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.

Physical Functioning, Mental Health, and Quality of Life in Different Congenital Heart Defects: Comparative Analysis in 3538 Patients From 15 Countries.

BACKGROUND: We compared physical functioning, mental health, and quality of life (QoL) of patients with different subtypes of congenital heart disease (CHD) in a large international sample and investigated the role of functional class in explaining the variance in outcomes across heart defects. METHODS: In the cross-sectional Assessment of Patterns of Patient-Reported Outcome in Adults with Congenital Heart Disease-International Study (APPROACH-IS), we enrolled 4028 adult patients with CHD from 15 countries. Diagnostic groups with at least 50 patients were included in these analyses, yielding a sample of 3538 patients (median age: 32 years; 52% women). Physical functioning, mental health, and QoL were measured with the SF-12 health status survey, Hospital Anxiety and Depression Scale (HADS), linear analog scale (LAS) and Satisfaction with Life Scale, respectively. Functional class was assessed using the patient-reported New York Heart Association (NYHA) class. Multivariable general linear mixed models were applied to assess the relationship between the type of CHD and patient-reported outcomes, adjusted for patient characteristics, and with country as random effect. RESULTS: Patients with coarctation of the aorta and those with isolated aortic valve disease reported the best physical functioning, mental health, and QoL. Patients with cyanotic heart disease or Eisenmenger syndrome had worst outcomes. The differences were statistically significant, above and beyond other patient characteristics. However, the explained variances were small (0.6% to 4.1%) and decreased further when functional status was added to the models (0.4% to 0.9%). CONCLUSIONS: Some types of CHD predict worse patient-reported outcomes. However, it appears that it is the functional status associated with the heart defect rather than the heart defect itself that shapes the outcomes.

Education as important predictor for successful employment in adults with congenital heart disease worldwide.

BACKGROUND: Conflicting results have been reported regarding employment status and work ability in adults with congenital heart disease (CHD). Since this is an important determinant for quality of life, we assessed this in a large international adult CHD cohort. METHODS: Data from 4028 adults with CHD (53% women) from 15 different countries were collected by a uniform survey in the cross-sectional APPROACH International Study. Predictors for employment and work limitations were studied using general linear mixed models. RESULTS: Median age was 32 years (IQR 25-42) and 94% of patients had at least a high school degree. Overall employment rate was 69%, but varied substantially among countries. Higher education (OR 1.99-3.69) and having a partner (OR 1.72) were associated with more employment; female sex (OR 0.66, worse NYHA functional class (OR 0.67-0.13), and a history of congestive heart failure (OR 0.74) were associated with less employment. Limitations at work were reported in 34% and were associated with female sex (OR 1.36), increasing age (OR 1.03 per year), more severe CHD (OR 1.31-2.10), and a history of congestive heart failure (OR 1.57) or mental disorders (OR 2.26). Only a university degree was associated with fewer limitations at work (OR 0.62). CONCLUSIONS: There are genuine differences in the impact of CHD on employment status in different countries. Although the majority of adult CHD patients are employed, limitations at work are common. Education appears to be the main predictor for successful employment and should therefore be encouraged in patients with CHD.

Patient-reported outcomes in adults with congenital heart disease: Inter-country variation, standard of living and healthcare system factors.

AIMS: Geographical differences in patient-reported outcomes (PROs) of adults with congenital heart disease (ConHD) have been observed, but are poorly understood. We aimed to: (1) investigate inter-country variation in PROs in adults with ConHD; (2) identify patient-related predictors of PROs; and (3) explore standard of living and healthcare system characteristics as predictors of PROs. METHODS AND RESULTS: Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS) was a cross-sectional, observational study, in which 4028 patients from 15 countries in 5 continents were enrolled. Self-report questionnaires were administered: patient-reported health (12-item Short Form Health Survey; EuroQOL-5D Visual Analog Scale); psychological functioning (Hospital Anxiety and Depression Scale); health behaviors (Health Behavior Scale-Congenital Heart Disease) and quality of life (Linear Analog Scale for quality of life; Satisfaction With Life Scale). A composite PRO score was calculated. Standard of living was expressed as Gross Domestic Product per capita and Human Development Index. Healthcare systems were operationalized as the total health expenditure per capita and the overall health system performance. Substantial inter-country variation in PROs was observed, with Switzerland having the highest composite PRO score (81.0) and India the lowest (71.3). Functional class, age, and unemployment status were patient-related factors that independently and consistently predicted PROs. Standard of living and healthcare system characteristics predicted PROs above and beyond patient characteristics. CONCLUSIONS: This international collaboration allowed us to determine that PROs in ConHD vary as a function of patient-related factors as well as the countries in which patients live.

Cost of Congenital Heart Disease Hospitalizations in Canada: A Population-Based Study.

BACKGROUND: The prevalence of congenital heart disease (CHD) is rising, and late complications are common. The impact of these factors on health-care costs is not well understood. We sought to describe inpatient CHD costs in Canada. METHODS: We conducted an observational retrospective cohort study. The Canadian Institute for Health Information (CIHI) Discharge Abstract Database was used for all Canadian provinces, except Quebec, between April 2004 and March 2014. We included hospitalizations with a main diagnosis of CHD (International Classification of Diseases, 10th revision, codes Q20.0-26.9) and hospitalizations having CHD as a secondary diagnosis if the main diagnosis was a comorbid condition related to CHD. CIHI patient cost estimates were used to provide dollar values. Costs were inflated to 2016 Canadian dollars. RESULTS: Among 59,917 hospitalizations, annual CHD costs increased by 21.6% from CAD$99.7 million (95% confidence interval [CI], $89.4-$110.1 million) in 2004 to $121.2 million (95% CI, $112.8-$129.6 million) in 2013 (P < 0.001). Costs were higher for children compared with adults. However, the cost increase was greater in adults (4.5%/y; P < 0.001) than in children (0.7%/y; P = 0.006). Adults accounted for 38.2% of costs in 2004 vs 45.8% in 2013 (P = 0.002). Costs increased most among adults with complex CHD (7.2%/y; P = 0.001). Adult men accounted for greater increases in costs relative to women (P < 0.001). Length of stay was unchanged over time. CONCLUSIONS: Inpatient CHD costs are increasing independent of inflation, particularly among adults with complex lesions. Although children still account for greater inpatient CHD costs, a larger increase was observed among adults. These data are important in allocating inpatient resources for adults with CHD.

Psychosocial Maturity, Autonomy, and Transition Readiness among Young Adults with Congenital Heart Disease or a Heart Transplant.

OBJECTIVE: The population of young adults with congenital heart disease (CHD) or a heart transplant (HTx) is growing rapidly. These survivors require lifelong cardiology care and must assume self-management responsibilities with respect to their health. Accordingly, we sought to assess psychosocial maturity and validity of the Transition Readiness Assessment Questionnaire (TRAQ) in this population. DESIGN: The study was designed as a cross-sectional observational study. SETTING: The study was set at tertiary-care pediatric and adult cardiology clinics in Edmonton and Toronto, Canada. PATIENTS: The patients were 18- to 25-year-olds with moderate or complex CHD or a HTx in childhood. OUTCOME MEASURES: Participants completed validated instruments including the TRAQ, Erickson's Psychosocial Stage Inventory (EPSI), and Kenny's Parental Attachment Questionnaire (PAQ). RESULTS: We enrolled 188 participants (41% women), 109 (58%) having moderate CHD, 64 (34%) complex CHD, and 15 (8%) with a HTx. Mean age for those followed in a pediatric clinic was 21.3 ± 2.3 years, compared with 21.8 ± 2.3 years for those attending an adult clinic (P = 0.20). All questionnaire scores were similar among participants with moderate CHD vs. complex CHD vs. an HTx, and among participants followed in a pediatric clinic vs. an adult clinic. EPSI and PAQ scores were similar to those of healthy populations, though perception of maternal overprotection was common. TRAQ scores increased with age. A response of "not needed for my care" to 5 or more of the 29 TRAQ items was provided by 110 (75%) participants. CONCLUSIONS: Age, but not disease severity should be a factor when considering the transition needs of young adults with heart disease. The TRAQ has important limitations in the adult CHD/HTx population and a cardiac-specific measure of transition readiness is needed.

Healthcare transition for youth with heart disease: a clinical trial.

OBJECTIVES: Adolescents with heart disease have complex health needs and require lifelong cardiology follow-up. Interventions to facilitate paediatric to adult healthcare transition are recommended, although outcomes are unknown. We sought to determine the impact of a transition intervention on improving knowledge and self-management skills among this population. METHODS: We conducted a clinical trial of 15-17 year olds with moderate or complex congenital heart disease (CHD) or cardiomyopathy. Participants were systematically allocated to either usual care (controls) or a 1 h nurse-led one-on-one teaching session about their heart. Allocation was determined by week of attendance in the cardiology clinic. The primary outcome was change in Transition Readiness Assessment Questionnaire (TRAQ) score at 6 months, possible scores ranging from 1 (low) to 5 (optimal). Cardiac knowledge (MyHeart score, range 0-100) was a secondary outcome. Analysis was intention to treat. RESULTS: Of 58 participants (48% female), 52 had CHD and 6 had cardiomyopathy. 27 were allocated to the intervention group; 3 declined the intervention and received usual care. When comparing the intervention group with the usual care group at 6 months postintervention, the mean self-management TRAQ score was 3.59 (±0.83) vs. 3.16 (±1.05), respectively (p=0.048, adjusted for baseline score); the mean self-advocacy TRAQ score was 4.38 (±0.56) vs. 4.01 (±0.95) (p=0.18) and the mean MyHeart score was 75% (±15) vs. 61% (±25) (p=0.019). CONCLUSIONS: A 1 h nurse-led transition intervention resulted in a significant improvement in self-management and cardiac knowledge scores. An educational intervention should be routine for youth with congenital or acquired heart disease. TRIAL REGISTRATION NUMBER: NCT01286480.

Effectiveness of prenatal screening for congenital heart disease: assessment in a jurisdiction with universal access to health care.

BACKGROUND: Neonates with certain forms of severe congenital heart disease (CHD) diagnosed prenatally might have better outcomes in comparison with those diagnosed after birth. The proportion of prenatally detected neonates with severe CHD and the effect of prenatal diagnosis on clinical outcomes have not been previously investigated in Canada. METHODS: We retrospectively studied infants in Alberta, Canada, who required surgical or catheter intervention for CHD at younger than 1 year of age, between January 2007 and December 2010, and pregnancy terminations affected by CHD. RESULTS: Of the 374 subjects identified (327 infants, 47 pregnancies with termination), 188 (50%) were detected prenatally. Failure of prenatal diagnosis was associated with anomalies not involving the 4-chamber view on ultrasound (odds ratio, 1.86; 95% confidence interval, 1.48-2.35; P < 0.001) and region of residence (P = 0.04). Prenatal detection was associated with fewer days to hospital admission (P < 0.001), fewer days to surgery (P = 0.003), and greater use of prostaglandins (P = 0.001). Infants diagnosed prenatally who underwent surgery within 15 days of age had higher preductal O2 saturations (P = 0.04), fewer days to admission (P = 0.03), and less frequently required preoperative intubation (P = 0.004), and inotropes (P = 0.001). Pregnancy termination occurred among 49% of fetuses detected before 24 weeks' gestation. CONCLUSIONS: Only 50% of fetuses and/or neonates with severe CHD managed in Alberta have a prenatal diagnosis. The likelihood of prenatal detection is influenced by the status of the 4-chamber view on ultrasound and the region of maternal residence indicating heterogeneous access to fetal echocardiography within Alberta. Prenatal detection might improve clinical outcomes for neonates with severe CHD.

Fast-track extubation after modified Fontan procedure.

OBJECTIVE: In 2007, we introduced a policy to plan to extubate all patients after a modified Fontan procedure in the operating room. Our objective was to review the feasibility, safety, and clinical outcomes of this approach. METHODS: Patients who underwent a modified Fontan operation between May 2004 and May 2010 were reviewed. RESULTS: Ninety-seven patients underwent a modified Fontan operation (mean age, 3.9 ± 2.2 years; mean weight, 15.1 ± 5.0 kg); 46 patients (47%) were extubated in the operating room (group A). Nineteen patients were extubated in the intensive care unit within 24 hours (group B), and 32 patients had delayed extubation (group C). The 3 groups were not significantly different with respect to preoperative characteristics. Twenty-four hours postoperatively, group A had a lower mean central venous pressure compared with patients in group B or C (13 vs 14 vs 17 mm Hg, respectively, P < .001); a higher base excess (0.4 vs -1.3 vs -3.4, P < .001); a lower fluid balance (234 vs 514 vs 730 mL, P < .001); and a lower inotrope score (4.6 vs 6.7 vs 10.8, P < .001). Group C had a longer median intensive care unit length of stay (2 vs 3 vs 6 nights, P = .01), kept their chest tubes longer (8 vs 9 vs 15 days, P = .001), and had a longer median hospital length of stay (9 vs 11 vs 21 days, P = .001). CONCLUSIONS: Extubation in the operating room after a modified Fontan procedure seems feasible. This approach is associated with improved early postoperative hemodynamics, earlier time to chest tube removal, and shorter intensive care unit and hospital lengths of stay.