The Impact of Social Determinants of Health on Transition from Pediatric to Adult Cardiology Care.

Social determinants of health (SDoH) are the economic, social, environmental, and psychosocial factors that influence health. Adolescents and young adults with congenital heart disease (CHD) require lifelong cardiology follow-up and therefore coordinated transition from pediatric to adult healthcare systems. However, gaps in care are common during transition, and driven in part by pervasive disparities in SDoH including race, ethnicity, socioeconomic status, access to insurance, and remote location of residence. These disparities often co-exist, and compound challenges faced by patients and families. For example, Black and Indigenous individuals are more likely to be subject to systemic racism and implicit bias within healthcare and other settings, be unemployed and poor, have limited access to insurance, and have a lower likelihood of transfer of care to adult CHD specialists. SDoH are also associated with acquired cardiovascular disease, a co-morbidity that adults with CHD face. This review summarizes existing evidence regarding the impact of SDoH on transition to adult care and proposes strategies at the individual, institutional, and population/system levels to reduce inequities faced by transition-age youth. These include routinely screening for SDoH in clinical settings with referral to appropriate services for those who screen positive, providing formal transition education for all transition-age youth including training on navigating complex medical systems, creating satellite cardiology clinics to facilitate access to care for those who live remote from tertiary centers, advocating for lifelong insurance coverage where applicable, mandating cultural sensitivity training for providers, and increasing the diversity of healthcare providers in pediatric and adult CHD care.

Acute kidney injury and renal recovery following Fontan surgery.

Objectives: Acute kidney injury has been described after Fontan surgery, but the duration and outcomes are unknown. We sought to describe the incidence of and risk factors for acute kidney injury and the phenotype of renal recovery, and evaluate the impact of renal recovery phenotype on outcomes. Methods: All children who underwent a Fontan operation at a single center between 2009 and 2022 were included. Data collected included Fontan characteristics, vasopressor use, all measures of creatinine, and postoperative outcomes. Logistic regression models were used to assess predictors of acute kidney injury and the association between acute kidney injury and outcomes. Results: We enrolled 141 children (45% female). Acute kidney injury occurred in 100 patients (71%). Acute kidney injury duration was transient (<48 hours) in 77 patients (55%), persistent (2-7 days) in 15 patients (11%), more than 7 days in 4 patients (3%), and unknown in 4 patients (3%). Risk factors for acute kidney injury included higher preoperative indexed pulmonary vascular resistance (odds ratio, 3.90; P = .004) and higher postoperative inotrope score on day 0 (odds ratio, 1.13, P = .047). Risk factors for acute kidney injury duration more than 48 hours included absence of a fenestration (odds ratio, 3.43, P = .03) and longer duration of cardiopulmonary bypass (odds ratio, 1.22 per 15-minute interval, P = .01). Acute kidney injury duration more than 48 hours was associated with longer length of stay compared with transient acute kidney injury (median 18 days [interquartile range, 9-62] vs 10 days [interquartile range, 8-16], P = .006) and more sternal wound infections (17% vs 4%, P = .049). Conclusions: Acute kidney injury after the Fontan operation is common. The occurrence and duration of acute kidney injury have significant implications for postoperative outcomes.

Anxiety and Depression in Adults With Congenital Heart Disease.

BACKGROUND: A comprehensive understanding of adult congenital heart disease outcomes must include psychological functioning. Our multisite study offered the opportunity to explore depression and anxiety symptoms within a global sample. OBJECTIVES: In this substudy of the APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults With Congenital Heart Disease-International Study), the authors we investigated the prevalence of elevated depression and anxiety symptoms, explored associated sociodemographic and medical factors, and examined how quality of life (QOL) and health status (HS) differ according to the degree of psychological symptoms. METHODS: Participants completed the Hospital Anxiety and Depression Scale, which includes subscales for symptoms of anxiety (HADS-A) and depression (HADS-D). Subscale scores of 8 or higher indicate clinically elevated symptoms and can be further categorized as mild, moderate, or severe. Participants also completed analogue scales on a scale of 0 to 100 for QOL and HS. Analysis of variance was performed to investigate whether QOL and HS differed by symptom category. RESULTS: Of 3,815 participants from 15 countries (age 34.8 ± 12.9 years; 52.7% female), 1,148 (30.1%) had elevated symptoms in one or both subscales: elevated HADS-A only (18.3%), elevated HADS-D only (2.9%), or elevations on both subscales (8.9%). Percentages varied among countries. Both QOL and HS decreased in accordance with increasing HADS-A and HADS-D symptom categories (P < 0.001). CONCLUSIONS: In this global sample of adults with congenital heart disease, almost one-third reported elevated symptoms of depression and/or anxiety, which in turn were associated with lower QOL and HS. We strongly advocate for the implementation of strategies to recognize and manage psychological distress in clinical settings. (Patient-Reported Outcomes in Adults With Congenital Heart Disease [APPROACH-IS]; NCT02150603).

Lower Socioeconomic Status is Associated with an Increased Incidence and Spectrum of Major Congenital Heart Disease and Associated Extracardiac Pathology.

Several studies have suggested an inverse relationship between lower socioeconomic status (SES) and the incidence of congenital heart disease (CHD) among live births. We sought to examine this relationship further in a Canada-wide population study, exploring CHD subtypes, trends, and associated noncardiac abnormalities. Infants born in Canada (less Quebec) from 2008 to 2018 with CHD requiring intervention in the first year were identified using ICD-10 codes through the Canadian Institute for Health Information Discharge Abstract Database. Births of CHD patients were stratified by SES (census-based income quintiles) and compared against national birth proportions using X2 tests. Proportions with extracardiac defects (ED) and nonlethal genetic syndromes (GS) were also explored. From 2008 to 2018, 7711 infants born with CHD were included. The proportions of major CHD distributed across SES quintiles were 27.1%, 20.1%, 19.2%, 18.6%, and 15.0% from lowest to highest, with significant differences relative to national birth proportions (22.0%, 20.0%, 20.6%, 20.7%, and 16.7% from lowest (1) to highest (5)) (p < 0.0001). No temporal trends in the CHD proportions across SES categories were observed over the study period. The distribution across SES quintiles was different only for specific CHD subtypes (double-outlet right ventricle (n = 485, p = 0.03), hypoplastic left heart syndrome (n = 547, p = 0.006), heterotaxy (n = 224, p = 0.03), tetralogy of Fallot (n = 1007, p = 0.008), truncus arteriosus (n = 126, p < 0.0001), and ventricular septal defect (n = 1916, p < 0.0001)), with highest proportions observed in the lowest quintile. The proportion of the total population with ED but not GS was highest in lower SES quintiles (< 0.0001) commensurate with increased proportion of CHD. Our study suggests a negative association between SES and certain CHD lesions and ED.

CanCHD Study of Hematopoietic Cancers in Children With and Without Genetic Syndromes.

BACKGROUND: Individuals with genetic syndromes can manifest both congenital heart disease (CHD) and cancer attributable to possible common underlying pathways. To date, reliable risk estimates of hematopoietic cancer (HC) among children with CHD based on large population-based data remain scant. This study sought to quantify the risk of HC by the presence of genetic syndrome among children with CHD. METHODS AND RESULTS: Data sources were the Canadian CHD database, a nationwide database on CHD (1999-2017), and the CCR (Canadian Cancer Registry). Standardized incidence ratios were calculated for comparing HC incidences in children with CHD with the general pediatric population. A modified Kaplan-Meier curve was used to estimate the cumulative incidence of HC with death as a competing risk. A total of 143 794 children (aged 0-17 years) with CHD were followed up from birth to age 18 years for 1 314 603 person-years. Of them, 8.6% had genetic syndromes, and 898 HC cases were observed. Children with known syndromes had a substantially higher risk of incident HC than the general pediatric population (standardized incidence ratio, 13.4 [95% CI, 11.7-15.1]). The cumulative incidence of HC was 2.44% (95% CI, 2.11-2.76) among children with a syndrome and 0.79% (95% CI, 0.72-0.87) among children without a syndrome. Acute myeloid leukemia had a higher cumulative incidence during early childhood than acute lymphoblastic leukemia. CONCLUSIONS: This is the first large population-based analysis documenting that known genetic syndromes in children with CHD are a significant predictor of HC. The finding could be essential in informing risk-stratified policy recommendations for cancer surveillance in children with CHD.

The Effect of Udenafil on Heart Rate and Blood Pressure in Adolescents With the Fontan Circulation.

The Fontan Udenafil Exercise Longitudinal (FUEL) trial showed that treatment with udenafil was associated with improved exercise performance at the ventilatory anaerobic threshold in children with Fontan physiology. However, it is not known how the initiation of phosphodiesterase 5 inhibitor therapy affects heart rate and blood pressure in this population. These data may help inform patient selection and monitoring after the initiation of udenafil therapy. The purpose of this study is to evaluate the effects of udenafil on vital signs in the cohort of patients enrolled in the FUEL trial. This international, multicenter, randomized, double-blind, placebo-controlled trial of udenafil included adolescents with single ventricle congenital heart disease who had undergone Fontan palliation. Changes in vital signs (heart rate [HR], systolic [SBP] and diastolic blood pressure [DBP]) were compared both to subject baseline and between the treatment and the placebo groups. Additional exploratory analyses were performed to evaluate changes in vital signs for prespecified subpopulations believed to be most sensitive to udenafil initiation. Baseline characteristics were similar between the treatment and placebo cohorts (n = 200 for each). The groups demonstrated a decrease in HR, SBP, and DBP 2 hours after drug/placebo administration, except SBP in the placebo group. There was an increase in SBP from baseline to after 6-min walk test in the treatment and placebo groups, and the treatment group showed an increase in HR (87.4 ± 15.0 to 93.1 ± 19.4 beats/min, p <0.01) after exercise. When comparing changes from baseline to the 26-week study visit, small decreases in both SBP (-1.9 ± 12.3 mm Hg, p = 0.03) and DBP (-3.0 ± 9.6 mm Hg, p <0.01) were seen in the treatment group. There were no clinically significant differences between treatment and placebo group in change in HR or blood pressure in the youngest age quartile, lightest weight quartile, or those on afterload-reducing agents. In conclusion, initiation of treatment with udenafil in patients with Fontan circulation was not associated with clinically significant changes in vital signs, implying that for patients similar to those enrolled in the FUEL trial, udenafil can be started without the requirement for additional monitoring after initial administration.

Patient factors and geographic barriers influencing excess time between paediatric and adult CHD care.

INTRODUCTION: Over 90% of children with CHD survive into adulthood and require lifelong cardiology care. Delays in care predispose patients to cardiac complications. We sought to determine the time interval to accessing adult CHD care beyond what was recommended by the referring paediatric cardiologist (excess time) and determine risk factors for prolonged excess time. MATERIALS AND METHODS: Retrospective cohort study including all patients in the province of Alberta, Canada, age 16-18 years at their last paediatric cardiology visit, with moderate or complex lesions. Excess time between paediatric and adult care was defined as the interval (months) between the final paediatric visit and the first adult visit, minus the recommended interval between these appointments. Patients whose first adult CHD appointment occurred earlier than the recommended interval were assigned an excess time of zero. RESULTS: We included 286 patients (66% male, mean age 17.6 years). Mean excess time was 7.9 ± 15.9 months. Twenty-nine (10%) had an excess time > 24 months. Not having a pacemaker (p = 0.03) and not needing cardiac medications at transfer (p = 0.02) were risk factors for excess time >3 months. Excess time was not influenced by CHD complexity. DISCUSSION: The mean delay to first adult CHD appointment was almost 8 months longer than recommended by referring paediatric cardiologists. Not having a pacemaker and not needing cardiac medication(s) were risk factors for excess time > 3 months. Greater outpatient resources are required to accommodate the growing number of adult CHD survivors.

Evaluation of a Video-Based Education Intervention for Parents of Children Undergoing Fontan Surgery: A Cluster Randomized Controlled Trial.

Background: Fontan surgery is performed at 2-4 years of age and is the third planned surgical intervention for children with a univentricular heart. Major challenges for children and parents after Fontan include (a) psychological distress, (b) prolonged pleural drainage, and (c) the need for postoperative anticoagulation. The aim of this study was to evaluate a pre-Fontan video-based intervention for parents to address these challenges. Methods: This study is a single-centre mixed-methods cluster randomized controlled trial. The intervention consisted of 3 brief whiteboard videos offered online from preadmission clinic to 1 month postoperatively. The parent's State Trait Anxiety Inventory score and the child's Post Hospital Behaviour Questionnaire score were measured 1 week and 1 month postoperatively. Semistructured interviews were conducted to obtain parental feedback on the videos. Results: We enrolled 26 children (13 female patients; 16 intervention group) and 1 parent per child. Mean State Trait Anxiety Inventory scores were similar between groups at both 1 week (52.8 vs 55.5, P = 0.25) and 1 month postoperatively (50.9 vs 53.9, P = 0.25). Post Hospital Behaviour Questionnaire scores were in the maladaptive range but did not differ between groups. Parents agreed or strongly agreed that the videos were helpful but should be provided earlier in the preoperative process. The main value of the videos was recognized as being a method for standardizing information provided to parents. Conclusions: A video-based education intervention did not impact State Trait Anxiety Inventory or Post Hospital Behaviour Questionnaire scores. However, the majority of parents agreed that the videos were helpful.

Contexte: L'opération de Fontan est réalisée à l'âge de 2 à 4 ans et constitue la troisième intervention chirurgicale planifiée chez les enfants qui présentent un ventricule unique. Les enfants et leurs parents font face à des défis importants après une opération de Fontan, dont a) la détresse psychologique, b) le drainage pleural prolongé et c) la nécessité de recourir à une anticoagulothérapie après la chirurgie. Notre étude visait à évaluer une intervention éducative préopératoire sous forme de vidéos présentées aux parents afin de leur permettre de mieux relever ces défis. Méthodologie: Nous avons mené un essai monocentrique à méthodes mixtes et à répartition aléatoire par grappes avec groupe témoin. L'intervention éducative consistait en une série de trois courtes vidéos sur tableau blanc disponibles en ligne à partir de la consultation clinique de préadmission et jusqu'à un mois après la chirurgie. Les scores des parents à l'inventaire d'anxiété situationnelle et de trait d'anxiété (State Trait Anxiety Inventory) et les scores des enfants à l'évaluation du comportement suivant l'hospitalisation (Post Hospital Behaviour Questionnaire) ont été mesurés une semaine et un mois après l'opération. Des entrevues semi-dirigées ont été réalisées afin de recueillir les commentaires des parents au sujet des vidéos. Résultats: Nous avons recruté 26 enfants (dont 13 filles; 16 enfants ont été affectés au groupe d'intervention éducative) et un parent pour chacun des enfants. Les scores moyens obtenus au State Trait Anxiety Inventory étaient comparables entre les deux groupes une semaine (52,8 vs 55,5, p = 0,25) et un mois (50,9 vs 53,9, p = 0,25) après l'opération. Les scores obtenus au Post Hospital Behaviour Questionnaire se situaient dans la fourchette des comportements mésadaptés, mais ne différaient pas entre les groupes. Les parents étaient d'accord ou fortement d'accord pour dire que les vidéos étaient utiles, mais qu'elles auraient dû être offertes plus tôt dans le processus préopératoire. La valeur principale des vidéos était selon eux qu'il s'agissait d'un moyen d'uniformiser l'information transmise aux parents. Conclusions: Une intervention éducative sous forme de vidéos n'a pas eu d'incidence sur les scores obtenus au State Trait Anxiety Inventory ou au Post Hospital Behaviour Questionnaire. Toutefois, la majorité des parents ont trouvé que les vidéos étaient utiles.

Transition readiness of youth with co-occurring chronic health and mental health conditions: A mixed methods study.

BACKGROUND: A large proportion of youth with chronic conditions have mental health comorbidities. However, the effect of these comorbidities on paediatric-adult transition readiness, and the relevance of widely used tools for measuring transition readiness, are unknown. OBJECTIVE: The objectives of this study were to describe and explore the transition readiness of youth with co-occurring chronic health and mental health conditions using a combination of quantitative data obtained from participants completing the Transition Readiness Assessment Questionnaire (TRAQ) and qualitative data. DESIGN AND PARTICIPANTS: A three-phase sequential explanatory mixed methods design was employed, with the qualitative strand taking priority. First, the TRAQ scores (range 1-5) of youth with co-occurring conditions (n = 61) enroled in a multisite randomized controlled trial were measured, followed by qualitative interviews with a sample of youth (n = 9) to explain the quantitative results. Results from both strands were then integrated, yielding comprehensive insights. RESULTS: Median TRAQ scores ranged from 2.86 on the appointment keeping subscale to 5.00 on the talking with providers subscale. The qualitative results uncovered the complexities faced by this group concerning the impact of a mental health comorbidity on transition readiness and self-management skills across TRAQ domains. The integrated findings identified a diverse and highly individualized set of strengths and challenges amongst this group that did not align with overarching patterns as measured by the TRAQ. CONCLUSIONS: This mixed methods study generated novel understandings about how youth with co-occurring conditions develop competencies related to self-care, self-advocacy and self-management in preparation for paediatric-adult service transitions. Results demonstrated the assessment of transition readiness using a generic scale does not address the nuanced and complex needs of youth with co-occurring chronic health and mental health conditions. Our findings suggest tailoring transition readiness practices for this group based on youths' own goals, symptoms, coping mechanisms and resources. PATIENT OR PUBLIC INVOLVEMENT: This study was conducted in collaboration with five young adult research partners (YARP) with lived experience transitioning from paediatric to adult health/mental health services. The YARP's contributions across study phases ensured the perspectives of young people were centred throughout data collection, analysis, interpretation and presentation of findings. All five YARP co-authored this manuscript.

The Fontan Udenafil Exercise Longitudinal Trial: Subgroup Analysis.

The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.5 mg twice daily). In this post hoc analysis, we evaluate whether subgroups within the population experienced a differential effect on exercise performance in response to treatment. The effect of udenafil on exercise was evaluated within subgroups defined by baseline characteristics, including peak oxygen consumption (VO2), serum brain-type natriuretic peptide level, weight, race, gender, and ventricular morphology. Differences among subgroups were evaluated using ANCOVA modeling with fixed factors for treatment arm and subgroup and the interaction between treatment arm and subgroup. Within-subgroup analyses demonstrated trends toward quantitative improvements in peak VO2, work rate at the ventilatory anaerobic threshold (VAT), VO2 at VAT, and ventilatory efficiency (VE/VCO2) for those randomized to udenafil compared to placebo in nearly all subgroups. There was no identified differential response to udenafil based on baseline peak VO2, baseline BNP level, weight, race and ethnicity, gender, or ventricular morphology, although participants in the lowest tertile of baseline peak VO2 trended toward larger improvements. The absence of a differential response across subgroups in response to treatment with udenafil suggests that the treatment benefit may not be restricted to specific sub-populations. Further work is warranted to confirm the potential benefit of udenafil and to evaluate the long-term tolerability and safety of treatment and to determine the impact of udenafil on the development of other morbidities related to the Fontan circulation.Trial Registration NCT0274115.

Just TRAC it! Transitioning Responsibly to Adult Care Using Smart Phone Technology.

PURPOSE: There is little evidence on whether smartphone technology influences transition readiness among adolescents with heart disease. Just TRAC it! is a method of using existing smartphone features (Notes, Calendar, Contacts, Camera) to manage personal health. We evaluated the impact of Just TRAC it! on self-management skills. METHODS: Randomized clinical trial of 16-18 year-olds with heart disease. Participants were randomly allocated 1:1 to either usual care (education session) or intervention (education session including Just TRAC it!). The primary outcome was change in TRANSITION-Q score between baseline, 3 and 6 months. Secondary outcomes were frequency of use and perceived usefulness of Just TRAC it! Analysis was intention-to-treat. RESULTS: We enrolled 68 patients (41% female, mean age 17.3 years), 68% having previous cardiac surgery and 26% had cardiac catheterization. TRANSITION-Q scores were similar at baseline and increased over time in both groups but were not significantly different between groups. Each additional point at the baseline score brought, on average, a 0.7-point increase in TRANSITION-Q score (95% CI 0.5-0.9) at each of 3 and 6 months. The Camera, Calendar and Notes apps were reported as most useful. All intervention participants would recommend Just TRAC it! to others. DISCUSSION: A nurse-led transition teaching with versus without Just TRAC it! improved transition readiness, with no significant difference between groups. Higher baseline TRANSITION-Q scores were associated with greater increase in scores over time. Participants had a positive reception to Just TRAC it! and would recommend it to others. Smartphone technology may be useful in transition education.

"They go hand in hand": a patient-oriented, qualitative descriptive study on the interconnectedness between chronic health and mental health conditions in transition-age youth.

BACKGROUND: Transition-age youth (TAY) with chronic health conditions frequently experience co-occurring mental health conditions. However, little is known about the perspectives of TAY with co-occurring diagnoses preparing to exit pediatric health and mental health services. Research is needed to understand the impact of a mental health condition on transition readiness and self-management in TAY with chronic health conditions. METHODS: TAY (aged 16-20 years) with co-occurring chronic health and mental health conditions were recruited in Alberta, Canada. Nine semi-structured individual interviews were completed by phone or videoconference, and transcribed verbatim. Guided by qualitative description, we analyzed the data using thematic analysis in partnership with five young adults with lived experience in the health/mental health systems. RESULTS: Participants shared their experiences living with simultaneous physical and mental health concerns and preparing for transition to adult care. Our analysis revealed three overarching themes: 1) "they're intertwined": connections between chronic health and mental health conditions in TAY, 2) impact of mental health on transition readiness and self-management, and 3) recommendations for service provision from the perspectives of TAY. CONCLUSIONS: Our findings highlighted the myriad ways in which physical and mental health are connected as TAY prepare for service transitions using specific examples and powerful metaphors. TAY endorsed the importance of providers discussing these connections in routine clinical care. Future research should involve co-designing and evaluating educational material addressing this topic with diverse TAY, caregivers, and service providers.

Patient-reported outcomes in the aging population of adults with congenital heart disease: results from APPROACH-IS.

The congenital heart disease (CHD) population now comprises an increasing number of older persons in their 6th decade of life and beyond. We cross-sectionally evaluated patient-reported outcomes (PROs) in persons with CHD aged 60 years or older, and contrasted these with PROs of younger patients aged 40-59 years and 18-39 years. Adjusted for demographic and medical characteristics, patients ≥60 years had a lower Physical Component Summary, higher Mental Component Summary, and lower anxiety (Hospital Anxiety and Depression Scale-Anxiety) scores than patients in the two younger categories. For satisfaction with life, older persons had a higher score than patients aged 40-59 years. Registration: ClinicalTrials.gov NCT02150603.

Incidence and impact of malnutrition in patients with Fontan physiology.

BACKGROUND: Single-ventricle patients require a series of surgeries, with the final stage being the Fontan. This form of circulation results in several long-term complications, but the impact and consequences of nutrition status remain unclear. We sought to evaluate the incidence of malnutrition in Fontan patients and the impact on outcomes. METHODS: This study was a retrospective cohort study of children who underwent Fontan surgery between 1997 and 2018. Clinical, demographic, and nutrition data were collected, including weight, height, body mass index (BMI), and their respective z scores (z score for weight-for-age [WAZ], z score for height-for-age [HAZ], and z score for BMI-for-age [BMIZ]) pre-Fontan, at discharge, 6 months, and 1, 5, and 10 years post-Fontan. Malnutrition status was categorized using the American Society for Parenteral and Enteral Nutrition guidelines and the Michigan MTool. Fontan failure was defined as listing for heart transplant or death. RESULTS: Of the 69 patients, moderate-severe malnutrition occurred at any time point in 11% (n = 8) by WAZ, 16% (n = 11) by HAZ, and 6% (n = 4) by BMIZ. Moderate-severe malnutrition persisted in 6.5%-12.9% at 10 years post-Fontan. Compared with the pre-Fontan period, there was no change in these parameters over time. There was no statistically significant difference in Fontan failure between degrees of pre-Fontan malnutrition. CONCLUSION: There is a 6%-16% incidence of moderate-severe malnutrition in Fontan patients. Malnutrition is a condition that remains present in follow-up. There was no association with anthropometric parameters and transplant-free survival. A prospective multi-institutional study is needed to understand the impact of malnutrition on long-term outcomes.

Practice Patterns in the Management of Pressure Restrictive Perimembranous Ventricular Septal Defects: A Multinational Survey.

Indications for the closure of pressure restrictive perimembranous ventricular septal defects (pmVSD) are not well established in the pediatric population. We sought to assess practice variability among pediatric cardiologists in the United States (US), Canada, Australia, and New Zealand. A survey ascertaining practice patterns, including case vignettes with incremental progression of disease severity, was designed and administered through representative professional cardiac organizations and email listservs in the designated countries. Among the 299 respondents, 209 (70.0%) were from the US, 65 (21.7%) were from Canada and 25 (8.3%) were from Australia and New Zealand. Indications for pressure restrictive pmVSD closure included the presence of left ventricular (LV) dilation for 81.6% (244/299) (defined as z-score ≥ 2 for 59.0% (144/244) and ≥ 3 for 40.2% (98/244)) and significant pulmonary-systemic flow ratio (QP:QS) for 71.2% (213/299) [defined as ≥ 1.5:1 for 36.2% (77/213) and ≥ 2 for 62% (132/213)]. US pediatric cardiologists elected to close restrictive pmVSD at lower LV z-score and QP:QS ratio cut-offs (p-value 0.0002 and 0.013, respectively). In a case vignette, 63.6% (173/272) chose to intervene if there was right coronary cusp prolapse with stable mild aortic regurgitation. Of the remaining cardiologists, 93% (92/99) intervened if the aortic regurgitation was progressive (from trivial to mild). Commonly identified indications with variable thresholds for closure of pressure restrictive pmVSDs included the presence or progression of LV dilation, significant volume loading, and aortic valve prolapse with regurgitation. US pediatric cardiologists may have a lower threshold for pmVSD closure.

Access to Specialized Care Across the Lifespan in Tetralogy of Fallot.

Individuals living with tetralogy of Fallot require lifelong specialized congenital heart disease care to monitor for and manage potential late complications. However, access to cardiology care remains a challenge for many patients, as does access to mental health services, dental care, obstetrical care, and other specialties required by this population. Inequities in health care access were highlighted by the COVID-19 pandemic and continue to exist. Paradoxically, many social factors influence an individual's need for care, yet inadvertently restrict access to it. These include sex and gender, being a member of a racial or ethnic historically excluded group, lower educational attainment, lower socioeconomic status, living remotely from tertiary care centres, transportation difficulties, inadequate health insurance, occupational instability, and prior experiences with discrimination in the health care setting. These factors may coexist and have compounding effects. In addition, many patients believe that they are cured and unaware of the need for specialized follow-up. For these reasons, lapses in care are common, particularly around the time of transfer from paediatric to adult care. The lack of trained health care professionals for adults with congenital heart disease presents an additional barrier, even in higher income countries. This review summarizes challenges regarding access to multiple domains of specialized care for individuals with tetralogy of Fallot, with a focus on the impact of social determinants of health. Specific recommendations to improve access to care within Canadian and American systems are offered.

Pour les personnes qui vivent avec la tétralogie de Fallot, des soins spécialisés en cardiopathie congénitale (CC) sont nécessaires pour surveiller et prendre en charge toute complication tardive éventuelle. Toutefois, l'accès à des soins en cardiologie demeure difficile pour de nombreux patients, tout comme aux services en santé mentale, aux soins dentaires, aux soins obstétriques et à d'autres soins spécialisés dont cette population a besoin. Des inégalités dans l'accès aux soins de santé ont été mises en lumière lors de la pandémie de COVID-19 et continuent d'exister. Paradoxalement, de nombreux facteurs sociaux agissent sur les besoins d'une personne en matière de soins, et limitent en même temps son accès à ces soins. Le sexe et le genre, l'appartenance à un groupe racial ou ethnique ayant vécu une exclusion par le passé, un niveau de scolarité ou un niveau socio-économique plus faible, l'éloignement géographique des centres de soins tertiaires, les difficultés de déplacement, une protection inadéquate en matière d'assurance de soins de santé, la précarité professionnelle et des expériences antérieures de discrimination en contexte de soins de santé font partie de ces facteurs, qui peuvent coexister et avoir un effet cumulatif. De plus, de nombreux patients croient être guéris et ne sont pas conscients qu'un suivi spécialisé est nécessaire pour eux. Pour ces raisons, les interruptions des soins sont fréquentes, en particulier au moment de la transition entre les soins pédiatriques et les soins destinés aux adultes. La pénurie de professionnels de la santé formés pour intervenir auprès d'adultes présentant une CC est un obstacle supplémentaire, même dans les pays à revenus élevés. Notre article de synthèse résume les obstacles vécus par les personnes vivant avec la tétralogie de Fallot dans l'accès à plusieurs types de soins spécialisés, en portant une attention particulière aux déterminants sociaux de la santé. Des recommandations concrètes pour améliorer l'accès aux soins en contexte canadien et américain sont présentées.

Impact of Udenafil on Echocardiographic Indices of Single Ventricle Size and Function in FUEL Study Participants.

BACKGROUND: The FUEL trial (Fontan Udenafil Exercise Longitudinal) demonstrated statistical improvements in exercise capacity following 6 months of treatment with udenafil (87.5 mg po BID). The effect of udenafil on echocardiographic measures of single ventricle function in this cohort has not been studied. METHODS: The 400 enrolled participants were randomized 1:1 to udenafil or placebo. Protocol echocardiograms were obtained at baseline and 26 weeks after initiation of udenafil/placebo. Linear regression compared change from baseline indices of single ventricle systolic, diastolic and global function, atrioventricular valve regurgitation, and mean Fontan fenestration gradient in the udenafil cohort versus placebo, controlling for ventricular morphology (left ventricle versus right ventricle/other) and baseline value. RESULTS: The udenafil participants (n=191) had significantly improved between baseline and 26 weeks visits compared to placebo participants (n=195) in myocardial performance index (P=0.03, adjusted mean difference [SE] of changes between groups -0.03[0.01]), atrioventricular valve inflow peak E (P=0.009, 3.95 [1.50]), and A velocities (P=0.034, 3.46 [1.62]), and annular Doppler tissue imaging-derived peak e' velocity (P=0.008, 0.60[0.23]). There were no significant differences in change in single ventricle size, systolic function, atrioventricular valve regurgitation severity, or mean fenestration gradient. Participants with a dominant left ventricle had significantly more favorable baseline values of indices of single ventricle size and function (lower volumes and areas, E/e' ratio, systolic:diastolic time and atrioventricular valve regurgitation, and higher annular s' and e' velocity). CONCLUSIONS: FUEL participants who received udenafil demonstrated a statistically significant improvement in some global and diastolic echo indices. Although small, the changes in diastolic function suggest improvement in pulmonary venous return and/or augmented ventricular compliance, which may help explain improved exercise performance in that cohort. REGISTRATION: URL: https://clinicaltrials.gov; Unique Identifier: NCT02741115.

Association between transition readiness and mental health comorbidity in youth with chronic health conditions.

PURPOSE: Between 33 and 59% of youth with chronic health conditions experience mental health conditions. Transition readiness, or the acquisition of knowledge and self-management skills, facilitates successful transition to adult care. Transition readiness among youth with co-occurring chronic health and mental health conditions has not been explored. DESIGN AND METHODS: This study used a sample of 201 patients (aged 16-21) with chronic conditions. All patients completed the Transition Readiness Assessment Questionniare (TRAQ) and were grouped into Cohort A: chronic health conditions only (n = 140), and Cohort B: co-occurring chronic health and mental health conditions (n = 61). A quantile regression at the 50th percentile was conducted to examine associations between TRAQ score and mental health comorbidity, age, gender and immigration status. RESULTS: The median TRAQ score for Cohort A was 3.87 (IQR 0.84) versus 4.00 (IQR 0.87) for Cohort B. Our analysis revealed that having a mental health comorbidity (b = 0.402, p = 0.034), being older in age (b = 0.540, p = 0.004) and being female (b = 0.388, p = 0.001) were associated with higher overall TRAQ score. CONCLUSIONS: The presence of a mental health comorbidity was associated with greater transition readiness as measured by the TRAQ in our sample. Future research should explore why youth with co-occurring chronic health and mental health conditions exhibit greater transition readiness. PRACTICE IMPLICATIONS: Youth with co-occurring chronic health and mental health conditions may develop transition readiness as a result of coping with mental health challenges. Practitioners could invite them to reflect on how their physical and mental health are related and affect their level of preparedness for adult care.