| 1. |
- Andersson, Alva, et al.
(författare)
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Functional muscle power in the lower extremity in adults with congenital heart disease
- 2023
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Ingår i: International Journal of Cardiology Congenital Heart Disease. - : Elsevier BV. - 2666-6685. ; 11
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Tidskriftsartikel (refereegranskat)abstract
- Background: We aimed to investigate functional muscular power in the lower extremity in adults with congenital heart disease (ACHD) and compare results with those of healthy persons. Secondarily, we set out to assess muscle power in relation to age, sex, and complexity of ACHD.Methods: Between 2013 and 2019, 1126 patients attended the ACHD Unit of Sahlgrenska University Hospital/Ostra and performed a test battery to determine physical fitness. Of these patients, 559 who performed the Timed-Stands Test (TST)-which requires 10 stands from a chair at the maximal possible speed-were included in the study.Results: Patients with ACHD performed the TST slower than reference (14.6 s [12.0-18.0] vs. 11.7 s [9.8-14.3], p < 0.001). Men with ACHD performed the TST more rapidly or according to reference in 8% of patients aged 18-39 years, 21% of patients aged 40-65 years, and 55% of patients aged >65 years. Women with ACHD performed the TST more rapidly or according to reference in 21% of patients aged 18-39 years, 56% of patients aged 40-65 years, and 32% of patients aged >65 years. Men with ACHD performed the TST significantly faster than women with ACHD (p < 0.001).Conclusions: Decreased functional muscle power was observed in patients with ACHD and was most pronounced in patients aged 18-39 years. Decreased muscle power is important to detect and requires further assessment because it may contribute to an increased risk of falling and developing lifestyle related diseases.
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| 5. |
- Daene, M, et al.
(författare)
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Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect
- 2023
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Ingår i: International Journal of Cardiology Congenital Heart Disease. - : Elsevier BV. - 2666-6685. ; 12
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Tidskriftsartikel (refereegranskat)abstract
- Background and aims Patients with Down Syndrome (DS) are frequently born with an atrioventricular septal defect (AVSD). Surgical repair of the defect aims to minimize mortality and morbidity. However, a surgical intervention, specifically in DS patients, is not without risk and a subgroup of patients underwent only conservative non-surgical treatment. Outcome data of these different approaches are scarce. The aim of this retrospective study was to compare the long-term outcome of DS patients with and without surgery for AVSD. Methods DS patients registered with AVSD in the hospital's database from January 1980 till December 2020 were selected. Patient characteristics, peri-operative if appropriate, and follow-up data were obtained from the medical files. Results In total, 72 unrepaired (36 male, 50%) and 134 repaired patients (61 male, 46%) were included. After a maximum of 60 years of follow-up, the all-cause mortality was 45.8% and 17.1%, respectively. Thirty-six percent and 13%, respectively, were labeled as non-cardiovascular death. Mean survival time for unrepaired AVSD was 40.7 years (95% CI 36.1–45.2) and for repaired AVSD 38.5 years (95% CI 35.3–41.6) (Log rank p = 0.465). However, the survival rate 35 years after birth was 62.1% for unrepaired patients versus 81.7% for repaired patients. Mortality rates were the highest the first months after surgical repair. Conclusions The mean survival rate of Down patients, born with an AVSD, did not differ between repair or not. However, long-term survival rate was higher in patients who underwent surgical repair. Mortality was highest the first months after surgery.
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| 6. |
- Johansson, Karna, 1982-, et al.
(författare)
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Reduced bone strength in adults with moderate or complex congenital heart disease
- 2021
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Ingår i: International Journal of Cardiology Congenital Heart Disease. - : Elsevier. - 2666-6685. ; 6
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Tidskriftsartikel (refereegranskat)abstract
- Background: We aimed to study bone mineral density (BMD) and skeletal strength in the growing population of adults with moderate or complex congenital heart disease (CHD).Methods: Peripheral quantitative computed tomography (pQCT) was performed on the radius and tibia in 49 adults with moderate or complex CHD, and in 49 age and sex matched controls (n = 23 [47%] female, mean age 36 +/- 15.5 years.) Strength in the radius and tibia were presented, respectively, in terms of Strength-Strain Index (SSI).Results: Patients had similar total BMD as controls in both the radius (807 +/- 82 vs. 792 +/- 75 mg/cm(3), p = 0.3) and tibia (663 +/- 86 vs. 689 +/- 67 mg/cm(3), p = 0.1). In the radius, patients had a lower xSSI than controls (154 +/- 46 vs. 175 +/- 54 mm(3), p = 0.04) but a similar ySSI (178 +/- 58 vs. 195 +/- 55 mm(3), p = 0.1). In the tibia, patients had a lower xSSI (1492 +/- 399 vs. 1780 +/- 372 mm(3), p < 0.001) as well as ySSI (1066 +/- 304 vs. 1250 +/- 281, p = 0.002). In a multiple linear regression model, only height was independently associated with decreased skeletal strength in patients tibia in terms of xSSI [F(2,46) = 40.002, p < 0.001, R-2 = 0.635].Conclusion: Adults with moderate or complex CHD had reduced strength in the radius and tibia, despite a normal BMD as assessed with pQCT. Their reduced bone strength could increase the risk of future fractures.
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| 7. |
- Karagianni, Alexia, 1980, et al.
(författare)
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Long-term outcome after closure of an atrial shunt in patients aged 60 years or older with ischemic stroke: A nationwide, registry-based, case-control study
- 2023
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Ingår i: International Journal of Cardiology Congenital Heart Disease. - Amsterdam : Elsevier. - 2666-6685. ; 11
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Tidskriftsartikel (refereegranskat)abstract
- Background: According to the current guidelines, evidence of the effects of transcatheter closure in patients aged ≥60 years with an atrial shunt and cryptogenic stroke is still limited. Methods: Using Swedish health registries, patients aged ≥60 years who had previously developed a cryptogenic cerebrovascular event and undergone transcatheter closure were identified. Patients with atrial fibrillation were excluded, and the remaining patients were propensity score-matched with patients of the same age and risk profile who had only undergone medical treatment and with controls from the general population. They were then followed up until 2017 (mean period of 7.1 ±3.9 years). Results: In total, 100 patients of the intervention group were matched with 100 patients of the medical treatment group and with 100 controls and followed up. The hazard ratio for a recurrent ischemic stroke in the intervention group compared with the medical treatment group was 0.8 (95% confidence interval, 0.3–2.1), and that compared with the controls was 2.3 (95% confidence interval, 0.6–8.9). Atrial fibrillation occurred at the same rate in the two treatment groups (odds ratio, 0.8; 95% confidence interval, 0.4–1.7). However, patients in the intervention group developed vascular disease at a lower rate (odds ratio, 0.5; 95% confidence interval, 0.25–0.85). Conclusions: Patients aged ≥60 years with cryptogenic stroke may undergo transcatheter closure of an atrial shunt after thorough screening for other potential causes of stroke. The incidence of vascular disease seems to be mitigated in these patients relative to medically treated patients. © 2023 The Authors. Published by Elsevier B.V.
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| 8. |
- Leibold, Allison, et al.
(författare)
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Pain in adults with congenital heart disease : An international perspective
- 2021
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Ingår i: International Journal of Cardiology Congenital Heart Disease. - : Elsevier. - 2666-6685. ; 5
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Tidskriftsartikel (refereegranskat)abstract
- Background: Patients with adult congenital heart disease (ACHD) have many risk factors for chronic pain such as prior cardiac interventions and adult comorbidities. However, the prevalence of chronic pain has not been well described in this population. We sought to determine the prevalence of pain in a large international cohort of patients with ACHD.Methods: Data from the APPROACH-IS dataset was utilized for this study which includes 4028 patients with ACHD from 15 different countries. The prevalence of pain was assessed under the health status patient reported outcome domain utilizing the EuroQol-5D 3 level version tool. Multivariable logistic regression was used to assess differences across countries in pain, taking into account country-level random effects for clustering across observations within each country.Results: A total of 3832 patients with ACHD met the study criteria, median age 32 years [IQR 25, 42], 52.6% females. The prevalence of at least moderate pain was reported by 28.9% (95% CO 27.5 = 30.3%) of participants. Pain was associated with country of origin, age, gender, background, education and marital status as well as several clinical variables including disease complexity, cardiac device presence, history of heart failure, psychiatric conditions and presence of other medical conditions. Those with pain had lower levels of perceived health and a lower quality of life score.Conclusion: Pain in patients with ACHD is common, impacting nearly one-third of patients. Given the far reaching implications of pain in patients with ACHD, further study of pain characteristics and treatment management appear warranted.
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| 11. |
- Nielsen, Susanne, 1969, et al.
(författare)
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Risk for first onset depression in adults with congenital heart disease
- 2022
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Ingår i: INTERNATIONAL JOURNAL OF CARDIOLOGY CONGENITAL HEART DISEASE. - : Elsevier BV. - 2666-6685. ; 9
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Tidskriftsartikel (refereegranskat)abstract
- Background: The long-term risk for depression among adults with congenital heart disease (ACHD) is unclear. Therefore, we assessed the risk for first onset of serious depression in patients with ACHD compared with a sex and age-matched control population without a congenital heart disease (CHD) diagnosis.Methods: We extracted all patients with CHD born from 1970 to 1999 who survived until age 18 years from the Swedish National Patient Register. For each case, 10 random controls without CHD were selected from the general population and matched by birth year and sex. Participants were followed-up until the first diagnosis of new onset serious depression requiring hospital or specialist outpatient care.Results: This study included 22,912 patients with ACHD and 224,259 controls. The mean age at depression onset was 30.1 years in the ACHD group and 30.3 years in controls. The overall associated risk for serious depression was higher among patients with ACHD compared with controls, with an adjusted hazard ratio (aHR) of 1.32 (95% confidence interval [CI]: 1.20-1.44). Patients in the complex lesion group had a 54% higher risk for depression (aHR 1.54, 95% CI: 1.10-2.18) compared with the control group. The corresponding result for the non-complex lesion group was aHR 1.30 (95% CI: 1.18-1.43).Conclusion: The long-term risk for serious depression was higher among young and middle-aged patients with ACHD compared with matched controls. The risk was particularly elevated among patients with complex lesions. Patients with ACHD need support through strategies to prevent depression.
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| 12. |
- Ozturk, Ayse-Gul, et al.
(författare)
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Long-term survival in patients with univentricular heart: A nationwide, register-based cohort study
- 2024
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Ingår i: INTERNATIONAL JOURNAL OF CARDIOLOGY CONGENITAL HEART DISEASE. - 2666-6685. ; 15
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Tidskriftsartikel (refereegranskat)abstract
- Background: Children with univentricular heart (UVH) have a limited life expectancy without early treatment. Long-term survival in UVH, in an unselected nationwide cohort, is unclear. Objectives: To determine long-term survival in patients with UVH including non -operated patients compared with a control population in Sweden. Methods: Patients with UVH born between 1970 and 2017 were identified from the National Registers and were matched for birth year and sex with 10 individuals without congenital heart disease. Follow-up was from birth until death, transplantation, or the end of study. Mortality risk was estimated by Cox proportional regression models and Kaplan-Meier survival analysis. Results: We included 5075 patients with UVH including 758 (14.9%) patients with hypoplastic left heart syndrome (HLHS), and 50,620 matched controls. Median follow-up time was 13.6 (IQR 0.7; 26.8) years. The hazard ratio for death in patients with UVH was 53.0 (95% confidence interval, 48.0-58.6), and for HLHS, 163.5 (95% CI, 124.3-215.2). In patients with HLHS, 84% of those who were born between 1982 and 1993 died or had transplantation during the first year of life compared with 29% born between 2006 and 2017. In patients with UVH without HLHS, death/transplantation in the first year of life declined from 36% in those born between 1970 and 1981 to 8.7% in those born between 2006 and 2017. Conclusions: The risk of mortality was >50 times higher in patients with UVH than in controls. The survival rate increased with a later decade of birth but was still <75% in patients born with HLHS.
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| 13. |
- Pardhan, Salma, 1979, et al.
(författare)
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Healthcare consumption in congenital heart disease: A temporal life-course perspective following pediatric cases to adulthood
- 2023
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Ingår i: International Journal of Cardiology Congenital Heart Disease. - : Elsevier BV. - 2666-6685. ; 11
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Tidskriftsartikel (refereegranskat)abstract
- Background Improvements in diagnosis, intervention, and care of congenital heart disease (CHD) have led to increased survivability and lifelong dependence on healthcare. This study aims to determine the extensiveness of inpatient care episodes across different life-stages and CHD severity compared to matched controls, and to explore how healthcare utilization among pediatric CHD cases have changed over time. Methodology National registry data was used to conduct a 1:9 matching analysis with age and sex matched controls. Then, Poisson timeseries analysis was used to conduct trend analysis for inpatient healthcare utilization among pediatric cases <18 years of age. Results Most CHD cases were non-complex (87.3%), with highest hospitalization rates occurring in infancy. Mean number of hospitalizations among complex cases were over twice that of non-complex cases. Also, as age progressed, mean hospitalization for non-complex cases began converging to the control population. In terms of trend analysis within this study period, healthcare utilization increased by 34% among the infant categories, but decreased by 12% and 32% among children between 1-9 years and 10–17 years, respectively. Also, utilization was not trending in one direction substantiating the claim that multiple time periods are required to assess temporal changes within this population. Conclusion Inpatient healthcare utilization among the CHD population appears to be decreasing over time in most cases, where non-complex cases transitioning to adult care are increasingly converging to the general population. Additionally, this study validates the need to use multiple time-periods when conducting longitudinal studies across the CHD population.
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| 14. |
- Sandberg, Camilla, et al.
(författare)
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Patients with complex congenital heart disease have slower calf muscle oxygenation during exercise
- 2021
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Ingår i: International Journal of Cardiology Congenital Heart Disease. - : Elsevier. - 2666-6685. ; 4
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Tidskriftsartikel (refereegranskat)abstract
- BackgroundPeripheral muscle factors are presumed to be contributors to the reduced exercise capacity in congenital heart disease (CHD), but the underlying mechanisms are poorly understood. The aim was to investigate if muscle oxygenation, at rest and during exercise, and the resting blood flow in the calf muscle is impaired in adults with complex CHD in comparison to controls.MethodSeventy-four adults with complex CHD (35.6 ± 14.3 years, females n = 22 [30%], males n = 52 [70%]) and seventy-four age and sex matched subjects were recruited. Muscle oxygenation was successfully determined using near-infrared spectroscopy on the medial portion of m. gastrocnemius in 63 patients and 67 controls. Measurements were made at rest, during venous occlusion to estimate blood flow (indicated by slope increase of total haemoglobin, HbT), and post arterial occlusion. Additionally, measurements were made at the onset of isotonic unilateral heel-lifts to exhaustion and during recovery post exercise.ResultsAdults with CHD had a slower desaturation rate at exercise onset (−7.7 ± 4.3%StO2x3.5sec−1 vs. −11.7 ± 5.8%StO2x3.5sec−1, p < 0.001) a slower half recovery time (28.6 ± 21.2s vs. 16.8 ± 11.1s, p < 0.001) and a slower resaturation rate post exercise in comparison to the control subjects (3.9 ± 3.7%StO2x3.5sec−1 vs. 6.1 ± 3.8%StO2x3.5sec−1, p = 0.002). In contrast, there were no differences in muscle oxygen kinetics at rest.ConclusionSlower muscle oxygenation kinetics during muscle exercise found in adults with complex CHD may give insight to the mechanisms for the reduced exercise capacity commonly found in this population. This finding may also provide implications for design of exercise training programs targeting muscle function for these patients.
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| 15. |
- Sandström, Anette, et al.
(författare)
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Exercise capacity in adult patients with tetralogy of Fallot
- 2021
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Ingår i: International Journal of Cardiology Congenital Heart Disease. - : Elsevier. - 2666-6685. ; 5
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Tidskriftsartikel (refereegranskat)abstract
- Background: On a group level, patients with repaired tetralogy of Fallot have impaired exercise capacity. Since exercise capacity is related to prognosis, and more patients survive into higher age, it is important to know the expected exercise capacity at different ages. Furthermore, factors associated with exercise capacity and the relation between exercise capacity and mortality need further evaluation.Methods: The national register of congenital heart disease was searched for exercise tests in adults with repaired tetralogy of Fallot. The results from exercise tests were compared with national reference data.Results: 314 patients were identified (median age 33.2 [IQR 24.8-44.7], 40.8% women). The mean percent of predicted workload was 74.8 (±19.6) % without change across ages. In multivariable analysis, NYHA class I (odds ratio [OR]4.2, 95% confidence interval [CI]1.7-10.0) and higher physical activity level (>3 h/week) (OR 3.6, 95%CI 1.8-7.3) were positively associated with higher exercise capacity, while ongoing cardiovascular therapy (OR 0.4, 95%CI 0.2-0.7) and male sex (OR 0.3, 95%CI 0.2-0.6) were negatively associated with higher exercise capacity. Both exercise capacity (HR 0.96, 95%CI 0.93-0.98) and heart rate reserve (HR 0.96, 95%CI 0.94-0.98) were associated with mortality.Conclusions: In patients with repaired tetralogy of Fallot the exercise capacity was approximately 75% of expected, regardless of age. Patients with better NYHA class and high physical activity level had a higher exercise capacity. Low exercise capacity and low heart rate reserve were associated with higher mortality. Therefore, evaluating exercise capacity as part of follow up is of importance.
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