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  • 1. Acuña Mora, Mariela
    et al.
    Sparud-Lundin, Carina
    Burström, Åsa
    Hanseus, Katarina
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Moons, Philip
    Bratt, Ewa-Lena
    Patient empowerment and its correlates in young persons with congenital heart disease2019In: European Journal of Cardiovascular Nursing, ISSN 1474-5151, E-ISSN 1873-1953, Vol. 18, no 5, p. 389-398, article id 1474515119835434Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE:: The objective of this study was to measure the level of empowerment and identify its correlates in young persons with congenital heart disease.

    STUDY DESIGN:: Patients aged 14-18 years with congenital heart disease, and under active follow-up in one of four paediatric cardiology centres in Sweden were invited to participate in a cross-sectional study. A total of 202 young persons returned the questionnaires. Patient empowerment was measured with the Gothenburg Young Persons Empowerment Scale that allows the calculation of total and subscale scores. Univariate and multivariate linear regression analyses were undertaken to analyse possible correlates, including: sex, age, health behaviours, knowledge of congenital heart disease, quality of life, patient-reported health, congenital heart disease complexity, transition readiness and illness perception.

    RESULTS:: The mean empowerment score was 54.6±10.6 (scale of 15-75). Univariate analyses showed that empowerment was associated with age, quality of life, transition readiness, illness perception, health behaviours and patient-reported health (perceived physical appearance, treatment anxiety, cognitive problems and communication issues). However, multivariable linear regression analyses identified that only transition readiness (β=0.28, P<0.001) and communication (β=0.36, P<0.001) had a positive association with patient empowerment. These variables were also significantly associated with the subscale scores of the empowerment scale of knowledge and understanding ( P<0.001), shared decision-making ( P<0.001) and enabling others ( P<0.01). The overall models' explained variance ranged from 8% to 37%.

    CONCLUSION:: Patient empowerment was associated with transition readiness and fewer problems communicating. While it is not possible to establish the directionality of the associations, interventions looking to increase empowerment could benefit from using these variables (or measurements) for evaluation purposes.

  • 2.
    Alenius Dahlqvist, Jenny
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Karlsson, Marcus
    Umeå University, Faculty of Medicine, Department of Radiation Sciences, Radiation Physics.
    Wiklund, Urban
    Umeå University, Faculty of Medicine, Department of Radiation Sciences, Radiation Physics.
    Hörnsten, Rolf
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Clinical Physiology.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Handheld ECG in analysis of arrhythmia and heart rate variability in children with Fontan circulation2014In: Journal of Electrocardiology, ISSN 0022-0736, E-ISSN 1532-8430, Vol. 47, no 3, p. 374-382Article in journal (Refereed)
    Abstract [en]

    Background: Our aim was to evaluate the intermittent use of a handheld ECG system for detecting silent arrhythmias and cardiac autonomic dysfunction in children with univentricular hearts. Methods: Twenty-seven patients performed intermittent ECG recordings with handheld devices during a 14-day period. A manual arrhythmia analysis was performed. We analyzed heart rate variability (HRV) using scatter plots of all interbeat intervals (Poincare plots) from the total observation period. Reference values of HRV indices were determined from Holter-ECGs in 41 healthy children. Results: One asymptomatic patient had frequent ventricular extra systoles. Another patient had episodes with supraventricular tachycardia (with concomitant palpitations). Seven patients showed reduced HRV. Conclusions: Asymptomatic arrhythmia was detected in one patient. The proposed method for pooling of intermittent recordings from handheld or similar devices may be used for detection of arrhythmias as well as for cardiac autonomic dysfunction.

  • 3.
    Alenius Dahlqvist, Jenny
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Karlsson, Marcus
    Umeå University, Faculty of Medicine, Department of Radiation Sciences, Radiation Physics.
    Wiklund, Urban
    Umeå University, Faculty of Medicine, Department of Radiation Sciences, Radiation Physics.
    Hörnsten, Rolf
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Clinical Physiology.
    Strömvall-Larsson, Eva
    Division of Cardiology, Department of Paediatrics, Sahlgrenska University Hospital/Queen Silvia Children’s Hospital, Göteborg University, Göteborg, Sweden.
    Berggren, Håkan
    Division of Cardiothoracic Surgery, Department of Paediatrics, Sahlgrenska University Hospital/Queen Silvia Children’s Hospital, Göteborg University, Göteborg, Sweden.
    Hanseus, Katarina
    Department of Paediatrics, Children’s Hospital, Lund University Hospital, Lund, Sweden.
    Johansson, Sune
    Paediatric Cardiac Surgical Unit, Children’s Hospital, Lund University Hospital, Lund, Sweden.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Heart rate variability in children with fontan circulation: lateral tunnel and extracardiac conduit2012In: Pediatric Cardiology, ISSN 0172-0643, E-ISSN 1432-1971, Vol. 33, no 2, p. 307-315Article in journal (Refereed)
    Abstract [en]

    The technique in Fontan surgery has developed from the lateral tunnel (LT) toward the extracardiac conduit (EC) used to reduce long-term complications such as atrial arrhythmia and sinus node dysfunction. Heart rate variability (HRV) examines cardiac nervous activity controlling the sinus node. This study aimed to investigate HRV in a cohort of children with univentricular hearts, focusing on the relation between HRV and surgical procedure. For 112 children with Fontan circulation, HRV was analyzed using power spectral analysis. Spectral power was determined in three regions: very-low-frequency (VLF), low-frequency (LF), and high-frequency (HF) regions. Patients were compared with 66 healthy controls subject. Patients with LT were compared with patients who had EC. The children with Fontan circulation showed a significantly reduced HRV including total power (P < 0.0001), VLF (P < 0.0001), LF (P < 0.0001), and HF (P = 0.001) compared with the control subjects. The LT and EC patients did not differ significantly. Reduced HRV was found in both the LT and EC patients. In terms of HRV reduction, EC was not superior to LT.

  • 4.
    Alenius Dahlqvist, Jenny
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences.
    Sunnegårdh, Jan
    Department of Cardiology, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Institute of Clinical Sciences, Gothenburg University, Gothenburg, Sweden.
    Hanséus, Katarina
    Department of Clinical Sciences Lund, Children Heart Center, Skåne University Hospital, Lund University, Lund, Sweden.
    Strömvall Larsson, Eva
    Department of Cardiology, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Institute of Clinical Sciences, Gothenburg University, Gothenburg, Sweden.
    Nygren, Anders
    Department of Cardiology, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Institute of Clinical Sciences, Gothenburg University, Gothenburg, Sweden.
    Dalén, Magnus
    Department of Molecular Medicine and Surgery, Department of Cardiac Surgery, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
    Berggren, Håkan
    Department of Pediatric Cardiac Surgery, Children's Heart Center, The Queen Silvia Children's Hospital, Gothenburg , Sweden.
    Johansson Ramgren, Jens
    Department of Pediatric Cardiac Surgery, Children´s Heart Center, Skånes University Hospital Lund, Sweden.
    Wiklund, Urban
    Umeå University, Faculty of Medicine, Department of Radiation Sciences.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences.
    Pacemaker treatment after Fontan surgery: a Swedish national study2019In: Congenital Heart Disease, ISSN 1747-079X, E-ISSN 1747-0803, Vol. 14, no 4, p. 582-589Article in journal (Other academic)
    Abstract [en]

    Objective: Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indications in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden.

    Methods: We retrospectively reviewed all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n = 599).

    Results: After a mean follow‐up of 12.2 years, 13% (78/599) of the patients with Fontan circulation had received pacemakers. Patients operated with the extracardiac conduit (EC) type of total cavopulmonary connection had a significantly lower prevalence of pacemaker implantation (6%) than patients with lateral tunnel (LT; 17%). Mortality did not differ between patients with (8%) and without pacemaker (5%). The most common pacemaker indication was sinus node dysfunction (SND) (64%). Pacemaker implantation due to SND was less common among patients with EC. Pacemaker implantation was significantly more common in patients with mitral atresia (MA; 44%), double outlet right ventricle (DORV; 24%) and double inlet left ventricle (DILV; 20%). In contrast, patients with pulmonary atresia with intact ventricular septum and hypoplastic left heart syndrome were significantly less likely to receive a pacemaker (3% and 6%, respectively).

    Conclusions: Thirteen percent of Fontan patients received a permanent pacemaker, most frequently due to SND. EC was associated with a significantly lower prevalence of pacemaker than LT. Permanent pacemaker was more common in patients with MA, DORV, and DILV.

  • 5.
    Alenius Dahlqvist, Jenny
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Wiklund, Urban
    Umeå University, Faculty of Medicine, Department of Radiation Sciences.
    Karlsson, Marcus
    Umeå University, Faculty of Medicine, Department of Radiation Sciences.
    Hanseus, Katarina
    Stromvall-Larsson, Eva
    Nygren, Anders
    Eliasson, Håkan
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Sinus node dysfunction in patients with Fontan circulation: could heart rate variability be a predictor for pacemaker implantation?2019In: Pediatric Cardiology, ISSN 0172-0643, E-ISSN 1432-1971, Vol. 40, no 4, p. 685-693Article in journal (Refereed)
    Abstract [en]

    Sinus node dysfunction (SND) causes significant morbidity in patients after Fontan surgery. Heart rate variability (HRV) reflects the autonomic regulation of the heart, and changes in HRV have been associated with SND in adults. We aimed to study whether changes in HRV could be detected in 24-h electrocardiographic (ECG) recordings in Fontan patients with SND. We compared HRV results from two patient groups; patients with Fontan circulation who later required a pacemaker due to severe SND (n = 12) and patients with Fontan circulation and SND, without indication for pacemaker treatment (n = 11), with two control groups; patients with Fontan circulation without SND (n = 90) and healthy controls (n = 66). The Poincare plot index SD2 (representing changes in heart rate over 24-h) and the very low-frequency (VLF) HRV component were significantly higher in both SND groups, both compared with healthy controls and patients with Fontan circulation without SND. In SND patients with pacemakers, SD2 and VLF were slightly reduced compared to SND patients without pacemaker (p = 0.06). In conclusion, in Fontan patients with SND the HRV is significantly higher compared to healthy controls and Fontan patients without SND. However, in patients with severe SND requiring pacemaker, SD2 and VLF tended to be lower than in patients with SND without pacemaker, which could indicate a reduced diurnal HRV in addition to the severe bradycardia. This is a small study, but our results indicate that HRV analysis might be a useful method in the follow-up of Fontan patients regarding development of SND.

  • 6.
    Alenius Dahlqvist, Jenny
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Wiklund, Urban
    Umeå University, Faculty of Medicine, Department of Radiation Sciences.
    Karlsson, Marcus
    Umeå University, Faculty of Medicine, Department of Radiation Sciences.
    Hanséus, Katarina
    Department of Clinical Sciences Lund, Children Heart Centre, Skåne University Hospital, Lund University, Lund, Sweden.
    Strömvall Larsson, Eva
    Department of Cardiology, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Institute of Clinical Sciences, Gothenburg University, Gothenburg, Sweden.
    Johansson Ramgren, Jens
    Department of Pediatric Cardiac Surgery, Children´s Heart Center, Skånes University Hospital Lund, Sweden.
    Berggren, Håkan
    Department of Pediatric Cardiac Surgery, Children's Heart Center, The Queen Silvia Children's Hospital, Gothenburg, Sweden.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Changes in heart rate variability during surgical stages to completed Fontan circulationManuscript (preprint) (Other academic)
    Abstract [en]

    Objectives: In patients with Fontan circulation, arrhythmia is a serious complication contributing to morbidity and mortality. Arrhythmia is related to heart rate variability (HRV), which reflects autonomic nervous regulation of the heart. Our hypothesis was that autonomic nervous ganglia, located at the junction of the superior vena cava’s entrance to the heart, may be affected during the bidirectional Glenn procedure (BDG), resulting in reduced HRV.

    Methods: 24-hour Holter ECG recordings were obtained before BDG (n=47), after BDG (n=47) and after total cavopulmonary connection (TCPC) (n=45) in patients, and in 38 healthy controls. HRV was analysed by spectral and Poincaré methods. Age-related z-scores were calculated and compared using linear mixed effects modeling.

    Results: HRV-parameters (Ptot, PVLF, PLF, PHF, PLF/PHF, SD2, and SD1/SD2) were significantly lower in patients before BDG when compared to healthy controls. The RR interval and SD2 were significantly increased in patients post  BDG compared to pre BDG. Compared to healthy controls; patients operated with BDG had significantly longer RR intervals and reduced Ptot and PLF. Patients post TCPC showed longer RR intervals, Ptot, PVLF, PLF, and PHF,  SD1, and SD1/SD2 compared with healthy controls. In patients post TCPC; Ptot, PVLF, PLF, PHF, PLF/PHF, SD2, and SD1/SD2 were decreased compared to pre TCPC.

    Conclusions: Heart rate was reduced after BDG procedure, and further reductions of HRV were seen post-TCPC. Our results indicate that autonomic regulation of cardiac rhythm is affected both after BDG and again after TCPC. This may be reflected as, and contribute to, postoperative arrhythmic events.

  • 7.
    Alenius Dahlqvist, Jenny
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Wiklund, Urban
    Umeå University, Faculty of Medicine, Department of Radiation Sciences.
    Karlsson, Marcus
    Umeå University, Faculty of Medicine, Department of Radiation Sciences.
    Hanséus, Katarina
    Department of Clinical Sciences Lund, Children Heart Centre, Skåne University Hospital, Lund University, Lund, Sweden.
    Strömvall Larsson, Eva
    Department of Cardiology, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Institute of Clinical Sciences, Gothenburg University, Gothenburg, Sweden.
    Nygren, Anders
    Department of Cardiology, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Institute of Clinical Sciences, Gothenburg University, Gothenburg, Sweden.
    Eliasson, Håkan
    Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Sinus node dysfunction in patients with Fontan circulation: could heart rate variability be a predictor for pacemaker implantation?Manuscript (preprint) (Other academic)
  • 8. Ambrosi, Aurelie
    et al.
    Salomonsson, Stina
    Eliasson, Håkan
    Zeffer, Elisabeth
    Dzikaite, Vijole
    Bergman, Gunnar
    Fernlund, Eva
    Theander, Elke
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Öhman, Annika
    Skogh, Thomas
    Rantapää-Dahlqvist, Solbritt
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Reumatology.
    Fored, Michael
    Blomqvist, Paul
    Ekbom, Anders
    Lindström, Ulla
    Melander, Mats
    Winqvist, Ola
    Gadler, Fredrik
    Jonzon, Anders
    Sonesson, Sven-Erik
    Wahren-Herlenius, Marie
    Influence of season of birth and maternal age in the development of congenital heart block in anti-Ro-SSA/La-SSB positive pregnancies2010In: Scandinavian Journal of Immunology, ISSN 0300-9475, E-ISSN 1365-3083, Vol. 72, no 3, p. 265-Article in journal (Refereed)
  • 9. Ambrosi, Aurélie
    et al.
    Salomonsson, Stina
    Eliasson, Håkan
    Zeffer, Elisabeth
    Skog, Amanda
    Dzikaite, Vijole
    Bergman, Gunnar
    Fernlund, Eva
    Tingström, Joanna
    Theander, Elke
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Skogh, Thomas
    Öhman, Annika
    Lundström, Ulla
    Mellander, Mats
    Winqvist, Ola
    Fored, Michael
    Ekbom, Anders
    Alfredsson, Lars
    Källberg, Henrik
    Olsson, Tomas
    Gadler, Fredrik
    Jonzon, Anders
    Kockum, Ingrid
    Sonesson, Sven-Erik
    Wahren-Herlenius, Marie
    Development of heart block in children of SSA/SSB-autoantibody-positive women is associated with maternal age and displays a season-of-birth pattern2012In: Annals of the Rheumatic Diseases, ISSN 0003-4967, E-ISSN 1468-2060, Vol. 71, no 3, p. 334-340Article in journal (Refereed)
    Abstract [en]

    OBJECTIVE: Congenital heart block may develop in the fetuses of Ro/SSA-positive and La/SSB-positive mothers. Recurrence rates of only 10-20% despite persisting maternal antibodies indicate that additional factors are critical for the establishment of heart block. The authors investigated the influence of other maternal and fetal factors on heart block development in a Swedish population-based cohort.

    METHODS: The influence of fetal gender, maternal age, parity and time of birth on heart block development was analysed in 145 families, including Ro/La-positive (n=190) and Ro/La-negative (n=165) pregnancies.

    RESULTS: There was a recurrence rate of 12.1% in Ro/La-positive women, and no recurrence in Ro/La-negative women. Fetal gender and parity did not influence the development of heart block in either group. Maternal age in Ro/La-positive pregnancies with a child affected by heart block was, however, significantly higher than in pregnancies resulting in babies without heart block (p<0.05).Seasonal timing of pregnancy influenced the outcome. Gestational susceptibility weeks 18-24 occurring during January-March correlated with a higher proportion of children with heart block and lower vitamin D levels during the same period in a representative sample of Swedish women and a corresponding higher proportion of children with heart block born in the summer (p<0.02). Maternal age or seasonal timing of pregnancy did not affect the outcome in Ro/La-negative pregnancies.

    CONCLUSION: This study identifies maternal age and seasonal timing of pregnancy as novel risk factors for heart block development in children of Ro/La-positive women. These observations may be useful for counselling when pregnancy is considered.

  • 10. Berghammer, Malin C
    et al.
    Brink, Eva
    Rydberg, Annika M
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Dellborg, Mikael
    Ekman, Inger
    Committed to Life: Adolescents' and Young Adults' Experiences of Living with Fontan Circulation2015In: Congenital Heart Disease, ISSN 1747-079X, E-ISSN 1747-0803, Vol. 10, no 5, p. 403-412Article in journal (Refereed)
    Abstract [en]

    Background: Single ventricle defects are among the most complex congenital heart defects and the development of advanced surgical procedures in recent decades has created the first generation of adolescents and young adults living with this condition. Yet little is known about how these individuals experience life and what impact the heart defect has on their life in general. Objective: The aim was to illuminate and gain a deeper understanding of adolescents' and young adults' experiences of living with a surgically palliated univentricular heart. Design: Seven open-ended in-depth interviews were conducted, transcribed, and analyzed according to the henomenological hermeneutical method. All adolescents and young adults operated before 1995 according to the Fontan procedure or the total cavo-pulmonary connection procedure at one pediatric cardiology unit were included in the study. They were 17-32 years of age (median age 22 years). Results: The interpretation of the interview transcripts showed that the participants experienced living with a surgically palliated univentricular heart in terms of feeling exceptional, strong, and healthy. This was supported by two structural analyses, where three themes emerged: happiness over being me, focusing on possibilities, and being committed to life. Conclusion: Living with a Fontan circulation included negative experiences but the analyses clearly demonstrated a feeling of being strong and healthy. An appreciation of having survived and being committed to life was found to be an integral part of the development of the interviewees' existential growth. This probably strengthens them further in their ability to balance expectations and hurdles in life. This study provides valuable insights into the experience of patients after the Fontan procedure and the importance of a positive health care environment throughout their lives.

  • 11.
    Birkeland, Anna-Lena
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Dahlgren, Lars
    Umeå University, Faculty of Social Sciences, Department of Sociology.
    Hägglöf, Bruno
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Child and Adolescent Psychiatry.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Breaking bad news: an interview study of paediatric cardiologists2011In: Cardiology in the Young, ISSN 1047-9511, E-ISSN 1467-1107, Vol. 21, no 3, p. 286-291Article in journal (Refereed)
    Abstract [en]

    Technical developments in paediatric cardiology over the last few decades have increased expectations on professionals, demanding of them more emotional competence and communicative ability. The aim of this study was to examine the approach of paediatric cardiologists in informing and communicating with the family of the patient.

    Method: A qualitative interview method was first tested in a pilot study with two paediatric cardiologists. There were nine subsequent semi-structured interviews that were carried out with paediatric cardiologists. A researcher performed all the interviews, which were taped, transcribed, decoded, and analysed.

    Results: Among paediatric cardiologists, how to break bad news to the family is an important concern, evident in findings regarding the significance of trust and confidence, the use of different emotional positions, and a common ambition to achieve skills to handle the situation. There is a need for reflection, education, and sharing of experiences. The cardiologists desire further development of teamwork and of skills in medical students and residents for delivering bad news.

    Conclusions: Doctors are expected to cope with the complexities of diagnoses and decisions, while simultaneously being sensitive to the feelings of the parents, aware of their own emotions, and able to keep it all under control in the context of breaking the bad news to the parents and keeping them informed. These conflicting demands create a need to expand the professional role of the doctor by including more training in emotional competence and communicative ability, beginning in medical school and continuing through consultancy.

  • 12.
    Birkeland, Anna-Lena
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Hagglöf, Bruno
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Child and Adolescent Psychiatry.
    Dahlgren, Lars
    Umeå University, Faculty of Social Sciences, Department of Sociology.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Interprofessional teamwork in Swedish pediatric cardiology: a national exploratory study2013In: Journal of Interprofessional Care, ISSN 1356-1820, E-ISSN 1469-9567, Vol. 27, no 4, p. 320-325Article in journal (Refereed)
    Abstract [en]

    This paper aims to describe the nature of pediatric cardiology teams (PCTs) based in Sweden through the use of a mixed methods approach. Questionnaires examining issues about the organization/ways of working, functions/tasks and attitudes were answered by 30 PCTs. Focus group interviews were conducted with six PCTs, selected purposefully by size and location, and information on experiences and attitudes on interprofessional teamwork was explored in depth. Results from the quantitative indicated that in 17 of the teams, where the nurse acted as the central coordinator, there was a positive attitude to the value of teamwork. In the interviews, different problems and needs of improvements were mentioned regarding structure, leadership, presence of physicians in the team as well as the team's mandate. All of the participants, however, agreed that interprofessional teams were required to manage the complexity of the children's care. In conclusion, this study suggests that PCTs need further support to develop structure, leadership and coordination of resources to function in a more effective manner. National plans or recommendations that mandate the organization and working methods of PCTs would be helpful for the ongoing development of PCTs in Sweden.

  • 13.
    Birkeland, Anna-Lena
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Hällgren Graneheim, Ulla
    Umeå University, Faculty of Medicine, Department of Nursing.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Hägglöf, Bruno
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Child and Adolescent Psychiatry.
    Dahlgren, Lars
    Umeå University, Faculty of Social Sciences, Department of Sociology.
    Facing bad news: a case study focusing on families having a child with congenital heart diseaseManuscript (preprint) (Other academic)
  • 14.
    Birkeland, Anna-Lena
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Dahlgren, Lars
    Umeå University, Faculty of Social Sciences, Department of Sociology.
    Hägglöf, Bruno
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Child and Adolescent Psychiatry.
    Teamwork in Swedish paediatric cardiology: a national exploratory study examining function and dynamicsManuscript (preprint) (Other academic)
  • 15.
    Birkeland, Anna-Lena
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Hägglöf, Bruno
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Child and Adolescent Psychiatry.
    The complexity of the psychosocial situation in children and adolescents with heart disease2005In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 94, no 10, p. 1495-1501Article in journal (Refereed)
    Abstract [en]

    Aim: To describe the psychosocial situation of children/adolescents with heart disease and their families, an inventory method was worked out.

    Methods: Ninety-seven children/adolescents with congenital heart disease (CHD) were graded into three categories with respect to complexity of CHD. Group I included 42 patients with malformations requiring standardized operations. Group II included 20 patients with more complicated malformations, and group III included 35 patients with very complex malformations. The patients were compared with controls without heart disease, matched for age and gender. The psychosocial impact of CHD was measured by the inventory.

    Results: The most frequent problems in the patient group were healthcare and treatment-related needs (71/97) in the external sphere, family symptoms (68/97) in the interpersonal sphere, and somatic symptoms (19/97) in the personal sphere. Corresponding numbers in the controls were treatment-related needs (15/97), family (9/97) and somatic symptoms (25/97). Fifty per cent of the symptoms in the patient groups were mild, 30% moderate and 20% severe. The most severe symptoms were found in the interpersonal sphere, where family symptoms constituted the most severe variable. The frequency of severe problems in the personal sphere was 11% in the patients and 1% in the controls. This inventory method differentiates the grades of medical complexity both regarding number and severity of psychosocial symptoms. It indicates severe personal problems in the most complex group and shows that they have severe personal problems independent of family problems.

    Conclusion: This study elucidates the psychosocial complexity in children/adolescents with CHD, which has clinical implications in developing a psychosocial care programme.

  • 16. Bratt, E. L.
    et al.
    Burström, Å.
    Hanseus, K.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Berghammer, M.
    Do not forget the parents: Parents' concerns during transition to adult care for adolescents with congenital heart disease2018In: Child Care Health and Development, ISSN 0305-1862, E-ISSN 1365-2214, Vol. 44, no 2, p. 278-284Article in journal (Refereed)
    Abstract [en]

    Background: Growing up with congenital heart disease (CHD) often means transfer to adult care and lifelong medical follow-up. An optimal transition process usually involves a multipart collaboration between the patient, their parents and other family members, and the healthcare providers. Taking an active role while knowing when it is time to step aside can be difficult for all the concerned parties, even the healthcare professionals. The aim of the present study therefore, was to explore parents' expectations and needs during their adolescent's transition to adult care.

    Method: Semi-structured interviews were conducted with 18 parents of 16 adolescents (aged 13-18years) with CHD in 4 pediatric cardiology settings in Sweden. The interviews were analysed with qualitative content analysis.

    Results: The analysis resulted in 2 main themes: (a) Feeling securethe importance of being prepared and informed. This theme focused on the need to be prepared and informed about transition and future transfer to adult care. (b) Recognizing when to hand over at the right time. This theme addressed the process of handing over the responsibility from the parent to the adolescents and contained handing over from pediatric care to adult care.

    Conclusion: Being prepared and informed about the upcoming transition process was essential. The parents underlined the importance of being involved in the transition planning for gradually handing over responsibility to the adolescent. They also considered establishing contact with the adult healthcare team before transfer as important and needed to be assured that CHD-related information of importance for the young person's daily life would be given.

  • 17. Burstrom, Asa
    et al.
    Mora, Mariela Acuna
    Ojmyr-Joelsson, Maria
    Sparud-Lundin, Carina
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Hanseus, Katarina
    Frenckner, Bjorn
    Nisell, Margret
    Moons, Philip
    Bratt, Ewa-Lena
    Ready for Transfer to Adult Care?: A Triadic Evaluation of Transition Readiness in Adolescents With Congenital Heart Disease and Their Parents2019In: Journal of Family Nursing, ISSN 1074-8407, E-ISSN 1552-549X, article id UNSP 1074840719864255Article in journal (Refereed)
    Abstract [en]

    Transfer to adult care for adolescents with chronic conditions ought to be determined by transition readiness. The aims of this study were (a) to describe the level of readiness for transition in adolescents with congenital heart disease, (b) to compare adolescents' assessment of transition readiness with their parents' assessments, and (c) to study potential correlates of transition readiness. A total of 157 triads of adolescents aged 14 to 18 years and their parents completed the Readiness for Transition Questionnaire. Adolescents scored higher on overall readiness than their parents. Multivariable analyses revealed that higher levels of adolescents' overall readiness were associated with a less threatening view of the illness, a higher level of empowerment, and with higher mothers' and fathers' overall readiness scores. Adolescents' responsibility scores were positively associated with age and parental adolescent responsibility scores. Parental involvement scores were negatively associated with adolescents' age and positively with the mothers' parental involvement scores. By using a triadic evaluation, the results of the present study significantly extend what is currently known about this population.

  • 18. Burström, Åsa
    et al.
    Bratt, Ewa-Lena
    Frenckner, Björn
    Nisell, Margret
    Hanséus, Katarina
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Öjmyr-Joelsson, Maria
    Adolescents with congenital heart disease: their opinions about the preparation for transfer to adult care2017In: European Journal of Pediatrics, ISSN 0340-6199, E-ISSN 1432-1076, Vol. 176, no 7, p. 881-889Article in journal (Refereed)
    Abstract [en]

    The aim of the study was to explore what adolescents with congenital heart disease (CHD) view as important in the preparation for the transfer to adult care. We performed interviews in four focus groups with adolescents (14-18 years old) at four university hospitals in Sweden. Data was analysed using qualitative content analysis. The analysis revealed one main category; Becoming a manager of the condition and four subcategories; Sufficient knowledge about the health, Be a participant in the care, Parental support, and Communicate with others about the health. The adolescents' ages differentiated the discussion in the groups. The older adolescents seemed to have more interest in transition planning, information and transfer. The younger described more frustrations about communication and handling the disease. Conclusion: To become a manager of the CHD in daily life, the adolescents want disease specific knowledge, which should be communicated in a developmentally appropriate way. Adolescents want to participate and be involved in the transition process. They need support and guidance in how to communicate their CHD. Parental support is fundamental but it change over time. Moreover, peer-support is becoming more significant during the transition process.

  • 19. Burström, Åsa
    et al.
    Mora, Mariela Acuna
    Öjmyr-Joelsson, Maria
    Sparud-Lundin, Carina
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Hanseus, Katarina
    Frenckner, Björn
    Nisell, Margret
    Moons, Philip
    Bratt, Ewa-Lena
    Parental uncertainty about transferring their adolescent with congenital heart disease to adult care2019In: Journal of Advanced Nursing, ISSN 0309-2402, E-ISSN 1365-2648, Vol. 75, no 2, p. 380-387Article in journal (Refereed)
    Abstract [en]

    Aims: To study parent's levels of uncertainty related to the transfer from pediatric to adult care in adolescents with congenital heart disease (CHD) and to identify potentially correlating factors. Background: Parents acknowledge that during transition they struggle with finding ways of feeling secure in handing over the responsibility and letting go of control. Well-prepared and informed parents who feel secure are most likely better skilled to support their adolescent and to hand over the responsibility. Design: A cross-sectional study. Methods: Overall, 351 parents were included (35% response rate). Parental uncertainty was assessed using a Linear Analogue Scale (0-100). Data were collected between January - August 2016. Potential correlates were assessed using the readiness for transition questionnaire and sociodemographic data. Results: The mean parental uncertainty score was 42.5. Twenty-four percent of the parents had a very low level of uncertainty (score 0-10) and 7% had a very high level (score 91-100). Overall, 26% of the mothers and 36% of the fathers indicated that they had not started thinking of the transfer yet. The level of uncertainty was negatively associated with the level of perceived overall readiness. Adolescents' age, sex, CHD complexity, and parental age were not related to uncertainty. Conclusion: A wide range in the levels of uncertainty was found. Parents who were less involved in the care, or perceived their adolescent as readier for the transition, felt less uncertain. Still, thirty percent of the parents had not started to think about the transfer to adult care.

  • 20.
    Charisopoulou, Dafni
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine. Department of Paediatric Cardiology, Leeds Teaching Hospitals NHS Trust, Leeds, UK.
    Koulaouzidis, George
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine. Department of Cardiology, Mid Yorkshire Hospitals NHS Trust, Wakefield, UK.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Henein, Michael
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine. Molecular and Clinical Sciences Research Institute, St George University London, London, UK; Brunel University, Middlesex, UK.
    Exercise worsening of electromechanical disturbances: a predictor of arrhythmia in long QT syndrome2019In: Clinical Cardiology, ISSN 0160-9289, E-ISSN 1932-8737, Vol. 42, no 2, p. 235-240Article in journal (Refereed)
    Abstract [en]

    Background; Electromechanical (EM) coupling heterogeneity is significant in long QT syndrome (LQTS), particularly in symptomatic patients; EM window (EMW) has been proposed as an indicator of interaction and a better predictor of arrhythmia than QTc. Hypothesis To investigate the dynamic response of EMW to exercise in LQTS and its predictive value of arrhythmia.

    Methods: Forty-seven LQTS carriers (45 +/- 15 years, 20 with arrhythmic events), and 35 controls underwent exercise echocardiogram. EMW was measured as the time difference between aortic valve closure on Doppler and the end of QT interval on the superimposed electrocardiogram (ECG). Measurements were obtained at rest, peak exercise (PE) and 4 minutes into recovery.

    Results: Patients did not differ in age, gender, heart rate, or left ventricular ejection fraction but had a negative resting EMW compared with controls (-42 +/- 22 vs 17 +/- 5 ms, P < 0.0001). EMW became more negative at PE (-89 +/- 43 vs 16 +/- 7 ms, P = 0.0001) and recovery (-65 +/- 39 vs 16 +/- 6 ms, P = 0.001) in patients, particularly the symptomatic, but remained unchanged in controls. PE EMW was a stronger predictor of arrhythmic events than QTc (AUC:0.765 vs 0.569, P < 0.001). B-blockers did not affect EMW at rest but was less negative at PE (BB: -66 +/- 21 vs no-BB: -113 +/- 25 ms, P < 0.001). LQT1 patients had worse PE EMW negativity than LQT2.

    Conclusion: LQTS patients have significantly negative EMW, which worsens with exercise. These changes are more pronounced in patients with documented arrhythmic events and decrease with B-blocker therapy. Thus, EMW assessment during exercise may help improve risk stratification and management of LQTS patients.

  • 21.
    Charisopoulou, Dafni
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Department of Paediatric Cardiology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London, UK.
    Koulaouzidis, George
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Henein, Michael Y.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology. Molecular & Clinical Sciences Research Institute, St George University, London, UK.
    Abnormal ventricular repolarization in long QT syndrome carriers is related to short left ventricular filling time and attenuated stroke volume response during exercise2018In: Echocardiography, ISSN 0742-2822, E-ISSN 1540-8175, Vol. 35, no 8, p. 1116-1123Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Long QT syndrome (LQTS) carriers are characterized by abnormal ventricular repolarization, prolonged systole, and mechanical dispersion. Prolonged left ventricular (LV) systole has been shown to result in disproportionate shortening of LV filling in other conditions. The aim of this study was to assess LV filling, diastolic function, and stroke volume (SV) response to dynamic exercise, in a group of LQTS carriers.

    METHODS: Forty-seven LQTS carriers (45 ± 15 years, 20 symptomatic) and 35 healthy individuals underwent bicycle stress echocardiogram. Electrocardiographic and echocardiographic measurements were obtained at rest, peak exercise, and 4 minutes into recovery.

    RESULTS: Long QT syndrome carriers and controls did not differ in age, gender, heart rate, QRS duration, or LV ejection fraction. At rest, LQTS carriers had longer QTc and shorter filling time (FT). At peak exercise, QTc increased and remained longer than controls at recovery. A negative correlation was found between QTc and FT (r = -.398, P = .001) with greater fall in FT in LQTS carriers than in controls at peak exercise (-23% ± 10 vs +2% ± 3, P < .0001). FT correlated with SV (r = +.27, P = .001), which increased more in controls than in LQTS carriers (+32% ± 4 vs +2% ± 1, P < .05). These differences were more pronounced in symptomatic LQTS carriers who had shorter FT and smaller SV at peak exercise and during recovery compared to asymptomatics (P < .05).

    CONCLUSIONS: Long QT syndrome carriers have longer QTc, but also shorter FT. These disturbances worsen at peak exercise (particularly in symptomatics) compromising LV filling and SV, hence a potential pathomechanism for adverse events.

  • 22. Chockalingam, Priya
    et al.
    Crotti, Lia
    Girardengo, Giulia
    Johnson, Jonathan N
    Harris, Katy M
    van der Heijden, Jeroen F
    Hauer, Richard NW
    Beckmann, Britt M
    Spazzolini, Carla
    Rordorf, Roberto
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Clur, Sally-Ann B
    Fischer, Markus
    van den Heuvel, Freek
    Kaeaeb, Stefan
    Blom, Nico A
    Ackerman, Michael J
    Schwartz, Peter J
    Wilde, Arthur AM
    Not all beta-blockers are equal in the management of Long QT Syndrome types 1 and 2: higher recurrence of events under metoprolol2012In: Journal of the American College of Cardiology, ISSN 0735-1097, E-ISSN 1558-3597, Vol. 60, no 20, p. 2092-2099Article in journal (Refereed)
    Abstract [en]

    Objectives The purpose of this study was to compare the efficacy of beta-blockers in congenital long QT syndrome (LQTS). Background Beta-blockers are the mainstay in managing LQTS. Studies comparing the efficacy of commonly used beta-blockers are lacking, and clinicians generally assume they are equally effective.

    Methods Electrocardiographic and clinical parameters of 382 LQT1/LQT2 patients initiated on propranolol (n = 134), metoprolol (n = 147), and nadolol (n = 101) were analyzed, excluding patients <1 year of age at beta-blocker initiation. Symptoms before therapy and the first breakthrough cardiac events (BCEs) were documented.

    Results Patients (56% female, 27% symptomatic, heart rate 76 +/- 16 beats/min, QTc 472 +/- 46 ms) were started on beta-blocker therapy at a median age of 14 years (interquartile range: 8 to 32 years). The QTc shortening with propranolol was significantly greater than with other beta-blockers in the total cohort and in the subset with QTc >480 ms. None of the asymptomatic patients had BCEs. Among symptomatic patients (n = 101), 15 had BCEs (all syncopes). The QTc shortening was significantly less pronounced among patients with BCEs. There was a greater risk of BCEs for symptomatic patients initiated on metoprolol compared to users of the other 2 beta-blockers combined, after adjustment for genotype (odds ratio: 3.95, 95% confidence interval: 1.2 to 13.1, p = 0.025). Kaplan-Meier analysis showed a significantly lower event-free survival for symptomatic patients receiving metoprolol compared to propranolol/nadolol.

    Conclusions Propranolol has a significantly better QTc shortening effect compared to metoprolol and nadolol, especially in patients with prolonged QTc. Propranolol and nadolol are equally effective, whereas symptomatic patients started on metoprolol are at a significantly higher risk for BCEs. Metoprolol should not be used for symptomatic LQT1 and LQT2 patients.

    (J Am Coll Cardiol 2012;60:2092-9) (C) 2012 by the American College of Cardiology Foundation

  • 23. Clur, Sally-Ann B
    et al.
    Vink, Arja S
    Etheridge, Susan P
    Robles de Medina, Pascale G
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Ackerman, Michael J
    Wilde, Arthur A
    Blom, Nico A
    Benson, D Woodrow
    Herberg, Ulrike
    Donofrio, Mary T
    Cuneo, Bettina F
    Left Ventricular Isovolumetric Relaxation Time Is Prolonged in Fetal Long-QT Syndrome2018In: Circulation: Arrhythmia and Electrophysiology, ISSN 1941-3149, E-ISSN 1941-3084, Vol. 11, no 4, article id e005797Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Long-QT syndrome (LQTS), an inherited cardiac repolarization disorder, is an important cause of fetal and neonatal mortality. Detecting LQTS prenatally is challenging. A fetal heart rate (FHR) less than third percentile for gestational age is specific for LQTS, but the sensitivity is only ≈50%. Left ventricular isovolumetric relaxation time (LVIRT) was evaluated as a potential diagnostic marker for fetal LQTS.

    METHODS AND RESULTS: <0.001), as was the N-LVIRT. The best cutoff to diagnose LQTS was N-LVIRT ≥11.3 at ≤20 weeks (92% sensitivity, 70% specificity). Simultaneous analysis of N-LVIRT and FHR improved the sensitivity and specificity for LQTS (area under the curve=0.96; 95% confidence interval, 0.82-1.00 at 21-30 weeks). N-LVIRT, LV myocardial performance index, and FHR trends differed significantly between LQTS fetuses and controls through gestation.

    CONCLUSIONS: The LVIRT is prolonged in LQTS fetuses. Findings of a prolonged N-LVIRT and sinus bradycardia can improve the prenatal detection of fetal LQTS.

  • 24.
    Diamant, Ulla-Britt
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Jensen, Steen M
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Winbo, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Stattin, Eva-Lena
    Umeå University, Faculty of Medicine, Department of Medical Biosciences, Medical and Clinical Genetics.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Vectorcardiographic recordings of the Q-T interval in a pediatric long Q-T syndrome population2013In: Pediatric Cardiology, ISSN 0172-0643, E-ISSN 1432-1971, Vol. 34, no 2, p. 245-249Article in journal (Refereed)
    Abstract [en]

    Measurements of the Q-T interval are less reliable in children than in adults. Identification of superior diagnostic tools is warranted. This study aimed to investigate whether a vectorcardiogram (VCG) recorded from three orthogonal leads (X, Y, Z) according to Frank is superior to a 12-lead electrocardiogram (ECG) in providing a correct long Q-T syndrome (LQTS) diagnosis in children. This LQTS group consisted of 35 genetically confirmed carriers of mutations in the KCNQ1 (n = 29) and KCNH2 (n = 6) genes. The control group consisted of 35 age- and gender-matched healthy children. The mean age was 7 years in the LQTS group and 6.7 years in the control group (range, 0.5-16 years). The corrected Q-T interval (QT(c)) was measured manually (QT(man)) by one author (A.W.). The 12-lead ECG automatic measurements (QT(ECG)) and interpretation (QT(Interpret)) of QT(c) were performed with the Mac5000 (GE Medical System), and the VCG automatic measurements (QT(VCG)) were performed with the Mida1000, CoroNet (Ortivus AB, Sweden). By either method, a QT(c) longer than 440 ms was considered prolonged and indicative of LQTS. Of the 35 children with genetically confirmed LQTS, 30 (86 %) received a correct diagnosis using QT(VCG), 29 (82 %) using QT(man), 24 (69 %) using QT(ECG), and 17 (49 %) using QT(Interpret). Specificity was 0.80 for QT(VCG), 0.83 for QT(man), 0.77 for QT(ECG), and 0.83 for QT(Interpret). The VCG automatic measurement of QT(c) seems to be a better predictor of LQTS than automatic measurement and interpretation of 12-lead ECG.

  • 25.
    Diamant, Ulla-Britt
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Vahedi, Farzad
    Sahlgrenska Akademin Göteborgs Universitet.
    Winbo, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Stattin, Eva-Lena
    Umeå University, Faculty of Medicine, Department of Medical Biosciences, Medical and Clinical Genetics.
    Jensen, Steen M
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Bergfeldt, Lennart
    Sahlgrenska Akademin Göteborgs Universitet.
    Electrophysiological phenotype in the LQTS mutations Y111C and R518X in the KCNQ1 gene2013In: Journal of applied physiology, ISSN 8750-7587, E-ISSN 1522-1601, Vol. 115, no 10, p. 1423-1432Article in journal (Refereed)
    Abstract [en]

    Long QT syndrome is the prototypical disorder of ventricular repolarization (VR), and a genotype-phenotype relation is postulated. Furthermore, although increased VR heterogeneity (dispersion) may be important in the arrhythmogenicity in long QT syndrome, this hypothesis has not been evaluated in humans and cannot be tested by conventional electrocardiography. In contrast, vectorcardiography allows assessment of VR heterogeneity and is more sensitive to VR alterations than electrocardiography. Therefore, vectorcardiography was used to compare the electrophysiological phenotypes of two mutations in the LQT1 gene with different in vitro biophysical properties, and with LQT2 mutation carriers and healthy control subjects. We included 99 LQT1 gene mutation carriers (57 Y111C, 42 R518X) and 19 LQT2 gene mutation carriers. Potassium channel function is in vitro most severely impaired in Y111C. The control group consisted of 121 healthy subjects. QRS, QT, and T-peak to T-end (Tp-e) intervals, measures of the QRS vector and T vector and their relationship, and T-loop morphology parameters were compared at rest. Apart from a longer heart rate-corrected QT interval (QT heart rate corrected according to Bazett) in Y111C mutation carriers, there were no significant differences between the two LQT1 mutations. No signs of increased VR heterogeneity were observed among the LQT1 and LQT2 mutation carriers. QT heart rate corrected according to Bazett and Tp-e were longer, and the Tp-e-to-QT ratio greater in LQT2 than in LQT1 and the control group. In conclusion, there was a marked discrepancy between in vitro potassium channel function and in vivo electrophysiological properties in these two LQT1 mutations. Together with previous observations of the relatively low risk for clinical events in Y111C mutation carriers, our results indicate need for cautiousness in predicting in vivo electrophysiological properties and the propensity for clinical events based on in vitro assessment of ion channel function alone.

  • 26.
    Diamant, Ulla-Britt
    et al.
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences. Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Winbo, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Stattin, Eva-Lena
    Umeå University, Faculty of Medicine, Department of Medical Biosciences, Medical and Clinical Genetics.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Kesek, Milos
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Jensen, Steen M
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
    Two automatic QT algorithms compared with manual measurement in identification of long QT syndrome2010In: Journal of Electrocardiology, ISSN 0022-0736, E-ISSN 1532-8430, Vol. 43, no 1, p. 25-30Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Long QT syndrome (LQTS) is an inherited disorder that increases the risk of syncope and malignant ventricular arrhythmias, which may result in sudden death.

    METHODS: We compared manual measurement by 4 observers (QT(manual)) and 3 computerized measurements for QT interval accuracy in the diagnosis of LQTS: 1. QT measured from the vector magnitude calculated from the 3 averaged orthogonal leads X, Y, and Z (QTVCG) and classified using the same predefined QTc cut-points for classification of QT prolongation as in manual measurements; 2. QT measured by a 12-lead electrocardiogram (ECG) program (QTECG) and subsequently classified using the same cut-points as in (1) above; 3. The same QT value as in (2) above, automatically classified by a 12-lead ECG program with thresholds for QT prolongation adjusted for age and sex (QTinterpret). The population consisted of 94 genetically confirmed carriers of KCNQ1 (LQT1) and KCNH2 (LQT2) mutations and a combined control group of 28 genetically confirmed noncarriers and 66 unrelated healthy volunteers.

    RESULTS: QT(VCG) provided the best combination of sensitivity (89%) and specificity (90%) in diagnosing LQTS, with 0.948 as the area under the receiver operating characteristic curve. The evaluation of QT measurement by the 4 observers revealed a high interreader variability, and only 1 of 4 observers showed acceptable level of agreement in LQTS mutation carrier identification (kappa coefficient >0.75).

    CONCLUSION: Automatic QT measurement by the Mida1000/CoroNet system (Ortivus AB, Danderyd, Sweden) is an accurate, efficient, and easily applied method for initial screening for LQTS.

  • 27.
    Grahn, K
    et al.
    Umeå University, Faculty of Medicine, Department of Odontology, Pediatric Dentistry.
    Wikström, S
    Umeå University, Faculty of Medicine, Department of Odontology, Pediatric Dentistry.
    Nyman, Linda
    Umeå University, Faculty of Medicine, Department of Odontology, Pediatric Dentistry.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Stecksén-Blicks, Christina
    Umeå University, Faculty of Medicine, Department of Odontology, Pediatric Dentistry.
    Attitudes about dental care among parents whose children suffer from severe congenital heart disease: a case-control study2006In: International Journal of Paediatric Dentistry, ISSN 0960-7439, E-ISSN 1365-263X, Vol. 16, no 4, p. 231-238Article in journal (Refereed)
    Abstract [en]

    OBJECTIVES: To examine attitudes and experiences of parents whose children have complex congenital heart disease (CHD) with respect to dental health information and advice, dental care, and service and to compare the results with data from an age- and gender-matched control group without any medical problems.

    SETTING: Faculty of Medicine (Paediatric Cardiology and Paediatric Dentistry), Umea University, Umea, Sweden.

    SAMPLE AND METHOD: Each group comprised parents of 33 children; the children's mean age was 9.4 years. All the cases and the controls resided in the county of Vasterbotten, northern Sweden. Data were collected with a questionnaire with 20 joint questions to both groups and four additional questions to the CHD group.

    RESULTS: Of the 20 joint questions, significant differences were displayed in the following areas: the professional group that provided the parents with dental health information and advice (P < 0.01), attitudes to reception at the dental clinic, and experience of sedation before operative dental treatment (P < 0.05). Parents to 11 children with CHD who were patients at a specialist clinic for paediatric dentistry scored the reception at the dental clinic as excellent in nine cases and satisfactory in two, compared to excellent (3), satisfactory (11), decent (4), and poor (4) among those who were patients in general dental practice (P < 0.01). No statistically significant differences in educational level or in parental experience of dental health were noted between the two groups (P > 0.05).

    CONCLUSION: Children with CHD in northern Sweden mainly receive their dental health information from a physician or a dentist, and healthy children mainly receive information from a dental hygienist indicating that children with CHD are given priority in the dental care system. Parental attitudes to reception in the dental service differed, and parents of healthy children scored the reception at the dental clinic better than parents of children with CHD. It is suggested that children with severe CHD should receive dental care in clinics for paediatric dentistry, particularly at early ages.

  • 28. Granberg, Marie
    et al.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Fisher, Anne G
    Activities in daily living and schoolwork task performance in children with complex congenital heart disease.2008In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 97, no 9, p. 1270-4Article in journal (Refereed)
    Abstract [en]

    AIM: To examine if children with complex congenital heart disease (CHD) differ significantly from healthy children in performance of activities in daily living (ADL) and schoolwork tasks. METHODS: Fourteen children with complex CHD (3-14 years) were matched in pairs by age and gender to healthy children (n = 14). The ADL Taxonomy was used to evaluate ability to perform ADL and the School Version of the Assessment of Motor and Process Skills (AMPS) to evaluate quality of schoolwork task performance. RESULTS: The children with complex CHD had significantly lower mean ADL, school motor and school process performance. Eight of the pairs differed significantly in school motor ability, ten in school process performance and nine in ability to perform ADL. CONCLUSION: This study indicates that there is a difference in overall performance both in ADL and schoolwork task performance between children with complex CHD and healthy children. These findings may suggest that early interventions should be provided for children with complex CHD, who often do not receive such services, in order to enhance participation in childhood ADL and schoolwork.

  • 29.
    Hansson, Lena
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Lind, Torbjörn
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Wiklund, Urban
    Umeå University, Faculty of Medicine, Department of Radiation Sciences.
    Öhlund, Inger
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Fluid restriction negatively affects energy intake and growth in very low birthweight infants with haemodynamically significant patent ductus arteriosus2019In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 108, no 11, p. 1985-1992Article in journal (Refereed)
    Abstract [en]

    Aim: We explored if fluid restriction in very low birthweight (VLBW) infants with a haemodynamically significant patent ductus arteriosus (PDA) affected energy and protein intakes and growth.

    Methods: Retrospectively, we identified 90 VLBW infants that were admitted to Umea University Hospital, Sweden, between 2009 and 2012: 42 with and 48 without haemodynamically significant PDA (hsPDA). Anthropometric, fluid, energy and protein intake data during the first 28 days of life were expressed as z‐scores.

    Results: In the 42 infants diagnosed with hsPDA, fluid intake was restricted after diagnosis, resulting in a decrease in energy and protein intake. No decrease was observed in the other 48 infants in the cohort. Multivariate analysis showed that the z‐score of weight change depended on both ductus arteriosus status and energy intake; thus, infants with hsPDA did not grow as expected with the energy provided to them.

    Conclusion: Energy and protein intake was diminished in prematurely born infants with hsPDA when fluid was restricted after diagnosis. The initial reduction in intakes may have contributed to the lower postnatal growth observed in these infants.

  • 30.
    Hansson, Lena
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Stecksen-Blicks, Christina
    Umeå University, Faculty of Medicine, Department of Odontology, Pediatric Dentistry.
    Oral microflora and dietary intake in infants with congenital heart disease: a case control study2012In: European Archives of Paediatric Dentistry, ISSN 1818-6300, E-ISSN 1996-9805, Vol. 13, no 5, p. 238-243Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Infants with moderate to severe congenital heart disease (CHD) are at a higher risk for growth failure and malnutrition due to increased metabolic demands and inadequate energy intake. This state requires meals that are more frequent and a special enriched diet, which may have negative implications on oral health.

    AIM: To examine the oral colonisation of some bacteria associated with caries development during infancy; mutans streptococci (MS) and lactobacilli (LCB) in infants with CHD and whether their dietary intake had an impact on the bacterial levels.

    DESIGN: This was a prospective case-control study. 11 infants with CHD and 22 healthy, age-matched infants were enrolled. Saliva samples and food diaries were collected at 6, 9, and 12 months of age. The total viable counts of MS and LCB in saliva were determined, and energy intake, meal frequency, intake of proteins, fat, carbohydrates and sucrose were calculated.

    RESULTS: At 12 months of age, the MS count was higher in the CHD group than in the controls (p<0.01), and MS constituted a higher ratio of the total viable count of oral bacteria (p<0.01). Meal frequency was higher in the CHD group at 6 and 9 months of age than in the controls (p<0.05). The intake of sucrose did not differ between the groups, while the total carbohydrate intake was higher in the control group at 6 and 12 months of age (p<0.05). Compared with the control group, which had six courses of antibiotic administration, the CHD infants had 21 courses (p<0.05).

    CONCLUSIONS: Infants with severe CHD have higher levels of MS at 12 months of age than the healthy controls. A higher meal frequency and use of diuretic medication and antibiotics may have influenced MS colonisation.

  • 31.
    Hansson, Lena
    et al.
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Öhlund, Inger
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Lind, Torbjörn
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Stecksén-Blicks, Christina
    Umeå University, Faculty of Medicine, Department of Odontology.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Dietary intake in infants with complex congenital heart disease: a case-control study on macro- and micronutrient intake, meal frequency and growth2016In: Journal of human nutrition and dietetics (Print), ISSN 0952-3871, E-ISSN 1365-277X, Vol. 29, no 1, p. 67-74Article in journal (Refereed)
    Abstract [en]

    BACKGROUND: Children with severe congenital heart disease (CHD) need considerable nutritional support to reach normal growth. The actual intake of macro- and micronutrients in outpatient CHD infants over a 6-month period in infancy is not described in the literature. The present study aimed to prospectively investigate the distribution between macro- and micronutrient intake, meal frequency and growth in children with CHD.

    METHODS: At 6, 9 and 12 months of age, a 3-day food diary and anthropometric data were collected in 11 infants with severe CHD and 22 healthy age- and feeding-matched controls. Macro- and micronutrient intake, meal frequency and growth were calculated.

    RESULTS: Compared to the healthy controls, CHD infants had a statistically significantly higher intake of fat at 9 months of age (4.8 versus 3.6 g kg(-1) day(-1) ), a higher percentage energy (E%) from fat, (40.6% versus 34.5%) and a lower E% from carbohydrates (46.1% versus 39.6%) at 12 months of age, and a lower intake of iron (7.22 versus 9.28 mg day(-1) ) at 6 months of age. Meal frequency was significantly higher at 6 and 9 months of age (P < 0.01). Mean Z-score weight for height, weight for age and body mass index for age were significant lower (P < 0.01) at all time points.

    CONCLUSIONS: Despite a higher intake of energy from fat and a higher meal frequency, the intake does not meet the needs for growth, and the results may indicate a low intake of micronutrients in CHD infants.

  • 32. Jansweijer, Joeri A.
    et al.
    van Spaendonck-Zwarts, Karin Y.
    Tanck, Michael W. T.
    van Tintelen, J. Peter
    Christiaans, Imke
    van der Smagt, Jasper
    Vermeer, Alexa
    Bos, J. Martijn
    Moss, Arthur J.
    Swan, Heikki
    Priori, Sylvia
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences.
    Tfelt-Hansen, Jacob
    Ackerman, Michael
    Olivotto, Iacopo
    Charron, Philippe
    Gimeno, Juan R.
    van den Berg, Maarten
    Wilde, Arthur
    Pinto, Yigal M.
    Heritability in genetic heart disease: the role of genetic background2019In: Open heart, E-ISSN 2053-3624, Vol. 6, no 1, article id UNSP e000929Article in journal (Refereed)
    Abstract [en]

    Background: Mutations in genes encoding ion channels or sarcomeric proteins are an important cause of hereditary cardiac disease. However, the severity of the resultant disease varies considerably even among those with an identical mutation. Such clinical variation is often thought to be explained largely by differences in genetic background or ‘modifier genes’. We aimed to test the prediction that identical genetic backgrounds result in largely similar clinical expression of a cardiac disease causing mutation, by studying the clinical expression of mutations causing cardiac disease in monozygotic twins.

    Methods: We compared first available clinical information on 46 monozygotic twin pairs and 59 control pairs that had either a hereditary cardiomyopathy or channelopathy.

    Results: Despite limited power of this study, we found significant heritability for corrected QT interval (QTc) in long QT syndrome (LQTS). We could not detect significant heritability for structural traits, but found a significant environmental effect on thickness of the interventricular septum in hypertrophic cardiomyopathy.

    Conclusions: Our study confirms previously found robust heritability for electrical traits like QTc in LQTS, and adds information on low or lacking heritability for structural traits in heritable cardiomyopathies. This may steer the search for genetic modifiers in heritable cardiac disease.

  • 33.
    Jashari, Haki
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Lannering, Katarina
    Ibrahimi, Pranvera
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Djekic, Demir
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Mellander, Mats
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Henein, Michael Y
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Persistent reduced myocardial deformation in neonates after CoA repair2016In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 221, no 15, p. 886-891Article in journal (Refereed)
    Abstract [en]

    INTRODUCTION: Surgical repair of coarctation of the aorta (CoA) is a safe procedure in children, however the condition is known for its potential recurrence and other related complications. The available evidence shows abnormal intrinsic properties of the aorta in CoA, thus suggesting additional effect, even after CoA repair, on left ventricular (LV) function. Accordingly, we sought to obtain a better understanding of LV myocardial mechanics in very early-corrected CoA using two-dimensional STE.

    METHODS AND RESULTS: We retrospectively studied 21 patients with corrected CoA at a median age of 9 (2-53) days at three time points: 1) just before intervention, 2) at short-term follow-up and 3) at medium-term follow-up after intervention and compared them with normal values. Speckle tracking analysis was conducted via vendor independent software, Tomtec. After intervention, LV function significantly improved (from -12.8±3.9 to -16.7±1.7; p<0.001), however normal values were not reached even at medium term follow-up (-18.3±1.7 vs. -20±1.6; p=0.002). Medium term longitudinal strain correlated with pre intervention EF (r=0.58, p=0.006). Moreover, medium term subnormal values were more frequently associated with bicuspid aortic valve (33.3% vs. 66.6%; p<0.05).

    CONCLUSION: LV myocardial function in neonates with CoA can be feasibly evaluated and followed up by speckle tracking echocardiography. LV subendocardial dysfunction however, remains in early infancy coarctation long after repair. Long-term follow-up through adulthood using myocardial deformation measurements should shed light on the natural history and consequences of this anomaly.

  • 34.
    Jashari, Haki
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Lannering, Katarina
    Mellander, Mats
    Ibrahimi, Pranvera
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Henein, Michael Y
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Coarctation repair normalizes left ventricular function and aorto-septal angle in neonates2017In: Congenital Heart Disease, ISSN 1747-079X, E-ISSN 1747-0803, Vol. 12, no 2, p. 218-225Article in journal (Refereed)
    Abstract [en]

    Background and aims: Patients with coarctation of the aorta (CoA) have increased left ventricular (LV) afterload that has been shown to impact the LV and ascending aortic function. We aimed to examine the effect of coarctation on LV function and aorto-septal angle (AoSA) before and after surgical repair.

    Methods: We retrospectively studied 21 patients with surgically repaired CoA at a median age of 9 (2-53) days at three time points: (1) just before intervention, (2) at short-term follow-up, and (3) at medium-term follow-up after intervention. AoSA was measured from the parasternal long axisview, at three time points during the cardiac cycle: (1) end diastole, (2) beginning of systole, and (3) at peak ejection in the descending aorta. In addition to conventional LV structure and function, global longitudinal strain, and strain rate were measured using STE technique and Tomtec soft-ware. Three groups of age matched healthy children served as controls at each time point.

    Results: AoSA was significantly wider before intervention, in particular at peak ejection in the descending aorta (1448 6 6.48 vs. 1368 6 4.18; P < .0001), and correlated with CoA pressure gradi-ent. After intervention, AoSA normalized and significantly correlated with the increase of LV cavity function and overall LV deformation parameters.

    Conclusions: AoSA is abnormally wide in neonates with CoA and is associated with severity ofobstruction, LV dysfunction and compromised LV global deformation.

  • 35.
    Jashari, Haki
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Ibrahimi, Pranvera
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Bajraktari, Gani
    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.
    Left ventricular response to pressure afterload in children: aortic stenosis and coarctation A systematic review of the current evidence2015In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 178, p. 203-209Article, review/survey (Refereed)
    Abstract [en]

    Congenital aortic stenosis (CAS) and Coarctation of Aorta (CoA) represent two forms of pressure afterload that affect the left ventricle (LV), hence require regular echocardiographic monitoring. Subclinical dysfunction of the LV exists even in asymptomatic patients with preserved left ventricular ejection fraction (EF), implying low sensitivity of EF in predicting optimum time for intervention. In this article we review patterns of LV myocardial deformation before and after correction of CAS and CoA in infants, children and adolescents, showing their important role in monitoring the course of LV dysfunction. A systematic search using PubMed was performed and suitable studies are presented on a narrative form. Normal EF and/or fractional shortening (FS), with subclinical myocardial dysfunction are reported in all studies before intervention. The short-term results, after intervention, were related to the type of procedure, with no improvement or further deterioration related to surgery but immediate improvement after balloon intervention. Long term follow-up showed further improvement but still subnormal function. Thus correction of CAS and CoA before irreversible LV dysfunction is vital, and requires longitudinal studies in order to identify the most accurate parameter for function prognostication. Until then, conventional echocardiographic parameters together with myocardial velocities and deformation parameters should continue to provide follow-up reproducible measures of ventricular function.

  • 36.
    Jashari, Haki
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Ibrahimi, Pranvera
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Bajraktari, Gani
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Kryeziu, Lindita
    Jashari, Fisnik
    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.
    Normal ranges of left ventricular strain in children: a meta-analysis2015In: Cardiovascular Ultrasound, ISSN 1476-7120, E-ISSN 1476-7120, Vol. 13, article id 37Article, review/survey (Refereed)
    Abstract [en]

    Aims: The definition of normal values of two-dimensional speckle-tracking echocardiography derived left ventricular (LV) deformation parameters, is of critical importance for the routine application of this modality in children. The objectives of this study were to perform a meta-analysis of normal ranges for longitudinal, circumferential and radial strain/strain rate values and to identify confounders that may contribute to differences in reported measures. Methods and Results: A systematic search was conducted. Studies describing normal healthy subjects and observational studies that used control groups as a comparison were included. Data were combined using a random-effect model. Effects of demographic, clinical and equipment variables were assessed through meta-regression. The search identified 1,192 subjects form 28 articles. Longitudinal strain (LS) normal mean values varied from -12.9 to -26.5 (mean, -20.5; 95 % CI, -20.0 to -21.0). Normal mean values of circumferential strain (CS) varied from -10.5 to -27.0 (mean, -22.06; 95 % CI, -21.5 to -22.5). Radial strain (RS) normal mean values varied from 24.9 to 62.1 (mean, 45.4; 95 % CI, 43.0 to 47.8). Meta-regression showed LV end diastolic diameter as a significant determinant of variation for LS. Longitudinal systolic strain rate (LSRs) was significantly determined by the age and RS by the type of vendor used. Conclusion: Variations among different normal ranges were dependent on the vendor used, LV end-diastolic diameter and age. Vendor-independent software for analyzing myocardial deformation in children, using images from different vendors would be the ideal solution for strain measurements or else using the same system for patient's follow up.

  • 37.
    Karlsson, Marcus
    et al.
    Umeå University, Faculty of Medicine, Department of Radiation Sciences, Radiation Physics.
    Hörnsten, Rolf
    Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Clinical Physiology.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Wiklund, Urban
    Umeå University, Faculty of Medicine, Department of Radiation Sciences, Radiation Physics.
    Automatic filtering of outliers in RR intervals before analysis of heart rate variability in Holter recordings: a comparison with carefully edited data.2012In: Biomedical engineering online, ISSN 1475-925X, E-ISSN 1475-925X, Vol. 11, no 2, p. 12-Article in journal (Refereed)
    Abstract [en]

    Background: Undetected arrhythmic beats seriously affect the power spectrum of the heart rate variability (HRV). Therefore, the series of RR intervals are normally carefully edited before HRV is analysed, but this is a time consuming procedure when 24-hours recordings are analysed. Alternatively, different methods can be used for automatic removal of arrhythmic beats and artefacts. This study compared common frequency domain indices of HRV when determined from manually edited and automatically filtered RR intervals.

    Methods and Results: Twenty-four hours Holter recordings were available from 140 healthy subjects of age 1-75 years. An experienced technician carefully edited all recordings. Automatic filtering was performed using a recursive procedure where RR intervals were removed if they differed from the mean of the surrounding RR intervals with more than a predetermined limit (ranging from 10% to 50%). The filtering algorithm was evaluated by replacing 1% of the beats with synthesised ectopic beats. Power spectral analysis was performed before and after filtering of both the original edited data and the noisy data set. The results from the analysis using the noisy data were used to define an age-based filtering threshold. The age-based filtration was evaluated with completely unedited data, generated by removing all annotations from the series of RR intervals, and then comparing the resulting HRV indices with those obtained using edited data. The results showed equivalent results after age-based filtration of both the edited and unedited data sets, where the differences in HRV indices obtained by different preprocessing methods were small compared to the mean values within each age group.

    Conclusions: The study showed that it might not be necessary to perform the time-consuming careful editing of all detected heartbeats before HRV is analysed in Holter recordings.

    In most subjects, it is sufficient to perform the regular editing needed for valid arrhythmia analyses, and then remove undetected ectopic beats and artefacts by age-based filtration of the series of RR intervals, particularly in subjects older than 30 years.

  • 38.
    Kesek, Milos
    et al.
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Jensen, Steen M
    Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
    Two Cases of LQT Syndrome with Malignant Syncope after Switch from Propranolol to Bisoprolol2016In: Pacing and Clinical Electrophysiology, ISSN 0147-8389, E-ISSN 1540-8159, Vol. 39, no 3, p. 305-306Article in journal (Refereed)
    Abstract [en]

    Propranolol in slow-release form has been the first-line treatment in long QT (LQT) until it was withdrawn from the market. We describe two cases where a switch to bisoprolol resulted in worsening of arrhythmia control: A man with LQT2, asymptomatic on propranolol, experienced syncope after switching to bisoprolol 5 mg daily. He switched back to propranolol and has remained asymptomatic during subsequent 12 months. A man with classical Jervell Lange-Nielsen syndrome, previous gangliectomy, and ICD implantation, switched to bisoprolol 5 mg daily. Four months later he experienced a tachycardia storm. He switched back to propranolol and has remained free from arrhythmias during subsequent 12 months.

  • 39. Mann, Stefan A.
    et al.
    Imtiaz, Mohammad
    Winbo, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Rydberg, Annika
    Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
    Perry, Matthew D.
    Couderc, Jean-Philippe
    Polonsky, Bronislava
    McNitt, Scott
    Zareba, Wojciech
    Hill, Adam P.
    Vandenberg, Jamie I.
    Convergence of models of human ventricular myocyte electrophysiology after global optimization to recapitulate clinical long QT phenotypes2016In: Journal of Molecular and Cellular Cardiology, ISSN 0022-2828, E-ISSN 1095-8584, Vol. 100, p. 25-34Article in journal (Refereed)
    Abstract [en]

    In-silico models of human cardiac electrophysiology are now being considered for prediction of cardiotoxicity as part of the preclinical assessment phase of all new drugs. We ask the question whether any of the available models are actually fit for this purpose. We tested three models of the human ventricular action potential, the O'hara-Rudy (ORD11), the Grandi-Bers (GB10) and the Ten Tusscher (TT06) models. We extracted clinical QT data for LQTS1 and LQTS2 patients with nonsense mutations that would be predicted to cause 50% loss of function in I-Ks and I-Kr respectively. We also obtained clinical QT data for LQTS3 patients. We then used a global optimization approach to improve the existing in silico models so that they reproduced all three clinical data sets more closely. We also examined the effects of adrenergic stimulation in the different LQTS subsets. All models, in their original form, produce markedly different and unrealistic predictions of QT prolongation for LQTS1, 2 and 3. After global optimization of the maximum conductances for membrane channels, all models have similar current densities during the action potential, despite differences in kinetic properties of the channels in the different models, and more closely reproduce the prolongation of repolarization seen in all LQTS subtypes. In-silico models of cardiac electrophysiology have the potential to be tremendously useful in complementing traditional preclinical drug testing studies. However, our results demonstrate they should be carefully validated and optimized to clinical data before they can be used for this purpose.

  • 40. Mohlkert, Lilly-Ann
    et al.