Moderate Congenital Heart Disease Research Articles

Abstract 12062: Additional Burden of Esophageal and Gastric Varices on Hospitalized Patients With Moderate or Severe Congenital Heart Disease

Introduction: Elevated central venous pressure (CVP) occurs in many forms of congenital heart disease (CHD) which may result in esophageal or gastric varices. We sought to describe the incidence of varices and acute outcomes for hospital admissions with moderate or severe CHD compared to those without CHD. Methods: Vizient Clinical Database was queried from 10/2018-12/2021 for admissions aged 1-89 years with ICD-10 codes for moderate or severe CHD with and without ICD-10 codes for esophageal (I85-I85.11) or gastric (I86.4) varices. For comparison (NO-CHD), admissions with no CHD codes with and without varices were queried. Data included demographics, hospital outcomes, principal procedure and costs. Results: There were 54,979 CHD admissions, 489 (0.9%) with varices and 28,282,750 NO-CHD admissions, 231,633 (0.8%) with varices ( p = 0.069); summary in Table 1. Compared to the NO-CHD population, admission with CHD and varices were younger, had longer lengths of stay, higher complication rates and hospital costs with no difference in in-hospital mortality. Table 2 shows the top 5 principal procedures for each group. Conclusions: Hospitalized patients with moderate or severe CHD and varices have worse acute hospital outcomes and higher costs compared to those without CHD. These findings suggest a potential additional disease burden in this already fragile population. Future work to identify and potentially treat modifiable factors could improve care for these patients.

Abstract 11061: Incidence of Cancer in Hospitalized Adults With Congenital Heart Disease

Introduction: Many patients with congenital heart disease (CHD) are repeatedly exposed to low-dose ionizing radiation for diagnosis, treatment and long-term management of their care. Hypothesis: We hypothesized that these repeated exposures put CHD patients at higher risk for radiation-associated cancers (RAC). Methods: Review of Vizient (a national, administrative database) from 12/2018 - 12/2021 for admissions aged 18-85 years with ICD-10 codes for moderate or severe CHD and RAC (bladder, breast, colon, esophageal, leukemia/myeloma, liver, lung, ovarian and stomach). Demographics, hospital outcomes and costs were compared to admissions with RAC and no CHD (NO-CHD). Results: Out of 34,793 CHD admissions, 6.4% had RAC, and out of 24,999,735 NO-CHD admissions, 11.2% had RAC (p<0.001; Table 1). CHD with RAC were younger, had longer length of stay, higher complications, in-hospital mortality and costs. Comparisons for each RAC type are in Table 2. There were higher rates of breast, liver and lung cancer in CHD (p<0.05 for all) and higher costs for all CHD RAC except esophageal. Conclusions: This national database review shows increased incidence of some types of RAC (lung, liver and breast) in adults with CHD, with worse hospital outcomes and higher costs for several cancer types. While frequent radiation in the CHD population may contribute to these findings, it will be important to explore further. These findings also highlight the importance of adherence to the “as low as reasonably achievable” (ALARA) principle in the care of children and adults with CHD and stresses the importance of imaging modalities that do not use ionizing radiation.

Abstract 15588: Effect of Covid-19 on the Mortality of Patients With Congenital Heart Disease

Introduction: Cardiovascular conditions have been recognized as risk factors for poor outcomes in association with COVID-19. However, the effect of the COVID-19 pandemic on different severity types of congenital heart disease (CHD) patients remains unknown. This study aims to examine whether patients with history of interventions for CHD experienced a higher mortality risk during the COVID-19 pandemic. Methods: This is a retrospective cohort study using data from the Pediatric Cardiac Care Consortium (PCCC). We included patients having US residence and direct identifiers, who were enrolled between 1982 and 2003 after undergoing transcatheter or surgical interventions for CHD. The observation window (2017-2020) was divided to a pre-SARSV2 and SARSV2 exposure period defined around the state specific onset of COVID-19 disease in 2020. Patients were categorized as having mild, moderate, severe-2 ventricle (2V) or single ventricle (SV) CHD. Standardized mortality ratios (SMRs) were used to describe the observed risk of mortality in CHD patients compared to the age- and sex-adjusted rates from the general U.S. population. Multivariable analysis was conducted using time-varying covariate Cox proportional hazards model with adjustment for age, sex and severity of CHD. Results: A total of 50,138 patients with history of interventions for CHD were included. Pooled pre-SARSV2 SMR was 2.38 [95% CI: 2.06, 2.75], while the SARSV2-exposure SMR was 2.07 [95% CI: 1.78, 2.35] (not significantly different). No significant differences in pooled SMRs were found by severity of CHD. The adjusted hazard risk for all-cause mortality in the SARSV2-exposure vs pre-SARSV2 period did not increase for patients with CHD (aHR: 0.59, 95%CI:0.19 - 1.84), and we did not find any differences in risk by severity subgroups or sex of patients. Conclusion: In this large cohort study monitoring long term outcomes after interventions for CHD, we could not detect any evidence of increased risk for all-cause mortality among this population during the early part of the COVID-19 pandemic. Additional data will be required over longer period to fully understand the impact of COVID-19 on adults with repaired CHD.

Acute Hospital Outcomes for Renal Transplantation in Patients With Moderate or Severe Congenital Heart Disease

Children and adults with congenital heart disease (CHD) are increasingly recognized to be at risk for acute and chronic renal injury. Some of these may progress to the need for renal transplantation. We hypothesized that patients with underlying moderate or severe CHD who undergo renal transplantation will have worse acute hospital outcomes. Using a national administrative database, we queried for admissions aged 0 to 50 years with moderate or severe CHD and renal transplantation and compared these to admissions without CHD. There were 56 admissions for renal transplantation in the CHD group (0.04%) and 26,285 admissions in the group without CHD (0.21%, p<0.001). The CHD group were younger, had a higher proportion of Whites, longer length of stay, higher complication rates, higher in-hospital mortality, and higher costs. In conclusion, although renal transplantation is still relatively uncommon in the CHD population, there is an increasing recognition of severe chronic renal disease in the setting of CHD, making it important to understand the potential implications of these findings.

End-of-life and palliative care provision to adults with congenital heart disease: mortality follow-back study using administrative data

Abstract Background Although many adults with congenital heart disease (CHD) still die prematurely, end-of-life care for these patients receives limited attention. There are indications that current care provision at the end of life is burdensome, expensive, and not in line with patients' needs and preferences. We sought to analyse end-of-life care in adult CHD patients to determine whether health services need to be optimized. Purpose This study aimed to describe patterns of healthcare consumption of adults with CHD who died in the last year of life. Methods This retrospective mortality follow-back study used data of the BELgian COngenital heart disease Database combining Administrative and Clinical data (BELCODAC), including individually linked healthcare claims, death certificates and clinical data from adults with CHD in Flanders (Belgium). For this study, adults with CHD who died between 2007 and 2016 from any cause except sudden death, accident or violence, were selected for inclusion. Accidental, violent, and sudden deaths were identified based on causes of death and healthcare use in the last 3 months of life. Healthcare consumption was based on nomenclature codes derived from healthcare claims data. Results A total of 327 eligible patients (median age: 58 y; 54% women; 43% mild CHD; 45% moderate CHD; 11% complex CHD; 49% cardiovascular cause of death) were identified. During the last year of life, healthcare use increased substantially (Fig. 1). During the last month of life, 54% of patients were hospitalised, 55% visited the emergency department, and 15% were admitted to an intensive care unit at least once (Fig. 2). A total of 8% and 5% of patients underwent heart surgery or catherization in the last month of life, respectively. Furthermore, 70% of patients had at least one encounter with a general practitioner and 11% with a CHD specialist in the last month of life. Specialist palliative care was provided to 13% of patients in the last month of life. When looking at the subgroup of patients with CHD that died due to a cardiovascular cause, proportions of patients that were hospitalised or had visits at the emergency department or intensive care unit in the last month of life were similar (Fig. 2). However, these patients underwent more heart surgeries (11%) and catherizations (8%), had more encounters with CHD specialists (15%), and received remarkably less specialized palliative care (4%) in the last month of life. Conclusion Resource utilization increased substantially during the last year of life, resulting in high acute healthcare consumption in the last month of life. It is remarkable that only a minority of patients received palliative care, especially when looking at patients who died due to a cardiovascular cause. Our findings motivate the need to assess if and how end-of-life is planned for adults with CHD. Future studies using qualitative analyses and survey methodology are needed to optimize the management of end-of-life care. Funding Acknowledgement Type of funding sources: Public grant(s) – EU funding. Main funding source(s): Research Foundation Flanders, European Society of Cardiology, Koning Boudewijnstichting, National Foundation on Research in Pediatric Cardiology, Swedish Research Council for Health, Working Life and Welfare-FORTE

Impact of Socioeconomic Status and Residence Distance on Infant Heart Disease Outcomes in Canada.

Background Socioeconomic status (SES) impacts clinical outcomes associated with severe congenital heart disease (sCHD). We examined the impact of SES and remoteness of residence (RoR) on congenital heart disease (CHD) outcomes in Canada, a jurisdiction with universal health insurance. Methods and Results All infants born in Canada (excluding Quebec) from 2008 to 2018 and hospitalized with CHD requiring intervention in the first year were identified. Neighborhood level SES income quintiles were calculated, and RoR was categorized as residing <100 km, 100 to 299 km, or >300 km from the closest of 7 cardiac surgical programs. In-hospital mortality at <1 year was the primary outcome, adjusted for preterm birth, low birth weight, and extracardiac pathology. Among 7711 infants, 4485 (58.2%) had moderate CHD (mCHD) and 3226 (41.8%) had sCHD. Overall mortality rate was 10.5%, with higher rates in sCHD than mCHD (13.3% versus 8.5%, respectively). More CHD infants were in the lowest compared with the highest SES category (27.1% versus 15.0%, respectively). The distribution of CHD across RoR categories was 52.3%, 21.3%, and 26.4% for <100 km, 100 to 299 km, and >300 km, respectively. Although SES and RoR had no impact on sCHD mortality, infants with mCHD living >300 km had a higher risk of mortality relative to those living <100 km (adjusted odds ratio [aOR], 1.43 [95% CI, 1.11-1.84]). Infants with mCHD within the lowest SES quintile and living farthest away had the highest risk for mortality (aOR, 1.74 [95% CI, 1.08-2.81]). Conclusions In Canada, neither RoR nor SES had an impact on outcomes of infants with sCHD. Greater RoR, however, may contribute to higher risk of mortality among infants with mCHD.

Dyslipidemia Among Adults With Congenital Heart Disease

BackgroundAtherosclerotic disease is an important cause of morbidity among adults with congenital heart disease (CHD). Prevalence of dyslipidemia in this group is poorly described. ObjectivesThis study aimed to describe the prevalence of dyslipidemia among adults with CHD. MethodsA prospective, outpatient screening study was conducted among adults aged ≥18 years at 4 New England ambulatory congenital cardiology centers. Participants were surveyed regarding cardiovascular risk factors. Nonfasting fingerstick samples were obtained for analysis using a point-of-care lipid analyzer. ResultsLipid screening was completed on 186 participants (median age 30 [range 18-71] years, 50% female). Eighteen (10%) had simple CHD anatomy, and 63 (34%) had complex anatomy. Only 15% of 169 respondents reported history of high cholesterol. Eighty-five (46%) participants met National Cholesterol Education Program definition of dyslipidemia with 60 (32%), 62 (34%), and 37 (20%) having low high-density lipoprotein cholesterol (HDL-C <40 mg/dL), high non–HDL-C (≥130 mg/dL), and high total cholesterol (TC ≥200 mg/dL), respectively. TC was higher among participants with simple CHD than among those with moderate and complex lesions (mean 178.4 ± 48.7 vs 170.1 ± 35.0 vs 157.6 ± 34.5 mg/dL; P = 0.03). HDL-C was lower among participants with complex CHD than among those with simple and moderate lesions (mean 44.1 ± 13.5 vs 46.9 ± 12.5 vs 49.8 ± 15.3 mg/dL; P = 0.05). ConclusionsDyslipidemia is highly prevalent among our cohort of adults with CHD, despite <15% reporting a prior diagnosis. Low HDL-C was more common in complex CHD, and high TC was more common in simple or moderate CHD. Lipid screening should be part of preventive health maintenance for all adults with CHD.

Transfer of congenital heart patients from paediatric to adult services in England

ObjectiveThis study assessed the transfer of patients from paediatric cardiac to adult congenital heart disease (ACHD) services in England and the factors impacting on this process.MethodsThis retrospective cohort study used...

Congenital Heart Disease: Growth Evaluation and Sport Activity in a Paediatric Population.

(1) Objective: To evaluate: (i) the associations of age and disease severity with anthropometric indices and weight status, (ii) the difference in the frequency of sports activity among different levels of disease severity in paediatric patients with congenital heart disease (CHD). (2) Methods: Clinical data of Caucasian children (aged 2–18 years) diagnosed with CHD (2005–2018) were retrospectively collected from the electronic register of outpatient visits. Of the 475 children with CHD, 368 children and their 1690 complete anthropometric measurements were eligible for inclusion in our analysis. (3) Results: Significant increase with age was observed for weight z-score [beta (95%CI): 0.03 (0.02, 0.05) for one-unit of age] and BMI z-score [0.06 (0.03, 0.08)] but not for height z-score. The probability of being underweight and overweight/obese increased and decreased with disease severity, respectively. The obesity probability of patients with mild CHD (0.06 [95%CI: 0.03, 0.08]) was not statistically distinguishable from that of patients with moderate CHD (0.03 [95%CI: 0.02, 0.05]), whereas it was lower in patients with severe CHD (0.004 [95%CI: 0.0, 0.009]). No obese patients with a univentricular heart defect were observed. Days spent in sport activities were equal to 1.9 [95%CI: 1.6, 2.2] days/week, 1.9 [1.5, 2.2], 1.4 [1.1, 1.7] and 0.7 [0.1, 1.3] in patients with mild, moderate, severe and univentricular CHD, respectively. (4) Conclusions: The risk of being overweight and obese should not be underestimated in paediatric patients diagnosed with CHD, especially in children with mild or moderate heart defects. It could be prevented or reduced by promoting a healthy lifestyle.

Repeat radiofrequency catheter ablation of atrial tachycardias in patients with congenital heart disease.

Atrial tachycardias (AT) in patients with congenital heart disease (CHD) are significantly contributing to morbidity and mortality. Aim of this study was to evaluate the long-term course of CHD patients requiring repeat ablation procedures (RAP) of AT. All 144 patients with CHD who had undergone ablation of AT at our center between January 2003 and October 2018 were enrolled. Patients were classified according to the complexity of CHD: complex CHD (cCHD), moderate CHD (mCHD), and simple CHD (sCHD). A total of 101 RAP were performed in 64 patients. One RAP was performed in n = 40, two in n = 13, three in n = 10, and five in n = 1. Acute success rate was 82% (83/101) and was not associated with the complexity of CHD (p = 1.0). Number of procedures was lower in patients with sCHD than in patients with mCHD and cCHD (sCHD 1.3 ± 0.6, mCHD 1.8 ± 1.0, and cCHD 1.8 ± 1.1, p = .04). RAP were most frequent in patients after Fontan palliation or Atrial switch procedure (2.0 ± 1.1 [n = 41] vs. 1.6 ± 0.9 all others, p = .016) and in patients with multiple unstable AT's (2.5 ± 1.1 [n = 11] vs. 1.7 ± 1.0, p = .008). Major complications occurred in 4/101 procedures. Complete follow-up was available in 125 patients. Since last RAP 73% of the patients were in sinus/atrial rhythm and 34/125 patients (27%) with AT recurrence did not require re-ablation with mean follow-up of 52 ± 40 months. Recurrences after ablation of AT in CHD patients were frequent. After RAP promising long-term results could be achieved. Data encourage repetitive ablation procedures in this patient population.

Hospital discharge codes and substantial underreporting of congenital heart disease

BackgroundHospital discharge codes are relied upon for research, accounting/invoicing and health systems planning. Congenital heart disease (CHD), however, is uniquely difficult for non-cardiologists to code due to the rarity, variety and complexity of lesions. It is therefore important that the accuracy of hospital discharge codes is regularly checked to ensure that the prevalence and burden of CHD is being correctly estimated and recorded. Methods and resultsWe identified all inpatient admissions of adults with CHD to Royal Prince Alfred Hospital in Sydney, Australia from January 2018 to March 2021 (257 admissions, 106 unique patients). The associated discharge coding summaries were extracted and compared to the codes in the separately collected and audited Adult CHD database. Only a quarter of discharge coding summaries contained any diagnosis of CHD, and just one-tenth accurately recorded all appropriate CHD diagnoses. Patients with simple lesions were most likely to have a coded diagnosis of CHD, while those with moderate and complex lesions were much less likely. Moreover, patients admitted under a cardiovascular specialty were twice as likely to have a coded diagnosis of CHD, compared with those admitted under non-cardiovascular specialties (p ​= ​0.006). Overall, less than half of patients had any hospital-coded diagnosis of CHD in any admission over the three-year study period. ConclusionsHospital discharge coding dramatically underreports CHD, especially for patients with moderate and severe CHD lesions and for admissions under non-cardiovascular specialties. This suggests that discharge coding-based estimates of the burden of CHD on hospitals and health systems may be substantially underestimated.

Transition readiness in congenital heart disease: Are teens and young adults getting the recommended information?

BackgroundThe 2011 American Heart Association best practices statement on transition for young adults with congenital heart disease (CHD) addresses both medical transfer of care and transition to independent adulthood. The statement specifically includes information regarding both medical and lifestyle issues. The aim of this study is to evaluate medical and lifestyle-related areas of knowledge of teens with CHD to help identify targets for potential intervention. MethodsMulticenter cohort study using a validated transition readiness assessment questionnaire, divided into medical, lifestyle and, for females, pregnancy/family planning knowledge. Results were reported as knowledge deficit, or percentage of items that respondents indicated they did not know. Results: 401 patients were included across four U.S. tertiary care centers (median age 18.7 years, range 14–25). Average perceived medical knowledge deficit was 19%, lifestyle knowledge deficit was 19%, and pregnancy/family planning knowledge deficit was 53%. Despite similar combined scores, females outperformed males in medical knowledge (P ​= ​0.03), while males outperformed females in lifestyle knowledge (P ​< ​0.001). Moderate CHD complexity patients scored highest in medical knowledge, while simple CHD scored lowest (P ​= ​0.01), and CHD complexity did not affect lifestyle scores. Taking cardiac medications or having an IEP, had no effect. ConclusionTeens and young adults with CHD exhibit similar overall knowledge between medical and lifestyle factors, but differences exist between males and females in each subcategory. Efforts to improve patient knowledge are needed, particularly for those with simple and complex CHD, and improving information regarding pregnancy and family planning.

Length of hospital stay after delivery among Danish women with congenital heart disease: a register-based cohort study

BackgroundThe literature about the impact of congenital heart disease (CHD) on the length of hospital stay after delivery is limited, and nonexisting in a country with free and equal access to healthcare. We aimed to examine the hypothesis that Danish women with CHD have a longer hospital stay after delivery compared to women without CHD. Secondarily, we aimed to examine the hypothesis that cesarean section modifies the association.MethodsThe study was a national cohort study using Danish nationwide registers in 1997–2014. Maternal CHD was categorized as simple, moderate, or complex CHD. The comparison group consisted of women without CHD. Outcome of interest was length of hospital stay after delivery registered in complete days. Mode of delivery was categorized as cesarean section or vaginal delivery. Data was analyzed using a generalized linear model with a Poisson distribution.ResultsWe included 939,678 births among 551,119 women. Women without CHD were on average admitted to the hospital for 3.6 (SD 3.7) days, whereas women with simple, moderate, and complex CHD were admitted for 3.9 (SD 4.4), 4.0 (SD 3.8) and 5.1 (SD 6.7) days, respectively. The adjusted length of hospital stay after delivery was 12% (relative ratio (RR) = 1.12, 95% confidence interval (CI) 1.07–1.18), 14% (RR = 1.14, 95% CI: 1.07–1.21), and 45% (RR = 1.45, 95% CI: 1.24–1.70) longer among women with simple, moderate, and complex CHD, respectively, compared to women without CHD. The association between maternal CHD and length of hospital stay was not modified by mode of delivery (p-value of interaction = 0.62). Women who gave birth by cesarean section were on average admitted to the hospital for 2.7 days longer compared to women with vaginal delivery.ConclusionThe hospital stay after delivery was significantly longer among women with CHD as compared to women without CHD. Further, higher complexity of CHD was associated with longer length of stay. Cesarean section did not modify the association.

PROTEA, A Southern African Multicenter Congenital Heart Disease Registry and Biorepository: Rationale, Design, and Initial Results.

Objectives: The PartneRships in cOngeniTal hEart disease (PROTEA) project aims to establish a densely phenotyped and genotyped Congenital Heart Disease (CHD) cohort for southern Africa. This will facilitate research into the epidemiology and genetic determinants of CHD in the region. This paper introduces the PROTEA project, characterizes its initial cohort, from the Western Cape Province of South Africa, and compares the proportion or “cohort-prevalences” of CHD-subtypes with international findings.Methods: PROTEA is a prospective multicenter CHD registry and biorepository. The initial cohort was recruited from seven hospitals in the Western Cape Province of South Africa from 1 April 2017 to 31 March 2019. All patients with structural CHD were eligible for inclusion. Descriptive data for the preliminary cohort are presented. In addition, cohort-prevalences (i.e., the proportion of patients within the cohort with a specific CHD-subtype) of 26 CHD-subtypes in PROTEA's pediatric cohort were compared with the cohort-prevalences of CHD-subtypes in two global birth-prevalence studies.Results: The study enrolled 1,473 participants over 2 years, median age was 1.9 (IQR 0.4–7.1) years. Predominant subtypes included ventricular septal defect (VSD) (339, 20%), atrial septal defect (ASD) (174, 11%), patent ductus arteriosus (185, 11%), atrioventricular septal defect (AVSD) (124, 7%), and tetralogy of Fallot (121, 7%). VSDs were 1.8 (95% CI, 1.6–2.0) times and ASDs 1.4 (95% CI, 1.2–1.6) times more common in global prevalence estimates than in PROTEA's pediatric cohort. AVSDs were 2.1 (95% CI, 1.7–2.5) times more common in PROTEA and pulmonary stenosis and double outlet right ventricle were also significantly more common compared to global estimates. Median maternal age at delivery was 28 (IQR 23–34) years. Eighty-two percent (347/425) of mothers used no pre-conception supplementation and 42% (105/250) used no first trimester supplements.Conclusions: The cohort-prevalence of certain mild CHD subtypes is lower than for international estimates and the cohort-prevalence of certain severe subtypes is higher. PROTEA is not a prevalence study, and these inconsistencies are unlikely the result of true differences in prevalence. However, these findings may indicate under-diagnosis of mild to moderate CHD and differences in CHD management and outcomes. This reemphasizes the need for robust CHD epidemiological research in the region.

Longitudinal Trends in Body Mass Index for Adults with Congenital Heart Disease.

Obesity is a modifiable, independent risk factor for adverse long-term outcomes in children and adults. Our objective was to determine the prevalence of overweight and obese status in a cohort of adults with congenital heart disease (CHD) as well as to assess longitudinal trends over a 20-year period. The study group consisted of patients 18years of age and older followed at our adult CHD clinic. Body mass index (BMI) data were collected from our index period, consisting of patient encounters from 2009 to 2012 (Period 2), as well as during 2000-2003 (Period 1) and 2017-2020 (Period 3) when available. The study cohort was subdivided into three groups per published guidelines: simple, moderate, and greater CHD complexity. The prevalence of obesity and overweight status was compared among the different groups as well as with published data (NHANES). Our cohort in Period 2 consisted of 261 subjects. The median age (25-75% interquartile range) for Period 2 was 27.6 (21.1-35.9) years and BMI was 25.2 (21.7-30.0) kg/m2 with 8.0% underweight, 40.0% with normal weight, 27.0% overweight, and 25% obese. 95 patients had follow-up data from each time period, with 96% of patients having moderate or greater complexity of CHD. The combined percentage of overweight and obese patients for the moderate and greater complex CHD groups increased from 42 and 37% in period 1 to 60% and 65% in period 3, respectively. The percentage of obese patients with moderate and greater CHD complexity increased by 250% and 55%, respectively, from Period 1 to 3. Our study cohort had a high prevalence of overweight and obese weight status. Given adults with CHD have high baseline cardiovascular morbidity, the presence of obesity can increase their risk for poor outcomes, highlighting the need for prevention of this modifiable risk factor.

Patient empowerment: a way to improve the communication between young persons and healthcare providers

Abstract Background Through patient empowerment, it is possible to support persons with congenital heart disease (CHD) be autonomous, become actively involved in care and gain higher knowledge about their condition, treatment and/or lifestyle recommendations. Conceptual models suggest that patient empowerment is associated with other patient-reported outcomes (PROs). However, available evidence has not concluded whether patient empowerment is leading to improvements in other PROs or viceversa. Purpose The present study aimed to examine the direction of effects between patient empowerment and PROs in young persons with CHD. Methods As part of the STEPSTONES-CHD trial, adolescents with CHD from seven pediatric cardiology centers in Sweden were included in a longitudinal observational study (n=132). Data were collected when patients were 16 (T0) and 17 years old (T1). The Gothenburg Young Persons Empowerment Scale (GYPES) was used to measure patient empowerment on a scale from 15–75, which higher scores denoting a higher level of patient empowerment. Cross-lagged analyses between patient empowerment and PROs were undertaken. These analyses provide information related to the predominant direction of effects over time by comparing standardized betas. The model includes three types of relations, within time relations, which are the correlations between patient empowerment and variable X at T0 and T1. Autoregressive relations, which represent the prediction of patient empowerment or variable X at T1 by their respective value at T0. Cross-lagged is the last type of relation and it is the prediction of variable X at T1 by patient empowerment at T0 or the prediction of patient empowerment at T1 by variable X at T0. The model was replicated four times and the PROs included in the analyses were quality of life (linear analog scale), patient-reported sale (PedsQL generic 4.0), communicating with the healthcare provider (PedsQL cardiac 4.0) and transition readiness (RTQ). Results The sample comprised of 60.6% (n=80) boys. Overall, 13.2% (n=17) had a mild CHD, 62.0% (n=80) a moderate CHD and 24.8% (n=32) a complex CHD. The mean empowerment score was 52.6 (±10.7) at T0 and 53.2 (±11.7) at T1. We found a significant cross-lagged effect between patient empowerment and communication skills (β=0.28), signifying that a higher level of patient empowerment predicted a higher level of communication skills (see Figure 1). No other cross-lagged relationships were found. Conclusions Clinicians interested in improving the communication skills of their patients, may consider whether they can achieve this by targeting patient empowerment. Patient empowerment gives patients the tools they need in order to feel comfortable asking questions and expressing their needs in the care process. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): Swedish Heart-Lung Foundation;Swedish Research Council for Health Working Life andWelfare;Swedish Children Heart Association;Swedish Research Council

JUST TRAC IT! TRANSITIONING RESPONSIBLY TO ADULT CARE USING SMART PHONE TECHNOLOGY

BACKGROUND Many adolescents living with heart disease face challenges when transitioning from pediatric to adult care. There is little evidence on how use of smartphone technology influences transition readiness. Just TRAC it! is a method of using existing features on a smartphone, including the Notes, Calendar, and Contacts apps, as a way for adolescents and adults to manage their health. We aimed to investigate the impact of nurse-led teaching with vs. without Just TRAC it! on self-management skills in this population. METHODS AND RESULTS We conducted a randomized clinical trial of 16-18 year olds with congenital heart disease (CHD) or acquired heart disease requiring surgery. Participants were randomly allocated 1:1 to either usual care (control; 1-hour education session) or the smartphone intervention (1-hour 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! at 3 and 6 months post intervention. Analysis was intention to treat. We enrolled 68 patients (41% female, mean age 17.3 years). Cardiac diagnosis was simple CHD (34%), moderate CHD (49%), complex CHD (13%), and acquired heart disease (4%). Sixty-eight percent had previous cardiac surgery and 26% had a previous cardiac catheterization. TRANSITION-Q scores were similar at baseline and increased over time in both groups (Figure) but were not significantly different between groups at 6 months. On average, TRANSITION-Q score at 6 months were 0.9 points higher than at 3 months (95% CI 0.1-1.7). 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) each at 3 months and at 6 months. Perceived usefulness of Just TRAC it! is summarized (Table). Ninety-seven percent of participants indicated they would recommend Just TRAC it! to others at 3 months and 100% at 6 months. CONCLUSION Nurse-led transition teaching with vs. without Just TRAC it! improved transition readiness based on TRANSITION-Q scores over time, with no significant difference between groups. The majority of participants found Just TRAC it! useful, with the camera, calendar and notes apps reported as the most useful. Participants had a positive reception to Just TRAC it! and would recommend it to others. Longer follow-up is needed to assess how smartphone technology is used by young adults to facilitate their entry into the adult healthcare system. Many adolescents living with heart disease face challenges when transitioning from pediatric to adult care. There is little evidence on how use of smartphone technology influences transition readiness. Just TRAC it! is a method of using existing features on a smartphone, including the Notes, Calendar, and Contacts apps, as a way for adolescents and adults to manage their health. We aimed to investigate the impact of nurse-led teaching with vs. without Just TRAC it! on self-management skills in this population. We conducted a randomized clinical trial of 16-18 year olds with congenital heart disease (CHD) or acquired heart disease requiring surgery. Participants were randomly allocated 1:1 to either usual care (control; 1-hour education session) or the smartphone intervention (1-hour 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! at 3 and 6 months post intervention. Analysis was intention to treat. We enrolled 68 patients (41% female, mean age 17.3 years). Cardiac diagnosis was simple CHD (34%), moderate CHD (49%), complex CHD (13%), and acquired heart disease (4%). Sixty-eight percent had previous cardiac surgery and 26% had a previous cardiac catheterization. TRANSITION-Q scores were similar at baseline and increased over time in both groups (Figure) but were not significantly different between groups at 6 months. On average, TRANSITION-Q score at 6 months were 0.9 points higher than at 3 months (95% CI 0.1-1.7). 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) each at 3 months and at 6 months. Perceived usefulness of Just TRAC it! is summarized (Table). Ninety-seven percent of participants indicated they would recommend Just TRAC it! to others at 3 months and 100% at 6 months. Nurse-led transition teaching with vs. without Just TRAC it! improved transition readiness based on TRANSITION-Q scores over time, with no significant difference between groups. The majority of participants found Just TRAC it! useful, with the camera, calendar and notes apps reported as the most useful. Participants had a positive reception to Just TRAC it! and would recommend it to others. Longer follow-up is needed to assess how smartphone technology is used by young adults to facilitate their entry into the adult healthcare system.

ASSOCIATION BETWEEN CLUSTERING OF LIFESTYLE RISK BEHAVIOURS AND RISK OF BEING OVERWEIGHT OR OBESE AMONG CHILDREN AND ADOLESCENTS WITH CONGENITAL HEART DISEASE ENROLLED IN A NOVEL PEDIATRIC EXERCISE MEDICINE PROGRAM

<h3>BACKGROUND</h3> Children and adolescents with congenital heart disease (CHD) are at a higher risk of being overweight or obese. Lifestyle risk behaviours are prevalent in this population and limited information is known about how the clustering of unhealthy lifestyle behaviours impact patients' weight status. Objective: To determine the association between unhealthy lifestyle behaviours and risk of being overweight or obese in pediatric patients with CHD. <h3>METHODS AND RESULTS</h3> We performed a cross-sectional analysis of children and adolescents (ages 8-17 years) referred from outpatient cardiology clinics who successfully enrolled in the Exercise Medicine (EM) Program at the Hospital for Sick Children between January 2018 – February 2020. Patients' demographic characteristics, medical history, anthropometry, and lifestyle behaviour data were retrospectively collected. CHD severity status was classified as simple, moderate, and severe based on the Bethesda Classification. The exposure variable was clustering of lifestyle risk behaviours (physical inactivity, excessive screen time, unhealthy dietary intake, and poor sleep hygiene). Participants were categorized into two groups: lesser clustering (one or two lifestyle risk behaviours) or greater clustering (three or four lifestyle risk behaviours). The outcome variable was body mass index z-score (zBMI) and patients were categorized into either normal body weight (-2 < zBMI < 2) or overweight/obese (>2 zBMI). Final multivariable logistic model was adjusted for age, sex, and CHD severity. There were 53 CHD patients included in the analysis. The mean age was 13±3 years, 27 (49%) were male, 19 (36%) were taking cardiac medication, and 30 (57%) had documented comorbidities. The proportion of simple, moderate, and severe CHD was 15%, 42%, and 43%, respectively. The proportion of patients with normal body weight was 72%, while 28% were overweight/obese. Of the 53 patients, 21 (53%) had greater clustering of unhealthy lifestyle patterns while 25 (48%) had lesser clustering. In our final multivariable logistic model, participants with greater clustering of lifestyle risk behaviours had 7.4 times the odds of being overweight/obese (95% CI 1.5, 54.4, P=0.025) than those participants with lesser clustering of lifestyle risk behaviours (Table 1). Age, sex, and CHD severity were not associated with weight status. <h3>CONCLUSION</h3> Clustering of multiple unhealthy lifestyle behaviour patterns are prevalent among children with CHD and this is associated with increased risk of being overweight/obese. Timely identification of lifestyle risk behaviours and targeted intervention through personalized lifestyle counseling and physical activity promotion may be an effective strategy to optimize body weight.

Long-term effectiveness of an mHealth-tailored physical activity intervention in youth with congenital heart disease: A randomized controlled trial.

To evaluate the long-term effectiveness of the Care & Organize Our Lifestyle (COOL) programme, a self-regulation theory-based mHealth programme, on improving disease knowledge and physical activity in youth with congenital heart disease (CHD). A three-arm parallel-group randomized controlled trial. A total 143 participants with simple and moderate CHD aged 15-24years were recruited from June 2016 to February 2018. The 12-month programme compared two active intervention groups to a standard-care control group (n=47). Participants in one active intervention group (n=49) were provided with COOL Passport, a mobile healthcare application. Those in the other group (n=47) were provided with access to the Health Promotion Cloud system and use of game-based interactive platforms along with COOL Passport. Outcomes were the Leuven Knowledge Questionnaire for Congenital Heart Disease and the International Physical Activity Questionnaire-Taiwan Show-Card Version. After 12months, 103 participants remained; the overall attrition rate was 28%. No significant differences were observed between the groups in any domain of disease knowledge after 6months or 1year. Neither active group exhibited significantly greater physical activity intensity than the standard-care control group in any month during the 1year. The mHealth-tailored intervention of the COOL programme did not improve disease knowledge or physical activity in young adults with CHD. The application of the COOL Passport and Health Promotion Cloud system and use of game-based interactive platforms must be modified and verified in future studies and may have clinical potential. The registry of clinical trials was ClinicalTrials.gov: NCT04264650.

The incidence of congenital heart disease in Azerbaijan: prospective epidemiological study

Background The incidence of Congenital Heart Disease (CHD) in Azerbaijan has never been calculated with a prospective study, using Echo-2D as a study method&#x0D; Aim First prospective epidemiology study calculating incidence and types of CHD in Baku, Azerbaijan.&#x0D; Population-method From June 2016 to August 2018, 2570 term neonates were screened in 2 major maternity units. The screening was randomized to equal females/males with no known previous obstetric alert regarding CHD. Scanning was done by two teams of pediatric cardiologists in echo-2D. Each team was ‘blinded’ to the findings of each other. All scans were recorded, and a third senior physician reevaluated them.&#x0D; Results From 2570 term neonates of the general population we detected 47 CHD’s. From them, 17 were c-CHD and 5, s-CHD. 25 were simple or moderate complex non-cyanotic CHD. The estimated incidence was 1.828%. 17/47 (36.2%) were cyanotic. The incidence of simple CHD was 25/47(53.2%). Analysis of the specific anatomy is presented in table 1.&#x0D; Conclusions This first-ever prospective epidemiology study in Azerbaijan involving a cohort equal to 1.65% of the annual living births of the country, estimated a high incidence. This is among the highest reported globally. The amount of critical &amp; cyanotic CHD was 46.8% and 36.2% respectable. These high numbers are possibly related to an isolated population and conjugated marriage customs of the country. This represents a public population health burden.