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- Björk, Anna, et al.
(författare)
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Incidence of diabetes mellitus and effect on mortality in adults with congenital heart disease
- 2024
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Ingår i: INTERNATIONAL JOURNAL OF CARDIOLOGY. - 0167-5273 .- 1874-1754. ; 401
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Tidskriftsartikel (refereegranskat)abstract
- Background: Worldwide, 1-2% of children are born with congenital heart disease (CHD) with 97% reaching adulthood. Objectives: This study aims to demonstrate the risk of diabetes in patients with CHD, and the influence of incident diabetes on mortality in CHD patients and controls. Methods: By combining data from patient registries, the incidence of adult -onset diabetes registered at age 35 or older, and subsequent mortality risk were analysed in two successive birth cohorts (born in 1930-1959 and 1960-1983), by type of CHD lesion and sex, compared with population -based controls matched for sex and year of birth and followed until a maximum of 87 years of age. Results: Out of 24,699 patients with CHD and 270,961 controls, 8.4% and 5.6%, respectively, were registered with a diagnosis of diabetes at the age of 35 or older, hazard ratio (HR) 1.47 (95% CI 1.40-1.54). The risk of diabetes was higher in the second birth cohort (HR of 1.74, 95% CI 1.54-1.95) and increased with complexity of CHD. After onset of DM, the total mortality among patients with CHD was 475 compared to 411/ 10,000 personyears among controls (HR 1.16, 95% CI 1.07-1.25). Conclusions: In this nationwide cohort of patients with CHD and controls, the incidence of diabetes was almost 50% higher in patients with CHD, with higher risk in the most recent birth cohort and in those with conotruncal defects, with the combination of CHD and diabetes associated with a significantly increased mortality compared to diabetic controls.
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- Björk, Anna, et al.
(författare)
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Incidence of Type 1 diabetes mellitus and effect on mortality in young patients with congenital heart defect – A nationwide cohort study
- 2020
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273. ; 310, s. 58-63
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Tidskriftsartikel (refereegranskat)abstract
- Background: 1% of all live born children are born with a congenital heart defect (CHD) and currently 95% reach adulthood. Type 1 diabetes mellitus (T1DM) is an autoimmune disease that can develop due to i.e. heredity, exposure to infections and stress-strain. The incidence of T1DM in patients with CHD is unknown and we analysed the risk of developing T1DM for patients with CHD, and how this influences mortality. Methods: By combining registries, the incidence of T1DM and the mortality was analysed in patients with CHD by birth cohort (1970–1993, 1970–1984 and 1984–1993) matched with population-based controls matched for sex, county and year of birth without CHD and followed from birth until a maximum of 42 years. Results: 221 patients with T1DM among 21,982 patients with CHD and 1553 patients with T1DM among 219,816 matched controls were identified. The hazard ratio (HR) for developing T1DM was 1.50 (95%, CI 1.31–1.73) in patients with CHD compared to the controls and the first birth cohort (1970–1984) had the highest risk for T1DM, HR 1.87 (95%, CI 1.56–2.24). After onset, mortality risk was 4.21 times higher (95%, CI 2.40–7.37) in patients with CHD and T1DM compared to controls with T1DM. Conclusion: From a nationwide cohort of patients with CHD and controls, the incidence of developing T1DM was 50% higher in patients with CHD, showing a significant increase in risk among birth cohort 1970–1984. The combination of CHD and T1DM was associated with a 4-fold increase in mortality compared to controls with only T1DM. © 2020
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- Dellborg, Mikael, 1954, et al.
(författare)
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Adults With Congenital Heart Disease: Trends in Event-Free Survival Past Middle Age
- 2023
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Ingår i: Circulation. - : Ovid Technologies (Wolters Kluwer Health). - 0009-7322 .- 1524-4539. ; 147:12, s. 930-938
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Tidskriftsartikel (refereegranskat)abstract
- Background:The survival of children with congenital heart disease has increased substantially over the past decades, with 97% currently reaching adulthood. The total effect of advanced treatment on future mortality and morbidity in adult survivors with congenital heart disease (CHD) is less well described. Methods:We used data from the Swedish National Inpatient, Outpatient, and Cause of Death Register to identify patients with CHD who were born between 1950 and 1999 and were alive at 18 years of age. Ten controls identified from the Total Population Register were matched for year of birth and sex and with each patient with CHD. Follow-up was from 1968 and 18 years of age until death or at the end of the study (2017). Survival percentage with 95% CI for all-cause mortality were performed with Kaplan-Meier survival function. Cox proportional hazard regression models with hazard ratios (HRs) and 95% CI were used to estimate the risk of all-cause mortality. Results:We included 37 278 patients with adult CHD (ACHD) and 412 799 controls. Mean follow-up was 19.2 years (+/- 13.6). Altogether, 1937 patients with ACHD (5.2%) and 6690 controls (1.6%) died, a death rate of 2.73 per 1000 person-years and 0.84 per 1000 person years, respectively. Mortality was 3.2 times higher (95% CI, 3.0-3.4; P<0.001) among patients with ACHD compared with matched controls. Up to the maximum of 50 years of follow-up, >75% of patients with ACHD were still alive. Mortality was highest among patients with conotruncal defects (HR, 10.13 [95% CI, 8.78-11.69]), but also significantly higher for the more benign lesions, with the lowest risk in patients with atrial septal defects (HR, 1.36 [95% CI, 1.19-1.55]). At least 75% of patients with ACHD alive at 18 years of age lived past middle age and became sexagenerians. Conclusions:In this large, nationwide, register-based cohort study of patients with ACHD surviving to 18 years of age, the risk of mortality up to 68 years of age was >3 times higher compared with matched controls without ACHD. Despite this, at least 75% of patients with CHD alive at 18 years of age lived past middle age and became sexagenerians. A notable risk decline in the mortality for patients with ACHD was seen for those born after 1975.
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- Eckerström, Filip, et al.
(författare)
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Mortality burden in patients born with Ebstein's anomaly: a 40-year nationwide cohort study.
- 2021
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Ingår i: European heart journal. Quality of care & clinical outcomes. - : Oxford University Press (OUP). - 2058-1742 .- 2058-5225. ; 7:3, s. 312-319
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Tidskriftsartikel (refereegranskat)abstract
- Survival rates for unoperated patients with Ebstein's anomaly (EA) are unknown. We estimated overall long-term mortality in operated and unoperated EA patients, compared with the general population in Sweden.Using national medical registries, Swedish individuals born 1970-1993 and diagnosed with EA between 1970 and 2011 were included. The hazard ratio for overall mortality for EA patients (n=216) vs. the matched comparison cohort (n=2160) was 43.7 (95% confidence interval (CI): 24.8-82.5). Mortality risk for EA patients (vs. controls) decreased as birth period progressed, with hazard ratios declining from 63.6 (95% CI: 26.3-191.8) for those born in the 1970s to 34.4 (95% CI: 15.8-83.1) for those born in the 1980s and 20.2 (95% CI: 1.6-632.5) for those born at the beginning of 1990s. The overall mortality hazard ratios for unoperated and operated patients with EA (vs. controls) were 30.2 (95% CI: 13.8-73.3) and 63.7 (95% CI: 28.1-172.5), respectively. The risk of mortality among unoperated EA patients (vs. controls) declined with progressing birth period, with hazard ratios declining from 58.4(95% CI: 15.1-415.2) in the 1970s to 22.9(95% CI: 8.0-75.3) in the 1980s and 0 (95% CI:0.0-70.2) in the 1990s.Overall all-cause mortality for patients with EA declined dramatically from 64 times to 20 times that of controls without EA, from the 1970s to the early 1990s. Unoperated patients with EA had better survival than did operated patients, possibly reflecting the higher severity of disease or more severe associated cardiac defects in patients undergoing surgery.
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- Eckerström, Filip, 1991, et al.
(författare)
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Mortality in Patients With Ebstein Anomaly
- 2023
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Ingår i: Journal of the American College of Cardiology. - 0735-1097. ; 81:25, s. 2420-2430
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND Low birth prevalence and referral bias constitute significant obstacles to elucidating the natural history of Ebstein anomaly (EA).OBJECTIVES An extensive 2-country register-based collaboration was performed to investigate the mortality in patients with EA.METHODS Patients born from 1970 to 2017 and diagnosed with EA were identified in Danish and Swedish nationwide medical registries. Each patient was matched by birth year and sex with 10 control subjects from the general population. Cumulative mortality and HR of mortality were computed using Kaplan-Meier failure function and Cox proportional regression model.RESULTS The study included 530 patients with EA and 5,300 matched control subjects with a median follow-up of 11 years. In the EA cohort, 43% (228) underwent cardiac surgery. Cumulative mortality was lower for patients diagnosed in the modern era (the year 2000 and later) than for those diagnosed in the prior era (P < 0.001). Patients with isolated lesion displayed lower cumulative mortality than patients with complex lesions did (P < 0.001). Patients with a presumed mild EA anatomy displayed a 35-year cumulative mortality of 11% (vs 4% for the matched control subjects; P < 0.001), yielding an HR for mortality of 6.0 (95% CI: 2.7-13.6), whereas patients with presumed severe EA demonstrated an HR of 36.2 (95% CI: 15.5-84.4) compared with control subjects and a cumulative mortality of 18% 35 years following diagnosis. CONCLUSIONS Mortality in patients with EA is high irrespective of presence of concomitant congenital cardiac malformations and time of diagnosis compared with the general population, but overall mortality has improved in the contemporary era.
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| 9. |
- Engsner, Stella, et al.
(författare)
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Impact of Down Syndrome on Survival Among Patients With Congenital Heart Disease
- 2024
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Ingår i: JOURNAL OF THE AMERICAN HEART ASSOCIATION. - 2047-9980. ; 13:2
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Tidskriftsartikel (refereegranskat)abstract
- BackgroundIncreasing survival among patients with congenital heart disease (CHD) has recently been reported. However, the impact of Down syndrome (DS) in patients with CHD is still debated. We aimed to estimate survival in patients with CHD with versus without DS compared with matched controls from the general population without CHD or DS.Methods and ResultsWe linked data from Swedish health registries to identify patients with CHD born between 1970 and 2017. Data from the Total Population Register were used to match each patient with CHD by sex and birth year with 8 controls without CHD or DS. A Cox proportional regression model was used to estimate mortality risk, and Kaplan-Meier curves were analyzed for the survival analysis. We identified 3285 patients with CHD-DS, 64 529 patients with CHD without DS, and 26 128 matched controls. The mortality risk was 25.1 times higher (95% CI, 21.3-29.5) in patients with CHD-DS versus controls. The mortality rate was 2 times higher (95% CI, 1.94-2.31) for patients with CHD with versus without DS. Lower mortality was found during the second versus first birth periods in patients with CHD-DS compared with controls; hazard ratio: 46.8 (95% CI, 29.5-74.0) and 17.7 (95% CI, 12.8-24.42) in those born between 1970 and 1989 versus 1990 and 2017, respectively.ConclusionsIn this retrospective cohort study, the mortality risk among patients with CHD-DS was 25 times higher compared with matched controls and 2 times higher compared with patients with CHD without DS. Survival was higher in patients with CHD-DS born after versus before 1990, coinciding with the modern era of congenital heart care.
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| 10. |
- Fedchenko, Maria, 1988, et al.
(författare)
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Cardiovascular risk factors in adults with coarctation of the aorta.
- 2019
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Ingår i: Congenital heart disease. - : Computers, Materials and Continua (Tech Science Press). - 1747-0803 .- 1747-079X. ; 14:4, s. 549-558
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Tidskriftsartikel (refereegranskat)abstract
- The aging patient with adult congenital heart disease (ACHD) faces the risk of developing atherosclerotic disease. Patients with coarctation of the aorta (CoA) are especially vulnerable because of an inherent high risk of developing hypertension. However, data on the prevalence of other cardiovascular risk factors are scarce. Therefore, this study aimed to describe the prevalence of traditional cardiovascular risk factors (diabetes, hypertension, hyperlipidemia, smoking, obesity, and sedentary lifestyle) in adult patients with CoA.Patients with CoA who were registered at the ACHD clinic in Gothenburg were asked to participate in a comprehensive cardiovascular risk assessment. This assessment included a glucose tolerance test, cholesterol profile, ambulatory blood pressure measurements, and a lifestyle questionnaire.A total of 72 patients participated. The median age was 43.5years and 58.3% were men. Sixty-six (91.7%) patients had ≥one cardiovascular risk factor and 40.3% had ≥three risk factors. Three (4.2%) patients were newly diagnosed with diabetes or impaired glucose tolerance. More than half of the patients had hyperlipidemia (n=42, 58.3%) and 35 patients (48.6%) were overweight or obese. Only three (4.2%) patients smoked regularly. Of the 60 patients who underwent 24-hour ambulatory blood pressure measurement, 33 (55.0%) were hypertensive. Of the 30 patients with known hypertension only 9 (30.0%) had well-controlled blood pressure on ambulatory blood pressure measurement.Cardiovascular risk factors among patients with CoA are prevalent. This may indicate a need for more aggressive screening strategies of traditional risk factors to minimize the risk of these patients also developing atherosclerotic disease.
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