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- Fedchenko, Maria, 1988, et al.
(författare)
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Ischemic heart disease in children and young adults with congenital heart disease in Sweden
- 2017
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273. ; 248, s. 143-148
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Tidskriftsartikel (refereegranskat)abstract
- Background: An 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. Methods: Using 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. Results: We 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. Conclusions: In 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. (C) 2017 Elsevier B.V. All rights reserved.
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| 2. |
- Mandalenakis, Zacharias, 1979, et al.
(författare)
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Survivorship in Children and Young Adults With Congenital Heart Disease in Sweden
- 2017
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Ingår i: JAMA internal medicine. - : American Medical Association (AMA). - 2168-6106 .- 2168-6114. ; 177:2, s. 224-230
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Tidskriftsartikel (refereegranskat)abstract
- Importance: 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. Objective: To determine survivorship in children and young adults with CHD compared with matched controls. Design, Setting, and Participants: 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 21982 patients with CHD in Sweden were identified. The mean (SD) follow-up time was 27.0 (8.86) years. Children serving as controls (n = 219816) (10 for each patient), matched for birth year, sex, and county, were randomly selected from the general population. Main Outcomes and Measures: Survivorship in young patients with CHD and controls. Results: Of the 21982 patients who were born between 1970 and 1993 and were registered with the diagnosis of CHD, 10650 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). Conclusions and Relevance: 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.
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| 3. |
- Skoglund, Kristofer, 1976, et al.
(författare)
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Long-term outcome after right ventricle to pulmonary artery conduit surgery and reintervention.
- 2017
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Ingår i: Scandinavian cardiovascular journal : SCJ. - : Informa UK Limited. - 1651-2006 .- 1401-7431. ; 51:5, s. 284-291
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Tidskriftsartikel (refereegranskat)abstract
- Reconstruction of the right ventricular outflow tract with a conduit is an established surgical procedure in congenital heart disease and reinterventions are common.An increasing number of patients have a conduit, but there are few population-based studies of long-term outcomes after conduit surgery, reoperations, and transcatheter pulmonary valve replacement.In April 2015, all adult patients with a conduit were identified in the Swedish National Registry for Congenital Heart Disease (SWEDCON). Data on patients who died before age of 16 years are not included in the registry and thus not included in the study.We found 574 patients with a mean age 36.1 years. The largest proportion had tetralogy of Fallot (45%). In total there were 762 operations and 50 transcatheter pulmonary valve replacements. Mean age at first conduit operation was 20.2 years. Long-term survival up to 48 years including perioperative mortality (<1%) was 93% at 20 years. The most common cause of death was cardiac-related. Higher age at first conduit operation was associated with increased mortality risk. Reintervention-free survival was 77% and 54% at 10 and 20 years, respectively. Conduit reinterventions were common. Ten-year reintervention-free survival after first conduit reintervention (n=176) was significantly lower than after first conduit operation (70% vs 77% p=.04). Higher age at first conduit operation was associated with a reduced risk of reintervention, whereas male sex and complex malformations were associated with increased risk of reintervention.The mortality of repeated conduit reinterventions is low. The need for reintervention of conduits is considerable, and reintervention-free survival after the first conduit reintervention is poorer than after first conduit implantation. The findings in this study only applies for patients reaching 16 years of age.
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| 4. |
- Skoglund, Kristofer, 1976, et al.
(författare)
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RV to PA conduits: impact of transcatheter pulmonary valve replacement in adults - a national register study
- 2017
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Ingår i: Scandinavian Cardiovascular Journal. - : Informa UK Limited. - 1401-7431 .- 1651-2006. ; 51:3, s. 153-158
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Tidskriftsartikel (refereegranskat)abstract
- Objective: The use of a conduit is an established surgical method for reconstruction of the right ventricular outflow tract in congenital heart disease; however, its limited durability makes reintervention almost inevitable. We studied the trends in new implantation, reoperation, and transcatheter pulmonary valve replacement (TPVR) from a Swedish national perspective. Design and results: The Swedish registry of congenital heart disease (SWEDCON) was used to collect data. From 2000-2014, there was an increase in adult patients with conduits from 122 to 536. There were 60 surgical conduit replacements, 40 TPVRs and 176 new conduit implantations in the study period. Perioperative mortality was < 1%. The yearly number of new implantations and reoperations both doubled over the study period. Patients with new implantations were older (mean age, 36 years) compared with the reoperation and TPVR groups (mean age, 26 years) with the majority of patients having tetralogy of Fallot. The majority of conduit reinterventions were surgical also after the introduction of TPVR in 2007, with no significant difference regarding diagnosis, gender, age, or previous number or longevity of conduits. Conclusion: The number of adults with conduits increased steadily with most conduit-related operations being new implantations in patients with tetralogy of Fallot. Surgical conduit replacements increased significantly and represented the majority of conduit reinterventions after the introduction of TPVR, indicating that TPVR is not used for the majority of patients with conduit failure. Diagnosis, gender, age, or previous number or longevity of conduits had no impact on choosing surgical replacement vs. TPVR.
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