 7 (35) 13 (65) In foster care, N (%) 3 (15) Cardiac diagnosis, N (%) Hypoplastic Left Heart Hypoplastic Right Heart Heterotaxy Syndrome 11 (55) 3 (15) 6 (30) Completed second stage palliative surgery, N (%) Completed third stage palliative surgery, N (%) 20 (100) 11 (55) Chronic non-cardiac conditions, median (range) 3 (0-5) Chronic technology dependence, N (%) Enteral and/or parenteral nutrition Respiratory support 13 (65) 9 (45) Home oxygen, N(%) 4 (20) Tracheostomy, N(%) 1 (5) Surgical feeding tube, N (15%) 3 (15) Growth impairment, N (%) 14 (70) Number of admissions in the last year of life, median (range) 2 (1-6) Days spend admitted to hospital in the last year of life, median (range) 122 (8-254) Number of surgeries in the last year of life 3 (1-11) The children were medically complex and many required chronic (> three months) medical treatment and the support of medical technology. Thirteen children (65%) required long-term enteral and/or parenteral nutrition, and nine (45%) required long-term respiratory support with oxygen and/or non-invasive ventilation. In the last year of their life, almost all children (19, 95%) had at least one active chronic non-cardiac condition and the median number of chronic non-cardiac conditions was three (0–5). As shown in Figure 2, developmental delay/intellectual impairment was most common (14, 70%), followed by digestive system conditions (7, 35%) and respiratory diseases (6, 30%). The category of circulatory system diseases excluded congenital heart disease diagnoses. None of the patients had a documented genetic syndrome. Second and third stage palliative surgery admission The second stage (Glenn procedure, N=20) and third stage (Fontan procedure, N=11) palliative surgery admissions are described in Table 2. The median total length of stay for second-stage surgical admissions was 30 days (range 4–250). There were ten children (50%) that were admitted for more than seven days pre-Glenn procedure including six children (30%) who were never discharged home between their first and second palliative surgeries. For the children that had a third stage surgical admission the median total length of stay was 60 days (range 14–178). In the post-operative period for both surgical stages, ICU days made up a significant proportion of the length of stay, with a large range for both days admitted to the ICU and days admitted to the ward. Table 2 Summary of second and third stage palliative surgery admissions Characteristics Second stage N=20 Third stage N=11 Total length of stay, median (range), d 30 (4-250) 60 (14-178) Post operative index surgery, median (range), d Total length of stay ICU initial ICU total Hospital ward 16 (4-114) 6.5 (1-99) 7 (1-99) 6.5 (0-82) 59 (7-177) 8 (2-70) 28 (2-165) 13 (0-83) Died, % (N) 35% (7) 55 % (6) Delayed sternal closure, % (N) 30% (6) 18% (2) Reoperation, % (N) > 1 reoperation 20% (4) 5% (1) 64% (7) 36% (4) Cardiac arrest, % (N) 15% (3) 9% (1) Mechanical circulatory support, % (N) 25% (5) 36% (4) Cardiac catheterization, % (N) 30% (6) 36% (4) Non-cardiac surgery, % (N) 45% (9) 45% (5) About a third of patients (7, 35%) that completed their Glenn procedure died during their Glenn admission. There were ten patients that were admitted for more than seven days before their Glenn surgery; half of these patients (5, 50%) died during their Glenn admission. Just over half (6, 55%) of patients who completed their Fontan procedure died during their Fontan admission. The rates of reoperation were high after both the Glenn and Fontan procedures (20% and 64% respectively) with about a third (4, 36%) of patients in the Fontan procedure admission requiring more than one reoperation. About half (45%) of children in each group had additional non-cardiac surgery in each of the second and third stage palliative surgery admissions. Five children (25%) required mechanical circulatory support during the Glenn admission, and four (36%) during Fontan admission. Approximately one third of children underwent one or more post-operative cardiac catheterizations (30% of patients during their index Glenn admission and 36% during their index Fontan admission) with most cardiac catheterizations being diagnostic and a smaller percentage being interventional. Additional admissions in the last year of life Of the 20 patients, 17 (85%) had admissions in the last year of life. These admissions are summarized in Table 3. Among the seventeen children, there were a total of 39 admissions with a median of one admission (0-6) per child. Overall, the median length of stay was 8 days (0-221), however it was longer for cardiac surgery admissions compared to admissions that did not involve cardiac surgery. However, within the admissions that did not include cardiac surgery (N=29), there were three admissions with a length of stay > 100 days including the longest admission (221 days) which were all for complications of congenital heart disease, specifically for protein losing enteropathy. Table 3 Additional admissions during the last year of life Characteristic Number (%) or median (range) Additional admissions in the last year of life, median (range) 1 (0-6) Length of stay, median (range), d ICU Hospital ward Admissions with cardiac surgery (N = 10) Admissions without cardiac surgery (N = 29) 8 (0-221) 2 (0-91) 3 (0-202) 41 (12-112) 4 (0-221) Reason for admission, N (%) Cardiac surgery Congenital heart disease-related comorbidities Cardiac investigations Infection Other 10 (26%) 12 (31%) 11 (28%) 4 (10%) 2 (5%) Acute comorbid conditions during hospital admissions Acute comorbidities were present in all admissions. Across all admissions (Glenn procedure, Fontan procedure and other admissions in the last year of life), approximately half of the children had an infection and vascular thrombosis. There were high rates of chylothorax (73%) and bleeding requiring reoperation (45%) in the Fontan admission cohort. A quarter of children (25%) had a central nervous system injury during their Glenn admission or other admission and 18% had a central nervous system injury during their Fontan admission. Additional details are found in Figure 3. Terminal admission and end-of-life experience The median length of stay for terminal admissions was 83 days (5–198) with the majority of inpatient days being spent in the ICU (see Table 4). Most children (18, 90%) died in the intensive care unit, while two (10%) died on the inpatient ward. Most children (13, 65%) died after withdrawal of life-sustaining therapies. Table 4 Terminal Admission Characteristic Number (%) or median (range) Length of terminal admission, median (range), d ICU Hospital ward 83 (5-198) 39 (6-165) 10 (0-91) Systolic heart function at terminal admission Normal Abnormal 11 (55) 9 (45) Transplant status Listed for transplant Not a candidate for transplant No transplant evaluation 5 (25%) 8 (40%) 7 (35%) CPR during terminal admission 9 (45%) MCS during terminal admission Time on MCS, median (Range), d 11 (55%) 10 (2-119) Palliative care consult 13 (65%) Time between initial palliative care consult and death, median (range), d 68 (2-470) Code status at the time of death No limitations or no documentation Limited resuscitation, no cardiac resuscitation Medical management without resuscitation Comfort-focused care 5 (25%) 3 (15%) 5 (25%) 7 (35%) Time between earliest end-of-life discussion and death (N=18), days 10 (1-264) Time between earliest change in code status and death (N=16), days 4 (0-257) Individuals involved in end-of-life conversations Family member Intensivist Cardiologist Social worker Palliative care Patient 20 (100%) 19 (95%) 15 (75%) 10 (50%) 6 (30%) 1 (5%) Location of death Cardiac ICU Hospital ward 18 (90%) 2 (10%) Mode of death Withdrawal of life sustaining therapies Non-escalation of therapies Failed resuscitation 13 (65%) 6 (30%) 1(5%) Family present at time of death Mother Father Additional family members Siblings 20 (100%) 19 (95%) 17 (85%) 10 (50%) 6 (30%) Children experienced multiple symptoms in the final days, with a median of five symptoms (3–12) per patient. The most common symptoms were pain (95%), anxiety or agitation (95%), respiratory distress (65%), and edema (65%) (Figure 4). Additional psychosocial and spiritual support was provided in some cases, including child life, social work, and spiritual services (Figure 5). The majority continued to receive intensive medical care in the final days of life. In the last week, 15 children (75%) were on inotropes, nine (45%) received mechanical circulatory support, and five (25%) underwent major surgery (Table 5). Cardiopulmonary resuscitation (CPR) was administered to six children (30%) during the final week, and in four cases (20%), this occurred within the last 48 hours of life. Two of the patients that received CPR in the last week of life were on MCS at the time of CPR initiation. Both received a prolonged period of CPR (45 and 70 minutes) leading to death. Of the nine children (45%) on MCS in the last week of life, almost half of them (4, 44%) were on MCS for more than 30 days prior to their death. All of the children that underwent major surgery in the last week of life (5, 25%) were on MCS at the time of surgery; three of them had been on MCS for more than 30 days, all were intubated, ventilated and sedated preoperatively and all of these children multi-organ dysfunction prior to surgery. Table 5 Interventions and treatments in the last week of life Characteristics Number (%) or median (range) Received CPR Duration of CPR, median (range) CPR while on MCS 6 (30%) 45 min (10-70 min) 2 (10%) Mechanical circulatory support Initiated during the last week of life ECPR 9 (45%) 5 (25%) 3 (15%) Renal replacement therapy (PD or CRRT) 6 (30%) Intubated and ventilated 13 (65%) Inotropes 15 (75%) Major surgery Cardiac Other 5 (25%) 2 (10%) 3 (15%) Nearly all children (18, 90%) had their goals of care, including resuscitation status, documented in the terminal hospital stay. Sixteen children (80%) had one or more changes in their goals of care status during the terminal admission with a median of two changes (1-5). At the time of death, most children’s (12, 60%) goals of care excluded resuscitation with five children (25%) having documented goals of active medical management without resuscitation (no cardiac resuscitation and no intubation) and seven children (35%) having documented goals of comfort focused care. Three children (15%) had documented limitations in cardiac resuscitation but included intubation and ventilation and five (25%) remained a full code (either by documented as such or by default because goals of care were undocumented). The median time between the earliest change in code status and death was four days with a wide range in timing (0-257 days), suggesting that goals-of-care transitions often occurred very close to the end of life. There were seven children (35%) that had a change to their goals of care status that occurred more than seven days before their death. Almost all changes in documented goals of care placed limits on medical interventions. In one case, there was a change from some limits to full resuscitation; this change occurred on the day the child died. End-of-life discussions were documented for the majority of patients, though the timing varied widely, with the median time between earliest end-of-life discussion and death being ten days (1-264). These discussions consistently involved family members (20, 100%) and the intensivist (19, 95%). Cardiologists participated in 15 cases (75%), while social work was present in 10 (50%) and palliative care in 6 (30%). Direct patient participation was rare, occurring in only one case (5%) (Table 4). Pediatric Palliative Care Involvement PPC was consulted for 13 children (65%) during the course of their illness (Figure 6). The most common reason for initial referral was for transplant assessment (53%), followed by refractory or complex symptom management (23%). Other reasons included tracheostomy decision-making, patient no longer being a surgical candidate, parental or patient request, and goals of care discussions (each 8%). The timing of consultation varied widely, with a median of 68 days (range 2–470) between initial palliative care involvement and death. For those who received palliative care support, the most common area of involvement was in complex decision-making (85%), followed by symptom management (77%) and support for family coping (69%). Discussion This study provides insight into the end-of-life experiences and health care utilization of children with SV anatomy at a large Canadian center. Additionally, it highlights some unique features of end-of-life symptoms and care for SV anatomy patients. These children spent much of their last year of life in hospital, with both surgical and non-surgical reasons for admission. The majority of children in our study died in the ICU, with many continuing to receive intensive therapies, such as inotropes, mechanical circulatory support, and major surgery, until end of life. Similar findings have been reported in other studies of children with advanced heart disease in the USA, Korea and Italy, where death occurring in ICU after withdrawal of life-sustaining therapies represents the vast majority of deaths [ 1 , 29 , 31 ]. Many of the children in this study received multiple intensive interventions in the last week of life. The mortality of patients with SV anatomy that receive MCS is high with survival to hospital discharge in this population being 30–50% [ 32 ]. Given this, judicious use of highly invasive interventions (major surgery) and resuscitation (CPR) for patients with SV anatomy on MCS is essential. Both the combination of interventions, and duration of combined intensive therapies, that children with SV anatomy received towards the end of life and raises questions about the benefits versus burdens of the combined interventions that some patients received. For most children, the intensive care received in the last week of life was provided with the goal of life prolongation. Prognostication is very challenging in these children, with many of them experiencing multiple periods of worsening and recovery, while providers and parents struggle to recognize the time point where they transition from a recoverable state to palliative care. This struggle may contribute to how end of life unfolds and the timing of the goals of care conversations happening late and close to death, as we and others have seen [ 29 , 33 – 34 ]. Recognizing there is uncertainty in their clinical trajectories, most of the children in our study had lengthy ICU stays with multiple acute conditions arising during their hospitalization. These findings, as well as other published risk factors [ 35 ] that diminish the likelihood of survival, could serve as triggers for earlier conversations around the child and family’s goals of care as well as the child’s resuscitation status. For children with SV anatomy, their diagnosis alone puts them at increased risk for death in childhood, and this risk increases with the additional complications and comorbid conditions that some develop over time. There is a notable gap in advanced care planning for patients with SV anatomy. There is an opportunity for improvements in advanced care planning in this unique population, which may then inform the course and conversations within hospital and critical care admissions. This study found there were some children with SV anatomy that transitioned to less interventional and more comfort focused goals of care earlier in their trajectory and that there was a very large range in the timing of this change in goals of care. In this cohort of children, about a third changed their goals of care status more than seven days prior to their death. Of these children, five were status-post Fontan procedure and the majority (80%) had failing Fontan physiology and were not transplant candidates. These two factors may appropriately lead to limitations in intervention and earlier conversations around goals of care. Two patients were status-post Glenn procedure, with one having progressive pulmonary stenosis and the second having complex comorbidities leading to ventilator dependency. In three cases, there was a discussion about having a planned out of hospital death however, none of these cases left hospital, all for different reasons. Additional factors that were prevalent in this cohort such as having long hospital and ICU stays and the need for readmission, as well as the length of time admitted prior to the Glenn procedure, could act as triggers for goals of care conversations. In the patients that had non-resuscitative goals of care, ICU-level care was often required in the end-of-life period to manage refractory symptoms or chronic complications. Symptom burden at the end of life was substantial in our cohort, with children experiencing a median of five symptoms in the final week, most commonly pain, respiratory distress, and anxiety. This is consistent with data from Molloy et al. (2021), who reported that pain, fatigue, and dyspnea were the most frequent and distressing symptoms experienced by children with advanced heart disease, particularly those children with single ventricle anatomy [ 36 ]. Despite this, certain symptoms such as fatigue and psychological distress, for which children in this population are at particularly high risk, are often underrecognized and undertreated [ 36 – 37 ]. There is a bias towards thinking that a good death occurs outside of an ICU setting however in this complex patient with SV anatomy, we found that the ICU setting was best suited to provide comfort focused care at the end of life given the types and number of medications required as well as the opportunity to provide one to one nursing care. More information is needed on how these complex patients can receive necessary care in alternate locations, including at home or in hospice, for those patients and families who may wish for their child’s end of life care and death outside of hospital. Beginning advanced care planning, and PPC involvement, from antenatal or neonatal period may help with better supporting these children and families, and also may normalize conversations around goals and values, which should occur early and be continually revisited over time. Moynihan et al (2022) found 70% of parents felt their child who died with advanced heart disease had a good death (34% agreed somewhat and 36% agreed strongly) and invasive therapies did not impact the families’ view on whether or not their child experienced a good death [ 34 ]. They found that a good death was associated with pain control, advanced care planning, greater preparedness and CPR avoidance. These goals can all be supported through both primary and subspecialty PPC. While palliative care was consulted in 65% of children in our study, referrals occurred relatively late, often triggered by transplant evaluation or when children were no longer surgical candidates.The pattern of limited PPC involvement and variable, often delayed, PPC consultation is consistent with existing literature, despite evidence that earlier integration is beneficial [ 22 ]. Although transplant assessment is a trigger for a PPC consult at our center, the absence of standardized PPC referral guidelines, or defined triggers outside of a transplant assessment, may contribute to missed opportunities of PPC involvement and/or late PPC involvement. Our study’s findings, there is a need to more clearly define what early, high-quality end of life care and palliative care involvement looks like for children with complex congenital heart disease, particularly those with single ventricle anatomy As partnerships between pediatric cardiac care, intensive care and palliative care continue to evolve, there is a growing need to reframe how care is conceptualized for this population, addressing persistent misperceptions among both families and providers regarding the role of early palliative involvement [ 25 ]. Emerging evidence supports the benefits of integrating PPC from the time of diagnosis in single-ventricle heart disease, as early consultation has been shown to foster therapeutic relationships, enhance family coping, and reduce parental anxiety [ 20 , 38 – 39 ]. Standardizing prenatal or early PPC consults, particularly for conditions such as hypoplastic left heart syndrome, has also been associated with improved family support, education, and care continuity without detracting from curative intent [ 39 ]. Collectively, these findings highlight the value of establishing palliative partnerships well before the terminal phase of illness. This study has several strengths, including the use of detailed, longitudinal chart review and a focus on a rare but high-risk population. Our hospital captures a large geographic area, making these findings broadly applicable to other Canadian centers with similar populations. However, limitations include the retrospective design, the lack of standardized symptom scoring tools for many symptoms, and potential gaps in data from care that patients received as outpatients or from other institutions. Despite these limitations, our findings provide valuable insight into the end-of-life experience of children with single ventricle anatomy and identify areas for improvement in care delivery. CONCLUSION In summary, this study adds to the growing literature supporting early, integrated palliative care for children with single ventricle anatomy, specifically in the Canadian context. By reframing ICU care as appropriate and necessary for comfort and end of life care, and by recognizing the role of early, more standardized PPC involvement, we can better align care with patient and family needs and goals. In the future, further work is needed to identify end of life quality indicators, create standardized triggers for PPC consultation, and improve education for care providers and families, ensuring that palliative care is viewed not as a last resort, but as an essential component of comprehensive, compassionate care. STATEMENTS AND DECLARATIONS Ethics Approval Ethics approval for this retrospective chart review was obtained from the University of Alberta Health Research Ethics Board (Pro00092382). The requirement for informed consent was waived. All data were de-identified prior to analysis and stored securely on password-protected servers in accordance with institutional and national ethical guidelines. The study adhered to the principles of the Declaration of Helsinki and all relevant regulations governing research involving human participants. Author Contributions All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by Lize-Mari Du Toit, Hayley Turnbull, Lindsay Ryerson, and Jennifer Conway. The first draft of the manuscript was written by Lize-Mari Du Toit and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript. Conflicts of Interest The authors have no relevant financial or non-financial interests to disclose. The authors have no conflicts of interest to declare that are relevant to the content of this article. All the authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials that are discussed in this manuscript. The authors have no financial or proprietary interests in any material discussed in this article. References Morell, E., Wolfe, J., Scheurer, M., Thiagarajan, R., Morin, C., Beke, D. M., Smoot, L., Cheng, H., Gauvreau, K., & Blume, E. D. (2012). Patterns of care at end of life in children with advanced heart disease. Archives of Pediatrics & Adolescent Medicine, 166 (8), 745–748. https://doi.org/10.1001/archpediatrics.2011.1829 O'Leary, P. W. (2002). Prevalence, clinical presentation, and natural history of children with single ventricle. 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Supplementary Files Figurenumbering.docx Cite Share Download PDF Status: Published Journal Publication published 16 Apr, 2026 Read the published version in Pediatric Cardiology → Version 1 posted Editorial decision: Revision requested 11 Feb, 2026 Reviews received at journal 22 Jan, 2026 Reviewers agreed at journal 09 Jan, 2026 Reviews received at journal 07 Jan, 2026 Reviewers agreed at journal 07 Jan, 2026 Reviewers invited by journal 07 Jan, 2026 Editor assigned by journal 06 Jan, 2026 Submission checks completed at journal 06 Jan, 2026 First submitted to journal 05 Jan, 2026 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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07:57:35","extension":"xml","order_by":19,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":135113,"visible":true,"origin":"","legend":"","description":"","filename":"b02cf4425d9c45ecacf59dd96974beac1structuring.xml","url":"https://assets-eu.researchsquare.com/files/rs-8524691/v1/da8d3590947e4b97728a2cda.xml"},{"id":100162861,"identity":"ed5aff0a-fa49-4024-9862-eb595fc0a431","added_by":"auto","created_at":"2026-01-13 15:09:46","extension":"html","order_by":20,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":148032,"visible":true,"origin":"","legend":"","description":"","filename":"earlyproof.html","url":"https://assets-eu.researchsquare.com/files/rs-8524691/v1/18c404b92a3c72738b377f0c.html"},{"id":100162857,"identity":"b54309af-1350-4a6a-9050-6a9bbeb2f414","added_by":"auto","created_at":"2026-01-13 15:09:45","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":74428,"visible":true,"origin":"","legend":"\u003cp\u003eTimeline of patient deaths by surgical stage\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-8524691/v1/47c1fd86bfa45285f9f7f73e.png"},{"id":100162802,"identity":"a9106556-d5bd-4524-abc0-1b1de18de889","added_by":"auto","created_at":"2026-01-13 15:09:38","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":61239,"visible":true,"origin":"","legend":"\u003cp\u003eSystem-based chronic comorbidities\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-8524691/v1/44628d900cf14c677e84e84b.png"},{"id":100162803,"identity":"bb325410-8e46-4628-be51-1354e25c7ce3","added_by":"auto","created_at":"2026-01-13 15:09:40","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":58808,"visible":true,"origin":"","legend":"\u003cp\u003eActive comorbid conditions during hospital admissions\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-8524691/v1/7af5dbdf4a54df5281f81500.png"},{"id":100162808,"identity":"74f500b3-7bde-4342-b5f8-5b75b21a6e35","added_by":"auto","created_at":"2026-01-13 15:09:40","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":45982,"visible":true,"origin":"","legend":"\u003cp\u003eSymptoms present in the last week of life\u003c/p\u003e","description":"","filename":"4.png","url":"https://assets-eu.researchsquare.com/files/rs-8524691/v1/219be55faa3a9dad9e22e3ec.png"},{"id":100162849,"identity":"2ef08992-78bc-4331-91d3-c92e95061730","added_by":"auto","created_at":"2026-01-13 15:09:43","extension":"png","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":31553,"visible":true,"origin":"","legend":"\u003cp\u003ePsychosocial and spiritual supports in the last week of life\u003c/p\u003e","description":"","filename":"5.png","url":"https://assets-eu.researchsquare.com/files/rs-8524691/v1/f743eadf2aefc7cf02e2bc87.png"},{"id":100162859,"identity":"ed900039-76e4-4f07-af06-e81cd8e6741f","added_by":"auto","created_at":"2026-01-13 15:09:46","extension":"png","order_by":6,"title":"Figure 6","display":"","copyAsset":false,"role":"figure","size":135900,"visible":true,"origin":"","legend":"\u003cp\u003e(a) Reason for initial palliative care consult and (b) Involvement of palliative care team\u003c/p\u003e","description":"","filename":"6.png","url":"https://assets-eu.researchsquare.com/files/rs-8524691/v1/ed7a716c4b704e477ad7194e.png"},{"id":107350845,"identity":"40c048ac-365e-4243-901c-53545272c939","added_by":"auto","created_at":"2026-04-20 16:05:44","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":899883,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8524691/v1/c112b955-d83d-4f5c-a970-f9a77e2c6658.pdf"},{"id":100368491,"identity":"be9da887-311f-4c95-aa3c-4e9a8bdcef9c","added_by":"auto","created_at":"2026-01-16 07:58:00","extension":"docx","order_by":1,"title":"","display":"","copyAsset":false,"role":"supplement","size":218974,"visible":true,"origin":"","legend":"","description":"","filename":"Figurenumbering.docx","url":"https://assets-eu.researchsquare.com/files/rs-8524691/v1/b1c894a4e45ba1b45c2d7d65.docx"}],"financialInterests":"No competing interests reported.","formattedTitle":"End of Life Care for Infants and Children with Single Ventricle Anatomy","fulltext":[{"header":"Introduction/Background","content":"\u003cp\u003eThere have been significant advances in the medical and surgical care of children with congenital and acquired heart disease leading to improved survival. However, cardiac disease remains a leading cause of non-accidental death in the pediatric population. The burden of disease is significant with most deaths occurring in hospital accompanied by costly and highly invasive interventions near the end of life [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Although single ventricle congenital heart defects account for only 8\u0026ndash;10% of all congenital heart disease (CHD), children with single ventricle (SV) anatomy face high morbidity and mortality during childhood and adolescence [\u003cspan additionalcitationids=\"CR3\" citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. Surgical management of these conditions generally involves a series of three palliative surgeries with the goal of using the single ventricle to pump systemic blood flow and transitioning to passive pulmonary blood flow. The Fontan procedure is the final palliative surgery; many patients with a Fontan circulation are now living into adulthood [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eFor children who receive a prenatal diagnosis of a congenital heart defect with SV anatomy, approximately 70% will survive to complete their Fontan palliation which is typically done around two to four years of age [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Death can occur at any age and at any stage of the surgical palliation, with the highest mortality rate (10\u0026ndash;20%) being in early infancy around the first palliative surgery [\u003cspan additionalcitationids=\"CR8\" citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. In children who have completed their third palliative surgery, recent studies have found that the estimated 20-year survival ranges from 60\u0026ndash;85% [\u003cspan additionalcitationids=\"CR11 CR12\" citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. During their life, children with SV anatomy have significant morbidity and often face multiple complications, many of which have a known impact on life expectancy [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e, \u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. Possible complications following third stage palliative surgery include arrhythmias, ventricular systolic dysfunction, protein losing enteropathy (PLE), hepatic dysfunction, renal dysfunction, thromboembolism, and plastic bronchitis [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan additionalcitationids=\"CR16\" citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]. In patients with Fontan circulation who develop protein losing enteropathy at any time after their Fontan surgery, the 20-year survival drops to 19% [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. Frequent complications and hospitalizations can negatively impact overall quality of life for these children [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e].\u003c/p\u003e \u003cp\u003ePediatric Palliative Care (PPC) is a philosophy of care that supports children living as well as possible, for as long as possible. PPC is being increasingly recognized as an important member of the care team for children with complex CHD. Palliative care integration has been shown to improve symptom burden and end-of-life experiences for children with complex CHD and their families [\u003cspan additionalcitationids=\"CR20 CR21 CR22\" citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e]. Although PPC teams are frequently consulted for psychosocial support and goals of care discussions, their involvement for symptom management and advanced care planning is often delayed [\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e]. Early and consistent involvement of PPC is crucial to ensure that care aligns with the patient\u0026rsquo;s and family\u0026rsquo;s goals and preferences [\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e]. Palliative care for children with single ventricle anatomy has been increasingly recognized as essential, yet it continues to be underutilized [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e, \u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAlthough the research around the quality of life of patients with SV anatomy is improving, there continues to be a substantial gap in the literature around what the end-of-life trajectory looks like for these children, and how to be support children with SV anatomy and their families through end-of-life and into bereavement. Mery et al. recently published a life-long journey map for patients with SV congenital heart disease and their families, in which the end-of -life, was notably absent despite death being universally part of the journey [\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e]. Several studies have shown that most children with advanced heart disease die in intensive care following the withdrawal of life-sustaining treatments, which reflects an aggressive approach to care near death [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]. The clinical trajectory and symptom burden towards the end of life for children with SV anatomy has not previously been described. This study aims to address these gaps by examining the clinical course, symptom experiences, and healthcare utilization of children with single ventricle anatomy towards the end of life.\u003c/p\u003e"},{"header":"Methods","content":"\u003cp\u003ePatient Population and Site:\u003c/p\u003e \u003cp\u003eA retrospective chart review was conducted of 20 children who received care at the Stollery Children\u0026rsquo;s Hospital in Edmonton, Alberta, Canada. The study included children (\u0026lt;\u0026thinsp;18 years) with complex CHD following a single-ventricle surgical pathway, who underwent at least their second stage palliative surgery (Glenn/cavopulmonary connection) and died in hospital between 2010 and 2023. Children were identified by a data analyst through an electronic database using surgical and disposition codes. Charts of identified children were reviewed by members of the research team to ensure children met inclusion criteria. Children were excluded if they died out of hospital or if they received a heart transplant.\u003c/p\u003e \u003cp\u003eStollery Children\u0026rsquo;s Hospital is a 236-bed quaternary hospital facility that provides inpatient and outpatient care for infants, children and youth up to age 18 years. The site has a comprehensive cardiac program that includes advanced therapies such as ECMO, VAD, and transplant and a pediatric palliative care (PPC) program that functions as a consult service. The PPC program has no access to pediatric hospice beds. Since 2015, a consult to pediatric palliative care has been included in the standard heart transplant assessment for all children undergoing this assessment.\u003c/p\u003e \u003cp\u003eData Extraction and Analysis:\u003c/p\u003e \u003cp\u003eData from electronic and paper medical records was collected from the terminal admission (the admission where the child died), any additional hospital admissions in the last year of life, as well as from the second and third stage palliative surgery admissions. Detailed chart review included review of operative records, discharge summaries, consult notes, daily progress notes written by physicians, nursing assessments and notes from allied health care team members (registered respiratory therapists, occupational therapists, physical therapists, registered social workers, spiritual care providers and certified child life specialists). Physician\u0026rsquo;s orders and medications administration records from the last week of life were reviewed for information about medication use. All available investigations, autopsy reports, procedure notes were reviewed to capture a high level of detail of the child\u0026rsquo;s care and course in each hospital admission.\u003c/p\u003e \u003cp\u003eVariables were chosen and recorded to document the burden of disease, treatments, health care utilization and the end-of-life trajectory. Extracted variables included patient demographics, cardiac diagnosis, reasons for admission, length of stay, procedures (arterial or venous line placement, intubation, chest tube placement, etc.), cardiac catheterizations and location of inpatient care. Operative procedures were recorded as definitive surgeries if they took place in an operating room and provided disease directed therapy. The number and type of surgeries were extracted from operative reports and were categorized as cardiac or non-cardiac. Surgeries directed at treatment of the congenital heart disease were categorized as cardiac. Non-cardiac surgeries included surgeries of other organ systems as well as surgeries related to complications of previous cardiac surgery (diaphragmatic plication, thoracic duct ligation, pacemaker insertion, thoracotomy and decortication and sternal wound debridement).\u003c/p\u003e \u003cp\u003eTo provide information on the medical complexity of these children we recorded chronic (defined as lasting\u0026thinsp;\u0026gt;\u0026thinsp;three months) non-cardiac comorbid conditions that were active (defined as requiring medical treatment, surgical treatment, and/or active follow up by a member of the child\u0026rsquo;s health care team) in the last year of their life. The chronic comorbid conditions, excluding the child\u0026rsquo;s congenital heart disease, were captured using a previously published system-based categories based on ICD-9 diagnoses [\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e] with one modification: mental conditions (ICD codes 290\u0026ndash;319) were split into two groups: 1. Mental health conditions (ICD codes 290\u0026ndash;314) and 2. Developmental delay/intellectual impairment (ICD codes 315\u0026ndash;319 and 783.4). The two conditions that were present in the cohort in the circulatory system diseases category were pulmonary hypertension and cerebrovascular disease. Children were defined as having growth impairment if their weight for age, length for age or body-mass-index (BMI) z-score was less than \u0026minus;\u0026thinsp;2. Additionally, to demonstrate the use of medical technology, life sustaining therapies and resuscitation, we documented chronic (\u0026gt;\u0026thinsp;three months) use of medical technology (enteral/parental nutrition and respiratory support of any kind) along with the use of cardiopulmonary resuscitation (CPR), and mechanical circulatory support (MCS). Mechanical circulatory support was defined as any of extracorporeal cardiopulmonary resuscitation (ECPR), extracorporeal membrane oxygenation (ECMO) as well as included short and long-term ventricular assist devices (VADs).\u003c/p\u003e \u003cp\u003eTo further delineate the burden of disease and intervention, acute comorbid conditions that were active (required medical and/or surgical treatment) were recorded for each admission. Acute comorbid conditions were defined as medical conditions that were newly diagnosed and treated during the admission, or, conditions previously diagnosed requiring new and/or escalating treatment during the admission. Definitions for the acute comorbid conditions were informed by the consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Health Disease 2008 [\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e]. The primary reason for admission was recorded from the discharge summary and these were categorized. Admission for cardiac investigations was defined as an admission for cardiac catheterization, cardiac MRI or cardiac transplant work-up. Admission for congenital heart disease-related comorbidities was defined as admissions for protein losing enteropathy, chylothorax, heart failure, ascites/edema secondary to congenital heart disease, thrombosis, plastic bronchitis and Fontan circulatory failure. Other categories were admission for cardiac surgery (surgery directed at treatment of the congenital heart disease) and infection.\u003c/p\u003e \u003cp\u003eIn order to document symptoms at the end of life, validated scores were used where available [FLACC Behavioral Pain Assessment Scale (Faces, Legs, Activity, Cry, Consolability scale), Wong-Baker FACES Pain Rating Scale, Numeric Pain Rating Scale (NPRS), Cornell Assessment of Pediatric Delirium (CAPD), Agitated Behavior Scale (ABS)]. Additional information about the prevalence of symptoms in the last week of life was gathered from written documentation from any member of the health care team and through medication administration records. End-of-life discussions, as defined by Lee et al. [\u003cspan citationid=\"CR29\" class=\"CitationRef\"\u003e29\u003c/span\u003e], and code status was documented as well as changes to the code status. Additional details on resuscitation, use of life sustaining treatments, PPC consultation and involvement, and psychosocial/spiritual supports were documented. The mode of death (categorized as withdrawal of life sustaining therapies, non-escalation of therapies, failed resuscitation, or death by neurological criteria as previously defined by Trowbridge et. al) and cause of death were recorded [\u003cspan citationid=\"CR30\" class=\"CitationRef\"\u003e30\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAll data were entered into a secure REDCap database hosted on a University of Alberta server. Identifiable information was maintained on a separate, encrypted master list accessible only to the research team, with de-identified data stored on password-protected external devices. Ethics approval was obtained from the University of Alberta Ethics Board (Pro00092382) and the Ethic Board provided a waiver for informed consent.\u003c/p\u003e \u003cp\u003eDescriptive statistics are presented as median (range) for continuous variables and as frequencies and percentages for categorical variables.\u003c/p\u003e"},{"header":"Results","content":"\u003cp\u003e\u003cstrong\u003ePatient Characteristics\u003c/strong\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eA total of 20 children with SV anatomy were included in the study cohort. The median age at death was 3.1 years (range 4 months\u0026ndash;16.7 years). Patient characteristics are summarized in Table 1. All 20 patients (100%) completed second-stage palliative surgery (Glenn procedure), and 11 (55%) completed third-stage palliative surgery (Fontan procedure). In their last year of life, children had a median of two admissions to hospital (1-6), spending a median of 122 days hospitalized (8-254) and experiencing multiple surgeries. The timeline of patient deaths within the stages of their surgical palliation is provided in Figure 1. \u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable 1\u0026nbsp;\u003c/strong\u003ePatient characteristics \u0026nbsp;\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"605\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eCharacteristic\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eNumber (%) or median (range)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eAge at death, median (range)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e3.1 years (4 months -16.7 years)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eSex, N (%)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Male\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Female\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e6 (30)\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e14 (70)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eLocation of residence, N (%)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Alberta\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Out of province\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e14 (70)\u003c/p\u003e\n \u003cp\u003e6 (30)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eDistance between home and surgical center (km), N (%)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;\u0026lt;100\u0026nbsp;\u003cbr\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; 101-500\u0026nbsp;\u003cbr\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026gt; 500\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e5 (25)\u003c/p\u003e\n \u003cp\u003e10 (50)\u003c/p\u003e\n \u003cp\u003e5 (25)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eSize of home community, N (%)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Rural/small (pop. \u0026lt;30,000)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Medium to large urban (pop. \u0026gt;30,000)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e7 (35)\u003c/p\u003e\n \u003cp\u003e13 (65)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eIn foster care, N (%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e3 (15)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eCardiac diagnosis, N (%)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Hypoplastic Left Heart\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Hypoplastic Right Heart\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Heterotaxy Syndrome\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e11 (55)\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e3 (15)\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e6 (30)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eCompleted second stage palliative surgery, N (%)\u003c/p\u003e\n \u003cp\u003eCompleted third stage palliative surgery, N (%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e20 (100)\u003c/p\u003e\n \u003cp\u003e11 (55)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eChronic non-cardiac conditions, median (range)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e3 (0-5)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eChronic technology dependence, N (%)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Enteral and/or parenteral nutrition\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Respiratory support\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e13 (65)\u003c/p\u003e\n \u003cp\u003e9 (45)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eHome oxygen, N(%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e4 (20)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eTracheostomy, N(%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e1 (5)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eSurgical feeding tube, N (15%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e3 (15)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eGrowth impairment, N (%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e14 (70)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eNumber of admissions in the last year of life, median (range)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e2 (1-6)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eDays spend admitted to hospital in the last year of life, median (range)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e122 (8-254)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 444px;\"\u003e\n \u003cp\u003eNumber of surgeries in the last year of life\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 161px;\"\u003e\n \u003cp\u003e3 (1-11)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eThe children were medically complex and many required chronic (\u0026gt; three months) medical treatment and the support of medical technology. Thirteen children (65%) required long-term enteral and/or parenteral nutrition, and nine (45%) required long-term respiratory support with oxygen and/or non-invasive ventilation. In the last year of their life, almost all children (19, 95%) had at least one active chronic non-cardiac condition and the median number of chronic non-cardiac conditions was three (0\u0026ndash;5). As shown in Figure 2, developmental delay/intellectual impairment was most common (14, 70%), followed by digestive system conditions (7, 35%) and respiratory diseases (6, 30%). The category of circulatory system diseases excluded congenital heart disease diagnoses. None of the patients had a documented genetic syndrome.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eSecond and third stage palliative surgery admission\u003c/strong\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe second stage (Glenn procedure, N=20) and third stage (Fontan procedure, N=11) palliative surgery admissions are described in Table 2. The median total length of stay for second-stage surgical admissions was 30 days (range 4\u0026ndash;250). There were ten children (50%) that were admitted for more than seven days pre-Glenn procedure including six children (30%) who were never discharged home between their first and second palliative surgeries. For the children that had a third stage surgical admission the median total length of stay was 60 days (range 14\u0026ndash;178). In the post-operative period for both surgical stages, ICU days made up a significant proportion of the length of stay, with a large range for both days admitted to the ICU and days admitted to the ward. \u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable 2\u003c/strong\u003e Summary of second and third stage palliative surgery admissions\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"605\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 340px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eCharacteristics\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 123px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eSecond stage\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003eN=20\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 142px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eThird stage\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003eN=11\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 340px;\"\u003e\n \u003cp\u003eTotal length of stay, median (range), d\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 123px;\"\u003e\n \u003cp\u003e30 (4-250)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 142px;\"\u003e\n \u003cp\u003e60 (14-178)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 340px;\"\u003e\n \u003cp\u003ePost operative index surgery, median (range), d\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Total length of stay \u0026nbsp; \u0026nbsp;\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;ICU initial\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;ICU total\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Hospital ward\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 123px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e16 (4-114)\u003c/p\u003e\n \u003cp\u003e6.5 (1-99)\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e7 (1-99)\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e6.5 (0-82)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 142px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e59 (7-177)\u003c/p\u003e\n \u003cp\u003e8 (2-70)\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e28 (2-165)\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e13 (0-83)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 340px;\"\u003e\n \u003cp\u003eDied, % (N)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 123px;\"\u003e\n \u003cp\u003e35% (7)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 142px;\"\u003e\n \u003cp\u003e55 % (6)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 340px;\"\u003e\n \u003cp\u003eDelayed sternal closure, % (N)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 123px;\"\u003e\n \u003cp\u003e30% (6)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 142px;\"\u003e\n \u003cp\u003e18% (2)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 340px;\"\u003e\n \u003cp\u003eReoperation, % (N)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;\u0026gt; 1 reoperation\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 123px;\"\u003e\n \u003cp\u003e20% (4)\u003c/p\u003e\n \u003cp\u003e5% (1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 142px;\"\u003e\n \u003cp\u003e64% (7)\u003c/p\u003e\n \u003cp\u003e36% (4)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 340px;\"\u003e\n \u003cp\u003eCardiac arrest, % (N)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 123px;\"\u003e\n \u003cp\u003e15% (3)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 142px;\"\u003e\n \u003cp\u003e9% (1)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 340px;\"\u003e\n \u003cp\u003eMechanical circulatory support, % (N)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 123px;\"\u003e\n \u003cp\u003e25% (5)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 142px;\"\u003e\n \u003cp\u003e36% (4)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 340px;\"\u003e\n \u003cp\u003eCardiac catheterization, % (N)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 123px;\"\u003e\n \u003cp\u003e30% (6)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 142px;\"\u003e\n \u003cp\u003e36% (4)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 340px;\"\u003e\n \u003cp\u003eNon-cardiac surgery, % (N)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 123px;\"\u003e\n \u003cp\u003e45% (9)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 142px;\"\u003e\n \u003cp\u003e45% (5)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eAbout a third of patients (7, 35%) that completed their Glenn procedure died during their Glenn admission. There were ten patients that were admitted for more than seven days before their Glenn surgery; half of these patients \u0026nbsp;(5, 50%) died during their Glenn admission. Just over half (6, 55%) of patients who completed their Fontan procedure died during their Fontan admission. The rates of reoperation were high after both the Glenn and Fontan procedures (20% and 64% respectively) with about a third (4, 36%) of patients in the Fontan procedure admission requiring more than one reoperation. About half (45%) of children in each group had additional non-cardiac surgery in each of the second and third stage palliative surgery admissions. Five children (25%) required mechanical circulatory support during the Glenn admission, and four (36%) during Fontan admission. Approximately one third of children underwent one or more post-operative cardiac catheterizations (30% of patients during their index Glenn admission and 36% during their index Fontan admission) with most cardiac catheterizations being diagnostic and a smaller percentage being interventional.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAdditional admissions in the last year of life\u003c/strong\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eOf the 20 patients, 17 (85%) had admissions in the last year of life. These admissions are summarized in Table 3. Among the seventeen children, there were a total of 39 admissions with a median of one admission (0-6) per child. Overall, the median length of stay was 8 days (0-221), however it was longer for cardiac surgery admissions compared to admissions that did not involve cardiac surgery. However, within the admissions that did not include cardiac surgery (N=29), there were three admissions with a length of stay \u0026gt; 100 days including the longest admission (221 days) which were all for complications of congenital heart disease, specifically for protein losing enteropathy. \u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable 3\u003c/strong\u003e Additional admissions during the last year of life\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"652\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 416px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eCharacteristic\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 236px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eNumber (%) or median (range)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 416px;\"\u003e\n \u003cp\u003eAdditional admissions in the last year of life, median (range)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 236px;\"\u003e\n \u003cp\u003e1 (0-6)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 416px;\"\u003e\n \u003cp\u003eLength of stay, median (range), d\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;ICU\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Hospital ward\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; Admissions with cardiac surgery (N = 10)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; Admissions without cardiac surgery (N = 29)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 236px;\"\u003e\n \u003cp\u003e8 (0-221)\u003c/p\u003e\n \u003cp\u003e2 (0-91)\u003c/p\u003e\n \u003cp\u003e3 (0-202)\u003c/p\u003e\n \u003cp\u003e41 (12-112)\u003c/p\u003e\n \u003cp\u003e4 (0-221)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 416px;\"\u003e\n \u003cp\u003eReason for admission, N (%)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Cardiac surgery\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; Congenital heart disease-related comorbidities\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Cardiac investigations\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Infection\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Other\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 236px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e10 (26%)\u003c/p\u003e\n \u003cp\u003e12 (31%)\u003c/p\u003e\n \u003cp\u003e11 (28%)\u003c/p\u003e\n \u003cp\u003e4 (10%)\u003c/p\u003e\n \u003cp\u003e2 (5%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u003cstrong\u003eAcute comorbid conditions during hospital admissions\u003c/strong\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAcute comorbidities were present in all admissions. Across all admissions (Glenn procedure, Fontan procedure and other admissions in the last year of life), approximately half of the children had an infection and vascular thrombosis. There were high rates of chylothorax (73%) and bleeding requiring reoperation (45%) in the Fontan admission cohort. A quarter of children (25%) had a central nervous system injury during their Glenn admission or other admission and 18% had a central nervous system injury during their Fontan admission. Additional details are found in Figure 3. \u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTerminal admission and end-of-life experience\u003c/strong\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe median length of stay for terminal admissions was 83 days (5\u0026ndash;198) with the majority of inpatient days being spent in the ICU (see Table 4). Most children (18, 90%) died in the intensive care unit, while two (10%) died on the inpatient ward. Most children (13, 65%) died after withdrawal of life-sustaining therapies.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable 4\u0026nbsp;\u003c/strong\u003eTerminal Admission\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"605\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eCharacteristic\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eNumber (%) or median (range)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eLength of terminal admission, median (range), d\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;ICU\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Hospital ward\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e83 (5-198)\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e39 (6-165)\u003c/p\u003e\n \u003cp\u003e10 (0-91)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eSystolic heart function at terminal admission\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Normal\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Abnormal\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e11 (55)\u003c/p\u003e\n \u003cp\u003e9 (45)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eTransplant status\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Listed for transplant\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Not a candidate for transplant\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;No transplant evaluation\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e5 (25%)\u003c/p\u003e\n \u003cp\u003e8 (40%)\u003c/p\u003e\n \u003cp\u003e7 (35%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eCPR during terminal admission\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e9 (45%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eMCS during terminal admission\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Time on MCS, median (Range), d\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e11 (55%)\u003c/p\u003e\n \u003cp\u003e10 (2-119)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003ePalliative care consult\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e13 (65%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eTime between initial palliative care consult and death, median (range), d\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e68 (2-470)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eCode status at the time of death\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;No limitations or no documentation\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Limited resuscitation, no cardiac resuscitation\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Medical management without resuscitation\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Comfort-focused care\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e5 (25%)\u003c/p\u003e\n \u003cp\u003e3 (15%)\u003c/p\u003e\n \u003cp\u003e5 (25%)\u003c/p\u003e\n \u003cp\u003e7 (35%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eTime between earliest end-of-life discussion and death (N=18), days\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e10 (1-264)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eTime between earliest change in code status and death (N=16), days\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e4 (0-257)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eIndividuals involved in end-of-life conversations\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Family member\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; Intensivist\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; Cardiologist\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Social worker\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; Palliative care\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Patient\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e20 (100%)\u003c/p\u003e\n \u003cp\u003e19 (95%)\u003c/p\u003e\n \u003cp\u003e15 (75%)\u003c/p\u003e\n \u003cp\u003e10 (50%)\u003c/p\u003e\n \u003cp\u003e6 (30%)\u003c/p\u003e\n \u003cp\u003e1 (5%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eLocation of death\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Cardiac ICU\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Hospital ward\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e18 (90%)\u003c/p\u003e\n \u003cp\u003e2 (10%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eMode of death\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; Withdrawal of life sustaining therapies\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; Non-escalation of therapies\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Failed resuscitation\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e13 (65%)\u003c/p\u003e\n \u003cp\u003e6 (30%)\u003c/p\u003e\n \u003cp\u003e1(5%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 331px;\"\u003e\n \u003cp\u003eFamily present at time of death\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Mother\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Father\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Additional family members\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Siblings\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 274px;\"\u003e\n \u003cp\u003e20 (100%)\u003c/p\u003e\n \u003cp\u003e19 (95%)\u003c/p\u003e\n \u003cp\u003e17 (85%)\u003c/p\u003e\n \u003cp\u003e10 (50%)\u003c/p\u003e\n \u003cp\u003e6 (30%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eChildren experienced multiple symptoms in the final days, with a median of five symptoms (3\u0026ndash;12) per patient. The most common symptoms were pain (95%), anxiety or agitation (95%), respiratory distress (65%), and edema (65%) (Figure 4). Additional psychosocial and spiritual support was provided in some cases, including child life, social work, and spiritual services (Figure 5). \u003c/p\u003e\n\u003cp\u003eThe majority continued to receive intensive medical care in the final days of life. In the last week, 15 children (75%) were on inotropes, nine (45%) received mechanical circulatory support, and five (25%) underwent major surgery (Table 5). Cardiopulmonary resuscitation (CPR) was administered to six children (30%) during the final week, and in four cases (20%), this occurred within the last 48 hours of life. Two of the patients that received CPR in the last week of life were on MCS at the time of CPR initiation. Both received a prolonged period of CPR (45 and 70 minutes) leading to death. Of the nine children (45%) on MCS in the last week of life, almost half of them (4, 44%) were on MCS for more than 30 days prior to their death. All of the children that underwent major surgery in the last week of life (5, 25%) were on MCS at the time of surgery; three of them had been on MCS for more than 30 days, all were intubated, ventilated and sedated preoperatively and all of these children multi-organ dysfunction prior to surgery.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable 5\u003c/strong\u003e Interventions and treatments in the last week of life\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"577\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 356px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eCharacteristics\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 221px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eNumber (%) or median (range)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 356px;\"\u003e\n \u003cp\u003eReceived CPR\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Duration of CPR, median (range)\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;CPR while on MCS\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 221px;\"\u003e\n \u003cp\u003e6 (30%)\u003c/p\u003e\n \u003cp\u003e45 min (10-70 min)\u003c/p\u003e\n \u003cp\u003e2 (10%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 356px;\"\u003e\n \u003cp\u003eMechanical circulatory support\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp;Initiated during the last week of life\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp;ECPR\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 221px;\"\u003e\n \u003cp\u003e9 (45%)\u003c/p\u003e\n \u003cp\u003e5 (25%)\u003c/p\u003e\n \u003cp\u003e3 (15%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 356px;\"\u003e\n \u003cp\u003eRenal replacement therapy (PD or CRRT)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 221px;\"\u003e\n \u003cp\u003e6 (30%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 356px;\"\u003e\n \u003cp\u003eIntubated and ventilated\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 221px;\"\u003e\n \u003cp\u003e13 (65%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 356px;\"\u003e\n \u003cp\u003eInotropes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 221px;\"\u003e\n \u003cp\u003e15 (75%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 356px;\"\u003e\n \u003cp\u003eMajor surgery\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Cardiac\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp;Other\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 221px;\"\u003e\n \u003cp\u003e5 (25%)\u003c/p\u003e\n \u003cp\u003e2 (10%)\u003c/p\u003e\n \u003cp\u003e3 (15%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eNearly all children (18, 90%) had their goals of care, including resuscitation status, documented in the terminal hospital stay. Sixteen children (80%) had one or more changes in their goals of care status during the terminal admission with a median of two changes (1-5). At the time of death, most children\u0026rsquo;s (12, 60%) goals of care excluded resuscitation with five children (25%) having documented goals of active medical management without resuscitation (no cardiac resuscitation and no intubation) and seven children (35%) having documented goals of comfort focused care. Three children (15%) had documented limitations in cardiac resuscitation but included intubation and ventilation and five (25%) remained a full code (either by documented as such or by default because goals of care were undocumented). The median time between the earliest change in code status and death was four days with a wide range in timing (0-257 days), suggesting that goals-of-care transitions often occurred very close to the end of life. There were seven children (35%) that had a change to their goals of care status that occurred more than seven days before their death. Almost all changes in documented goals of care placed limits on medical interventions. In one case, there was a change from some limits to full resuscitation; this change occurred on the day the child died.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eEnd-of-life discussions were documented for the majority of patients, though the timing varied widely, with the median time between earliest end-of-life discussion and death being ten days (1-264). These discussions consistently involved family members (20, 100%) and the intensivist (19, 95%). Cardiologists participated in 15 cases (75%), while social work was present in 10 (50%) and palliative care in 6 (30%). Direct patient participation was rare, occurring in only one case (5%) (Table 4).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003ePediatric Palliative Care Involvement\u003c/strong\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003ePPC was consulted for 13 children (65%) during the course of their illness (Figure 6). The most common reason for initial referral was for transplant assessment (53%), followed by refractory or complex symptom management (23%). Other reasons included tracheostomy decision-making, patient no longer being a surgical candidate, parental or patient request, and goals of care discussions (each 8%). The timing of consultation varied widely, with a median of 68 days (range 2\u0026ndash;470) between initial palliative care involvement and death. For those who received palliative care support, the most common area of involvement was in complex decision-making (85%), followed by symptom management (77%) and support for family coping (69%). \u0026nbsp;\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003e This study provides insight into the end-of-life experiences and health care utilization of children with SV anatomy at a large Canadian center. Additionally, it highlights some unique features of end-of-life symptoms and care for SV anatomy patients. These children spent much of their last year of life in hospital, with both surgical and non-surgical reasons for admission. The majority of children in our study died in the ICU, with many continuing to receive intensive therapies, such as inotropes, mechanical circulatory support, and major surgery, until end of life. Similar findings have been reported in other studies of children with advanced heart disease in the USA, Korea and Italy, where death occurring in ICU after withdrawal of life-sustaining therapies represents the vast majority of deaths [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR29\" class=\"CitationRef\"\u003e29\u003c/span\u003e, \u003cspan citationid=\"CR31\" class=\"CitationRef\"\u003e31\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eMany of the children in this study received multiple intensive interventions in the last week of life. The mortality of patients with SV anatomy that receive MCS is high with survival to hospital discharge in this population being 30\u0026ndash;50% [\u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e]. Given this, judicious use of highly invasive interventions (major surgery) and resuscitation (CPR) for patients with SV anatomy on MCS is essential. Both the combination of interventions, and duration of combined intensive therapies, that children with SV anatomy received towards the end of life and raises questions about the benefits versus burdens of the combined interventions that some patients received.\u003c/p\u003e \u003cp\u003eFor most children, the intensive care received in the last week of life was provided with the goal of life prolongation. Prognostication is very challenging in these children, with many of them experiencing multiple periods of worsening and recovery, while providers and parents struggle to recognize the time point where they transition from a recoverable state to palliative care. This struggle may contribute to how end of life unfolds and the timing of the goals of care conversations happening late and close to death, as we and others have seen [\u003cspan citationid=\"CR29\" class=\"CitationRef\"\u003e29\u003c/span\u003e, \u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR34\" class=\"CitationRef\"\u003e34\u003c/span\u003e]. Recognizing there is uncertainty in their clinical trajectories, most of the children in our study had lengthy ICU stays with multiple acute conditions arising during their hospitalization. These findings, as well as other published risk factors [\u003cspan citationid=\"CR35\" class=\"CitationRef\"\u003e35\u003c/span\u003e] that diminish the likelihood of survival, could serve as triggers for earlier conversations around the child and family\u0026rsquo;s goals of care as well as the child\u0026rsquo;s resuscitation status. For children with SV anatomy, their diagnosis alone puts them at increased risk for death in childhood, and this risk increases with the additional complications and comorbid conditions that some develop over time. There is a notable gap in advanced care planning for patients with SV anatomy. There is an opportunity for improvements in advanced care planning in this unique population, which may then inform the course and conversations within hospital and critical care admissions.\u003c/p\u003e \u003cp\u003eThis study found there were some children with SV anatomy that transitioned to less interventional and more comfort focused goals of care earlier in their trajectory and that there was a very large range in the timing of this change in goals of care. In this cohort of children, about a third changed their goals of care status more than seven days prior to their death. Of these children, five were status-post Fontan procedure and the majority (80%) had failing Fontan physiology and were not transplant candidates. These two factors may appropriately lead to limitations in intervention and earlier conversations around goals of care. Two patients were status-post Glenn procedure, with one having progressive pulmonary stenosis and the second having complex comorbidities leading to ventilator dependency. In three cases, there was a discussion about having a planned out of hospital death however, none of these cases left hospital, all for different reasons. Additional factors that were prevalent in this cohort such as having long hospital and ICU stays and the need for readmission, as well as the length of time admitted prior to the Glenn procedure, could act as triggers for goals of care conversations.\u003c/p\u003e \u003cp\u003eIn the patients that had non-resuscitative goals of care, ICU-level care was often required in the end-of-life period to manage refractory symptoms or chronic complications. Symptom burden at the end of life was substantial in our cohort, with children experiencing a median of five symptoms in the final week, most commonly pain, respiratory distress, and anxiety. This is consistent with data from Molloy et al. (2021), who reported that pain, fatigue, and dyspnea were the most frequent and distressing symptoms experienced by children with advanced heart disease, particularly those children with single ventricle anatomy [\u003cspan citationid=\"CR36\" class=\"CitationRef\"\u003e36\u003c/span\u003e]. Despite this, certain symptoms such as fatigue and psychological distress, for which children in this population are at particularly high risk, are often underrecognized and undertreated [\u003cspan citationid=\"CR36\" class=\"CitationRef\"\u003e36\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR37\" class=\"CitationRef\"\u003e37\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThere is a bias towards thinking that a good death occurs outside of an ICU setting however in this complex patient with SV anatomy, we found that the ICU setting was best suited to provide comfort focused care at the end of life given the types and number of medications required as well as the opportunity to provide one to one nursing care. More information is needed on how these complex patients can receive necessary care in alternate locations, including at home or in hospice, for those patients and families who may wish for their child\u0026rsquo;s end of life care and death outside of hospital. Beginning advanced care planning, and PPC involvement, from antenatal or neonatal period may help with better supporting these children and families, and also may normalize conversations around goals and values, which should occur early and be continually revisited over time.\u003c/p\u003e \u003cp\u003eMoynihan et al (2022) found 70% of parents felt their child who died with advanced heart disease had a good death (34% agreed somewhat and 36% agreed strongly) and invasive therapies did not impact the families\u0026rsquo; view on whether or not their child experienced a good death [\u003cspan citationid=\"CR34\" class=\"CitationRef\"\u003e34\u003c/span\u003e]. They found that a good death was associated with pain control, advanced care planning, greater preparedness and CPR avoidance. These goals can all be supported through both primary and subspecialty PPC. While palliative care was consulted in 65% of children in our study, referrals occurred relatively late, often triggered by transplant evaluation or when children were no longer surgical candidates.The pattern of limited PPC involvement and variable, often delayed, PPC consultation is consistent with existing literature, despite evidence that earlier integration is beneficial [\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e]. Although transplant assessment is a trigger for a PPC consult at our center, the absence of standardized PPC referral guidelines, or defined triggers outside of a transplant assessment, may contribute to missed opportunities of PPC involvement and/or late PPC involvement. Our study\u0026rsquo;s findings, there is a need to more clearly define what early, high-quality end of life care and palliative care involvement looks like for children with complex congenital heart disease, particularly those with single ventricle anatomy\u003c/p\u003e \u003cp\u003eAs partnerships between pediatric cardiac care, intensive care and palliative care continue to evolve, there is a growing need to reframe how care is conceptualized for this population, addressing persistent misperceptions among both families and providers regarding the role of early palliative involvement [\u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e]. Emerging evidence supports the benefits of integrating PPC from the time of diagnosis in single-ventricle heart disease, as early consultation has been shown to foster therapeutic relationships, enhance family coping, and reduce parental anxiety [\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e, \u003cspan citationid=\"CR38\" class=\"CitationRef\"\u003e38\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR39\" class=\"CitationRef\"\u003e39\u003c/span\u003e]. Standardizing prenatal or early PPC consults, particularly for conditions such as hypoplastic left heart syndrome, has also been associated with improved family support, education, and care continuity without detracting from curative intent [\u003cspan citationid=\"CR39\" class=\"CitationRef\"\u003e39\u003c/span\u003e]. Collectively, these findings highlight the value of establishing palliative partnerships well before the terminal phase of illness.\u003c/p\u003e \u003cp\u003eThis study has several strengths, including the use of detailed, longitudinal chart review and a focus on a rare but high-risk population. Our hospital captures a large geographic area, making these findings broadly applicable to other Canadian centers with similar populations. However, limitations include the retrospective design, the lack of standardized symptom scoring tools for many symptoms, and potential gaps in data from care that patients received as outpatients or from other institutions. Despite these limitations, our findings provide valuable insight into the end-of-life experience of children with single ventricle anatomy and identify areas for improvement in care delivery.\u003c/p\u003e"},{"header":"CONCLUSION","content":"\u003cp\u003eIn summary, this study adds to the growing literature supporting early, integrated palliative care for children with single ventricle anatomy, specifically in the Canadian context. By reframing ICU care as appropriate and necessary for comfort and end of life care, and by recognizing the role of early, more standardized PPC involvement, we can better align care with patient and family needs and goals. In the future, further work is needed to identify end of life quality indicators, create standardized triggers for PPC consultation, and improve education for care providers and families, ensuring that palliative care is viewed not as a last resort, but as an essential component of comprehensive, compassionate care.\u003c/p\u003e"},{"header":"STATEMENTS AND DECLARATIONS","content":"\u003cp\u003e\u003cstrong\u003eEthics Approval\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eEthics approval for this retrospective chart review was obtained from the University of Alberta Health Research Ethics Board (Pro00092382). The requirement for informed consent was waived. All data were de-identified prior to analysis and stored securely on password-protected servers in accordance with institutional and national ethical guidelines. The study adhered to the principles of the Declaration of Helsinki and all relevant regulations governing research involving human participants.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor Contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by Lize-Mari Du Toit, Hayley Turnbull, Lindsay Ryerson, and Jennifer Conway. The first draft of the manuscript was written by Lize-Mari Du Toit and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConflicts of Interest\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors have no relevant financial or non-financial interests to disclose. The authors have no conflicts of interest to declare that are relevant to the content of this article. All the authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials that are discussed in this manuscript. The authors have no financial or proprietary interests in any material discussed in this article.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eMorell, E., Wolfe, J., Scheurer, M., Thiagarajan, R., Morin, C., Beke, D. M., Smoot, L., Cheng, H., Gauvreau, K., \u0026amp; Blume, E. D. (2012). Patterns of care at end of life in children with advanced heart disease. \u003cem\u003eArchives of Pediatrics \u0026amp; Adolescent Medicine, 166\u003c/em\u003e(8), 745\u0026ndash;748. https://doi.org/10.1001/archpediatrics.2011.1829\u003c/li\u003e\n\u003cli\u003eO\u0026apos;Leary, P. W. (2002). 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(2022). Complications and management of functional single ventricle children with Fontan circulation: From the surgeon\u0026rsquo;s point of view. \u003cem\u003eFrontiers in Cardiovascular Medicine, 9\u003c/em\u003e, 917059. https://doi.org/10.3389/fcvm.2022.917059\u003c/li\u003e\n\u003cli\u003eMedina, C. K., Prabhu, N. K., Alderete, I. S., et al. (2024). Days alive and out of hospital for children born with single-ventricle heart disease. \u003cem\u003eCardiology in the Young, 34\u003c/em\u003e(7), 1445\u0026ndash;1450. https://doi.org/10.1017/S1047951124000118\u003c/li\u003e\n\u003cli\u003eBlume, E. D., Kirsch, R., Cousino, M. K., Walter, J. K., Steiner, J. M., Miller, T. A., ... \u0026amp; American Heart Association Pediatric Heart Failure and Transplantation Committee of the Council on Lifelong Congenital Heart Disease and Heart Health in the Young. (2023). Palliative care across the life span for children with heart disease: A scientific statement from the American Heart Association. \u003cem\u003eCirculation: Cardiovascular Quality and Outcomes, 16\u003c/em\u003e(2), e000114. https://doi.org/10.1161/HCQ.0000000000000114\u003c/li\u003e\n\u003cli\u003eDavis, J. A. M., Bass, A., Humphrey, L., Texter, K., \u0026amp; Garee, A. (2020). Early integration of palliative care in families of children with single ventricle congenital heart defects: A quality improvement project to enhance family support. \u003cem\u003ePediatric Cardiology, 41\u003c/em\u003e(1), 114\u0026ndash;122. https://doi.org/10.1007/s00246-019-02231-y\u003c/li\u003e\n\u003cli\u003eTing, J., Songer, K., Bailey, V., Rotman, C., Lipsitz, S., Rosenberg, A. R., Delgado-Corcoran, C., \u0026amp; Moynihan, K. M. (2024). Impact of subspecialty pediatric palliative care on children with heart disease: A systematic review and meta-analysis. \u003cem\u003ePediatric Cardiology\u003c/em\u003e. Advance online publication. https://doi.org/10.1007/s00246-024-03535-4\u003c/li\u003e\n\u003cli\u003eSonger, K. L., Wawrzynski, S. E., Olson, L. M., Harousseau, M. E., Meeks, H. D., Moresco, B. L., \u0026amp; Delgado-Corcoran, C. (2025). Association of palliative care timing with end-of-life quality in children with heart disease. \u003cem\u003eJournal of Pain and Symptom Management, 69\u003c/em\u003e(4), 402\u0026ndash;408. https://doi.org/10.1016/j.jpainsymman.2025.01.003\u003c/li\u003e\n\u003cli\u003e\u003cstrong\u003eMarcus, K. L., Balkin, E. M., Al-Sayegh, H., et al. (2018).\u003c/strong\u003e Patterns and outcomes of care in children with advanced heart disease receiving palliative care consultation. \u003cem\u003eJournal of Pain and Symptom Management, 55\u003c/em\u003e(2), 351\u0026ndash;358. https://doi.org/10.1016/j.jpainsymman.2017.09.015\u003c/li\u003e\n\u003cli\u003eBlume, E. D., Morell, E. B., Aiyagari, R., Ziniel, S., Beke, D. M., Thiagarajan, R., Taylor, L., Kulik, T., Pituch, K., \u0026amp; Wolfe, J. (2014). Parental perspectives on suffering and quality of life at end-of-life in children with advanced heart disease: An exploratory study. \u003cem\u003ePediatric Critical Care Medicine, 15\u003c/em\u003e(4), 336\u0026ndash;342. https://doi.org/10.1097/PCC.0000000000000072\u003c/li\u003e\n\u003cli\u003eWan, A., Weingarten, K., \u0026amp; Rapoport, A. (2020). \u0026ldquo;Palliative care?! But this child\u0026rsquo;s not dying\u0026rdquo;: The burgeoning partnership between pediatric cardiology and palliative care. \u003cem\u003eCanadian Journal of Cardiology, 36\u003c/em\u003e(12), 1992\u0026ndash;1999. https://doi.org/10.1016/j.cjca.2020.07.013\u003c/li\u003e\n\u003cli\u003eMery, C. M., Berdusco, E., Gibson, R., Huffman, L. C., Harrington, K. R., Menahem, S., \u0026amp; Kelleher, K. J. (2023). Examining the real-life journey of individuals and families affected by single-ventricle congenital heart disease. \u003cem\u003eJournal of the American Heart Association, 12\u003c/em\u003e(5), e027556. https://doi.org/10.1161/JAHA.122.027556 \u003c/li\u003e\n\u003cli\u003eDing, Z., Zhu, J., Ding, Y., et al. (2023). Comorbidities in congenital heart disease: Different patterns in childhood and adulthood. \u003cem\u003eBMC Cardiovascular Disorders, 23\u003c/em\u003e, 613. https://doi.org/10.1186/s12872-023-03654-5\u003c/li\u003e\n\u003cli\u003e\u003cem\u003ePart IV \u0026ndash; The dictionary of definitions of complications associated with the treatment of patients with congenital cardiac disease.\u003c/em\u003e (2008). \u003cem\u003eCardiology in the Young, 18\u003c/em\u003e(S2), 282\u0026ndash;530. https://doi.org/10.1017/S1047951108003351\u003c/li\u003e\n\u003cli\u003eLee, J., Kim, G. B., Song, M. K., Lee, S. Y., Kim, M. S., \u0026amp; Bae, E. J. (2020). End-of-life care of hospitalized children with advanced heart disease. \u003cem\u003eJournal of Korean Medical Science, 35\u003c/em\u003e(16), e107. https://doi.org/10.3346/jkms.2020.35.e107 \u003c/li\u003e\n\u003cli\u003eTrowbridge, A., Walter, J. K., McConathey, E., Morrison, W., \u0026amp; Feudtner, C. (2018). Modes of death within a children\u0026rsquo;s hospital. \u003cem\u003ePediatrics, 142\u003c/em\u003e(4), e20174182. https://doi.org/10.1542/peds.2017-4182 \u003c/li\u003e\n\u003cli\u003eAgosto, C., Benedetti, F., De Tommasi, V., Milanesi, O., Stellin, G., Padalino, M. A., \u0026amp; Benini, F. (2021). End-of-life care for children with complex congenital heart disease: Parents\u0026rsquo; and medical caregivers\u0026rsquo; perceptions. \u003cem\u003eJournal of Paediatrics and Child Health, 57\u003c/em\u003e(5), 696\u0026ndash;701. https://doi.org/10.1111/jpc.15208 \u003c/li\u003e\n\u003cli\u003eMiller, J. R., Lancaster, T. S., Callahan, C., Abarbanell, A. M., \u0026amp; Eghtesady, P. (2018). An overview of mechanical circulatory support in single-ventricle patients. \u003cem\u003eTranslational Pediatrics, 7\u003c/em\u003e(2), 151\u0026ndash;161. https://doi.org/10.21037/tp.2018.03.01 \u003c/li\u003e\n\u003cli\u003eValente, I. E., Fisher, G., Wolf, B. J., \u0026amp; Tanious, M. K. (2023). Code Status Discussions in Pediatric Patients With Heart Disease During Terminal Admissions. \u003cem\u003ePediatrics\u003c/em\u003e, \u003cem\u003e152\u003c/em\u003e(6), e2023063221.\u003c/li\u003e\n\u003cli\u003eMoynihan, K. M., Ziniel, S. I., Johnston, E., Morell, E., Pituch, K., \u0026amp; Blume, E. D. (2022). A \u0026ldquo;good death\u0026rdquo; for children with cardiac disease. \u003cem\u003ePediatric Cardiology, 43\u003c/em\u003e(4), 744\u0026ndash;755. https://doi.org/10.1007/s00246-021-02769-6 \u003c/li\u003e\n\u003cli\u003ePoh, C. L., Millar, J., d\u0026rsquo;Udekem, Y., \u0026amp; Brizard, C. (2021). Pre- and post-operative determinants of transplantation-free survival after Fontan: The Australia and New Zealand experience. \u003cem\u003eInternational Journal of Cardiology: Heart \u0026amp; Vasculature, 35\u003c/em\u003e, 100825. https://doi.org/10.1016/j.ijcha.2021.100825\u003c/li\u003e\n\u003cli\u003eMolloy, M. A., DeWitt, E. S., Morell, E., Reichman, J. R., Brown, D. W., Kobayashi, R., Sleeper, L. A., Elia, E. G., Samsel, C., \u0026amp; Blume, E. D. (2021). Parent-reported symptoms and perceived effectiveness of treatment in children hospitalized with advanced heart disease. \u003cem\u003eThe Journal of Pediatrics, 238\u003c/em\u003e, 221\u0026ndash;227.e1. https://doi.org/10.1016/j.jpeds.2021.06.077\u003c/li\u003e\n\u003cli\u003eMcCormick, A. D., Wilde, M. M., \u0026amp; Charpie, C. E. (2022). Psychological functioning in pediatric children with single-ventricle heart disease: A systematic review. \u003cem\u003eCardiology in the Young, 32\u003c/em\u003e(2), 173\u0026ndash;184. https://doi.org/10.1017/S1047951121003844 \u003c/li\u003e\n\u003cli\u003eHancock, H. S., Pituch, K., Uzark, K., Bhat, P., Fifer, C., Silveira, M., \u0026amp; Aiyagari, R. (2018). A randomized trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease. \u003cem\u003eCardiology in the Young, 28\u003c/em\u003e(4), 561\u0026ndash;570. https://doi.org/10.1017/S1047951117002605\u003c/li\u003e\n\u003cli\u003eLowenstein, S., Macauley, R., Perko, K., \u0026amp; Ronai, C. (2020). Provider perspective on the role of palliative care in hypoplastic left heart syndrome. \u003cem\u003eCardiology in the Young, 30\u003c/em\u003e(3), 377\u0026ndash;382. https://doi.org/10.1017/S1047951119002019\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"pediatric-cardiology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pedc","sideBox":"Learn more about [Pediatric Cardiology](http://link.springer.com/journal/246)","snPcode":"246","submissionUrl":"https://submission.nature.com/new-submission/246/3","title":"Pediatric Cardiology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"Pediatric Palliative Care, Pediatric Cardiology, End-of-life Care, Single-ventricle anatomy","lastPublishedDoi":"10.21203/rs.3.rs-8524691/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8524691/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eIntroduction:\u003c/p\u003e\n\u003cp\u003eCare 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.\u003c/p\u003e\n\u003cp\u003eMethods:\u003c/p\u003e\n\u003cp\u003eThis 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 (\u0026lt; 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.\u003c/p\u003e\n\u003cp\u003eResults:\u003c/p\u003e\n\u003cp\u003eMedian 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%).\u003c/p\u003e\n\u003cp\u003eConclusions:\u003c/p\u003e\n\u003cp\u003eChildren 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.\u003c/p\u003e","manuscriptTitle":"End of Life Care for Infants and Children with Single Ventricle Anatomy","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2026-01-13 15:08:41","doi":"10.21203/rs.3.rs-8524691/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2026-02-11T14:25:07+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2026-01-22T18:58:32+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"336102893177480015365093197936399832663","date":"2026-01-09T17:43:22+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2026-01-07T14:59:30+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"41270713857566319055151855633743707058","date":"2026-01-07T13:07:08+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2026-01-07T08:34:39+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2026-01-06T12:04:10+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2026-01-06T12:03:43+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Cardiology","date":"2026-01-05T20:26:32+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"pediatric-cardiology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pedc","sideBox":"Learn more about [Pediatric Cardiology](http://link.springer.com/journal/246)","snPcode":"246","submissionUrl":"https://submission.nature.com/new-submission/246/3","title":"Pediatric Cardiology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"f2ee931e-4016-49d2-a156-7451759e8097","owner":[],"postedDate":"January 13th, 2026","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2026-04-20T16:02:58+00:00","versionOfRecord":{"articleIdentity":"rs-8524691","link":"https://doi.org/10.1007/s00246-026-04242-y","journal":{"identity":"pediatric-cardiology","isVorOnly":false,"title":"Pediatric Cardiology"},"publishedOn":"2026-04-16 15:59:38","publishedOnDateReadable":"April 16th, 2026"},"versionCreatedAt":"2026-01-13 15:08:41","video":"","vorDoi":"10.1007/s00246-026-04242-y","vorDoiUrl":"https://doi.org/10.1007/s00246-026-04242-y","workflowStages":[]},"version":"v1","identity":"rs-8524691","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8524691","identity":"rs-8524691","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}