| 1. |
- Hebert, Anders, et al.
(författare)
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Hemodynamic causes of exercise intolerance in Fontan patients.
- 2014
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273. ; 175:3, s. 478-483
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Tidskriftsartikel (refereegranskat)abstract
- Exercise intolerance is frequent among Fontan patients and an important determinant for quality of life. This study investigated the hemodynamic causes of impaired exercise capacity in Fontan patients with particular focus on the influence of stroke volume index (SVI) and heart rate (HR).
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| 2. |
- Jensen, A. S., et al.
(författare)
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Fibrinogen function is impaired in whole blood from patients with cyanotic congenital heart disease
- 2013
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273 .- 1874-1754. ; 167:5, s. 2210-2214
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Tidskriftsartikel (refereegranskat)abstract
- Background: Patients with cyanotic congenital heart disease (CCHD) have haemostatic abnormities associated with bleeding and thrombo-embolic events. The haemostatic abnormalities are not fully understood, but recent studies indicate that elevated haematocrit and fibrinogen function may be of importance. The aim of this study was to characterise the haemostatic profile and examine the potential role of haematocrit on clot formation and strength in CCHD patients. Furthermore to examine whether CCHD patients with history of haemoptysis have diminished fibrinogen function compared to those without haemoptysis. Methods: In a prospective study 75 adult CCHD patients had haematocrit, platelet count, and plasma fibrinogen concentration examined. Furthermore thrombelastography(TEG) as well as TEG Functional Fibrinogen(TEG FF) assay evaluating fibrinogen function(FLEV) was performed. Data were compared with historical data regarding previous haemoptysis in CCHD patients. Results: Haematocrit was 57 +/- 8% and platelet counts in the lower normal range. TEG revealed a hypocoagulable condition with impaired clot formation. TEG values were correlated to haematocrit, indicating that elevated haematocrit causes impaired clot formation and strength. Despite high levels of plasma fibrinogen, TEG FF demonstrated that FLEV was diminished and negatively correlated to haematocrit. Furthermore CCHD patients with previous history of haemoptysis had significantly lower FLEV compared to CCHD patients without haemoptysis. Conclusion: Patients with CCHD are hypocoagulable mainly due to impaired fibrinogen function. Despite a low platelet count, platelet function does not seem to be severely affected in CCHD patients. Haemostasis, and especially fibrinogen function, is negatively affected by elevated haematocrit, and fibrinogen function is diminished in CCHD patients with haemoptysis. (C) 2012 Elsevier Ireland Ltd. All rights reserved.
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| 3. |
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| 4. |
- Jensen, A. S., et al.
(författare)
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The haematocrit - an important factor causing impaired haemostasis in patients with cyanotic congenital heart disease
- 2013
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273 .- 1874-1754. ; 167:4, s. 1317-1321
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Tidskriftsartikel (refereegranskat)abstract
- Background: Patients with cyanotic congenital heart disease(CCHD) have haemostatic abnormalities, which result in an increased risk of bleeding. The cause is unknown, but recent studies have indicated that an elevated haematocrit, which is present in cyanotic patients, could be an important factor. The aim of this study was to characterize the haemostatic profile, examine how changes in haematocrit affect the haemostatic profile, and whether a haematocrit reduction could terminate bleeding in CCHD patients. Methods: This was a prospective, multicenter study. The haemostatic profile consisting of haematocrit, platelet count and thrombelastography(TEG) was characterized in ninety-eight CCHD patients. To evaluate the influence of haematocrit on the haemostatic profile, 21 of the patients underwent phlebotomy and 16 patients received treatment with an iron supplement. Furthermore ten patients with haemoptysis underwent phlebotomy. The haemostatic profile was reevaluated after interventions. Results: TEG revealed that patients with CCHD and elevated haematocrit were hypocoagulable due to reduced clot formation and strength. Furthermore a positive correlation between elevated haematocrit and hypocoagulability was present. Interventions such as phlebotomy and treatment with supplemental iron causing significant haematocrit changes confirmed the correlation between haematocrit and the haemostatic profile. Finally a haematocrit reduction by phlebotomy successfully terminated haemoptysis in ten CCHD patients. Conclusion: Patients with CCHD and elevated haematocrit are hypocoagulable. The hypocoagulable haemostatic profile is positively correlated to increasing haematocrit. An intervention, which increases or decreases haematocrit, changes the haemostatic profile. A haematocrit reduction seems to improve the haemostatic profile, and may thereby terminate bleeding. However, these results warrant further studies. (C) 2012 Elsevier Ireland Ltd. All rights reserved.
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| 5. |
- Larsson, David, et al.
(författare)
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The diagnostic and prognostic value of brain natriuretic peptides in adults with a systemic morphologically right ventricle or Fontan-type circulation.
- 2007
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273. ; 114:3, s. 345-351
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Tidskriftsartikel (refereegranskat)abstract
- Background: In acquired heart disease, brain natriuretic peptide (BNP) and N-Teminal pro-brain natriuretic peptide (NT-proBNP) are increasingly used as diagnostic and prognostic markers. In adult Congenital heart disease, the abnormal anatomy and physiology complicate assessment of cardiac function. We Studied the clinical correlates of measurement of natriuretic peptides (NP) in adults with a right ventricle in the systemic position or with Fontan-type physiology. Methods: A prospective longitudinal study (follow up time 23 +/- 13 months, mean +/- S.D.) was conducted in a specialised centre on 61 patients (age 26 +/- 8 years; NYHA class 1.5 +/- 0.6) including Senning/Mustard corrected transposition, congenitally corrected transposition and Fontan/ total cavopulmonary connection. Plasma NP concentration was compared with NYHA class, exercise capacity and echocardiographically determined systemic systolic ventricular function. Results: Neurohormone concentrations were generally elevated (mean=290% of upper reference limit) and related to NYHA class (P < 0.001, NYHA I vs. II-IV). No clinically significant relationship to ventricular function or exercise capacity was found however. An NP measurement could not predict the future Course of the disease in terms of functional status or ventricular function. Conclusion: In contrast to patients with acquired heart disease, measurement of NP seems to have low clinical value in adults with a right ventricle in the systemic position or with Fontan-type physiology.
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| 6. |
- Larsson, Lena, et al.
(författare)
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Aerobic capacity in adolescence is associated with time to intervention in adult men with atrial septal defects
- 2019
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Ingår i: International Journal of Cardiology. - : Elsevier. - 0167-5273 .- 1874-1754. ; 280, s. 57-60
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Tidskriftsartikel (refereegranskat)abstract
- Background: Atrial septal defect (ASD) is a congenital heart lesion that often remains undiagnosed until adulthood. The reasons for this may be multifactorial. It is, however, known that closure of a hemodynamically significant ASD improves exercise capacity. This study aimed to explore whether the aerobic capacity in late adolescence is associated with time to diagnosis and intervention in adult men with late diagnosis of an atrial shunt.Methods: The Swedish Military Conscription Service Register contains data on exercise tests performed in late adolescence. By linking these data with the National Patient Register, 254 men with a later intervention for an ASD were identified.Results: Interventions were performed at a mean of 26.5 +/- 7.9 years after the initial exercise tests. The mean absolute workload among those with a later diagnosed ASD was similar to those without a later diagnosed ASD (274 +/- 51W vs. 276 +/- 52 W, p = 0.49). Men with a higher exercise capacity (>= 1 SD) had their intervention earlier (21.9 +/- 8.6 years vs. 27.5 +/- 7.4 years, p < 0.001).Conclusions: The aerobic exercise capacity was similar in adolescent men with later interventions for ASD compared to the reference population. Furthermore, those with high exercise capacity appeared to be diagnosed earlier. Thus, low exercise capacity may not be a feature of ASD during adolescence, but rather develop later in life as a natural progression of the disease.
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| 7. |
- Rinnström, Daniel, 1982-, et al.
(författare)
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Left ventricular hypertrophy in adults with previous repair of coarctation of the aorta : association with systolic blood pressure in the high normal range
- 2016
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273 .- 1874-1754. ; 37, s. 369-369
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Tidskriftsartikel (refereegranskat)abstract
- Background: Arterial hypertension is common in adults with repaired coarctation of the aorta (CoA). The associations between the diagnosis of hypertension, actual blood pressure, other factors affecting left ventricular overload, and left ventricular hypertrophy (LVH) are not yet fully explored in this population. Material and results: From the national register for congenital heart disease, 506 adult patients (>= 18 years old) with previous repair of CoA were identified (37.0% female, mean age 35.7 +/- 13.8 years, with an average of 26.8 +/- 12.4 years post repair). Echocardiographic data were available for all patients, and showed LVH in 114 (22.5%) of these. Systolic blood pressure (SBP) (mm Hg) (OR 1.02, CI 1.01-1.04), aortic valve disease, (OR 2.17, CI 1.33-3.53), age (years) (OR 1.03, CI 1.01-1.05), diagnosis of arterial hypertension (OR 3.02, CI 1.81-5.02), and sex (female) (OR 0.41, CI 0.24-0.72) were independently associated with LVH. There was an association with LVH at SBP within the upper reference limits [ 130, 140] mm Hg (OR 2.23, CI 1.05-4.73) that further increased for SBP > 140 mm Hg (OR 8.02, CI 3.76-17.12). Conclusions: LVH is common post repair of CoA and is associated with SBP even below the currently recommended target level. Lower target levels may therefore become justified in this population. ORCID Id: 0000-0003-0976-6910
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| 8. |
- Sandberg, Camilla, et al.
(författare)
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Height, weight and body mass index in adults with congenital heart disease
- 2015
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273 .- 1874-1754. ; 187, s. 219-226
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Tidskriftsartikel (refereegranskat)abstract
- Background: High BMI is a risk factor for cardiovascular disease and, in contrast, low BMI is associated with worse prognosis in heart failure. The knowledge on BMI and the distribution in different BMI-classes in adults with congenital heart disease (CHD) are limited. Methods and results: Data on 2424 adult patients was extracted from the Swedish Registry on Congenital Heart Disease and compared to a reference population (n = 4605). The prevalence of overweight/obesity (BMI >= 25) was lower in men with variants of the Fontan procedure, pulmonary atresia (PA)/double outlet right ventricle (DORV) and aortic valve disease (AVD) (Fontan 22.0% and PA/DORV 15.1% vs. 43.0%, p = 0.048 and p < 0.001) (AVD 37.5% vs. 49.3%, p < 0.001). Overt obesity (BMI >= 30) was only more common in women with AVD (12.8% vs. 9.0%, p = 0.005). Underweight (BMI < 18.5) was generally more common in men with CHD (complex lesions 4.9% vs. 0.9%, p < 0.001 and simple lesions 3.2% vs. 0.6%, <0.001). Men with complex lesions were shorter than controls in contrast to females that in general did not differ from controls. Conclusion: Higher prevalence of underweight in men with CHD combined with a lower prevalence of over-weight/obesity in men with some complex lesions indicates that men with CHD in general has lower BMI compared to controls. In women, only limited differences between those with CHD and the controls were found. The complexity of the CHD had larger impact on height in men. The cause of these gender differences as well as possible significance for prognosis is unknown. (C) 2015 Elsevier Ireland Ltd. All rights reserved.
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| 9. |
- Sandberg, Camilla, et al.
(författare)
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Low bone mineral density in adults with complex congenital heart disease
- 2020
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Ingår i: International Journal of Cardiology. - : Elsevier. - 0167-5273 .- 1874-1754. ; 319, s. 62-66
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Tidskriftsartikel (refereegranskat)abstract
- Aims: The majority of children with complex congenital heart disease (CHD) survive into adulthood due to advances in medical care. Adult patients with CHD have an increased incidence of diagnoses related to ageing such as heart failure, dementia, cancer and sarcopenia, despite a relatively low age. They also have a shorter life expectancy. It is unknown if their bone structures also show signs of premature ageing. We therefore investigated Bone Mineral Content (BMC) and bone mineral density (BMD) in an adult population with complex CHD.Methods: The total body BMC and BMD was examined using dual energy X-ray absorptiometry (DXA) in 73 adults with complex CHD (mean age 35.8 ± 14.3, women n = 22) and 73 age and sex matched controls.Results: The adults with complex CHD had lower total body BMC (2.6 ± 0.5 kg vs. 2.9 ± 0.5 kg, p < 0.001) and BMD (1.18 ± 0.12 g/cm2 vs. 1.26 ± 0.11 g/cm2, p < 0.001) compared to controls. BMD was lower for patients with single ventricle physiology and for the other complex diagnoses, and it persisted after correction for most common risk factors for osteoporosis.Conclusion: Adults with complex CHD have reduced total body BMC and BMD compared to healthy controls. These results are a sign of frailty that conforms with other previously reported signs of premature ageing. The risk of osteoporosis is low in our relatively young population, but it is assumed to increase with ageing. We recommend that clinicians pay close attention to risk factors for osteoporosis, and are generous in administering DXA-measurements in order to prevent future fractures among adults with complex CHD.
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| 10. |
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