This comprehensive systematic review and meta-analysis summarizes the published literature on the role of SPPC in pediatric heart disease. Overall, we identified limited empiric data, generally low in quality, solely from the US, and mostly single center studies with small sample sizes. Even with a paucity of studies, meta-analysis identified benefits of SPPC at both a patient- and family-level for decedents and survivors, with individual studies supporting system-level in-hospital cost reduction and improved staff rating of quality of life prior to death. Pediatric patients who received SPPC were less likely to experience active resuscitation at EOL, and were more likely to have documentation of ACP and resuscitation limits. Additionally, SPPC involvement was found to decrease parental stress. Identified gaps and results offer opportunities for future research and interventions to improve holistic care for children with cardiac disease.

Data supporting benefits of SPPC for children with heart disease mirror results in other populations. The evaluated studies show significant increases in ACP documentation and limits to resuscitation for pediatric patients with AHD who received SPPC, compared to those who did not receive SPPC. This is similar to findings in oncology patients and children with complex chronic conditions who subsequently die as inpatients [18, 43]. One included study evaluated the perspectives of parents of children heart disease who died and found they are more likely to perceive a “good death” experience if they felt prepared, participated in ACP, and reported non-cure-oriented goals-of care [38]. This is consistent with research in parents of children with other complex chronic conditions that shows that parents highly value ACP early in the illness course and that this improves parent-reported EOL outcomes [44].

Of pediatric cardiac patients who died, those who received SPPC were half as likely to experience active resuscitation at EOL. Similar observations were noted in general pediatric patients and children with cancer with SPPC involvement who were both less likely to experience resuscitative events prior to death [18, 45]. Importantly, one included study showed active CPR negatively influenced a family’s perception of a “good death” experience for their child with heart disease [38]. Taken together, core principles of palliative care may promote goal-concordant EOL experiences in this population.

Although EOL and SPPC are often falsely conflated, our meta-analysis demonstrated benefits of SPPC extend beyond decedents. Among parents, parental stress decreased with the receipt of SPPC services [40, 42]. Patients with SVHD have among the highest risk of mortality in AHD, so unsurprisingly this population received increased attention. The randomized control trial of early SPPC intervention for mothers of children with SVHD found decreased maternal anxiety and improved communication and family relationships [40] while parental depression and anxiety did not decrease with the intervention in all-comer neonates with AHD [42]. As part of a quality improvement initiative aiming to improve collaboration between one institution’s Single Ventricle (SV) team and SPPC team, a preliminary survey of Heart Center staff showed that 88% of respondents agreed or strongly agreed that routine involvement of the SPPC team with SV patients has improved the overall psychosocial and/or decision-making support provided to families [46]. While this study lacked a control, it suggests that providers of pediatric cardiac care see benefit from routine involvement for families of children with SVHD.

AHD is a leading cause of disease-related death in US children, and issues around EOL for these patients deserve attention [47]. Despite identifying eleven review articles specifically discussing the role of palliative care in the pediatric cardiac population emphasizing this as a key emerging area and the benefits described, overall SPPC referral rates were low [5, 10,11,12,13,14, 21,22,23,24,25]. Though SPPC involvement has been increasing in children with AHD [37], referral rates have historically been low compared to other pediatric complex conditions [18, 45]. Barriers to SPPC involvement include concerns from pediatric cardiologists of “undermining parental hope” and parents perceiving they are “giving up” [48, 49]. Notably, in a study evaluating features of SPPC involvement for children with AHD, most families expressed that life prolongation was a priority at the time of initial consultation, which suggests that parents can retain hope and concurrently receive SPPC services [19]. Our evidence suggests that SPPC involvement leads to increased ACP, modes/locations of death that may be more goal-concordant, and improvements in psychosocial support for families of children with AHD. Comparable benefits of SPPC to other patient populations should prompt improved integration of SPPC into the care of pediatric patients with AHD to improve the experience of patients and families as early as immediately after diagnosis, up to end-of-life.

There are limitations to consider when interpreting results. Studies are heterogenous in included populations (e.g., CICU decedents vs VAD patients) and the outcome measures examined with no standardized approach to evaluating EOL metrics in pediatric AHD. Overall studies were of low-quality with only 1 RCT, limiting ability to make causal inferences. All studies were conducted in the US in English language, and all were single center apart from one study evaluating parental perspectives from two institutions [38] and a state-based SPPC program implementation study [34], limiting generalizability. Clinicians may be more likely to consult SPPC for children with higher illness severity and/or challenging social situations; we could not assess the impact of these and other potential confounders on mortality and LOS. The fact that benefits of SPPC were observed in retrospective studies where selection bias for referral may exist could suggest larger true benefits. While definitions of SPPC differed between study designs, variable care delivery between hospitals is universal. This review was unable to examine individual interventions or frequency of interventions. Furthermore, patients in the control group may have had effective “primary” palliative care (comprehensive care with a palliative approach delivered by the primary cardiology/intensive care team) which cannot be quantified. When aggregated, statistical findings were stronger for several outcomes despite only two included studies, indicating small sample sizes may also preclude accurate conclusions without meta-analyses. We were unable to include hospice involvement in the meta-analysis due to different definitions of hospice in the included studies, i.e., home with hospice care versus institutional hospice care. This limitation is significant, as dying at home or under hospice conditions is one of the potential outcomes of interest of SPPC involvement described in other pediatric populations [18].

More work is needed in exploring logistics of optimal SPPC delivery models across different cardiac care environments including when and how subspecialty teams should to be involved versus primary models of delivery. Considering this must span prenatal, intensive care, inpatient, and outpatient involvement, there needs to be parallel efforts to both enhance primary palliative care through training of cardiology and critical care fellows [50, 51] and to consider novel integration approaches [15].

Additional studies are necessary to deepen the body of literature describing the impact of SPPC on the care of children with AHD. Outcomes in retrospective studies primarily centered around SPPC impact on EOL, so an opportunity exists for further research to evaluate the impacts on more holistic benefits extending to survivors or bereavement support after death. Additionally, EOL outcomes studied for pediatric patients are often extrapolated from adult EOL care priorities, such as the location of death being at home. Parental perspectives about preference for location of death has not been studied in pediatric heart disease, and future studies should identify whether preferred location of death was elicited, and if the ultimate location of death was concordant with that preference. There is a dearth of studies evaluating the perspectives of children and adolescents with heart disease receiving SPPC services.

Barriers to more generalizable research through multicenter studies include limitations to ICD-10 coding and cardiac registry data collection. Collaboration with pediatric cardiology and cardiac surgery registries could move the field forward by significantly facilitating larger scale studies [52, 53]. Defining high-quality pediatric EOL care is a priority offering opportunities for standardization of outcomes, which would allow for improved analysis of SPPC impact across institutions [54]. Despite quality metrics for EOL care in oncology [54, 55], no measures are defined for children with AHD, which is a critical gap demanding further research.

Our systematic review and meta-analysis shows benefits of SPPC for these patients, at both a patient- and family-level with decreased parental stress, less active resuscitation at EOL, and increased ACP documentation and resuscitation limits compared to those without SPPC. Children with AHD are a unique subpopulation of pediatric patients with potentially limited lifespans and high intensity of care through EOL. Overall we identified a paucity of high-quality data studying the influence of SPPC, however findings correlate with literature in other pediatric populations. Results illuminate gaps and future opportunities for research.