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Patients with Fontan failure are high-risk candidates for heart transplantation and other advanced therapies. Understanding the outcomes following initial heart failure consultation can help define appropriate timing of referral for advanced heart failure care.
This is a survey study of heart failure providers seeing any Fontan patient for initial heart failure care. Part 1 of the survey captured data on clinical characteristics at the time of heart failure consultation, and Part 2, completed 30 days later, captured outcomes (death, transplant evaluation outcome, and other interventions). Patients were classified as “too late” (death or declined for transplant due to being too sick) and/or “care escalation” (ventricular assist device implanted, inotrope initiated, and/or listed for transplant), within 30 days. “Late referral” was defined as those referred too late and/or had care escalation.
Between 7/2020 and 7/2022, 77 Fontan patients (52% inpatient) had an initial heart failure consultation. Ten per cent were referred too late (6 were too sick for heart transplantation with one subsequent death, and two others died without heart transplantation evaluation, within 30 days), and 36% had care escalation (21 listed ± 5 ventricular assist device implanted ± 6 inotrope initiated). Overall, 42% were late referrals. Heart failure consultation < 1 year after Fontan surgery was strongly associated with late referral (OR 6.2, 95% CI 1.8–21.5, p=0.004).
Over 40% of Fontan patients seen for an initial heart failure consultation were late referrals, with 10% dying or being declined for transplant within a month of consultation. Earlier referral, particularly for those with heart failure soon after Fontan surgery, should be encouraged.
Racial and ethnic disparities are well described in paediatric cardiac critical care outcomes. However, understanding the mechanisms behind these outcomes and implementing interventions to reduce and eliminate disparities remain a gap in the field of paediatric cardiac critical care. The Pediatric Cardiac Critical Care Consortium (PC4) established the Equity, Diversity, and Inclusion (EDI) Committee in 2020 to promote an equity lens to its aim of improving paediatric cardiac critical care quality and outcomes across North America. The PC4 EDI Committee is working to increase research, quality improvement, and programming efforts to work towards health equity. It also aims to promote health equity considerations in PC4 research. In addition to a focus on patient outcomes and research, the committee aims to increase the inclusion of Black, Indigenous, and People of Color (BIPOC) members in the PC4 collaborative. The following manuscript outlines the development, structure, and aims of the PC4 EDI Committee and describes an analysis of social determinants of health in published PC4 research.
The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) lacks a rigorous enrollment audit process, unlike other collaborative networks. Most centers require individual families to consent to participate. It is unknown whether there is variation across centers or biases in enrollment.
We used the Pediatric Cardiac Critical Care Consortium (PC4) registry to assess enrollment rates in NPC-QIC for those centers participating in both registries using indirect identifiers (date of birth, date of admission, gender, and center) to match patient records. All infants born 1/1/2018–12/31/2020 and admitted 30 days of life were eligible. In PC4, all infants with a fundamental diagnosis of hypoplastic left heart or variant or who underwent a surgical or hybrid Norwood or variant were eligible. Standard descriptive statistics were used to describe the cohort and center match rates were plotted on a funnel chart.
Of 898 eligible NPC-QIC patients, 841 were linked to 1,114 eligible PC4 patients (match rate 75.5%) in 32 centers. Match rates were lower in patients of Hispanic/Latino ethnicity (66.1%, p = 0.005), and those with any specified chromosomal abnormality (57.4%, p = 0.002), noncardiac abnormality (67.8%, p = 0.005), or any specified syndrome (66.5%, p = 0.001). Match rates were lower for patients who transferred to another hospital or died prior to discharge. Match rates varied from 0 to 100% across centers.
It is feasible to match patients between the NPC-QIC and PC4 registries. Variation in match rates suggests opportunities for improvement in NPC-QIC patient enrollment.
Burnout is well characterised in physicians and residents but not in paediatric cardiology fellows, and few studies follow burnout longitudinally. Training-specific fears have been described in paediatric cardiology fellows but also have not been studied at multiple time points. This study aimed to measure burnout, training-specific fears, and professional fulfilment in paediatric cardiology fellows with the attention to time of year and year-of-training.
This survey-based study included the Professional Fulfillment Index and the Impact of Events Scale as well as an investigator-designed Fellow Fears Questionnaire. Surveys were distributed at three-time points during the academic year to paediatric cardiology fellows at a large Midwestern training programme. Fellow self-reported gender and year-of-training were collected. Descriptive analyses were performed.
10/17 (59%) of fellows completed all surveys; 60% were female, 40% in the first-year class, 40% in the second-year class, and 20% in the third-year class. At least half of the fellows reported burnout at each survey time point, with lower mean professional fulfilment scores. The second-year class, who rotate primarily in the cardiac ICU, had higher proportions of burnout than the other two classes. At least half of fellows reported that they “often” or “always” worried about not having enough clinical knowledge or skills and about work–life balance.
Paediatric cardiology fellows exhibit high proportions of burnout and training-specific fears. Interventions to mitigate burnout should be targeted specifically to training needs, including during high-acuity rotations.
Health-related quality of life in children who have undergone the Ross procedure has not been well characterised. The aim of this study was to characterise health-related quality of life in this cohort and compare to children with other CHD.
In this cross sectional, single-centre study, health-related quality of life was assessed in patients who underwent a non-neonatal Ross procedure using the Pediatric Quality of Life Inventory. Ross cohort scores were compared with healthy norms, patients with CHD requiring no surgical intervention or had curative surgery (Severity 2, S2) and patients who were surgically repaired with ≥1 surgical procedure and with significant residual lesion or need for additional surgery (Severity 3, S3). Associations between Pediatric Quality of Life Inventory score and patient factors were also examined.
68 patients completed surveys. Nearly one-sixth of patients had overall scores below the cut-off for at-risk status for impaired health-related quality of life. There was no difference in overall health-related quality of life score between the Ross cohort and healthy children (p = 0.56) and S2 cohort (p = 0.97). Health-related quality of life was significantly higher in the Ross cohort compared to S3 cohort (p = 0.02). This difference was driven by a higher psychosocial health-related quality of life in the Ross cohort as compared to S3 cohort (p = 0.007). Anxiety scores were significantly worse in the Ross cohort compared to both S2 (p = 0.001) and S3 (p = 0.0017), respectively.
Children who have undergone a Ross procedure report health-related quality of life equivalent to CHD not requiring therapy and superior to CHD with residual lesions. Despite these reassuring results, providers should be aware of potential anxiety among Ross patients.
Prolonged effusions post-Fontan procedure are associated with morbidity. Fontan patients have higher pro-inflammatory cytokines in chest tube drainage compared to controls. Colchicine, an anti-inflammatory medication, decreases effusions in adults after cardiac surgery. We hypothesised that patients post-Fontan treated with colchicine would have decreased pro-inflammatory cytokine levels and shorter duration of chest tube drainage.
This pilot clinical trial enrolled nine patients (intention to treat); five completed the protocol (per protocol). Post-operative Fontan patients 20 months to 5 years receiving colchicine were compared to a previously published control cohort (n = 25). Per protocol patients received 0.6 mg colchicine daily starting post-operative day 2, ending 1 day after chest tube removal. Chest tube samples were taken on days 1–4, 7 and 10, or until removal and analysed with a 17-cytokine Bio-Plex Assay. Descriptive statistics and basic univariate comparisons were made.
There was no difference in duration of chest tube drainage or length of stay between intention to treat patients and controls. Per protocol patients had shorter duration of chest tube drainage compared to controls (6 days [interquartile range 4.7–7], versus 10 days [7–11], p = 0.007) and shorter length of stay (7 days [5.5–9] versus 9 days [9–13], p = 0.005). Pro-inflammatory cytokines trended lower in per protocol patients.
In this pilot cohort, patients who completed the colchicine protocol post-Fontan procedure had shortened duration of chest tube drainage and length of stay. A decrease of pro-inflammatory cytokines may contribute to the mechanism of this change.
Patients with single ventricle heart disease are living into adulthood due to medical and surgical advancements but have significant physical comorbidities and an increased risk for psychological comorbidities compared to healthy subjects or those with other CHD diagnoses. This study aimed to systematically review psychological functioning in paediatric single ventricle heart disease.
Literature was searched using PubMed, Embase, PsycInfo, CINAHL Complete and Scopus. Peer-reviewed articles that included patients ages 0–25 years with single ventricle heart disease, and quantitative measures of psychological outcomes were included. Meta-analysis using a fixed-effect model was conducted for internalising and externalising t-scores, utilised by the Achenbach Child Behavior Checklist.
Twenty-nine records met the criteria for inclusion. 13/24 studies demonstrated increased risk for internalising disorders, such as anxiety/depression; 16/22 studies demonstrated risk for externalising disorders, such as attention or behavioural problems. Meta-analysis of four studies revealed that paediatric single ventricle heart disease patients had no significant difference in internalising and externalising t-scores compared to normative values.
The current review demonstrates the need for further studies to better understand psychological functioning in patients with single ventricle heart disease, with a majority of studies showing increased risk for psychological problems despite no difference seen in a small meta-analysis. This summary of the literature underscores the need for regular psychological screening, earlier intervention and integrated mental health therapies in paediatric single ventricle heart disease.
Understanding the impact of the COVID-19 pandemic on paediatric non-COVID-19-related care, as well as patient and caregiver concerns and stressors, is critical for informing healthcare delivery. It was hypothesised that high care disruptions and psychological stress would be observed among paediatric and adult CHD patients in the early phase of the pandemic.
A cross-sectional, international, electronic survey study was completed. Eligible participants included parents of children with acquired or CHD, adults with CHD, or caregivers of adults with CHD.
A total of 1220 participants from 25 countries completed the survey from 16 April to 4 May, 2020. Cardiac care disruption was significant with 38% reporting delays in pre-pandemic scheduled cardiac surgeries and 46% experiencing postponed cardiac clinic visits. The majority of respondents (75%) endorsed moderate to high concern about the patient with heart disease becoming ill from COVID-19. Worry about returning for in-person care was significantly greater than worry of harm to patient due to postponed care. Clinically significant psychological stress was high across the sample including children (50%), adults with CHD (42%), and caregivers (42%).
The early phase of the COVID-19 pandemic contributed to considerable disruptions in cardiac care for patients with paediatric and adult CHD. COVID-19-related fears are notable with potential to impact willingness to return to in-person care. Psychological stress is also very high necessitating intervention. Further study of the impact of delays in care on clinical outcomes is warranted.
Lymphatic vessel dysplasia is associated with Fontan-associated protein-losing enteropathy. Extra nodal non-Hodgkin lymphomas including mucosa-associated lymphoid tissue (MALT lymphoma) are associated with lymphatic vessel dysplasia. Here, we describe the case of a 7-year-old with Fontan-associated protein-losing enteropathy who developed MALT lymphoma with a clinical course indicative of interaction between these pathologies and improvement in protein-losing enteropathy after MALT lymphoma treatment. This case suggests a pathophysiologic overlap which has implications for the management of Fontan-associated protein-losing enteropathy.
Social media is any type of communication utilising electronic technology that follows two guiding principles: free publishing or sharing of content and ideas and group collaboration and inter-connectedness. Over the last 10 years, social media technology has made tremendous inroads into all facets of communication. Modalities such as Facebook, YouTube, and Twitter are no longer viewed as new communication technologies. Owing to their tremendous usage, they are now common ways to conduct a dialogue with individuals and groups. Greater than 91% of teenagers and 89% of young adults routinely use social media. Further, 24% of teenagers reported being online “almost constantly”. These forms of communication are readily used by individuals cared for in the field of paediatric cardiology; thus, they should carry significant interest for cardiology care providers; however, social media’s influence on medicine extends beyond use by patients. It directly affects all medical providers, both users and non-users. Further, social media has the ability to improve care for patients with paediatric heart disease. This article details social media’s current influence on paediatric cardiology, including considerations for professional use of social media and potential opportunities to improve cardiac care.
Numerous data sets collect information on patients with paediatric cardiovascular disease, including paediatric heart failure and transplant patients. This review discusses methodologies available for linking and integrating information across data sets, which may help facilitate answering important questions in the field of paediatric heart failure and transplant that cannot be answered with individual data sets or single-centre data alone.
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