Congenital Heart Disease In Sweden Research Articles

Outcomes after cancer diagnosis in children and adult patients with congenital heart disease in Sweden: a registry-based cohort study

ObjectivePatients with congenital heart disease (CHD) have an increased cancer risk. The aim of this study was to determine cancer-related mortality in CHD patients compared with non-CHD controls, compare ages...

Long-term survival in patients with univentricular heart: a nationwide, register-based cohort study

Abstract Background Univentricular heart (UVH) is a broad term that covers various cardiac structural abnormalities in which one ventricle is severely underdeveloped, or a ventricular septal wall did not form, leading to a functional single-ventricle physiology. Patients with UVH have a very limited life expectancy if not surgically treated. While the outcome in children with UVH have been extensively studied, data regarding the long-term outcomes in an unselected, nationwide cohort is still lacking. Purpose The aim of the present study was to determine long-term survival in patients with UVH compared to an age- and sex-matched control population without congenital heart disease in Sweden. Methods We linked data from the Swedish Patient and Cause of Death Register to identify patients with UVH that were born between 1970 and 2017. Each patient with UVH was matched with ten individuals without congenital heart disease for year of birth and sex. The mortality risk in patients with UVH compared with matched controls was analyzed using Cox proportional regression models and Kaplan-Meier survival analysis. Results Of a total of 5,075 patients with UVH and 50,620 matched controls, 1,992 (37.9%) and 485 (1.0%) died, respectively. The mean follow-up (standard deviation) was 15.7 (± 14.2) years. A total of 758 patients (14.9%) of the UVH population were diagnosed with hypoplastic left heart syndrome (HLHS). The mortality risk was 53.01 (95% CI, 48.0–58.6) times higher in UVH and 163.5 (95% CI, 124.3–215.2) times higher in HLHS compared to controls. According to birth period, highest mortality was found at the earliest UVH cohort (1970–1981) 59.8%, and decreased by later birth periods. The lowest mortality was in the latest birth period, 2006–2017 (16.3%). Conclusions In this nationwide, register-based cohort study, the overall risk of mortality was more than 50 times higher in patients with UVH compared to matched controls. However, an increased survival was observed over time and by later birth periods. Quadragenarians with UVH is still a rare group but are expected to increase during the next decades. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Swedish heart lung foundation

Qualitative study of facilitators and barriers for continued follow-up care as perceived and experienced by young people with congenital heart disease in Sweden

In-depth understanding of factors perceived by young people with congenital heart disease (CHD) to affect continued follow-up care is needed to tailor preventive strategies for discontinuation of follow-up care. To...

Burden of Ischemic Stroke in Patients With Congenital Heart Disease: A Nationwide, Case-Control Study.

BackgroundPatients with congenital heart disease (CHD) are at increased risk of developing ischemic stroke (IS) compared with controls without CHD. However, the long‐term outcomes after IS, including IS recurrence and mortality risk, remain unclear.Methods and ResultsWe identified all patients with CHD in Sweden who were born between 1930 and 2017 using the Swedish National Patient Register and the Cause of Death Register. Ten controls without CHD were randomly selected from the general population and matched for birth year and sex for each patient with CHD. The follow‐up of the study population was performed between January 1970 and December 2017. In total, 88 700 patients with CHD (50.6% men) and 890 450 matched controls (51.0%) were included in this study. During a mean follow‐up of 25.1±22.0 years, patients with CHD had a 5‐fold higher risk of developing an index IS (hazard ratio [HR], 5.01; 95% CI, 4.81–5.22) compared with controls. However, the risk of developing a recurrent IS was lower in patients with CHD compared with controls (HR, 0.66; 95% CI, 0.56–0.78), an observation that persisted after adjustment for cardiovascular risk factors and comorbidities. Patients with CHD were also at a significantly lower risk of all‐cause mortality after index IS than controls (HR, 0.53; 95% CI, 0.49–0.58).ConclusionsPatients with CHD had a 5‐fold higher risk of developing index IS compared with matched controls. However, the risk of recurrent IS stroke and all‐cause mortality were 34% and 47% lower, respectively, in patients with CHD compared with controls.

Mechanisms of impact and experiences of a person-centred transition programme for adolescents with CHD: the Stepstones project

BackgroundDuring the past decade there has been some evaluation of transition programmes for adolescents with chronic conditions. However, this has rarely involved process evaluations focusing on mechanisms leading to outcomes, thus hampering implementation of these complex interventions. Our aim was to (I) describe adolescents’ and parents’ experiences of participating in a person-centred transition programme aiming to empower them in transition to adulthood and (II) explore the mechanisms of impact.MethodsA qualitative process evaluation was performed, embedded in a randomized controlled trial evaluating the effectiveness of a transition programme for adolescents with congenital heart disease in Sweden. A purposive sample of 14 adolescents and 12 parents randomized to the intervention group were interviewed after participation in the programme. Data were analysed deductively and inductively in NVivo v12.ResultsExperiences of participation in the transition programme were generally positive. Meeting a transition coordinator trained in person-centred care and adolescent health and embarking on an educational process based on the adolescents’ prerequisites in combination with peer support were considered key change mechanisms. However, support to parents were not sufficient for some participants, resulting in ambivalence about changing roles and the unmet needs of parents who required additional support.ConclusionsParticipants experienced increased empowerment in several dimensions of this construct, thus demonstrating that the transition programme was largely implemented as intended and the evidence-based behaviour-change techniques used proved effective in reaching the outcome. These findings can inform future implementation of transition programmes and illuminate challenges associated with delivering a complex intervention for adolescents with chronic conditions.

Risk of ischemic stroke in adult patients with congenital heart disease

Abstract Introduction With an increasing proportion of adults with congenital heart disease (CHD) surviving into middle age and beyond, CHD patients will be at increased risk of acquired cardiovascular conditions, such as ischemic stroke. Compared to controls, patients with CHD have a higher prevalence of arrhythmias, persistent shunts enabling paradoxical embolization, heart failure, mechanical valves as well as potentially hypercoagulable states, all of which can further increase the risk of stroke. Purpose The aim of our study was to investigate the risk of developing ischemic stroke in adults with CHD in Sweden compared to controls from the general population. Methods We used data from the Swedish National Patient and Cause of Death registries to identify all CHD patients ≥18 years of age, born during the period 1930–1998, with a first time diagnosis of ischemic stroke. Follow-up started in January 1970 and went on until December 2017. Approximately ten controls matched for age and sex were randomly selected from the general population for each patient with CHD. CHD diagnoses were classified into six lesion groups according to a previously published hierarchical classification system. Results In total, 43,110 patients with CHD and 474,267 controls were included in the study (51.4% men) and mean follow up time was 25.4±18.4 years. Patients with CHD had a 6 times higher risk of developing an ischemic stroke compared with controls (hazard ratio 6.0, 95% confidence interval 5.8–6.2, p≤0.001), with altogether 8.8% (n=3785) of CHD patients developing ischemic stroke compared with 1.6% (n=7516) of controls. Ischemic stroke was more common in all CHD lesion groups; however, patients with atrial septal defects/patent foramen ovale had the highest incidence rate of ischemic stroke with an incidence rate of 76.1 events/10,000 patient years compared with 8.7 in controls. Patients with CHD and ischemic stroke had markedly less hypertension, diabetes mellitus and hypercholesterolemia, compared with controls (7.1%, 2.0%, 2.9% respectively in CHD patients, compared with 19.6%, 6.6%, 5.3%, in controls, p≤0.001 for all). In addition, atrial fibrillation and heart failure were only slightly more common in CHD patients with ischemic stroke compared to controls (atrial fibrillation: 12.0% in CHD vs 10.4% in controls, p=0.01; heart failure: 8.7% in CHD vs 7.3% in controls, p=0.009). Conclusion In this large nationwide study, we found that the risk of ischemic stroke in adult patients with CHD was six times higher than in controls, despite a lower prevalence of common risk factors for stroke such as hypertension, diabetes mellitus and hypercholesterolemia. In addition, atrial fibrillation and heart failure were only slightly more common in CHD patients compared with controls. This implies that the etiology of ischemic stroke might be different in CHD patients compared with controls. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): This work was funded by the Swedish state under an agreement between the Swedish government and county councils, the ALF agreement (Grant number: 236611) and the Swedish Heart-Lung Foundation (Grant Number: 20090724).

Validation of myocardial infarction diagnosis in patients with congenital heart disease in Sweden

BackgroundThe population of adults with congenital heart disease (CHD) is growing, and increasingly more patients with CHD reach older ages. Patients with CHD are at an increased risk of myocardial infarction (MI) with increased age. Diagnosing MI in patients with CHD can be challenging in clinical practice owing to a high prevalence of aberrant electrocardiograms, ventricular hypertrophy, and heart failure, among other factors. The National Swedish Patient Register (NPR) is widely used in epidemiological studies; however, MI diagnoses specifically in patients with CHD have never been validated in the NPR.MethodsWe contacted hospitals and medical archive services to request medical records for 249 patients, born during 1970–2012, with both CHD and MI diagnoses and who were randomly selected from the NPR by the Swedish National Board of Health and Welfare. Follow-up was until 2015. We performed a medical chart review to validate the MI diagnoses; we also validated CHD diagnoses to ensure that only patients with confirmed CHD diagnoses were included in the MI validation process.ResultsWe received medical records for 96.4% (n = 238/249) of patients for validation of CHD diagnoses. In total, 74.8% (n = 178/238) had a confirmed CHD diagnosis; of these, 70.2% (n = 167) had a fully correct CHD diagnosis in the NPR; a further 4.6% (n = 11) had a CHD diagnosis, but it was misclassified. MI diagnoses were validated in 167 (93.8%) patients with confirmed CHD. Of the patients with confirmed CHD, 88.0% (n = 147/167) had correct MI diagnoses. Patients with non-complex CHD diagnoses had more correct MI diagnoses than patients with complex CHD (91.0%, n = 131 compared with 69.6%, n = 16). The main cause for incorrect MI diagnoses was typographical error, contributing to 50.0% of the incorrect diagnoses.ConclusionsThe validity of MI diagnoses in patients with confirmed CHD in the NPR is high, with nearly 9 of 10 MI diagnoses being correct (88.0%). MI in patients with CHD can safely be studied using the NPR.

Ischemic heart disease in children and young adults with congenital heart disease in Sweden

BackgroundAn increasing proportion of congenital heart disease (CoHD) patients survive to an age associated with increased risk of developing ischemic heart disease (IHD). The aim was to investigate the risk of developing IHD among children and young adults with CoHD. MethodsUsing the Swedish National Patient Register, we created a cohort of all CoHD patients born between January 1970 and December 1993. Ten controls matched for age, sex, county were randomly selected from the general population for each patient (n=219,816). Patients and controls were followed from birth until first IHD event, death, or December 31, 2011. ResultsWe identified 21,982 patients with CoHD (51.6% men), mean follow-up was 26.4 (21.2–33.9) years. CoHD patients had 16.5 times higher risk of being hospitalized with or dying from IHD compared to controls (95% CI: 13.7–19.9), p<0.0001. Patients with conotruncal defects and severe nonconotruncal defects, had the highest IHD incidence rate (71.1 and 56.3 cases per 100,000 person-years, respectively, compared to 2.9 and 2.3 in controls). Hypertension and diabetes were less common among CoHD patients with IHD than among controls with IHD (hypertension 9.7% vs 19.7%, diabetes 1.8% vs 7.7% in CoHD patients and controls). Patients with aortic coarctation did not have a specific increase in the risk of developing IHD or acute myocardial infarction. ConclusionsIn this large case-control cohort study, the relative risk of developing IHD was markedly higher in CoHD patients than in controls. However, the absolute risk was low in both groups.

Survivorship in Children and Young Adults With Congenital Heart Disease in Sweden

Mortality in patients with congenital heart disease (CHD) has markedly decreased during recent decades because of advancement in pediatric care. However, there are limited data on survival trends in children and young adults with CHD compared with the general population. To determine survivorship in children and young adults with CHD compared with matched controls. A registry-based, prospective, matched-cohort study was conducted in Sweden. Data from the national patient and cause of death registers were linked to identify individuals with CHD born between January 1, 1970, and December 31, 1993, who were registered at or after birth. Follow-up and comorbidity data were collected until December 31, 2011. Survival analyses were performed with the Cox proportional hazards model; these analyses were performed from January 1, 1970, to December 31, 2011. A total of 21 982 patients with CHD in Sweden were identified. The mean (SD) follow-up time was 27.0 (8.86) years. Children serving as controls (n = 219 816) (10 for each patient), matched for birth year, sex, and county, were randomly selected from the general population. Survivorship in young patients with CHD and controls. Of the 21 982 patients who were born between 1970 and 1993 and were registered with the diagnosis of CHD, 10 650 were female (48.4%). Median age at index registration was 4.22 years (interquartile range, 17.07 years). Survivorship among children younger than 5 years was increased from 96% in those born in 1970-1979 to 98% in those born in 1990-1993. Hazard ratios (HRs) of death in relation to that in control individuals decreased from 225.84 (95% CI, 136.84-372.70) to 33.47 (95% CI, 22.54-49.70). A substantial, but less pronounced, absolute and relative increase in survivorship was found in older patients (HRs ranged from 24.52; 95% CI, 11.72-51.26, at 5-9 years to 4.27; 95% CI, 2.29-7.95, at 18-29 years). According to a hierarchical CHD classification, the group of patients with the most severe complex defects (ie, common arterial trunk, transposition of the great vessels, double inlet ventricle, hypoplastic left heart syndrome, tetralogy of Fallot, and atrioventricular septal defect) had the highest risk for death (HR, 64.07; 95% CI, 53.39-76.89). Despite substantially increasing absolute and relative survivorship in children and young adults with CHD, the mortality risk remains high compared with the risk in matched controls. Further research on reducing the death rate in this vulnerable group is required.

Impact of a standardized training program on midwives' ability to assess fetal heart anatomy by ultrasound.

BackgroundStudies of prenatal detection of congenital heart disease (CHD) in the UK, Italy, and Norway indicate that it should be possible to improve the prenatal detection rate of CHD in Sweden. These studies have shown that training programs, visualization of the outflow tracts and color-Doppler all can help to speed up and improve the detection rate and accuracy. We aimed to introduce a more accurate standardized fetal cardiac ultrasound screening protocol in Sweden.MethodsA novel pedagogical model for training midwives in standardized cardiac imaging was developed, a model using a think-aloud analysis during a pre- and post-course test and a subsequent group reflection. The self-estimated difficulties and knowledge gaps of two experienced and two beginner midwives were identified. A two-day course with mixed lectures, demonstrations and hands-on sessions was followed by a feedback session three months later consisting of an interview and check-up. The long-term effects were tested two years later.ResultsAt the post-course test the self-assessed uncertainty was lower than at the pre-course test. The qualitative evaluation showed that the color Doppler images were difficult to interpret, but the training seems to have improved their ability to use the new technique. The ability to perform the method remained at the new level at follow-up both three months and two years later.ConclusionsOur results indicate that by implementing new imaging modalities and providing hands-on training, uncertainty can be reduced and examination time decreased, but they also show that continuous on-site training with clinical and technical back-up is important.

Abstract 19136: Incidence of Diabetes Mellitus in Patients with Congenital Heart Disease

Introduction: Today 0.8-1 % of all children are born with congenital heart defects (CHD) and most live into adulthood, with estimations and registry data indicating that 90-95% of all CHD now reach adulthood. CHD may increase the risk of infections and subsequently higher risk of diabetes type 1. Diabetes type 2 (T2D) increases and CHD could lead to a more sedentary lifestyle with a higher risk of developing T2D. Rhe relation between CHD and diabetes has previously not been investigated. Hypotheses: We estimated the risk of developing diabetes in a large cohort of patients with CHD as found in the nationwide diabetes registry in Sweden, NDR Methods: the National Diabetes Registry, NDR has an estimated coverage above 70% for Sweden. We identified patients with CHD in the NDR and compared to two controls groups, control group 1 never hospitalized paired patients with T1D or T2D and a second control group randomly selected paired patients with diabetes T1D or T2D and previous hospitalization for any reason. Both control groups were adjusted by age and gender. The analyzed characteristics were age, gender, type of diabetes, diabetes duration, systolic blood pressure, BMI, waist measurement, HbA1c, smoking, microalbuminuria, antihypertensive, lipid lowering drug use and type of hypoglycaemic treatment. Results: We found 1860 patients in the NDR with CHD as additional diagnosis. With a presumed prevalence of between 0.3 and 0.4% of the adult population, diabetes was found in 6.2 to 8.2% of all adults with CHD, for an odds-ratio of 1.35 (1.3-1.4, p&lt;0.001) for diabetes of any kind to occur in patients with CHD as compared to adults without CHD. CHD and diabetes with the Swedish population at age 18-100 years CHD Total pop OR P Total no 25.000 (est) 7.562.648 With diabetes 1860 425.375 with diabetes 7.4% 5.6% 1.35 (1.3-1.4) &lt;0.0001 Conclusion. Based on estimated prevalence of adults with congenital heart disease in Sweden, the prevalence of diabetes seems to be higher in the CHD population than in the population without CHD, given an OR of 1.35.