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Context Although access to subspecialty pediatric palliative care (PPC) is increasing, little is known about the role of PPC for children with advanced heart disease (AHD). Objectives The objective of this study was to examine features of subspecialty PPC involvement for children with AHD. Methods This is a retrospective single-institution medical record review of patients with a primary diagnosis of AHD for whom the PPC team was initially consulted between 2011 and 2016. Results Among 201 patients, 87% had congenital/structural heart disease, the remainder having acquired/nonstructural heart disease. Median age at initial PPC consultation was 7.7 months (range 1 day–28.8 years). Of the 92 patients who were alive at data collection, 73% had received initial consultation over one year before. Most common indications for consultation were goals of care (80%) and psychosocial support (54%). At initial consultation, most families (67%) expressed that their primary goal was for their child to live as long and as comfortably as possible. Among deceased patients (n = 109), median time from initial consultation to death was 33 days (range 1 day–3.6 years), and children whose families expressed that their primary goal was for their child to live as comfortably as possible were less likely to die in the intensive care unit (P = 0.03) and more likely to die in the setting of comfort care or withdrawal of life-sustaining interventions (P = 0.008). Conclusion PPC involvement for children with AHD focuses on goals of care and psychosocial support. Findings suggest that PPC involvement at end of life supports goal-concordant care. Further research is needed to clarify the impact of PPC on patient outcomes.

Studies have shown that pediatricians in all stages of training are uncomfortable managing patients at end of life. Our goal was to create and test a portable reference card to improve pediatric resident education in comprehensive care for children nearing end of life.We evaluated the impact of the Pediatric End-of-Life Care Management Reference Card on residents' perceived comfort and knowledge through pre- and post-intervention surveys. The preintervention questionnaires and pocket cards were distributed to all first- and second-year residents, and then a follow-up survey was provided six months later. Based on Likert scales, questions focused on self-reported understanding of palliative care principles and knowledge regarding and comfort in performing end-of-life symptom management.Twenty-six pediatric residents completed pre- and post-intervention surveys. Following receipt of the reference card, no significant changes were noted consistently across all groups of residents. The majority of improvements were noted when comparing second to third year residents, including knowledge and comfort related to pain management, comfort in managing secretions and nausea, and documentation following death. The first to second year residents demonstrated improvement in knowing what language to use to tell a family that their child has died.This study demonstrates that a portable reference card may be a convenient, simple, and useful component of education for pediatric residents in end-of-life care management. This reference card is a foundation from which to develop a standardized educational tool. Additional research is required to assess the impact of this type of intervention in pediatric palliative care education.

Despite advances in therapy, outcomes for children with pulmonary hypertension remain poor. We sought to assess the validity of a pediatric-specific functional classification system for pulmonary hypertension (PH) in a heterogeneous population of children with PH diagnosed by echocardiogram or cardiac catheterization. A single-center, retrospective study of 65 infants and children with PH was performed. Pediatric Functional Class (FC) at diagnosis, at last visit, and change in FC over time were evaluated for their association with mortality and PH-associated morbidity in univariate, time-to-event, and multivariate regression analyses. Median age at PH diagnosis was 5.3 months (0 days–12.7 years). Twenty-five children (38 %) had idiopathic PH or PH secondary to congenital heart disease, one (2 %) had left heart disease, and 39 (60 %) had PH secondary to respiratory disease. Mortality was 25 % (16/63), primarily in the first year of follow-up. FC at diagnosis was not significantly associated with survival (p = 0.22), but higher FC (more impaired) at last visit (p

Background Studies have shown that children with cancer globally lack access to palliative care. Little is known regarding physicians’ perceptions of palliative care, treatment access, and self-reported competence in providing palliative care. Procedure Members of the Global Neuroblastoma Network (online tumor board) were surveyed. Eighty-three respondents met inclusion criteria; 53 (64%) completed the survey. Results Most respondents trained in high-income countries (HIC) but practice in low- and middle-income countries (LMIC), and care for more than five patients with neuroblastoma annually. WHO Essential Medicines in palliative care varied in availability, with incomplete access across LMIC centers. Nonpharmacologic therapies were inconsistently available. Contrary to international definitions, 17% of respondents inappropriately considered palliative care as that initiated only after curative therapy is stopped. Mean physician competence composite score (Likert scale 1–5, 5 = very competent) in providing symptomatic relief and palliative care across phases of care was 2.93 (95% CI 2.71–3.22). Physicians reported significantly greater competence in symptom management during cure-directed therapy than during end-of-life (P = 0.02) or when patients are actively dying (P = 0.007). Practicing in HIC, prior palliative care training, having access to radiotherapy, and not having to turn patients away due to bed shortages were significantly predictive of perceived competence in providing palliative care at end of life. Conclusions An international sample identified gaps in treatment and palliative care service availability, in understanding the definition of palliative care, and in self-reported competence in providing palliative care. Increased perceived competence was associated with training, which supports the need for increased palliative care education and advocacy, especially in LMIC.

OBJECTIVES The disease burden and mortality of children with pulmonary hypertension are significantly higher than for the general PICU population. We aimed to develop a risk-adjustment tool predicting PICU mortality for pediatric pulmonary hypertension patients: the Pediatric Index of Pulmonary Hypertension Intensive Care Mortality score. DESIGN Retrospective analysis of prospectively collected multicenter pediatric critical care data. SETTING One-hundred forty-three centers submitting data to Virtual Pediatric Systems database between January 1, 2009, and December 31, 2015. PATIENTS Patients 21 years old or younger with a diagnosis of pulmonary hypertension. INTERVENTIONS Twenty-one demographic, diagnostic, and physiologic variables obtained within 12 hours of PICU admission were assessed for inclusion. Multivariable logistic regression with stepwise selection was performed to develop the final model. Receiver operating characteristic curves were used to compare the Pediatric Index of Pulmonary Hypertension Intensive Care Mortality score with Pediatric Risk of Mortality 3 and Pediatric Index of Mortality 2 scores. MEASUREMENTS AND MAIN RESULTS Fourteen-thousand two-hundred sixty-eight admissions with a diagnosis of pulmonary hypertension were included. Primary outcome was PICU mortality. Fourteen variables were selected for the final model: age, bradycardia, systolic hypotension, tachypnea, pH, FIO2, hemoglobin, blood urea nitrogen, creatinine, mechanical ventilation, nonelective admission, previous PICU admission, PICU admission due to nonsurgical cardiovascular disease, and cardiac arrest immediately prior to admission. The receiver operating characteristic curve for the Pediatric Index of Pulmonary Hypertension Intensive Care Mortality model (area under the curve = 0.77) performed significantly better than the receiver operating characteristic curves for Pediatric Risk of Mortality 3 (area under the curve = 0.71; p < 0.001) and Pediatric Index of Mortality 2 (area under the curve = 0.69; p < 0.001), respectively. CONCLUSIONS The Pediatric Index of Pulmonary Hypertension Intensive Care Mortality score is a parsimonious model that performs better than Pediatric Risk of Mortality 3 and Pediatric Index of Mortality 2 for mortality in a multicenter cohort of pediatric pulmonary hypertension patients admitted to PICUs. Application of the Pediatric Index of Pulmonary Hypertension Intensive Care Mortality model to pulmonary hypertension patients in the PICU might facilitate earlier identification of patients at high risk for mortality and improve the ability to prognosticate for patients and families.

While the importance of pediatric palliative care (PPC) for children with life-threatening illness is increasingly recognized, little is known about physicians' attitudes toward palliative care for children with heart disease.To compare the perspectives of PPC physicians and pediatric cardiologists regarding palliative care in pediatric heart disease.Cross-sectional web-based surveys.Responses from 183 pediatric cardiologists were compared to those of 49 PPC physicians (response rates 31% [183/589] and 28% [49/175], respectively). Forty-eight percent of PPC physicians and 63% of pediatric cardiologists agreed that availability of PPC is adequate (p = 0.028). The majority of both groups indicated that PPC consultation occurs "too late." Compared with pediatric cardiologists, PPC physicians reported greater competence in all areas of advance care planning, communication, and symptom management. PPC physicians more often described obstacles to PPC consultation as "many" or "numerous" (42% vs. 7%, p 0.001). PPC physicians overestimated how much pediatric cardiologists worry about PPC introducing inconsistency in approach (60% vs. 11%, p 0.001), perceive lack of added value from PPC (30% vs. 7%, p 0.001), believe that PPC involvement will undermine parental hope (65% vs. 44%, p = 0.003), and perceive that PPC is poorly accepted by parents (53% vs. 27%, p 0.001).There are significant differences between pediatric cardiologists and PPC physicians in perception of palliative care involvement and perceived barriers to PPC consultation. An intervention that targets communication and exchange of expertise between PPC and pediatric cardiology could improve care for children with heart disease.

Despite advances in the diagnosis and management of pediatric pulmonary hypertension (PH), children with PH represent a growing inpatient population with significant morbidity and mortality. To date, no studies have described the clinical characteristics of children with PH in the pediatric intensive care unit (PICU). A retrospective multicenter cohort study of 153 centers in the Virtual PICU Systems database who submitted data between 1 January 2009 and 31 December 2015 was performed. A total of 14,880/670,098 admissions (2.2%) with a diagnosis of PH were identified. Of these, 2190 (14.7%) had primary PH and 12,690 (85.3%) had secondary PH. Mortality for PH admissions was 6.8% compared to 2.3% in those admitted without PH (odds ratio = 3.1; 95% confidence interval = 2.9–3.4). Compared to patients admitted to the PICU without PH, those with PH were younger, had longer length of stay, higher illness severity scores, were more likely to receive invasive mechanical ventilation, cardiopulmonary resuscitation, extracorporeal membrane oxygenation, and more likely to have co-diagnoses of sepsis, heart failure, and respiratory failure. In a multivariate model, factors significantly associated with mortality for children with PH included age 16 years, invasive mechanical ventilation, and co-diagnoses of heart failure, sepsis, hemoptysis, disseminated intravascular coagulation, stroke, and multi-organ dysfunction syndrome. Despite therapeutic advances, the disease burden and mortality of children with PH remains significant. Further investigation of the risk factors associated with clinical deterioration and mortality in this population could improve the ability to prognosticate and inform clinical decision-making.

While availability of palliative care consultation for children with advanced heart disease increases, little is known about cardiologist attitudes towards palliative care. We sought to describe perspectives of cardiologists regarding palliative care and to characterize their perceived competence in palliative care concepts. A cross-sectional survey of pediatric cardiologists and cardiac surgeons from 19 pediatric medical centers was performed. Overall response rate was 31% (183/589). Respondents had a median of 18 years of experience since medical school (range 2–49) and most practiced at academic centers (91%). Sixty-percent of respondents felt that palliative care consultations occur “too late” and the majority (85%) agreed that palliative care consultations are helpful. Barriers to requesting palliative care consultation were most frequently described as “referring to palliative care services too early will undermine parents’ hope” (45%) and “concern that parents will think I am giving up on their child” (56%). Only 33% of cardiologists reported feeling “very” or “moderately” competent in prognosticating life expectancy while over 60% felt competent caring for children with heart disease around end of life, and nearly 80% felt competent discussing goals of care and code status. Greater perceived competence was associated with subspecialty (heart failure/intensivist vs. other) (OR 3.6, 95% CI 1.6–8.1, p = 0.003) and didactic training (OR 6.27, 95% CI 1.8–21.8, p = 0.004). These results underscore the need for further training in palliative care skills for pediatric cardiologists. Enhancing palliative care skills among cardiologists and facilitating partnership with subspecialty palliative care teams may improve overall care of children with advanced heart disease.

Purpose Patient perceived effectiveness of care is increasingly recognized as a measure of quality. There is little data on the frequency or treatment of symptoms in children with advanced heart disease. We sought to evaluate parental report of symptoms and successful treatment of those symptoms in their children hospitalized with advanced heart disease (AHD). Methods We performed a prospective cohort survey of parents of children hospitalized with AHD at a single institution. Parents were approached if their child was (1) admitted for > 7 days with single ventricle physiology, pulmonary vein stenosis, or pulmonary hypertension, or (2) had ICU stay >30d, mechanical circulatory support, >3 surgeries/ admissions in the last year, mechanical ventilator support >14d, or listed for heart transplant. Information was collected regarding symptoms, suffering from those symptoms, and whether symptoms were addressed and treated by medical providers. Results 112 parents participated, with the majority ward admissions (60%). 51% of parents reported that their child was experiencing ‘a great deal’ or ‘a lot’ of symptoms related to their heart disease. Frequent symptoms reported included: fatigue (76%), feeding difficulties (67%), irritability (66%), pain (65%), and dyspnea (64%). The percentage suffering from the symptoms was fairly consistent across individual symptoms (37-47%). The least often treated symptoms were sadness/depression (47%), fatigue (49%), and sleep disturbances (52%), and these were some of the least likely to be reported as successfully treated (31-33%) (Fig). Conclusion Fatigue, pain, dyspnea, irritability and feeding difficulties are the symptoms most frequently identified by parents caring for children with AHD in the hospital setting, with pain and dyspnea most often being the symptoms treated successfully. Our data suggest that cardiologists may inadequately assess and treat mental health symptoms associated with hospitalization for advanced heart disease.

Objective To characterize patterns of care at the end of life for children and young adults with life-threatening complex chronic conditions (LT-CCCs) and to compare them by LT-CCC type. Study design Cross-sectional survey of bereaved parents (n = 114; response rate of 54%) of children with noncancer, noncardiac LT-CCCs who received care at a quaternary care children's hospital and medical record abstraction. Results The majority of children with LT-CCCs died in the hospital (62.7%) with more than one-half (53.3%) dying in the intensive care unit. Those with static encephalopathy (AOR, 0.19; 95% CI, 0.04-0.98), congenital and chromosomal disorders (AOR, 0.28; 95% CI, 0.09-0.91), and pulmonary disorders (AOR, 0.08; 95% CI, 0.01-0.77) were significantly less likely to die at home compared with those with progressive central nervous system (CNS) disorders. Almost 50% of patients died after withdrawal or withholding of life-sustaining therapies, 17.5% died during active resuscitation, and 36% died while receiving comfort care only. The mode of death varied widely across LT-CCCs, with no patients with pulmonary disorders dying receiving comfort care only compared with 66.7% of those with CNS progressive disorders. A majority of patients had palliative care involvement (79.3%); however, in multivariable analyses, there was distinct variation in receipt of palliative care across LT-CCCs, with patients having CNS static encephalopathy (AOR, 0.07; 95% CI, 0.01-0.68) and pulmonary disorders (AOR, 0.07; 95% CI, 0.01-.09) significantly less likely to have palliative care involvement than those with CNS progressive disorders. Conclusions Significant differences in patterns of care at the end of life exist depending on LT-CCC type. Attention to these patterns is important to ensure equal access to palliative care and targeted improvements in end-of-life care for these populations.

To describe parent perspectives regarding the end-of-life experience of children with advanced heart disease.Cross-sectional multicenter survey study of bereaved parents.Two tertiary care pediatric hospitals.Parents of children younger than 21 years with primary cardiac diagnoses who died in the hospital 9 months to 4 years before the survey date. Parents were excluded if they were non-English speakers or had previously denied permission to contact.The Survey for Caring for Children with Advanced Heart Disease was developed, piloted, and then sent to parents of all children who died at two sites.Fifty bereaved parents responded (39% response rate) a mean of 2.7 years after their child's death. Median age at death was 6 months (3.6 d to 20.4 yr). At end-of-life, 86% of children were intubated and 46% were receiving mechanical circulatory support. Seventy-eight percent died during withdrawal of life-sustaining interventions and 16% during resuscitative efforts. Parents realized that their child had no realistic chance of survival a median of 2 days prior to death (0-30 d). According to parents, 47% of children suffered "a great deal," "a lot," or "somewhat" during the end-of-life period. The symptoms parents perceived to be causing the most suffering were breathing and feeding difficulties in children under 2 years and fatigue and sleeping difficulties in older children. Seventy-one percent of parents described the quality of life of their child during the last month of life as "poor" or "fair." Most parents (84%) described the quality of care delivered as "very good" or "excellent."According to their parents, many children with advanced heart disease experience suffering in the end-of-life care period. For most, realization that their child has no realistic chance of survival does not occur until late, some not until death is imminent. Once this realization occurs, however, parents perceive peacefulness, a "good death," and excellent quality of care. Strategies for improved communication around symptom management, quality of life, prognosis, and advance care planning are needed for families of children with advanced heart disease.