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  • 1.
    Andersson, Jonas
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Mellberg, Caroline
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Otten, Julia
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Ryberg, Mats
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Rinnström, Daniel
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Larsson, Christel
    Lindahl, Bernt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Occupational and Environmental Medicine.
    Hauksson, Jon
    Umeå University, Faculty of Medicine, Department of Radiation Sciences. d Department of Radiography and Biomedical Science, Faculty of Medicine, University of Iceland, Reykjavik, Iceland.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Olsson, Tommy
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Left ventricular remodelling changes without concomitant loss of myocardial fat after long-term dietary intervention2016In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 216, 92-96 p.Article in journal (Refereed)
    Abstract [en]

    Background: Accumulation of myocardial triglycerides (MTG) is associated with impaired left ventricular (LV) remodelling and function in obese and diabetic subjects. The role of MTG accumulation in development of heart failure in this group of patients is unknown. Short-term studies suggest that diets that lead to weight loss could mobilize MTG, with a favourable effect on cardiac remodelling. In a 24-month, randomized, investigator-blinded study, we assessed the effect of two different diets and subsequent weight loss on cardiac function and MTG in postmenopausal women. Methods: Sixty-eight healthy postmenopausal women with body mass index [BMI] >= 27 kg/m(2) were randomized to an ad libitum Palaeolithic diet (PD) or a Nordic Nutrition Recommendation (NNR) diet for 24 months. Morphology, cardiac function, and MTG levels were measured using magnetic resonance (MR) scanning, including proton spectroscopy at baseline and 6 and 24 months. Results: Despite mean weight losses of 4.9 (1.0) kg (NNR) and 7.8 (1.1) kg (PD), the MTG content did not change over time (p = 0.98 in the NNR and p = 0.11 in the PD group at 24 months). Reduced left ventricular mass was observed in both diet groups over 24 months. Blood pressure was reduced at 6 months, but returned to baseline levels at 24 months. End diastolic volume, stroke volume, and cardiac output decreased over time. No differences between diet groups were observed. Conclusions: Diet intervention and moderate weight loss over 24 months improved LV remodelling but did not alter MTG levels in overweight/obese postmenopausal women.

  • 2. Apers, Silke
    et al.
    Kovacs, Adrienne H.
    Luyckx, Koen
    Alday, Luis
    Berghammer, Malin
    Budts, Werner
    Callus, Edward
    Caruana, Maryanne
    Chidambarathanu, Shanthi
    Cook, Stephen C.
    Dellborg, Mikael
    Enomoto, Junko
    Eriksen, Katrine
    Fernandes, Susan M.
    Jackson, Jamie L.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Khairy, Paul
    Kutty, Shelby
    Menahem, Samuel
    Rempel, Gwen
    Sluman, Maayke A.
    Soufi, Alexandra
    Thomet, Corina
    Veldtman, Gruschen
    Wang, Jou-Kou
    White, Kamila
    Moons, Philip
    Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS): Rationale, design, and methods2015In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 179, 334-342 p.Article in journal (Refereed)
    Abstract [en]

    Background: Data on patient-reported outcomes (PROs) in adults with congenital heart disease (CHD) are inconsistent and vary across the world. Better understanding of PROs and their differences across cultural and geographic barriers can best be accomplished via international studies using uniform research methods. The APPROACH-IS consortium (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study) was created for this purpose and investigates PROs in adults with CHD worldwide. This paper outlines the project rationale, design, and methods. Methods/design: APPROACH-IS is a cross-sectional study. The goal is to recruit 3500-4000 adults with CHD from 15 countries in five major regions of the world (Asia, Australia, Europe, North and South America). Self-report questionnaires are administered to capture information on PRO domains: (i) perceived health status (12-item Short-form Health Survey & EuroQOL-5D); (ii) psychological functioning (Hospital Anxiety and Depression Scale); (iii) health behaviors (Health-Behavior Scale-Congenital Heart Disease); and (iv) quality of life (Linear Analog Scale & Satisfaction With Life Scale). Additionally, potential explanatory variables are assessed: (i) socio-demographic variables; (ii) medical history (chart review); (iii) sense of coherence (Orientation to Life Questionnaire); and (iv) illness perceptions (Brief Illness Perception Questionnaire). Descriptive analyses and multilevel models will examine differences in PROs and investigate potential explanatory variables. Discussion: APPROACH-IS represents a global effort to increase research understanding and capacity in the field of CHD, and will have major implications for patient care. Results will generate valuable information for developing interventions to optimize patients' health and well-being. 

  • 3.
    Backman, Christer
    et al.
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Clinical Physiology.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Tossavainen, Erik
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Henein, Michael Y
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Unusual arrhythmogenic myocardial disease2014In: International Cardiovascular Forum Journal, ISSN 2410-2636, Vol. 1, no 4, 195-196 p.Article in journal (Refereed)
  • 4.
    Bäckstrom, Björn
    et al.
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Forensic Medicine. Department of Forensic Medicine, National Board of Forensic Medicine, PO Box 7616, SE-907 12, Umeå.
    Johansson, Bengt
    Eriksson, Anders
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Forensic Medicine.
    Death from Nitrous Oxide2015In: Journal of Forensic Sciences, ISSN 0022-1198, E-ISSN 1556-4029, Vol. 60, no 6, 1662-1665 p.Article in journal (Refereed)
    Abstract [en]

    Nitrous oxide is an inflammable gas that gives no smell or taste. It has a history of abuse as long as its clinical use, and deaths, although rare, have been reported. We describe two cases of accidental deaths related to voluntary inhalation of nitrous oxide, both found dead with a gas mask covering the face. In an attempt to find an explanation to why the victims did not react properly to oncoming hypoxia, we performed experiments where a test person was allowed to breath in a closed system, with or without nitrous oxide added. Vital signs and gas concentrations as well as subjective symptoms were recorded. The experiments indicated that the explanation to the fact that neither of the descendents had reacted to oncoming hypoxia and hypercapnia was due to the inhalation of nitrous oxide. This study raises the question whether nitrous oxide really should be easily, commercially available.

  • 5.
    Camilla, Sandberg
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Pomeroy, Jeremy
    Thilén, Ulf
    Gradmark, Anna
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Wadell, Karin
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Habitual Physical Activity in Adults with Congenital Heart Disease Compared with Age- and Sex- Matched Controls2016In: Canadian Journal of Cardiology, ISSN 0828-282X, E-ISSN 1916-7075, Vol. 32, no 4, 547-553 p.Article in journal (Refereed)
    Abstract [en]

    Background: Most adult patients with congenital heart disease (CHD) have reduced aerobic exercise capacity. Their habitual physical activity (PA) level is, however, less well studied. In this study habitual PA level in a cohort of adults with CHD compared to healthy age and gender matched controls was investigated.

    Methods: Eighty adults with CHD, classed as either “complex” (n=40) or “simple” (n=40), and 42 healthy controls were studied with a combined uniaxial accelerometer and heart rate monitor worn during 4 consecutive days. We analysed 1) the time spent during ≥ moderate/vigorous PA, 2) accelerometer counts/day and 3) to what extent the World Health Organization recommendations on PA were reached.

    Results: Patients with simple lesions had higher total accelerometer counts/day compared to both patients with complex lesions and controls (simple lesions; median (IQR) 107.7(63.4) vs. complex lesions; 72.8(53.5) and controls; 78.3(49.6), p≤0.001 and p=0.002). Furthermore, no differences in time spent during ≥ moderate-to-vigorous PA was found between patients and controls. In addition 46% of the patients with simple lesions, 55% of the patients with complex lesions and 44% of the controls did not reach the W.H.O.-recommended level of daily PA, but no significant differences between groups were found. There were no differences in achieving recommended PA level between patients in NYHA I vs. NYHA II+III.

    Conclusions: Patients with CHD follow the same PA-level pattern as the general population. Broad strategies promoting an active lifestyle are needed across the population and especially for patients with complex CHD and impaired NYHA class.

  • 6. Diller, Gerhard-Paul
    et al.
    Dimopoulos, Konstantinos
    Okonko, Darlington
    Li, Wei
    Babu-Narayan, Sonya V
    Broberg, Craig S
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Bouzas, Beatriz
    Mullen, Michael J
    Poole-Wilson, Philip A
    Francis, Darrel P
    Gatzoulis, Michael A
    Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication.2005In: Circulation, ISSN 0009-7322, E-ISSN 1524-4539, Vol. 112, no 6, 828-35 p.Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Although some patients with adult congenital heart disease (ACHD) report limitations in exercise capacity, we hypothesized that depressed exercise capacity may be more widespread than superficially evident during clinical consultation and could be a means of assessing risk.

    METHODS AND RESULTS: Cardiopulmonary exercise testing was performed in 335 consecutive ACHD patients (age, 33+/-13 years), 40 non-congenital heart failure patients (age, 58+/-15 years), and 11 young (age, 29+/-5 years) and 12 older (age, 59+/-9 years) healthy subjects. Peak oxygen consumption (peak VO2) was reduced in ACHD patients compared with healthy subjects of similar age (21.7+/-8.5 versus 45.1+/-8.6; P<0.001). No significant difference in peak VO2 was found between ACHD and heart failure patients of corresponding NYHA class (P=NS for each NYHA class). Within ACHD subgroups, peak VO2 gradually declined from aortic coarctation (28.7+/-10.4) to Eisenmenger (11.5+/-3.6) patients (P<0.001). Multivariable correlates of peak VO2 were peak heart rate (r=0.33), forced expiratory volume (r=0.33), pulmonary hypertension (r=-0.26), gender (r=-0.23), and body mass index (r=-0.19). After a median follow-up of 10 months, 62 patients (18.5%) were hospitalized or had died. On multivariable Cox analysis, peak VO2 predicted hospitalization or death (hazard ratio, 0.937; P=0.01) and was related to the frequency and duration of hospitalization (P=0.01 for each).

    CONCLUSIONS: Exercise capacity is depressed in ACHD patients (even in allegedly asymptomatic patients) on a par with chronic heart failure subjects. Lack of heart rate response to exercise, pulmonary arterial hypertension, and impaired pulmonary function are important correlates of exercise capacity, as is underlying cardiac anatomy. Poor exercise capacity identifies ACHD patients at risk for hospitalization or death.

  • 7.
    Hannuksela, Matias
    et al.
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Anaesthesiology.
    Stattin, Eva-Lena
    Umeå University, Faculty of Medicine, Department of Medical Biosciences, Medical and Clinical Genetics.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Carlberg, Bo
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Screening for familial thoracic aortic aneurysms with aortic imaging does not detect all potential aarriers of the disease2015In: Aorta, ISSN 2325-4637, Vol. 3, no 1, 1-8 p.Article in journal (Refereed)
    Abstract [en]

    Background: About 20% of patients with thoracic aortic aneurysm or dissection (TAAD) have a first-degree relative with a similar disease. The familial form (FTAAD) of the disease is inherited in an autosomal-dominant pattern. Current guidelines for thoracic aortic disease recommend screening of first-degree relatives of TAAD patients. In known familial disease, screening of both first- and second-degree relatives is recommended. However, the outcomes of such a screening program are unknown.

    Methods: We screened all first- and second-degree relatives in seven families with known FTAAD with echo- cardiography. No underlying gene defect had been detected in these families.

    Results: Of 119 persons investigated, 13 had known thoracic aortic disease. In the remaining 106 cases, we diagnosed 19 additional individuals with a dilated ascending thoracic aorta; for an autosomal-dominant disease, the expected number of individuals in this group would have been 40 (p<0.0001). Further, only one of the 20 first-degree relatives younger than 40 years had a dilated aorta, although the expected number of individuals with a disease-causing mutation would have been 10.

    Conclusions: In most families with TAAD, a diagnosis still relies on measuring the diameter of the thoracic aorta. We show that a substantial number of previously unknown cases of aortic dilatation can be identified by screening family members. It is, however, not possible to consider anyone free of the condition, even if the aortic diameter is normal, especially at a younger age.

  • 8.
    Hellström, Martin
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine. Umeå University, Faculty of Medicine, Department of Radiation Sciences, Oncology.
    Engström-Laurent, Anna
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Mörner, Stellan
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Hyaluronan and collagen in human hypertrophic cardiomyopathy: a morphological analysis2012In: Cardiology Research and Practice, ISSN 2090-8016, E-ISSN 2090-0597, Vol. 2012, 545219- p.Article in journal (Refereed)
    Abstract [en]

    The hypertrophic cardiomyopathy (HCM) disease process is not only limited to cardiomyocyte abnormalities but also engages the extracellular matrix. Hyaluronan (HA) and its receptor CD44 are involved in cellular growth and tissue proliferation but have so far been less studied in myocardial hypertrophy. In HCM, collagens are abundant but their histological distribution and relation to hyaluronan have not been described. Material and Methods. Myocardial specimens from 5 patients with symptomatic left ventricular tract obstruction undergoing myectomy due to HCM were processed for histochemistry and immunohistochemistry. Results. HA staining was more intense in HCM patients. The histological distribution of HA was the same in patients and controls, that is, interstitial staining including the space between cardiomyocytes, in fibrous septa, and in the adventitia of intramyocardial blood vessels. CD44 was not detected in the myocardium of patients or controls. Collagen I showed the same general localisation as HA but detailed distribution differed. Conclusions. This is the first study that describes the distribution of hyaluronan in human HCM. HA staining is more intense in HCM patients but without coexpression of its receptor CD44, at least not in the chronic phase of HCM. HA and collagen I have the same localisation.

  • 9.
    Hellström, Martin
    et al.
    Umeå University, Faculty of Medicine, Department of Radiation Sciences, Oncology.
    Ericsson, Madelene
    Umeå University, Faculty of Medicine, Department of Medical Biosciences, Physiological chemistry.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Faraz, Mahmood
    Umeå University, Faculty of Medicine, Department of Radiation Sciences, Oncology.
    Anderson, Fredrick
    Umeå University, Faculty of Medicine, Department of Medical Biosciences, Physiological chemistry.
    Henriksson, Roger
    Umeå University, Faculty of Medicine, Department of Radiation Sciences, Oncology. Regional Cancer Center Stockholm/Gotland, Stockholm, Sweden.
    Nilsson, Stefan K.
    Umeå University, Faculty of Medicine, Department of Medical Biosciences, Physiological chemistry.
    Hedman, Håkan
    Umeå University, Faculty of Medicine, Department of Radiation Sciences, Oncology.
    Cardiac hypertrophy and decreased high-density lipoprotein cholesterol in Lrig3-deficient mice2016In: American Journal of Physiology. Regulatory Integrative and Comparative Physiology, ISSN 0363-6119, E-ISSN 1522-1490, Vol. 310, no 11, R1045-R1052 p.Article in journal (Refereed)
    Abstract [en]

    Genetic factors confer risk for cardiovascular disease. Recently, large genome-wide population studies have shown associations between genomic loci close to LRIG3 and heart failure and plasma high-density lipoprotein (HDL) cholesterol level. Here, we ablated Lrig3 in mice and investigated the importance of Lrig3 for heart function and plasma lipid levels. Quantitative reverse transcription-polymerase chain reaction (RT-PCR) was used to analyze Lrig3 expression in the hearts of wild-type and Lrig3-deficient mice. In addition, molecular, physiological, and functional parameters such as organ weights, heart rate, blood pressure, heart structure and function, gene expression in the heart, and plasma insulin, glucose, and lipid levels were evaluated. The Lrig3-deficient mice were smaller than the wild-type mice but otherwise appeared grossly normal. Lrig3 was expressed at detectable but relatively low levels in adult mouse hearts. At 9 mo of age, ad libitum-fed Lrig3-deficient mice had lower insulin levels than wildtype mice. At 12 mo of age, Lrig3-deficient mice exhibited increased blood pressure, and the Lrig3-deficient female mice displayed signs of cardiac hypertrophy as assessed by echocardiography, heart-to-body weight ratio, and expression of the cardiac hypertrophy marker gene Nppa. Additionally, Lrig3-deficient mice had reduced plasma HDL cholesterol and free glycerol. These findings in mice complement the human epidemiological results and suggest that Lrig3 may influence heart function and plasma lipid levels in mice and humans.

  • 10.
    Hellström, Martin
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Engström Laurent, Anna
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Hyaluronan and its receptor CD44 in the heart of newborn and adult rats.2006In: Anatomical record part A: discoveries in molecular, cellular, and evolutionary biology, ISSN 1552-4884, Vol. 288, no 6, 587-592 p.Article in journal (Refereed)
  • 11.
    Henein, Michael
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Bengrid, Tarek
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Nicoll, Rachel
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Zhao, Y.
    Ultrasound Department, Capital Medical University, Beijing, China.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Schmermund, A.
    Medicine, Bethanien Hospital, Frankfurt, Germany.
    Extensive coronary calcification compromises myocardial perfusion in the absence of high grade stenosis2014In: Atherosclerosis, ISSN 0021-9150, E-ISSN 1879-1484, Vol. 235, no 2, E68-E68 p.Article in journal (Other academic)
  • 12.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Cardiovascular Magnetic Resonance in Heart Failure2010In: Heart Failure in Clinical Practice / [ed] Michael Y. Henein, Springer London, 2010, 241-253 p.Chapter in book (Refereed)
    Abstract [en]

    Cardiovascular magnetic resonance (CMR) is an increasingly used imaging tool in cardiology. Initially, technical resources and access to trained staff were limited to a few centers. As scanners become more available and thanks to widespread training of cardiologists and technicians, CMR can now be performed in most cardiology centers. Without exposing the patient to ionizing radiation, a comprehensive cardiac study can be performed in 30–45 min, depending on clinical problem and scanner capabilities. A typical cardiac exam consists of anatomical images for gross anatomy and cine images for the evaluation of ventricular function. Depending on the clinical problem, a number of additional techniques can be added. Using chelated gadolinium contrast agents, first pass myocardial perfusion can be analyzed during pharmacological stress for the diagnosis of coronary artery disease. After injection of contrast in a peripheral vein, high resolution angiography can be performed. Phase contrast velocity maps (which despite the terminology it does not include contrast agents) can also be used to analyze intrathoracic vascular blood flow and flow velocity.

  • 13.
    Johansson, Bengt
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Babu-Narayan, Sonya V
    Kilner, Philip J
    The effects of breath-holding on pulmonary regurgitation measured by cardiovascular magnetic resonance velocity mapping.2009In: Journal of Cardiovascular Magnetic Resonance, ISSN 1097-6647, E-ISSN 1532-429X, Vol. 11, no 1, 1- p.Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Pulmonary regurgitation is a common and clinically important residual lesion after repair of tetralogy of Fallot. Cardiovascular magnetic resonance (CMR) phase contrast velocity mapping is widely used for measurement of pulmonary regurgitant fraction. Breath-hold acquisitions, usually acquired during held expiration, are more convenient than the non-breath-hold approach, but we hypothesized that breath-holding might affect the amount of pulmonary regurgitation. METHODS: Forty-three adult patients with a previous repair of tetralogy of Fallot and residual pulmonary regurgitation were investigated with CMR. In each, pulmonary regurgitant fraction was measured from velocity maps transecting the pulmonary trunk, acquired during held expiration, held inspiration, by non-breath-hold acquisition, and also from the difference of right and left ventricular stroke volume measurements. RESULTS: Pulmonary regurgitant fraction was lower when measured by velocity mapping in held expiration compared with held inspiration, non-breath-hold or stroke volume difference (30.8 vs. 37.0, 35.6, 35.4%, p = 0.00017, 0.0035, 0.026). The regurgitant volume was lower in held expiration than in held inspiration (41.9 vs. 48.3, p = 0.0018). Pulmonary forward flow volume was larger during held expiration than during non-breath-hold (132 vs. 124 ml, p = 0.0024). CONCLUSION: Pulmonary regurgitant fraction was significantly lower in held expiration compared with held inspiration, free breathing and stroke volume difference. Altered airway pressure could be a contributory factor. This information is relevant if breath-hold acquisition is to be substituted for non-breath-hold in the investigation of patients with a view to re-intervention.

  • 14.
    Johansson, Bengt
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Babu-Narayan, Sonya V
    Kilner, Philip J
    Cannell, Timothy M
    Mohiaddin, Raad H
    3-dimensional time-resolved contrast-enhanced magnetic resonance angiography for evaluation late after the mustard operation for transposition2010In: Cardiology in the Young, ISSN 1047-9511, E-ISSN 1467-1107, Vol. 20, no 1, 1-7 p.Article in journal (Refereed)
    Abstract [en]

    3-dimensional dynamic angiography is a useful method for detecting anatomically moderate-to-severe, but not mild, obstructions in the systemic venous channels following Mustard repair for transposition. This technique can be used as a single imaging method and/or as complimentary to standard two dimensional cardiovascular magnetic resonance techniques for detection of clinically important obstructions in the systemic venous channels.

  • 15.
    Johansson, Bengt
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Bédard, E
    Intracardiac Thrombus in the fontan circulation2010In: Cases in adult congenital heart disease / [ed] Gatzoulis M Michael A., Gary D. Webb, MD, Craig Broberg, MD and Hideki Uemura, London: Churchill Livingstone , 2010Chapter in book (Other academic)
  • 16.
    Johansson, Bengt
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Engström, Karl-Gunnar
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Surgery.
    Dellborg, M.
    Kronvall, T.
    Kvidal, P.
    Mattsson, E.
    Thilen, U.
    Functional class, symptoms, medications, arrhythmia devices and quality of life in adults with congenital aortic valve disease. Data from the national registry of congenital heart disease2013In: European Heart Journal, ISSN 0195-668X, E-ISSN 1522-9645, Vol. 34, no Supplement: 1, 375-375 p.Article in journal (Other academic)
    Abstract [en]

    Purpose: Despite the relative high prevalence of congenital aorticvalve disease, the outcome in contemporary cohorts of adults is not well established. In the present study, we have analysed data in the National Registry on Adult Congenital Heart Disease in order to elucidate the long-term outcome regarding functional class,symptoms, quality of life, medications and need for arrhythmiadevices in this cohort.

    Methods: Six hundred fifty-one adult patients with isolated congenitalaortic valve disease met the criteria and were grouped according to: if their first aortic valve intervention was < 18 years (group 1) (n=152), first aortic valve intervention > 18 years (group 2) (n=129) or no aorticvalve intervention (group 0) (n=370).

    Results: 92% of the patients were in NYHA I. Symptoms were reportedin 12.7% but more commonly in group 2 compared with group 1 (20.7% vs. 9.6%, p = 0.039). The overall quality of life assessed with EQ-VAS was 90% and equal between groups. The use of cardiovascularmedications, anticoagulation excluded, was higher in group 2 than ingroups 0 and 1 (29.1% vs. 9.1% and 11.6%, p = 0.001, p < 0.001). Warfarin was prescribed in 55.3% of the patients in group 2, in 34.5% ingroup 1 and 1.7% in group 0 (p < 0.001 for all comparisons) whichindicates that non-mechanical valve prostheses or other alternatives are common in group 1 and 2. Implanted arrhythmia devices were more common in group 2 compared with group 0 (5.1 vs. 0.6%, p = 0.01).

    Conclusion: Functional status and quality of life is generally goodand not obviously related to previous interventions. Symptoms, cardiovascular medications, including warfarin, and anti-arrhythmiadevices were more common in patients with their initial valveintervention in adult age. Many patients with a previous intervention have alternatives to mechanical heart valve prostheses and may thus need future re-interventions.

  • 17.
    Johansson, Bengt
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Henein, Michael
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Damus-Kaye-Stansel anastomosis in a patient with tricuspid atresia, transposition of the great arteries, VSD and total cavo-pulmonary connection (TCPC).2009In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 140, no 3, e43-e44 p.Article in journal (Refereed)
    Abstract [en]

    A Damus-Kaye-Stansel (DKS) anastomosis, i.e. an end-to-side anastomosis between the pulmonary artery and the aorta, has been applied in a wide spectrum of congenital heart disease including the Fontan circulation. We hereby present a 19-year-old woman with tricuspid atresia, transposition of great arteries, hypoplastic right ventricle, and a ventricular septal defect who was operated with total cavo-pulmonary connection (TCPC) and a DKS anastomosis. The Cardiovascular Magnetic Resonance (CMR) study showed that the systemic ventricular outflow tract is not obstructed with a good overall result of the previous interventions. CMR therefore, is an ideal mean for studying detailed anatomy and physiology without any need for radiation or contrast media.

  • 18.
    Johansson, Bengt
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Holmer, Fredrik
    Department of Thoracic Surgery, Umeå University Hospital, Umeå, Sweden.
    Ottander, Per
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Lund, Björn
    Diagnostic Radiology, Umeå University Hospital, Umeå, Sweden.
    Henein, Michael Y
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    CMR diagnosis of coronary graft fistula2014In: International Cardiovascular Forum Journal, ISSN 2410-2636, Vol. 1, no 3, 157-158 p.Article in journal (Refereed)
  • 19.
    Johansson, Bengt
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Holmgren, Anders
    Hedström, Magnus
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Engström-Laurent, Anna
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Engström, Karl Gunnar
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Surgery.
    Evaluation of hyaluronan and calcifications in stenotic and regurgitant aortic valves.2011In: European Journal of Cardio-Thoracic Surgery, ISSN 1010-7940, E-ISSN 1873-734X, Vol. 39, no 1, 27-32 p.Article in journal (Refereed)
    Abstract [en]

    Objective: Hyaluronan (HA) is a major component of the interstitium and has been observed in normal heart valves. The function of HA in heart valves is unknown but contribution to biomechanical function has been proposed. The purpose of this investigation was to study the distribution of HA in relation to calcifications in diseased human aortic valves. Methods: Human aortic valves were collected at aortic valve replacement, of whom nine patients had regurgitation and 13 stenotic disease. The valves were decalcified and stained for the visualisation of HA. The specimens were macroscopically evaluated for magnitude of calcification using image analysis. The microscopic amount and distribution of HA and calcifications were semiquantitatively evaluated using histochemistry. Results: The overall HA staining showed an inverse relationship against the magnitude of observed valve calcifications (p<0.001) and type of disease (p=0.014). Multiple-group comparison revealed regionally reduced HA staining in diffuse and heavy calcified regions inside the valve (both p<0.001) compared with normal-structured parts of the valve. HA was concentrated on the ventricular side of the valve (p=0.002). Conclusions: The content of HA was reduced in calcified aortic valves and had a heterogeneous distribution, potentially contributing to poor valve function. HA may also be involved in the pathophysiological process in degenerative aortic stenosis.

  • 20.
    Johansson, Bengt
    et al.
    Umeå University, Faculty of Medicine, Public Health and Clinical Medicine.
    Maceira, Alicia M
    Babu-Narayan, Sonya V
    Moon, James C
    Pennell, Dudley J
    Kilner, Philip J
    Clefts can be seen in the basal inferior wall of the left ventricle and the interventricular septum in healthy volunteers as well as patients by cardiovascular magnetic resonance.2007In: Journal of the American College of Cardiology, ISSN 0735-1097, E-ISSN 1558-3597, ISSN 1558-3597, Vol. 50, no 13, 1294-1295 p.Article in journal (Refereed)
  • 21.
    Johansson, Bengt
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine. Umeå University, Faculty of Medicine, Department of Integrative Medical Biology (IMB), Anatomy.
    Mörner, Stellan
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Waldenström, Anders
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Stål, Per
    Umeå University, Faculty of Medicine, Department of Integrative Medical Biology (IMB), Anatomy.
    Myocardial capillary supply is limited in hypertrophic cardiomyopathy: a morphological analysis2008In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 126, no 2, 252-257 p.Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: To clarify the morphological basis of the limited coronary reserve in hypertrophic cardiomyopathy (HCM). BACKGROUND: Some of the symptoms in Hypertrophic cardiomyopathy (HCM), such as chest pain, dyspnea and arrhythmia, may be explained by myocardial ischemia. Many patients with HCM are known to exhibit these symptoms in the absence of atherosclerosis in the major coronary vessels. Decreased myocardial perfusion has been demonstrated in HCM, however, little is known about the myocardial capillary morphology in this disease. METHODS: Using immunohistochemistry and morphometry, we analysed capillaries and cardiomyocytes in myectomy specimens from 5 patients with HCM with moderate hypertrophy and left ventricular outflow tract obstruction and in 5 control hearts. RESULTS: The number of capillaries per cardiomyocyte (p<0.009) and number of capillaries per cardiomyocyte area unit, reflecting cardiomyocyte mass (p=0.009), were lower in individuals with HCM, i.e. indicating loss of capillaries. In HCM, the capillary density was 33% lower (p<0.05). CONCLUSIONS: Our morphologic findings show that the capillary supply, and thus the coronary reserve, is impaired in HCM with moderate hypertrophy and left ventricular outflow tract obstruction. These data may partly explain the limitation of myocardial perfusion in HCM, which is associated with worse prognosis. Furthermore, we present evidence of actual loss of myocardial capillaries in HCM and a defective capillary growth.

  • 22.
    Lysell-Bergström, Catharina
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Widman, Lars
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Johnson, Owe
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Mörner, Stellan
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Progressive conduction disease late after adriamycin treatment of lymphoma, despite normalised ejection fraction2014In: International Cardiovascular Forum Journal, ISSN 2410-2636, Vol. 1, no 3, 160-161 p.Article in journal (Refereed)
  • 23.
    Mörner, Stellan
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Henein, Michael
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Arrhythmogenic left ventricular apical aneurysm in hypertrophic cardiomyopathy2010In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 151, no 1, e8-e9 p.Article in journal (Refereed)
    Abstract [en]

    A 70-year old lady with prior myectomy for hypertrophic obstructive cardiomyopathy presented with sustained ventricular tachycardia. She was found to have a large left ventricular (LV) apical aneurysm. Surgical intervention was not advised, due to the risk of creating a small LV cavity after surgery and ICD was not advised based on the risk of injuring a very thin walled aneurysm. The patient's arrhythmia settled on medical therapy, but unfortunately she suffered an unwitnessed death three months later.

    This case represents a rare complication to a rare disease with limited management options. In such patients evidence based medicine is of little help, if any.

  • 24.
    Mörner, Stellan
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Henein, Michael
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Letter to editor: Tako-tsubo cardiomyopathy in the setting of pre-existing myocardial disease2010In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 145, no 3, 605- p.Article, review/survey (Other academic)
  • 25.
    Nilsson, Ulf
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Eriksson, Berne
    Göteborgs Universitet.
    Blomberg, Anders
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Pulmonary Medicine.
    Lundbäck, Bo
    Göteborgs Universitet.
    Lindberg, Anne
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Pulmonary Medicine.
    Ischemic heart disease among subjects with and without chronic obstructive pulmonary disease: ECG-findings in a population-based cohort study2015In: BMC Pulmonary Medicine, ISSN 1471-2466, E-ISSN 1471-2466, Vol. 15, 156Article in journal (Refereed)
    Abstract [en]

    Background: Cardiovascular comorbidity in COPD is common and contributes to increased mortality. A few population-based studies indicate that ischemic electrocardiogram (ECG)-changes are more prevalent in COPD, while others do not.

    The aim of the present study was to estimate the presence of ischemic heart disease (IHD) in a population-based COPD-cohort in comparison with subjects without COPD.

    Methods: All subjects with obstructive lung function (COPD, n = 993) were identified together with age- and sex-matched controls (non-COPD, n = 993) from population-based cohorts examined in 2002–04. In 2005, data from structured interview, spirometry and ECG were collected from 1625 subjects. COPD was classified into GOLD 1–4 after post-bronchodilator spirometry. Ischemic ECG-changes, based on Minnesota-coding, were classified according to the Whitehall criteria into probable and possible IHD.

    Results: Self-reported IHD was equally common in COPD and non-COPD, and so were probable and possible ischemic ECG-changes according to Whitehall. After excluding subjects with restrictive spirometric pattern from the non-COPD-group, similar comparison with regard to presence of IHD performed between those with COPD and those with normal lung-function did neither show any differences. There was a significant association between self-reported IHD (p = 0.007) as well as probable ischemic ECG-changes (p = 0.042), and increasing GOLD stage. In COPD there was a significant association between level of FEV1 percent of predicted and self-reported as well as probable ischemic ECG-changes, and this association persisted for self-reported IHD also after adjustment for sex and age.

    Conclusion: In this population-based study, self-reported IHD and probable ischemic ECG-changes were associated with COPD disease severity assessed by spirometry.

  • 26.
    Rinnström, Daniel
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Engström, Gunnar
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Surgery. Heart centre, Umeå university.
    Ugander, Martin
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Factors associated with left ventricular hypertrophy in adults with surgically repaired coarctation of the aorta2013In: International Cardiovascular Forum Journal, ISSN 2410-2636, Vol. 1, no 2, 79-83 p.Article in journal (Refereed)
    Abstract [en]

    Introduction: Most patients with repaired coarctation of the aorta (CoA) live normal lives and have good physical performance. However, even after a successful surgical intervention, long-term cardiovascular risks including left ventricular hypertrophy remain. The aim of the study was to identify factors associated with increased left ventricular mass (LVM) in patients with surgically repaired CoA.

    Methods: Consecutive cardiovascular magnetic resonance investigations in 51 patients with surgically repaired CoA (age 37+/-15 years, age at intervention 9.7 ± 6.8 years, 45% female) were reviewed. LVM was measured and indexed to body surface area. The association between increased LVM index and clinical, anatomic and functional variables was investigated with logistic regression analysis.

    Results: In this population, 14/51 (27%) patients had a LVM index above normal limits. Factors associated with an increased LVM index in univariate analysis were higher systolic blood pressure (odds ratio (OR) = 1.04, 95 % confidence interval(CI) 1.00-1.08, p = 0.03), descending aortic diameter (OR = 1.48, CI 1.14-1.90, p = 0.003) and more than mild aortic valve disease or previous aortic valve intervention (OR = 15.1, CI 2.50-48.4, p=0.002), but not diastolic blood pressure, diameter of ascending aorta, diameter or ratio of CoA, velocity in descending aorta, smoking or bicuspid aortic valve (p > 0.05 for all). In multivariate analysis, only systolic blood pressure (p = 0.05) and aortic valve disease (p = 0.006) remained significant, yielding R2 = 0.47, p = 0.002 for the model.

    Conclusion: Increased LVM is a common late finding after surgically repaired CoA. This study showed that LVM was associated with modifiable factors; systolic blood pressure and aortic valve disease. As most patients are young, and increased LVM will eventually affect ventricular function, close attention to blood pressure optimization may be of particular importance in the surgically repaired CoA population.

  • 27.
    Rinnström, Daniel
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Heart Centre, Umeå.
    Engström, Karl Gunnar
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Surgery. Cardiothoracic Surgery, Heart Centre.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Heart Centre, Umeå.
    Subtypes of bicuspid aortic valves in coarctation of the aorta2014In: Heart and Vessels, ISSN 0910-8327, E-ISSN 1615-2573, Vol. 29, no 3, 354-363 p.Article in journal (Refereed)
    Abstract [en]

    Bicuspid aortic valves (BAVs) represent a wide morphologic and functional spectrum. In coarctation of the aorta, BAVs are common, but the proportion of BAV subtypes and their relation to aortic dimensions and development of late valve dysfunction are unknown. Sixty-two cardiovascular magnetic resonance investigations of patients with coarctation of the aorta were reviewed with respect to aortic valve morphology, aortic valve function, and aortic dimensions. BAVs were identified in 45 patients (72.6 %), of which 13 (20.9 %) were type-0 (two commissures), 28 (45.1 %) type-1 (three commissures but fusion of one commissure with a raphe) and 4 (6.5 %) valves were bicuspid but not possible to classify further. Patients with BAVs type-0 had larger dimensions in their sinus of Valsalva (35.5 ± 6.8 vs. 29.7 ± 2.7 mm, p = 0.002), ascending aorta (33.1 ± 6.2 vs. 26.0 ± 4.3 mm, p = 0.005) and sino-tubular junction (29.3 ± 7.4 vs. 24.2 ± 3.5 mm, p = 0.040) compared with tricuspid aortic valves (TAVs). Moderate and severe aortic valve disease was more common in BAV type-0 compared with BAV type-1 (p = 0.030) and TAV (p = 0.016). In a multivariate linear regression model BAV type-0 (p = 0.005), BAV type-1 (p = 0.011), age (p < 0.001), patient height (p = 0.009), and aortic valve disease (p = 0.035) were independently associated with increased diameter of the ascending aorta (R (2) of the model 0.54, p < 0.001). BAV type-0 is relatively common in coarctation of the aorta. Both BAV type-0 and type-1 are associated with increased diameter of the ascending aorta but this association is stronger for BAV type-0. Development of aortic valve disease is more common in BAV type-0 than in BAV type-1. Discrimination between BAV subtypes may potentially provide clinical and prognostic information in patients with coarctation of the aorta.

  • 28.
    Rinnström, Daniel
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå Heart Centre.
    Engström, Karl-Gunnar
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Surgery.
    Ugander, M.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå Heart Centre.
    Factors associated with left ventricular hypertrophy in adults with surgically repaired coarctation of the aorta2013In: European Heart Journal, ISSN 0195-668X, E-ISSN 1522-9645, Vol. 34, no Supplement: 1, 322-322 p.Article in journal (Other academic)
    Abstract [en]

    Purpose: Most patients with repaired coarctation of the aorta live normal lives and have good physical performance. However, even after surgical intervention, there are long-term cardiovascular risks including arterial hypertension and left ventricular hypertrophy.

    Methods: Fifty-one cardiovascular magnetic resonance investigations inadult patients with repaired coarctation of the aorta were reviewed and left ventricular mass was calculated. Using binary logistic regression, factors associated with left ventricular mass index abovethe reference limit were analysed among clinical, anatomic and functional variables.

    Results: In this population, 14 (27.5%) of the patients had leftventricular mass index above the upper reference limit. Higher systolic blood pressure (OR=1.042, p=0.028), > mild aortic valve disease/previous aortic valve intervention (OR=1.042, p=0.002), and diameter of the descending aorta (OR=1.475, p=0.003) wereindependently associated with left ventricular mass index above theupper reference limit. In a post hoc model where systolic blood pressure was categorised in four levels, only high systolic blood pressure (>160 mmHg) (OR=31.913, p=0.020), together with > mild aortic valve disease or previous aortic valve intervention (OR=25.493, p=0.002) remainedindependently associated with left ventricular mass index above theupper reference limit.

    Conclusion: Increased left ventricular mass is a common finding late after repair in coarctation of the aorta and is associated with themodifiable factors blood pressure and aortic valve disease. As most patients are young, and increased mass will affect the left ventricle over decades, attention to blood pressure is important in this population.

  • 29.
    Rydberg, Annika
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Eriksson, Anders
    Heart Centre, Umeå university hospital.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Heart Centre, Umeå university hospital.
    Andersson, Jonas
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Heart Centre, Umeå university hospital.
    ALCAPA: a need for guidelines for managing the adult type2013In: International Cardiovascular Forum Journal, ISSN 2410-2636, Vol. 1, no 2, 104-106 p.Article, review/survey (Refereed)
  • 30.
    Sandberg, Camilla
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Engström, Karl Gunnar
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Surgery.
    Dellborg, Mikael
    Thilén, Ulf
    Wadell, Karin
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Heart Centre, Umeå.
    The level of physical exercise is associated with self-reported health status (EQ-5D) in adults with congenital heart disease2015In: European Journal of Preventive Cardiology, ISSN 2047-4873, E-ISSN 2047-4881, Vol. 22, no 2, 240-248 p.Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: The prognosis in adults with congenital aortic valve disease is usually favourable; nevertheless, a number of medical and social factors might hamper long-term prognosis and quality of life. With a focus on physical exercise level, data from the Swedish National Registry on Congenital Heart Disease (SWEDCON) were analysed and variables associated with health-related quality of life in adults with congenital aortic valve disease were identified.

    METHODS: In this registry study, SWEDCON was searched for adult patients with isolated congenital aortic valve disease and valid EuroQol-5Dimensions health questionnaire (EQ-5D) data.

    RESULTS: This study identified 315 patients. The majority (n = 202, 64%) reported best possible health status (EQ-5Dindex = 1) whereas 113 (35%) reported some impairment (EQ-5Dindex < 1) with mean EQ-5Dindex 0.73 ± 0.17. In a multivariate logistic regression model, self-reported physical exercise > 3 h/week was independently associated with best possible health status (EQ-5Dindex = 1; p = 0.013). Moreover presence of cardiovascular symptoms (p < 0.001), active smoking (p = 0.002), history of valve surgery (p = 0.017), low educational level (p = 0.022), and higher systolic blood pressure (p = 0.029) were independently associated with impaired health status (EQ-5Dindex < 1).

    CONCLUSIONS: Physical exercise >3 h/week was, as a single variable, associated with best possible health status in adults with congenital aortic valve disease. In contrast, a number of medical and social factors are associated with worse self-reported health status. Among these, symptoms, smoking, and educational level are potential targets for modification and intervention. There is a need for studies investigating the effect of increased level of physical exercise in patients with congenital aortic valve disease.

  • 31.
    Sandberg, Camilla
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine.
    Hedström, Magnus
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine.
    Dellborg, M.
    Magnusson, A.
    Zetterstöm, A. -K
    Wadell, Karin
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine.
    Increased endurance capacity in adults with complex congenital heart disease after home-based interval exercise training on ergometer cycle2015In: European Heart Journal, ISSN 0195-668X, E-ISSN 1522-9645, Vol. 36, 458-458 p.Article in journal (Other academic)
  • 32.
    Sandberg, Camilla
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Hedström, Magnus
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Wadell, Karin
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Dellborg, Mikael
    Magnusson, Anders
    Zetterström, Anna-Klara
    Ljungqvist, Amanda
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Home-Based Interval Training Increases Endurance Capacity in Adults with Complex Congenital Heart DiseaseManuscript (preprint) (Other academic)
  • 33.
    Sandberg, Camilla
    et al.
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy. Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Rinnström, Daniel
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Dellborg, Mikael
    Thilén, Ulf
    Sörensson, Peder
    Nielsen, Niels-Erik
    Christersson, Christina
    Wadell, Karin
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Height, weight and body mass index in adults with congenital heart disease2015In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 187, 219-226 p.Article in journal (Refereed)
    Abstract [en]

    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.

  • 34.
    Sandberg, Camilla
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå Heart Centre.
    Thilen, U.
    Wadell, Karin
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Engström, Karl-Gunnar
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Surgery.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå Heart Centre.
    Complex adult congenital heart disease is associated with impaired skeletal muscle function2013In: European Heart Journal, ISSN 0195-668X, E-ISSN 1522-9645, Vol. 34, no Supplement: 1, 383-383 p.Article in journal (Refereed)
    Abstract [en]

    Purpose: Complex congenital heart disease is often associatedwith impaired physical functioning, usually measured as peak oxygen uptake in an exercise test. Skeletal muscle function is, however, less studied in these patients.

    Methods: Unilateral isotonic shoulder flexion was tested in 79 adultpatients (mean age 36.6±14.8 years, 31 females) with congenital heartdisease, classed as either "complex" (n=41, 51.9%) or "simple" (n=38, 48.1%). The patients were sitting comfortably in a chair with their back touching the wall and holding a weight (2 kg for women and 3 kg for men) in the hand of the tested side. The patients were asked to elevate the arm, from 0 to 90 degrees flexion, as many times as possible. The pace of 20 contractions per minute was held using a metronome.

    Results: Patients with complex lesions performed less shoulder flexions compared with patients with simple lesions (29.2±10.0 vs. 54.6±25.8, p<0.001). In univariate analysis including a number of demographic and clinical variables, only complexity of cardiac lesion (p<0.001) and on-going cardiac medications (p=0.012) were associated with shouldermuscle function, of which complexity (p<0.001) remained significant in multivariate analysis.

    Conclusion: There is a marked difference in shoulder muscle functionbetween patients with complex and simple congenital heart disease. Such differences might affect ability to perform daily activities and contribute to impaired overall physical functioning. Rehabilitation targeting muscle function may be indicated in patients with complexcongenital heart disease.

  • 35.
    Sandberg, Camilla
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Thilen, U.
    Wadell, Karin
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation.
    Gradmark, Anna
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Adults with congenital heart disease have lower habitual physical activity level compared to healthy age and gender matched controls2014In: European Heart Journal, ISSN 0195-668X, E-ISSN 1522-9645, Vol. 35, no Supplement 1, Meeting abstract P1352, 237-237 p.Article in journal (Other academic)
  • 36.
    Sandberg, Camilla
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine. Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Thilen, U.
    Wadell, Karin
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Adults with complex congenital heart disease have impaired skeletal muscle function and lower confidence in performing exercise training compared to healthy age and gender matched controls2014In: European Heart Journal, ISSN 0195-668X, E-ISSN 1522-9645, Vol. 35, no Supplement 1, Meeting abstract P3279, 579-579 p.Article in journal (Other academic)
  • 37.
    Sandberg, Camilla
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Thilén, Ulf
    Wadell, Karin
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Adults with complex congenital heart disease have impaired skeletal muscle function and reduced confidence in performing exercise training2015In: European Journal of Preventive Cardiology, ISSN 2047-4873, E-ISSN 2047-4881, Vol. 22, no 12, 1523-1530 p.Article in journal (Refereed)
    Abstract [en]

    Background Adults with congenital heart disease (ACHD) usually have reduced aerobic exercise capacity compared with controls. However, their skeletal muscle function is less studied. Material and methods In this cross-sectional study, unilateral isotonic shoulder flexion, unilateral isotonic heel-lift, maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) were tested in 85 patients with ACHD (35 women, mean age 36.814.8 years), classed as either complex' (n=43) or simple' (n=42), and 42 age and gender matched controls (16 women, mean age 36.914.9). Maximum number of shoulder flexions and heel-lifts were measured. MIP/MEP was tested using a handheld respiratory pressure meter. Exercise self-efficacy, measuring confidence in performing exercise training, was evaluated. Results Adults with complex lesions performed fewer shoulder flexions compared with controls and patients with simple lesions (28.2 +/- 11.1 vs. 63.6 +/- 40.4, p<0.001 and 28.2 +/- 11.1 vs. 54.9 +/- 24.9, p<0.001), as well as fewer heel-lifts compared with controls and patients with simple lesions (17.6 +/- 7.7 vs. 26.3 +/- 12.8, p<0.001 and 17.6 +/- 7.7 vs. 23.2 +/- 7.0, p=0.024), lower MIP than controls (80.7 +/- 26.7 vs. 111.1 +/- 29.9cm H2O, p<0.001) and lower MEP compared with controls (110.8 +/- 39.9 vs. 141.8 +/- 39.5, p<0.001). Their exercise self-efficacy was lower than controls (28.0 +/- 8.3 vs. 33.4 +/- 6.1, p=0.002). In a linear regression model complex heart lesions were independently associated with impaired limb muscle function. Conclusion Adults with complex congenital heart disease have impaired skeletal muscle function compared with patients with simple lesions and healthy controls. They also had lower confidence in performing exercise training. Thus, this population might have a potential for rehabilitation focusing on improving muscle function and confidence in performing exercise training.

  • 38.
    Sandberg, Camilla
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå Heart Centre.
    Wadell, Karin
    Umeå University, Faculty of Medicine, Department of Community Medicine and Rehabilitation, Physiotherapy.
    Gradmark, Anna
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå Heart Centre.
    Thilen, U.
    Engström, Karl-Gunnar
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Surgery.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Umeå Heart Centre.
    Physical activity level in adults with congenital heart disease: effects of gender and complexity of heart lesion2013In: European Heart Journal, ISSN 0195-668X, E-ISSN 1522-9645, Vol. 34, no Supplement: 1, 382-383 p.Article in journal (Refereed)
    Abstract [en]

    Purpose: Many adults with congenital heart disease have reduced exercise capacity but only little is known about habitual physical activityin this group. The aim of this study was to investigate habitual physicalactivity level in a cohort of adults with congenital heart disease.

    Methods: Seventy-five adult patients (29 females) aged 37.2±15.1 yearswith congenital heart disease classed as either simple or complex were studied with a combined accelerometer and heart rate monitor (Actiheart). The patients carried the Actiheart during 5 consecutive days, and where encouraged to proceed with their usual daily activities. Data was analysed with the Actiheart Software version 2.2. A mean ofPhysical Activity Level (PAL) (PAL=Total Energy Expenditure/Resting Energy Expenditure) over 4 days was calculated. PAL < 1.45 was categorised as low, 1.45-1.6 as moderate and >1.6 as high.

    Results: Forty-three patients (57,3%) had low PAL, 18 (24%) moderate PAL and 14 (16,7%) high PAL. PAL was lower in women compared to men (1.32±0.10 vs. 1.52±0.18, p < 0.001). PAL was higher in patients withsimple compared with complex congenital heart disease (1.50±0.21 vs. 1.39±0.14, p = 0.019). Among men, active smoking (p=0.015) and lower age (p=0.04) were independently associated with higher PAL, whereas no such associations were observed among women.

    Conclusion: The majority of the observed patients had low PAL and women had lower PAL compared to men. PAL was related to complexity ofheart lesion. Efforts to increase habitual physical activity may beindicated in this population, especially in women and patients withcomplex congenital heart disease.

  • 39.
    Stål, Per
    et al.
    Umeå University, Faculty of Medicine, Department of Integrative Medical Biology (IMB), Anatomy.
    Lindman, Rolf
    Department of Oral and Maxillofacial Surgery and Jaw Orthopedics, Malmö University Hospital, Malmö, Sweden.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine. Umeå University, Faculty of Medicine, Department of Integrative Medical Biology (IMB), Anatomy.
    Capillary supply of the soft palate muscles is reduced in long-term habitual snorers2009In: Respiration, ISSN 0025-7931, E-ISSN 1423-0356, Vol. 77, no 3, 303-310 p.Article in journal (Refereed)
    Abstract [en]

    Background: Snoring and obstructive sleep apnea (OSA) cause vibration and stretch of the upper airway tissues that may result in neuromuscular damage and changes in the microcirculation. Objectives: The aim of this investigation was to test whether long-term snoring affects capillary supply in soft palate muscles. Methods: Samples from the palatopharyngeus (PP) and uvula (UV) muscles were collected from 8 patients undergoing uvulo-palatopharyngoplasty because of habitual snoring and OSA. Control samples were obtained at autopsy. The muscles were analyzed using immunohistochemistry and morphometry. Results: The patients' palate muscles had a lower capillary density (PP 443 vs. 711 capillaries/mm(2), p < 0.001, and UV 452 vs. 624 capillaries/mm(2), p = 0.009), a lower number of capillaries related to an individual muscle fiber (PP 1.3 vs. 2.7, p = 0.003, and UV 1.0 vs. 1.9, p = 0.03) and a lower number of capillaries related to the fiber size (PP 0.9 vs. 2.1, p = 0.001, and UV 0.6 vs. 1.9, p = 0.002). Conclusions: Our results indicate that reduced capillary supply of palate muscles plays a pathophysiological role in long-term snorers and OSA. The cause of the low capillary supply is unclear, but neuromuscular injury due to repeated vibratory and stretch trauma of the soft palate during snoring is a plausible mechanism.

  • 40.
    Stål, Per S
    et al.
    Umeå University, Faculty of Medicine, Department of Integrative Medical Biology (IMB), Anatomy.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Abnormal Mitochondria Organization and Oxidative Activity in the Palate Muscles of Long-Term Snorers with Obstructive Sleep Apnea2012In: Respiration, ISSN 0025-7931, E-ISSN 1423-0356, Vol. 83, no 5, 407-417 p.Article in journal (Refereed)
    Abstract [en]

    Background: Histopathological alterations and a reduced number of capillaries have been observed in the palate muscles of snorers with obstructive sleep apnea syndrome (OSAS). These changes may create a substrate for decreased microcirculation, impaired aerobic metabolism and muscle dysfunction and contribute to upper airway obstruction during sleep. Objectives: The aim was to analyze mitochondria distribution and oxidative enzyme activity in relation to capillary supply in the palate muscles of patients with a history of long-term snoring and OSAS. Methods: Palatopharyngeus (PP) and uvula (UV) muscle samples were obtained from 8 patients undergoing uvulopalatopharyngoplasty due to habitual snoring and OSAS. The muscles were analyzed with enzyme- and immunohistochemistry and morphometry. Results: Abnormalities in the internal organization of mitochondria and oxidative activity were observed in 39 ± 15% of the fibers in the PP and 4 ± 3% in the UV, but not in control samples. The majority of these fibers had a lobulated contour and trabecular internal organization of mitochondria. The number of capillaries around abnormal fibers (PP 0.9 ± 0.3, UV 0.4 ± 0.1) was lower than in fibers of a normal appearance in both patients (PP 1.4 ± 0.6, UV 1.2 ± 0.3) and references (PP 2.7 ± 0.7, UV 1.9 ± 0.9) (p < 0.05). Conclusions: Abnormal mitochondrial distribution, a low capillary supply and signs of impaired oxidative activity suggest that muscle dysfunction of the palate muscles in long-term snorers may contribute to the upper airway obstruction during sleep. The cause of these abnormalities remains unclear, but local muscle and nerve trauma due to vibration and stretch is a possible etiology.

  • 41. Trouw, Leendert A
    et al.
    Okroj, Marcin
    Kupreishvili, Koba
    Landberg, Göran
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Niessen, Hans W M
    Blom, Anna M
    C4b-binding protein is present in affected areas of myocardial infarction during the acute inflammatory phase and covers a larger area than C32008In: PLoS ONE, ISSN 1932-6203, Vol. 3, no 8, e2886- p.Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: During myocardial infarction reduced blood flow in the heart muscle results in cell death. These dying/dead cells have been reported to bind several plasma proteins such as IgM and C-reactive protein (CRP). In the present study we investigated whether fluid-phase complement inhibitor C4b-binding protein (C4BP) would also bind to the infarcted heart tissue. METHODS AND FINDINGS: Initial studies using immunohistochemistry on tissue arrays for several cardiovascular disorders indicated that C4BP can be found in heart tissue in several cardiac diseases but that it is most abundantly found in acute myocardial infarction (AMI). This condition was studied in more detail by analyzing the time window and extent of C4BP positivity. The binding of C4BP correlates to the same locations as C3b, a marker known to correlate to the patterns of IgM and CRP staining. Based on criteria that describe the time after infarction we were able to pinpoint that C4BP binding is a relatively early marker of tissue damage in myocardial infarction with a peak of binding between 12 hours and 5 days subsequent to AMI, the phase in which infiltration of neutrophilic granulocytes in the heart is the most extensive. CONCLUSIONS: C4BP, an important fluid-phase inhibitor of the classical and lectin pathway of complement activation binds to jeopardized cardiomyocytes early after AMI and co-localizes to other well known markers such as C3b.

  • 42. Tsang, Wendy
    et al.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Public Health and Clinical Medicine.
    Salehian, Omid
    Holm, Johan
    Webb, Gary
    Gatzoulis, Michael A
    Therrien, Judith
    Intracardiac thrombus in adults with the Fontan circulation.2007In: Cardiology in the Young, ISSN 1047-9511, E-ISSN 1467-1107, Vol. 17, no 6, 646-651 p.Article in journal (Refereed)
  • 43. Vejlstrup, N.
    et al.
    Sorensen, K.
    Mattsson, E.
    Sondergaard, L.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Thilen, U.
    Kvidal, P.
    Dellborg, M.
    Eriksson, P.
    Population-based study of long-term outcome of mustard/senning correction for transposition of the great arteries (TGA)2014In: European Heart Journal, ISSN 0195-668X, E-ISSN 1522-9645, Vol. 35, no Supplement 1, Meeting abstract P3314, 588-588 p.Article in journal (Other academic)
  • 44.
    Vejlstrup, Niels
    et al.
    Copenhagen, Denmark .
    Sörensen, Keld
    Aarhus, Denmark .
    Mattsson, Eva
    Stockholm, Sweden .
    Thilen, Ulf
    Lund University, Sweden.
    Kvidal, Per
    Uppsala, Sweden.
    Johansson, Bengt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Iversen, Kasper
    Herlev County Hospital, Denmark .
    Söndergaard, Lars
    Copenhagen, Denmark .
    Dellborg, Mikael
    Göteborg, Sweden .
    Eriksson, Peter
    Göteborg, Sweden .
    Long-Term Outcome of Mustard/Senning Correction for Transposition of the Great Arteries in Sweden and Denmark2015In: Circulation, ISSN 0009-7322, E-ISSN 1524-4539, Vol. 132, no 8, 633-638 p.Article in journal (Refereed)
    Abstract [en]

    Background The atrial switch operation, the Mustard or Senning operation, for the transposition of the great arteries (TGA) was introduced in the late 1950s and was the preferred surgery for TGA until the early 1990s. The Mustard and Senning operation involves extensive surgery in the atria and leaves the right ventricle as the systemic ventricle. The Mustard and Senning cohort is now well into adulthood and we begin to see the long-term outcome. Methods and Results All the 6 surgical centers that performed Mustard and Senning operations in Sweden and Denmark identified all operated TGA patients. Information about death was obtained in late 2007 and early 2008 from the Danish and Swedish Centralised Civil Register by using the patients' unique national Civil Registration Numbers. Four hundred sixty-eight patients undergoing the atrial switch operation were identified. Perioperative 30-day mortality was 20%, and 60% were alive after 30 years of follow-up. Perioperative mortality was significantly increased by the presence of a ventricular septal defect, left ventricular outflow obstruction, surgery early in the Mustard and Senning era. However, only pacemaker implantation is predictive of long-term outcome (hazard ratio, 1.90; 95% confidence interval, 1.05-3.46, P=0.04), once the TGA patient has survived the perioperative period. The risk of reoperation was correlated to the presence of associated defects and where the first Mustard/Senning operation was performed. Conclusions The long-term survival of patients with Mustard and Senning correction for TGA appears to be primarily determined by factors in the right ventricle and tricuspid valve and not the timing of or the type of surgery in childhood. Cardiac function necessitating the implantation of a pacemaker is associated with an increase in mortality.

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