| 1. |
|
|
| 2. |
- Ashman Kröönström, Linda, 1982, et al.
(författare)
-
Exercise capacity, physical activity, and health-related quality of life in adults with CHD
- 2020
-
Ingår i: Cardiology in the Young. - 1047-9511 .- 1467-1107. ; 30:5, s. 668-673
-
Tidskriftsartikel (refereegranskat)abstract
- Objectives: The aim of this study was to assess exercise capacity, physical activity, and health-related quality of life within a broad and unselected group of adults with CHD.Design: From April 2009 to February 2014, 1310 patients were assessed for suitability to participate in this single-centre cross-sectional study. Seven hundred and forty-seven (57%) patients were included, performed a submaximal bicycle test, and answered questionnaires regarding physical activity and health-related quality of life. Exercise capacity, physical activity, and health-related quality of life were compared with reference values and correlations were studied.Results: The exercise capacities of men and women with CHD were 58.7 and 66.3%, respectively, of reference values. Approximately, 20-25% of the patients did not achieve the recommended amount of physical activity. In addition, men scored significantly less points on 7 out of 10 scales of health-related quality of life and women in 6 out of 10 scales, compared with reference values. The strongest correlation was between exercise capacity and the Short Form-36 (physical function).Conclusions: Exercise capacity was impaired in all adults with CHD, including those with less complicated CHD. One-quarter of the patients did not achieve the recommended levels of physical activity. Exercise tests followed by individualised exercise prescriptions may be offered to all patients with CHD aiming to increase exercise capacity, levels of physical activity, improve health-related quality of life, and reduce the risk of acquired life-style diseases.
|
|
| 3. |
- Bay, Annika, 1970-, et al.
(författare)
-
It ́s like balancing on a slackline : A description from adults living with congenital heart disease
- 2018
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 28:Suppl. S1, s. S37-S37
-
Tidskriftsartikel (refereegranskat)abstract
- Introduction: Several studies have shown that adults with congenital heart disease have reduced exercise capacity and do not reach the recommended daily level of physical activity. With this in view, it is of great importance to investigate how this population experiences physical activity. The aim of the study is to illuminate how adults with congenital heart disease describes themselves in relation to physical activity.Methods: Semi-structured interviews with fourteen adults with complex congenital heart disease were performed. Patients were recruited from the clinic waiting list, based on their scheduled follow up and diagnosis. Interviews were analysed by qualitative content analysis.Results: The overall theme It´s like balancing on a slackline illustrates how adults with congenital heart disease described themselves in relation to physical activity. The overall theme consists of four themes: Being an adventurer- enjoying the challenges of physical activity, Being a realist- adapting to physical ability, Beinga non-doer- lacking prerequisites for physical activity and Being an outsider- feeling excluded depending on physical ability.Conclusions: The descriptions on themselves as a physically active were not constant or one-dimensional and the descriptions varied during the interviews, related to different time periods in life. It meant that they could described themselves as being an adventurer liking tough challenges, but at the same time describing themselves as being a non-doer with uncertainty over their physical strength. The findings point out specific factors for adults with CHD that might constitute as obstacles, but also possibilities for being physically active.
|
|
| 4. |
- Berghammer, Malin, 1970-, et al.
(författare)
-
Comparison of participants and non-participants in patient-reported outcome surveys : the case of Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study
- 2017
-
Ingår i: Cardiology in the Young. - : CAMBRIDGE UNIV PRESS. - 1047-9511 .- 1467-1107. ; 27:3, s. 427-434
-
Tidskriftsartikel (refereegranskat)abstract
- Background: The last decade has seen a vast increase in the use of patient-reported outcomes. As patientreported outcomes are used in order to capture patients' perspectives of their health and illness, it is a prerequisite for accurate patient-reported outcome evaluations to use representative samples. In order to evaluate representativeness, the present study focussed on the comparison between participants and non-participants in the Swedish branch of the international study APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study), regarding demographic, clinical, and health status characteristics. Methods: Eligible patients for APPROACH-IS were identified and selected from SWEDCON, the Swedish registry for congenital heart disease (CHD). Overall, 912 eligible patients were identified, of whom 471 participated, 398 did not participate, and 43 were either unreachable or declined to participate in APPROACH-IS. The participants and nonparticipants were compared in terms of statistical significance and effect sizes. Results: Significant differences were observed between participants and non-participants for sex, age, primary diagnosis, number of cardiac operations, and fatigue; however, the effect sizes were in general small, except for the difference in primary diagnosis. No differences between the two groups were found in number of catheterisations, implanted device, the distribution of NYHA functional class, or health status and symptoms. Conclusions: This study shows that participants and non-participants are relatively comparable groups, which confirms the representativeness of the participants. The Swedish data from APPROACH-IS can therefore be reliably generalised to the population of adults with CHD in Sweden.
|
|
| 5. |
- Birkeland, Anna-Lena, 1955-, et al.
(författare)
-
Breaking bad news : an interview study of paediatric cardiologists
- 2011
-
Ingår i: Cardiology in the Young. - 1047-9511 .- 1467-1107. ; 21:3, s. 286-291
-
Tidskriftsartikel (refereegranskat)abstract
- 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.
|
|
| 6. |
- Björk, Anna, et al.
(författare)
-
Type 1 diabetes mellitus and associated risk factors in patients with or without CHD: a case-control study
- 2017
-
Ingår i: Cardiology in the Young. - : Cambridge University Press (CUP). - 1047-9511 .- 1467-1107. ; 27:9, s. 1670-1677
-
Tidskriftsartikel (refereegranskat)abstract
- Background: Approximately 1% of children are born with CHD, and 90-95% reach adulthood. Increased exposure to infections and stress-strain can contribute to an increased risk of developing type 1 diabetes mellitus. CHD may increase the risk of more serious infections, stress-strain, and increased risk of developing type 1 diabetes mellitus. Methods: We analysed the onset of and the risk of mortality and morbidity associated with concurrent CHD in patients with type 1 diabetes mellitus compared with patients with type 1 diabetes mellitus without CHD. The study combined data from the National Diabetes Register and the National Patient Register. Results: A total of 104 patients with CHD and type 1 diabetes mellitus were matched with 520 controls. Patients with CHD and type 1 diabetes mellitus had an earlier onset of diabetes (13.9 versus 17.4 years, p < 0.001), longer duration of diabetes (22.4 versus 18.1 years, p < 0.001), higher prevalence of retinopathy (64.0 versus 43.0%, p = 0.003), higher creatinine levels (83.5 versus 74.1 mu mol/L, p = 0.03), higher mortality (16 versus 5%, p = 0.002), and after onset of type 1 diabetes mellitus higher rates of co-morbidity (5.28 versus 3.18, p <= 0.01), heart failure (9 versus 2%, p = 0.02), and stroke (6 versus 2%, p = 0.048) compared with controls. Conclusions: From a nationwide register of patients with type 1 diabetes mellitus, the coexistence of CHD and type 1 diabetes mellitus was associated with an earlier onset, a higher frequency of microvascular complications, co-morbidity, and mortality.
|
|
| 7. |
- Bratt, Ewa-Lena, 1970, et al.
(författare)
-
Effects of lifestyle changes and high-dose β-blocker therapy on exercise capacity in children, adolescents, and young adults with hypertrophic cardiomyopathy
- 2015
-
Ingår i: Cardiology in the Young. - 1047-9511 .- 1467-1107. ; 25:3, s. 501-510
-
Tidskriftsartikel (refereegranskat)abstract
- Aim: The use of β-blocker therapy in asymptomatic patients with hypertrophic cardiomyopathy is controversial. This study evaluates the effect of lifestyle changes and high-dose β-blocker therapy on their exercise capacity. Methods and results: A total of 29 consecutive newly diagnosed asymptomatic patients with familial hypertrophic cardiomyopathy, median age 15 years (range 7–25), were recruited. In all, 16 patients with risk factors for sudden death were treated with propranolol if no contraindications, or equivalent doses of metoprolol; 13 with no risk factors were randomised to metoprolol or no active treatment. Thus, there were three treatment groups, non-selective β-blockade (n=10, propranolol 4.0–11.6 mg/kg/day), selective β-blockade (n=9, metoprolol 2.7–5.9 mg/kg/day), and randomised controls (n=10). All were given recommendations for lifestyle modifications, and reduced energetic exercise significantly (p=0.002). Before study entry, and after 1 year, all underwent bicycle exercise tests with a ramp protocol. There were no differences in exercise capacity between the groups at entry, or follow-up, when median exercise capacity in the groups were virtually identical (2.4, 2.3, and 2.3 watt/kg and 55, 55, and 55 watt/(height in metre)2 in control, selective, and non-selective groups, respectively. Maximum heart rate decreased in the selective (−29%, p=0.04) and non-selective (−24%, p=0.002) groups. No patient developed a pathological blood-pressure response to exercise because of β-blocker therapy. Boys were more frequently risk-factor positive than girls (75% versus 33%, p=0.048) and had higher physical activity scores than girls at study-entry (p=0.011). Conclusions: Neither selective nor non-selective β-blockade causes significant reductions in exercise capacity in patients with hypertrophic cardiomyopathy above that induced by lifestyle changes.
|
|
| 8. |
- Bratt, Ewa-Lena, 1970, et al.
(författare)
-
Parental reactions, distress, and sense of coherence after prenatal versus postnatal diagnosis of complex congenital heart disease
- 2019
-
Ingår i: Cardiology in the Young. - 1047-9511 .- 1467-1107. ; 29:11, s. 1328-1334
-
Tidskriftsartikel (refereegranskat)abstract
- Introduction: A diagnosis of congenital heart disease (CHD) in offspring triggers psychological distress in parents. Results of previous studies have been inconsistent regarding the psychological impact of a prenatal versus a postnatal diagnosis. The aim of this study was to evaluate the influence of the time of diagnosis on levels of parental distress. Methods: Pregnant women and their partners with a fetus diagnosed with complex CHD, parents of children with postnatally diagnosed CHD, and pregnant women and their partners with uncomplicated pregnancies were invited to participate. Data were collected during pregnancy and 2–6 months after delivery using the Hospital Anxiety and Depression Scale, sense of coherence, life satisfaction, and Dyadic Adjustment Scale. Results: During pregnancy, the prenatal group scored lower sense of coherence compared to controls (p=0.044). Postnatally the prenatal group scored lower on sense of coherence compared to the postnatal group and controls (p=0.001; p=0.001). Postnatally, the prenatal and postnatal groups had higher levels of anxiety compared to controls (p=0.025; p=0.0003). Life satisfaction was lower in the prenatal group compared to that in the postnatal group and in controls (p=0.000; p=0.0004). Conclusion: Parents with a prenatal diagnosis of CHD in offspring report a low sense of coherence already during pregnancy which decreased further at follow-up. The same group reported a lower satisfaction with life compared to parents of a child with postnatal diagnosis of CHD and parents of a healthy child. This motivates further efforts to improve counselling and support during pregnancy and for parents after a prenatal diagnosis.
|
|
| 9. |
- Bratt, Ewa-Lena, 1970, et al.
(författare)
-
Parents' experiences of having an asymptomatic child diagnosed with hypertrophic cardiomyopathy through family screening.
- 2011
-
Ingår i: Cardiology in the young. - 1467-1107 .- 1047-9511. ; 21:1, s. 8-14
-
Tidskriftsartikel (refereegranskat)abstract
- Hypertrophic cardiomyopathy is hereditary and the commonest medical cause of sudden death in childhood and adolescence, which is the reason for recommending screening in children with an affected parent. A diagnosis of hypertrophic cardiomyopathy implies lifestyle modifications, restrictions that may bring profound changes to the affected individual and impacts on the whole family.
|
|
| 10. |
- Bratt, Ewa-Lena, 1970, et al.
(författare)
-
The experience of being diagnosed with hypertrophic cardiomyopathy through family screening in childhood and adolescence.
- 2012
-
Ingår i: Cardiology in the young. - 1467-1107 .- 1047-9511. ; 22:5, s. 528-535
-
Tidskriftsartikel (refereegranskat)abstract
- AimTo describe the experiences of children and adolescents being screened positive for hypertrophic cardiomyopathy and how this impacts their daily life. BACKGROUND: Hypertrophic cardiomyopathy is a hereditary disease and the most common medical cause of sudden death in childhood and adolescence. This is the reason for recommending screening in children with an affected first-degree relative. A diagnosis of hypertrophic cardiomyopathy implies lifestyle modifications, restrictions that may bring profound changes to the daily life of the affected individual. DESIGN: This is a descriptive qualitative interview study. METHODS: We interviewed 13 asymptomatic children or adolescents diagnosed with hypertrophic cardiomyopathy through family screening 12-24 months after the diagnosis. Analysis was conducted with qualitative content analysis. RESULTS: Children described an involuntary change, which affected their daily life with limitations and restrictions in life, both in the individual and social context. Lifestyle recommendations had the most severe impact on daily life and affected their social context. They tried to navigate in a world with new references, and after reorientation they felt hope and had faith in the future. CONCLUSIONS: Children diagnosed with hypertrophic cardiomyopathy through family screening went through an involuntary change resulting in limitations and restrictions in life. This study indicates that there is a need for support and that healthcare professionals have to consider the specific needs in these families. Our findings thus give guidance in how best to improve support to the patients and their family. Diagnosis in asymptomatic children should be accompanied by ideally multi-professional follow-up, focusing not only on medical issues.
|
|
| 11. |
|
|
| 12. |
- Broberg, Olof, et al.
(författare)
-
Circulating leptin is associated with adverse vascular changes in young adult survivors of childhood cancer
- 2024
-
Ingår i: Cardiology in the Young. - 1047-9511 .- 1467-1107.
-
Tidskriftsartikel (refereegranskat)abstract
- INTRODUCTION: Proteomics may help discover novel biomarkers and underlying mechanisms for cardiovascular disease. This could be useful for childhood cancer survivors as they show an increased risk of cardiovascular disease. The aim of this study was to investigate circulating cardiovascular proteins in young adult survivors of childhood cancer and their relationship to previously reported subclinical cardiovascular disease.METHODS: Ninety-two cardiovascular proteins were measured in 57 childhood cancer survivors and in 52 controls. For proteins that were significantly different between childhood cancer survivors and controls, we performed correlations between protein levels and measures of peripheral arterial stiffness (carotid distensibility and stiffness index, and augmentation index) and endothelial dysfunction (reactive hyperemia index).RESULTS: Leptin was significantly higher in childhood cancer survivors compared to controls (normalized protein expression units: childhood cancer survivors 6.4 (1.5) versus 5.1 (1.7), p < 0.0000001) after taking multiple tests into account. Kidney injury molecule-1, MER proto-oncogene tyrosine kinase, selectin P ligand, decorin, alpha-1-microglobulin/bikunin precursor protein, and pentraxin 3 showed a trend towards group differences (p < 0.05). Among childhood cancer survivors, leptin was associated with anthracycline treatment after adjustment for age, sex, and body mass index (p < 0.0001). Higher leptin correlated with lower carotid distensibility after adjustment for age, sex, body mass index, and treatments with radiotherapy and anthracyclines (p = 0.005).CONCLUSION: This proteomics approach identified that leptin is higher in young asymptomatic adult survivors of childhood cancer than in healthy controls and is associated with adverse vascular changes. This could indicate a role for leptin in driving the cardiovascular disease burden in this population.
|
|
| 13. |
- Ekman-Joelsson, Britt-Marie, 1956, et al.
(författare)
-
Immunological differences between heart- and kidney-transplanted children: a cross-sectional study.
- 2023
-
Ingår i: Cardiology in the young. - 1047-9511 .- 1467-1107. ; 33:5, s. 787-792
-
Tidskriftsartikel (refereegranskat)abstract
- Post-transplantation lymphoproliferative is a potentially mortal complication after heart transplantation in children. As the immune system plays a crucial role in the development of lymphoma, we explored the influence of thymus function in relation to immunosuppressive treatment in organ-transplanted children and healthy control subjects. A prospective case-control study was performed at a single centre, in which 36 children who had undergone heart transplantation were compared to two control groups: 34 kidney-transplanted children and 33 healthy age- and sex-matched children. T- and B-lymphocyte subtypes and monocytes were analysed by flow cytometry, and T-cell receptor excision circles were assessed using quantitative polymerase chain reaction. Heart-transplanted children had a lymphocyte profile characterised by reduced or absent thymic function with low numbers of T-cell receptor excision circles and total and naïve T cells, together with immune activation against the allograft. Despite similar immunosuppressive treatment, the kidney-transplanted group showed an activated T-lymphocyte compartment.
|
|
| 14. |
- Eslami, Bahareh, et al.
(författare)
-
Gender differences in health conditions and socio-economic status of adults with congenital heart disease in a developing country
- 2013
-
Ingår i: Cardiology in the Young. - 1047-9511 .- 1467-1107. ; 23:02, s. 209-218
-
Tidskriftsartikel (refereegranskat)abstract
- Background Providing appropriate care for adults with congenital heart disease requires the evaluation of their current situation. There is limited research in Iran about these patients, particularly in relation to gender differences in the demographic/socio-economic and lifestyle factors, as well as disease parameters.Materials and methods The sample consisted of 347 congenital heart disease patients in the age group of 18–64 years, including 181 women, assessed by an analytical cross-sectional study. The patients were recruited from the two major heart hospitals in Tehran. Data were collected using questionnaires.Results The mean age of the patients was 33.24 years. Women were more often married and more often had offspring than men (p < 0.001). Educational level and annual income were similar between women and men. Unemployment was higher among women (p < 0.001), but financial strain was higher among men (p < 0.001). Smoking, alcohol, and water-pipe use was higher among men than among women (p < 0.001). Cardiac factors, for example number of cardiac defects, were similar among women and men, except that there were more hospitalisations owing to cardiac problems, for example arrhythmia, among men. Disease was diagnosed mostly at the hospital (57.4%). Most medical care was provided by cardiologists (65.1%). Only 50.1% of patients had knowledge about their type of cardiac defect.Conclusion Gender differences exist in the socio-economic and lifestyle characteristics of adults with congenital heart disease, in some cases related to the disease severity. Our findings also point to the need for interventions to increase patients’ knowledge about, and use of, healthier lifestyle behaviours, irrespective of gender. Furthermore, providing appropriate jobs, vocational training, and career counselling may help patients to be more productive.
|
|
| 15. |
- Frid, Christina, et al.
(författare)
-
Long-term survival in children with atrioventricular septal defect and common atrioventricular valvar orifice in Sweden.
- 2004
-
Ingår i: Cardiology in the young. - : Cambridge University Press (CUP). - 1047-9511 .- 1467-1107. ; 14:1, s. 24-31
-
Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: The survival for patients with atrioventricular septal defect has improved markedly over the last decades and, during the same period, the survival of children with Down's syndrome has also increased. The aim of our study was to investigate long-term survival in patients having atrioventricular septal defect with common valvar orifice, but without associated significant congenital heart defects, in the setting of Down's syndrome, comparing the findings to those in chromosomally normal children with the same malformation. METHODS AND RESULTS: In a population-based retrospective study, we scrutinised the medical records from 801 liveborn children with atrioventricular septal defect born in Sweden during the period 1973 through 1997. Data on gender, presence or absence of Down's syndrome, associated congenital heart defects, date of birth, operation and death were recorded and followed up until 2001. An isolated atrioventricular septal defect with common atrioventricular valvar orifice was present in 502 children, of whom 86% had Down's syndrome. We found a significant reduc tion over time in age at operation, and in postoperative mortality at 30 days, from 28 to 1%. Using a multiple logistic regression model, we found no significant differences in mortality between genders, nor between those with or without Down's syndrome. Early corrective surgery could not be identified as a significant independent factor for survival. The 5-year postoperative survival in patients with Down's syndrome increased from 65% over the period from 1973 through 1977, to about 90% in the period 1993 through 1997, and the same trend was observed in chromosomally normal patients. CONCLUSIONS: Survival in uncomplicated atrioventricular septal defect with common atrioventricular valvar orifice has greatly increased, and surgical correction is now equally successful in patients with Down's syndrome and chromosomally normal patients, and for both genders. Death in connection with surgery is no longer the major threat, and focus must now be on long-term follow-up.
|
|
| 16. |
- Hansson, Lena, 1967-, et al.
(författare)
-
Vitamin D, liver-related biomarkers, and distribution of fat and lean mass in young patients with Fontan circulation
- 2022
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 32:6, s. 861-868
-
Tidskriftsartikel (refereegranskat)abstract
- Introduction/aim: Young patients with Fontan circulation may have low serum 25-hydroxyvitamin D levels, an affected liver, and unhealthy body compositions. This study aimed to explore the association between vitamin D intake/levels, liver biomarkers, and body composition in young Fontan patients.Method: We collected prospective data in 2017 to 2018, obtained with food-frequency questionnaires, biochemical analyses of liver biomarkers, and dual-energy X-ray absorptiometry scans in 44 children with Fontan circulation. Body compositions were compared to matched controls (n = 38). Linear regression analyses were used to investigate associations of biomarkers, leg pain, and lean mass on serum levels of 25-hydroxyvitamin D. Biomarkers were converted to z scores and differences were evaluated within the Fontan patients.Results: Our Fontan patients had a daily mean vitamin D intake of 9.9 µg and a mean serum 25-hydroxyvitamin D of 56 nmol/L. These factors were not associated with fat or lean mass, leg pain, or biomarkers of liver status. The Fontan patients had significantly less lean mass, but higher fat mass than controls. Male adolescents with Fontan circulation had a greater mean abdominal fat mass than male controls and higher cholesterol levels than females with Fontan circulation.Conclusion: Vitamin D intake and serum levels were not associated with body composition or liver biomarkers in the Fontan group, but the Fontan group had lower lean mass and higher fat mass than controls. The more pronounced abdominal fat mass in male adolescents with Fontan circulation might increase metabolic risks later in life.
|
|
| 17. |
|
|
| 18. |
|
|
| 19. |
- Olofsson, Cecilia Kjellberg, et al.
(författare)
-
Life satisfaction, health-related quality of life and physical activity after treatment for valvular aortic stenosis
- 2023
-
Ingår i: Cardiology in the Young. - : Cambridge University Press (CUP). - 1047-9511 .- 1467-1107. ; 33:3, s. 403-409
-
Tidskriftsartikel (refereegranskat)abstract
- Objective: To investigate health-related quality of life and life satisfaction in children and adolescents treated for isolated congenital valvular aortic stenosis compared to healthy peers. Our second aim was to investigate the relationship between objectively measured physical activity, health-related quality of life and life satisfaction in the same group. Methods: Forty-eight patients, 8-18 years of age, were recruited, as well as 43 healthy peers matched for age, gender and residential area. Health-related quality of life was assessed by the KIDSCREEN-52 self-report and parent proxy report, and life satisfaction was evaluated with the Satisfaction With Life Scale. Physical activity was measured with an accelerometer for 7 days. Sports participation was self-reported. Results: No differences in the health-related quality of life domains were found between patients and controls in the self-reports. In the proxy reports, parents of the adolescents rated their child's autonomy lower than did the parents of the healthy controls. A negative relationship was found between moderate to vigorous physical activity, sports participation, life satisfaction and the psychological well-being domain in adolescent patients. In children there was a positive relationship between moderate physical activity and the physical and psychological well-being domains. Conclusion: Overall, children and adolescents treated for valvular aortic stenosis reported similar life satisfaction and health-related quality of life as their healthy peers. The negative relationships between intense physical activity and sports participation with health-related quality of life and life satisfaction in adolescent patients might be explained by both physical and psychological factors in these teenagers with complex, lifelong heart disease.
|
|
| 20. |
- Rinnström, Daniel, 1982-, et al.
(författare)
-
High prevalence of ascending aortic dilation in adults with repaired coarctation of the aorta
- 2021
-
Ingår i: Cardiology in the Young. - Cambridge, United Kingdom : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 31:6, s. 992-997
-
Tidskriftsartikel (refereegranskat)abstract
- Background: Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients. Methods and results: From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]). Conclusions: Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure. © 2021 The Author(s),. Published by Cambridge University Press.
|
|
| 21. |
- Rinnström, Daniel, 1982-, et al.
(författare)
-
Poor blood pressure control in adults with repaired coarctation of the aorta and hypertension : a register-based study of associated factors
- 2017
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 27:9, s. 1708-1715
-
Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- Arterial hypertension is common in adults with repaired coarctation of the aorta, and is associated with several severe complications.This study aimed to investigate the prevalence of poorly controlled (⩾140/90 mmHg) blood pressure among patients with diagnosed hypertension and to identify associated factors.In the national register for CHD, adults with repaired coarctation of the aorta and diagnosed hypertension – defined as a registry diagnosis and/or use of anti-hypertensive prescription medication – were identified. Logistic regression analysis was used to identify variables associated with poorly controlled blood pressure.Of the 243 included patients, 27.2% were female, the mean age was 45.4±15.3 years, and 52.3% had poorly controlled blood pressure at the last registration. In a multivariable model, age (years) (OR 1.03, CI 1.01–1.06, p=0.008) was independently associated with poorly controlled blood pressure and so was systolic arm–leg blood pressure gradient in the ranges [10, 20] mmHg (OR 4.92, CI 1.76–13.79, p=0.002) to >20 mmHg (OR 9.93, CI 2.99–33.02, p<0.001), in comparison with the reference interval [0, 10] mmHg. Patients with poorly controlled blood pressure had, on average, more types of anti-hypertensive medication classes prescribed (1.9 versus 1.5, p=0.003).Poorly controlled blood pressure is common among patients with repaired coarctation of the aorta and diagnosed hypertension, despite what seems to be more intensive treatment. A systolic arm–leg blood pressure gradient is associated with poorly controlled blood pressure, even at low levels usually not considered for intervention, and may be an indicator of hypertension that is difficult to treat.
|
|
| 22. |
- Rydberg, Annika, et al.
(författare)
-
Serial assessment of variability in heart rate in children with the Fontan circulation.
- 2005
-
Ingår i: Cardiology in the Young. - London : Greenwich Medical Media. - 1047-9511 .- 1467-1107. ; 15:5, s. 498-503
-
Tidskriftsartikel (refereegranskat)abstract
- Autonomic nervous control of the heart can be studied by analysing variability in heart rate. Although earlier studies have shown reduced variability in patients with the Fontan circulation, we are not aware of any previous study examining longitudinal changes in such children. We have examined 13 patients who had undergone total cavopulmonary connection, and 37 healthy controls matched for age and gender. The examinations included complete echocardiography, and 24-hour ambulatory electrocardiogram for analysis of the parameters for variability in heart rate. After the Fontan procedure, three follow-up examinations were performed at a mean of 4.4 years, 5.6 and 7.2 years. Reduced variability was found in those with the Fontan circulation. A significant difference was found between patients and their controls with respect to high-frequency power at the second, p equal to 0.05, and third, p equal to 0.03, examination. The ratio of low-to-high-frequency components progressively increased in those with the Fontan circulation, a phenomenon that led to a significant difference, p equal to 0.03, at the third examination. Our study shows that, in patients with the Fontan circulation, routine ambulatory electrocardiographic monitoring including analysis of variability in heart rate, detects over time a progressive sympatovagal imbalance.
|
|
| 23. |
- Rönning, Helén, et al.
(författare)
-
Educational needs in adults with congenitally malformed hearts
- 2008
-
Ingår i: Cardiology in the Young. - 1047-9511 .- 1467-1107. ; 18:5, s. 473-479
-
Tidskriftsartikel (refereegranskat)abstract
- Background and aim: The number of adults with congenitally malformed hearts is growing, and there is an increasing demand for their continuous follow-up. At present, different programmes have been established for adults with congenital cardiac disease, but there is a lack of knowledge regarding how education and psychosocial support should be given to achieve effects. Before developing educational programmes, it is necessary to be aware of the perspective of the patients. The aim of our study, therefore, was to describe how adults with congenitally malformed hearts experience their educational needs. Methods: The study had a qualitative design. We interviewed 16 adults, aged from 19 to 55 years, with congenitally malformed hearts. Results: Two-way communication emerged as crucial to individualising education. Without good communication, those with congenitally malformed hearts, receiving information from providers of healthcare, are unable to transfer the information received. Thus, individualised education gives access to knowledge and the tools required to manage important areas in life, such as the congenital cardiac malformation, physical activity, the situation of life, treatment, and resources available for healthcare. The information given should provide easy access to knowledge through proper educational materials and methods, and be given with respect for the individual. This is facilitated if the education is tailored to the requirements of the individual in a holistic approach, and is provided through good communication. Conclusion: Our investigation shows that a structured educational programme needs to start from the perspective of the individual patient, and that two-way Communication needs to be taken into consideration to enhance knowledge.
|
|
| 24. |
- Sandberg, Camilla, et al.
(författare)
-
Despite reductions in muscle mass and muscle strength in adults with CHD, the muscle strength per muscle mass relationship does not differ from controls
- 2021
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 31:5, s. 792-798
-
Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Patients with CHD exhibit reduced isometric muscle strength and muscle mass; however, little is known how these parameters relate. Therefore, the aim was to investigate the relation between isometric limb muscle strength and muscle mass for patients in comparison to age- and sex-matched control subjects.METHODS: Seventy-four patients (35.6 ± 14.3 years, women n = 22) and 74 matched controls were included. Isometric muscle strength in elbow flexion, knee extension, and hand grip was assessed using dynamometers. Lean mass, reflecting skeletal muscle mass, in the arms and legs was assessed with dual-energy x-ray absorptiometry.RESULTS: Compared to controls, patients had lower muscle strength in elbow flexion, knee extension, and hand grip, and lower muscle mass in the arms (6.6 ± 1.8 kg versus 5.8 ± 1.7 kg, p < 0.001) and legs (18.4 ± 3.5 kg versus 15.9 ± 3.2 kg, p < 0.001). There was no difference in achieved muscle force per unit muscle mass in patients compared to controls (elbow flexion 0.03 ± 0.004 versus 0.03 ± 0.005 N/g, p = 0.5; grip strength 0.008 ± 0.001 versus 0.008 ± 0.001 N/g, p = 0.7; knee extension 0.027 ± 0.06 versus 0.028 ± 0.06 N/g, p = 0.5). For both groups, muscle mass in the arms correlated strongly with muscle strength in elbow flexion (patients r = 0.86, controls, r = 0.89), hand grip (patients, r = 0.84, controls, r = 0.81), and muscle mass in the leg to knee extension (patients r = 0.64, controls r = 0.68).CONCLUSION: The relationship between isometric muscle strength and limb muscle mass in adults with CHD indicates that the skeletal muscles have the same efficiency as in healthy controls.
|
|
| 25. |
- Sandberg, Camilla, et al.
(författare)
-
Impaired knee extension muscle strength in adolescents but not in children with Fontan circulation
- 2020
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 30:8, s. 1138-1143
-
Tidskriftsartikel (refereegranskat)abstract
- Introduction: Impaired isometric muscle strength was previously reported in adults with Fontan circulation. However, it is unclear if this impairment is present in children and adolescents with Fontan circulation. We investigated isometric muscle strength of the lower limb in patients (6–18 years) with Fontan circulation in comparison with healthy controls.Method: In this cross-sectional study, 43 patients (6–18 years) with Fontan circulation and 43 age- and sex-matched controls were included. Isometric knee extension and plantar flexion muscle strength were assessed using dynamometry (Newton, N). Lean mass of the legs was assessed with dual-energy X-ray absorptiometry. Analyses were performed on group level (n = 43), and for subgroups that included children aged 6–12 years (n = 18) and adolescents aged 13–18 years (n = 25).Results: On group level, the patients with Fontan circulation had impaired isometric knee extension strength in comparison with the controls (p = 0.03). In subgroup analyses, impaired isometric knee extension strength was present in the adolescents (p = 0.009) but not in the children groups. For plantar flexion, there was no difference between patients and controls. There was no difference in lean mass between patients and controls (9.6 ± 4.3 kg vs. 10.8 ± 5.6 kg, p = 0.31). However, the lean mass was highly correlated to isometric knee extension strength (patients r = 0.89, controls r = 0.96, p < 0.001) and isometric plantar flexion strength (patients r = 0.7, controls r = 0.81, p < 0.001).Conclusion: The finding of impaired isometric knee extension muscle strength in adolescents (13–18 years) with Fontan circulation and no corresponding impairment in the children group (6–12 years) could imply that isometric muscle strength gets more impaired with age.
|
|
| 26. |
|
|
| 27. |
- Svensson, Birgitta, et al.
(författare)
-
Health-related quality of life in children with surgery for CHD : a study from the Swedish National Registry for Congenital Heart Disease
- 2017
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 27:2, s. 333-343
-
Tidskriftsartikel (refereegranskat)abstract
- As survival of children with CHD needing surgery has improved significantly, the need for follow-up in terms of health-related quality of life has become increasingly important. In this study, we sought to describe health-related quality of life in children with CHD in relation to cardiac surgery. Methods: A retrospective Swedish National Registry for Congenital Heart Disease survey measured using DISABKIDS chronic generic measure-short version included 337 children (age 9-17 years; 39% girls). The majority (n= 319, 95%) of children had a biventricular heart, whereas the remaining had a univentricular heart. Cardiac surgery was performed in 197 (58%) children. Health-related quality of life was expressed as total score (100 highest) and given as medians and 10-90th percentiles. Results: The overall total score was 95 (88-100). Children with a biventricular heart who had undergone three or more surgeries (n= 31; 9%) had the lowest total score of 81 (61-97; p< 0.001). Children with two or more surgeries and those with univentricular heart were classified in NYHA II more frequently than children with one or no cardiac surgery (p= 0.005 and < 0.001, respectively). Children with three or more surgeries and those with univentricular heart needed more help at school (p< 0.001). Compared with children with other chronic diseases, children with CHD had a high total score except for children with three or more surgeries who had comparable total scores with children with other chronic diseases. Conclusion: Children with three or more cardiac surgeries and those with a univentricular heart appear to have lower health-related quality of life, cognitive ability, and NYHA classification.
|
|
| 28. |
- Svensson, Birgitta, et al.
(författare)
-
"The only thing I wonder is when I will have surgery again" : everyday life for children with right ventricle outflow tract anomalies during assessment for heart surgery
- 2023
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 33:3, s. 396-401
-
Tidskriftsartikel (refereegranskat)abstract
- Many children with complex right ventricle outflow tract anomalies such as Tetralogy of Fallot, common arterial trunk, and pulmonary atresia with ventricular septal defect require repeated heart surgeries early in life, but also later throughout their lifetime, thereby emphasising the importance of careful life-long follow-up. The need for repeated heart surgeries during childhood is recognised as a heavy burden on the child. Optimising the time point for re-intervention is important, since delaying re-intervention for these children can lead to complications such as ventricular arrhythmias, heart failure, and death. To this purpose, thorough pre-operative assessment (henceforth named as assessment) including clinical examination, echocardiography, MRI, and exercise test need to be performed whenever the indication for reoperation is suspected. It is likely to believe that children who are going through this kind of assessment that may lead to heart surgery need extra support. According to previous research, children with complex heart disease fear for the possibility of surgery and the thought of future repeated heart surgery is associated with anxiety. This might have an impact on children's everyday life and in research nowadays involving children with CHD, the focus has changed from survival to how these children experience their everyday life. Earlier studies have shown that they experience physical activities limitation and feelings of isolation, but no study has yet studied how children with right ventricle outflow tract anomalies experience their everyday life. To be able to provide support, studies are needed to explore how these children experience the period from assessment to decision, as well as the months thereafter. Therefore, the aim of this study was to explore how children diagnosed with complex right ventricle outflow tract anomalies experience their heart disease and their everyday life during the assessment and after the decision on whether to perform a new cardiac surgery.
|
|
| 29. |
- Söderlund, Fredrik, et al.
(författare)
-
Preoperative heart failure is not associated with impaired coagulation in paediatric cardiac surgery
- 2021
-
Ingår i: Cardiology in the Young. - 1047-9511 .- 1467-1107. ; 31:6, s. 979-984
-
Tidskriftsartikel (refereegranskat)abstract
- Objective: The objectives of the present study were to determine whether there was any association between the grade of heart failure, as expressed by preoperative levels of brain natriuretic peptide and Ross score, and the preoperative coagulation status in patients with non-restrictive ventricular shunts and determine whether there were any postoperative disturbances of the coagulation system in these patients, as measured by thromboelastometry and standard laboratory analyses of coagulation. Design: Perioperative coagulation was analysed with laboratory-based coagulation tests and thromboelastometry before, 8 hours after, and 18 hours after cardiac surgery. In addition, brain natriuretic peptide was analysed before and 18 hours after surgery. Patients: 40 children less than 12 months old with non-restrictive congenital ventricular or atrio-ventricular shunts scheduled for elective repair of their heart defects. Results: All coagulation parameters measured were within normal ranges preoperatively. There was a significant correlation between brain natriuretic peptide and plasma fibrinogen concentration preoperatively. There was no statistically significant correlation between brain natriuretic peptide and INTEM-MCF, FIBTEM-MCF, plasma fibrinogen, activated partial thromboplastin time, prothrombin time, or platelet count at any other time point, either preoperatively or postoperatively. Postoperatively, fibrinogen plasma concentration and FIBTEM-MCF decreased significantly at 8 hours, followed by a large increase at 18 hours to higher levels than preoperatively. Conclusions: There was no evidence of children with non-restrictive shunts having coagulation abnormalities before cardiac surgery. Brain natriuretic peptide levels or Ross score did not correlate with coagulation parameters in any clinically significant way.
|
|
| 30. |
- Tanghöj, Gustaf, et al.
(författare)
-
Adverse events within 1 year after surgical and percutaneous closure of atrial septal defects in preterm children
- 2019
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 29:5, s. 626-636
-
Tidskriftsartikel (refereegranskat)abstract
- Introduction: Atrial septal defect is the third most common CHD. A hemodynamically significant atrial septal defect causes volume overload of the right side of the heart. Preterm children may suffer from both pulmonary and cardiac comorbidities, including altered myocardial function. The aim of this study was to compare the rate of adverse events following atrial septal defect closure in preterm- and term-born children.Method: We performed a retrospective cohort study including children born in Sweden, who had a surgical or percutaneous atrial septal defect closure at the children’s hospitals in Lund and Stockholm, between 2000 and 2014, assessing time to the first event within 1 month or 1 year. We analysed differences in the number of and the time to events between the preterm and term cohort using the Kaplan–Meier survival curve, a generalised model applying zero-inflated Poisson distribution and Gary-Anderson’s method.Results: Overall, 413 children were included in the study. Of these, 93 (22.5%) were born prematurely. The total number of adverse events was 178 (110 minor and 68 major). There was no difference between the cohorts in the number of events, whether within 1 month or within a year, between major (p = 0.69) and minor (p = 0.84) events or frequencies of multiple events (p = 0.92).Conclusion: Despite earlier procedural age, larger atrial septal defects, and higher comorbidity than term children, preterm children appear to have comparable risk for complications during the first year after surgical or percutaneous closure.
|
|
| 31. |
- Tanghöj, Gustaf, et al.
(författare)
-
Risk factors for adverse events within one year after atrial septal closure in children : a retrospective follow-up study
- 2020
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 30:3, s. 303-312
-
Tidskriftsartikel (refereegranskat)abstract
- Introduction: Secundum atrial septal defect is one of the most common congenital heart defects. Previous paediatric studies have mainly addressed echocardiographic and few clinical factors among children associated with adverse events. The aim of this study was to identify neonatal and other clinical risk factors associated with adverse events up to one year after closure of atrial septal defect.Methods: This retrospective case-control study includes children born in Sweden between 2000 and 2014 that were treated surgically or percutaneously for an atrial septal defect. Conditional logistic regression was used to evaluate the association between major and minor adverse events and potential risk factors, adjusting for confounding factors including prematurity, neonatal sepsis, neonatal general ventilatory support, symptomatic atrial septal defects, and pulmonary hypertension.Results: Overall, 396 children with 400 atrial septal defect closures were included. The median body weight at closure was 14.5 (3.5-110) kg, and the median age was 3.0 (0.1-17.8) years. Overall, 110 minor adverse events and 68 major events were recorded in 87 and 49 children, respectively. Only symptomatic atrial septal defects were associated with both minor (odds ratio (OR) = 2.18, confidence interval (CI) 95% 1.05-8.06) and major (OR = 2.80 CI 95% 1.23-6.37) adverse events.Conclusion: There was no association between the investigated neonatal comorbidities and major or minor events after atrial septal defect closure. Patients with symptomatic atrial septal defects had a two to four times increased risk of having a major event, suggesting careful management and follow-up of these children prior to and after closure.
|
|
| 32. |
- Ternrud, Linda, et al.
(författare)
-
Prevalence of fatigue in adults with congenital heart disease
- 2022
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 32, s. 1432-1439
-
Tidskriftsartikel (refereegranskat)abstract
- Aims: The aim of this cross-sectional study was to examine the prevalence of the multidimensional phenomenon of fatigue in adults with congenital heart disease.Background: Adults with congenital heart disease are a growing population, and patient-reported outcomes can provide valuable information about the patient's experience of living with CHD. Fatigue is a multidimensional phenomenon that can be described as an overwhelming feeling of exhaustion with a reduced capacity of mental and physical work. Fatigue can be observed clinically in adults with congenital heart disease, but the actual prevalence is unknown.Methods: Fatigue was assessed by the Multidimensional Fatigue Inventory which enables the respondent to report the presence of fatigue according to five dimensions: "general fatigue,""physical fatigue,""mental fatigue,""reduced motivation,"and "reduced activity."The questionnaire was sent to 463 patients in Lund and Umeå. Four groups with complex CHD and two groups with moderately complex CHD were included. The reliability (internal consistency) of the Multidimensional Fatigue Inventory was tested for all dimensions and groups of diagnosis.Results: The response rate was 56.6% (n= 262). In patients with complex CHD, 40.0-59.4% reported severe to very severe general fatigue, and patients with a single ventricle reported the highest prevalence (59.4%). Among patients with complex CHD, 29.2-40.0% reported severe to very severe mental fatigue. The Multidimensional Fatigue Inventory had a high reliability measured with Cronbach's alpha.Conclusions: The study findings show a relatively high prevalence of fatigue in adults with congenital heart disease, and general fatigue was the most prevalent. Further studies are needed regarding fatigue and its causes and consequences in adults with congenital heart disease.Relevance to clinical practice: The Multidimensional Fatigue Inventory proved to be an instrument with high reliability and low internal loss, which suggests that the instrument may be suitable to use as a patient-reported outcome in the care of adults with congenital heart disease, preferably at repeated occasions.
|
|
| 33. |
|
|
| 34. |
- Wikner, Anna, et al.
(författare)
-
Lower bone strength in young patients with Fontan circulation compared to controls
- 2024
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107.
-
Tidskriftsartikel (refereegranskat)abstract
- Objectives: Previous reports indicate bone deficits in patients with Fontan circulation. However, the consequences of these deficits on bone strength and when these changes occur are unclear.Aim: To compare the tibial bone strength-strain index between young patients (6-19 years) with Fontan circulation and age- and sex-matched controls, and to determine strength-strain-index in subgroups of children (6-12 years) and adolescents (13-19 years) versus controls.Method: The tibia was examined with peripheral quantitative CT. Based on the assessed data, bone strength-strain index was calculated in the lateral and anterior-posterior directions.Results: Twenty patients with Fontan and twenty controls (mean age 13.0 ± 4.4 years; 50% females) were examined. Patients had a lower strength-strain index in the lateral direction compared to controls (808.4 ± 416.8mm3 versus 1162.5 ± 552.1mm3, p = 0.043). Subgroup analyses showed no differences regarding strength-strain index in children (6-12 years) with Fontan circulation compared to controls. However, the adolescents (13-19 years) with Fontan circulation had lower strength-strain indexes in both the lateral and anterior-posterior directions compared to controls (1041.4 ± 299.8mm3 versus 1596.4 ± 239.6mm3, p < 0.001, and 771.7 ± 192.4mm3 versus 1084.9 ± 215.0mm3, p = 0.004). When adjusted for height, there were differences between patients (6-19 years) and controls in strength-strain indexes in both the lateral and anterior-posterior directions. In subgroup analyses, the results remained robust.Conclusion: Young patients (6-19 years) with Fontan circulation have a lower strength-strain index in the tibia compared to controls. Subgroup analyses show that this deficit is mainly driven by the differences in adolescents (13-19 years), which might suggest that bone strength decreases with age.
|
|
| 35. |
- Winbo, Annika, et al.
(författare)
-
Iron-deficiency anaemia, gastric hyperplasia, and elevated gastrin levels due to potassium channel dysfunction in the Jervell and Lange-Nielsen Syndrome
- 2013
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 23:3, s. 325-334
-
Tidskriftsartikel (refereegranskat)abstract
- Aim We investigated extra-cardiac clinical symptoms and signs in the rare Jervell and Lange-Nielsen Syndrome, characterised by impaired KCNQ1 function, a gene essential for gastric acid secretion. METHODS: All Swedish Jervell and Lange-Nielsen cases with double KCNQ1 mutations (14 cases) were investigated by medical record review, an interview, and were offered laboratory testing for iron-deficiency anaemia and gastrointestinal markers. RESULTS: A history of iron-deficiency anaemia in 12 of 14 patients and subjective gastrointestinal symptoms in 13 of 14 patients was revealed. Previous endoscopy in five cases had revealed no case of coeliac or inflammatory bowel disease but three cases of mucosal hyperplasia/dysplasia. Current signs of anaemia or iron substitution were present in 9 of 12 tested cases. Elevated levels of gastrin in seven of nine cases, pepsinogen in six of seven cases, and faecal calprotectin in nine of nine cases were present. A significant correlation between elevated gastrin levels and concurrent iron-deficiency and/or anaemia was revealed (p-value 0.039). CONCLUSIONS: A high frequency of extra-cardiac clinical symptoms and previous medical investigations was found. We propose that the Jervell and Lange-Nielsen Syndrome phenotypically includes gastrointestinal symptoms/signs and secondary iron-deficiency anaemia owing to hypochlorhydria on the basis of KCNQ1 mutations. The resultant elevated gastrin level is a potential risk factor for later gastrointestinal cancer. Clinical monitoring with regard to developing anaemia and hypergastrinaemia should be considered in the Jervell and Lange-Nielsen Syndrome.
|
|
| 36. |
- Öhman, Annika, et al.
(författare)
-
Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia : A Swedish national cohort study
- 2020
-
Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 30:3, s. 353-360
-
Tidskriftsartikel (refereegranskat)abstract
- Background: Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia. Methods: Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993-2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016. Results: The 1993-2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome. Conclusion: The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.
|
|
| 37. |
- Öhman, Annika, et al.
(författare)
-
Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study
- 2020
-
Ingår i: Cardiology in the Young. - : Cambridge University Press (CUP). - 1047-9511 .- 1467-1107. ; 30:3
-
Tidskriftsartikel (refereegranskat)abstract
- Background: Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia. Methods: Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993-2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016. Results: The 1993-2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome. Conclusion: The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.
|
|
| 38. |
- Östman-Smith, Ingegerd, 1947, et al.
(författare)
-
Echocardiographic and electrocardiographic identification of those children with hypertrophic cardiomyopathy who should be considered at high-risk of dying suddenly
- 2005
-
Ingår i: Cardiol Young. - 1047-9511. ; 15:6, s. 632-642
-
Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Hypertrophic cardiomyopathy is a common cause of sudden death in children. In this study, we aimed to identify clinical measures for stratification of this risk in childhood. PATIENTS AND METHODS: By means of a retrospective cohort study from six regional centres of paediatric cardiology, we identified 128 patients with hypertrophic cardiomyopathy presenting below 19 years of age, with a mean follow-up of 10.8 years. Of the patients, 31 had died, 16 suddenly, with a median age at sudden death of 13.3 years. RESULTS: Cox regression shows that electrocardiographic voltages, analysed as the sum of the R and S waves in all six limb leads (p equal to 0.001), and septal thickness expressed as proportion of the 95th centile for age (p equal to 0.036), were independent predictors of sudden death. When the sum of the R and S waves is over 10 millivolts, the odds ratio for sudden death was 8.4, with 95% confidence intervals from 2.2 to 33.7 (p equal to 0.0012), and finding a septal thickness over 190% of 95th centile for age gives an odds ratio of 6.2, with confidence intervals from 1.5 to 25.1 (p equal to 0.011). Noonan's syndrome, with a p value equal to 0.043, and the ratio of the left ventricular wall to its cavity in diastole, with a p value equal to 0.005, were independent predictors of death in cardiac failure, with a ratio of the mural thickness to the dimension of the cavity over 0.30 giving an odds ratio of 36.0, with confidence limits from 4.2 to 311, and a p value equal to 0.00009. At follow-up, patients deemed to be at a high risk of dying suddenly were identified by the combination of the sum of the R and S waves greater than 10 millivolts and septal thickness over 190%, with a sensitivity of 91%, specificity of 78%, positive predictive value of 50%, and a negative predictive value of 97%. CONCLUSIONS: Children at high risk of dying suddenly with hypertrophic cardiomyopathy, with a subsequent annual mortality of 6.6%, can be distinguished at the time of diagnosis from those patients having a low risk of sudden death, the latter with an annual mortality of 0.27%.
|
|
| 39. |
- Aburawi, Elhadi, et al.
(författare)
-
A transthoracic Doppler echocardiography study of C-reactive protein and coronary microcirculation in children after open heart surgery.
- 2007
-
Ingår i: Cardiology in the Young. - 1467-1107. ; 17:5, s. 472-477
-
Tidskriftsartikel (refereegranskat)abstract
- Background Systemic inflammation has been suggested to underlie in part the elevated risk of arrhythmias and myocardial dysfunction during the first weeks after cardiac surgery. Recent transthoracic Doppler studies from our centre indicated increased basal coronary arterial flow in children 5 days after cardiopulmonary bypass surgery. In these children, we investigated whether the inflammatory mediator, C-reactive protein, could influence this association. Methods The peak flow velocity, velocity time-integral in diastole and systole, and basal blood flow in the proximal part of the left anterior descending artery, were assessed by transthoracic Doppler echocardiography 1 day before, and 5 days after, cardiac surgery in 17 children with ventricular and atrioventricular septal defects whose mean age at surgery was 6 months. Levels of C-reactive protein in the plasma were measured at both time-points. Results Prior to surgery, all children had levels of C-reactive protein under the limit for detection, that is less than 0.8 milligrams per litre. The levels of the protein had increased significantly by the second day, when the median value was 25, and the range from 20 to 142 milligrams per litre. They remained elevated on the fifth day after surgery, when the median was 11, and the range from 3 to 20 milligrams per litre. On the fifth day, the percentage increase in velocity time integral corrected for left ventricular mass was significantly lower in those patients with C-reactive protein greater than or equal to 10 milligrams per litre than in the remaining patients. Also, both the velocity time integral and the velocity of diastolic peak flow correlated inversely with log C-reactive protein, r being equal to −0.54 and p less than 0.02 and r equal to −0.74 and p less than 0.01, respectively, particularly among those patients in whom clamping of the aorta lasted for more than 1 hour, r for this statistic being equal to −0.8. Conclusion The postsurgical increase in the velocity of coronary arterial flow in children is inversely associated with rising levels of C-reactive protein. The duration of the aortic cross-clamping during surgery strengthens the association between levels of C-reactive protein and the microcirculatory changes.
|
|
| 40. |
- Ahlström, L., et al.
(författare)
-
Surgical age and morbidity after arterial switch of transposition of the great arteries
- 2014
-
Ingår i: Cardiology in the Young. - 1467-1107. ; 24:S1, s. 151-152
-
Konferensbidrag (refereegranskat)abstract
- Background: Although transposition of the great arteries (TGA) accounts for less than 5% of congenital heart disease, the clinical course is often dramatic with need for early diagnosis and careful preoperative care as well as advanced surgical correction and postoperative support. Since 1993, Lund is one of the two tertiary referral centers for pediatric cardiac surgery in Sweden, with nearly 400 surgical procedures each year. Methods: A single-institution 12-year retrospective survey of 127 neonates and infants (median for birth weight, gestational week, and age at surgery: 3.5 kg, 39 weeks, and 4 days, respectively) with TGA corrected via arterial switch operation (ASO). Postoperative morbidity and mortality during the hospital stay were reviewed. Patients with double outlet right ventricle and chromosome abnormalities were excluded. “Major postoperative morbidity” (MPM) was defined as presence of 1 or more of the following: prolonged mechanical ventilation (MV), delayed sternum closure, reoperation, CPAP/NIV after extubation, and ECMO. Patients were grouped based on distance between Lund and referral clinic as follows: “local”- within 200 km radius (n=67), and “external” >200 km (n=60). Results: There was only 1 death, born preterm (gestational week 34) with a body weight 7 days, n=25) surgical age had impact on MPM (p>0.4). Among those without fetal diagnosis of TGA, neither age at surgery (p=0.8) nor MPO (p=0.5) differed between “local” and “external” groups. Conclusion: ASO can be performed safely in full term neonates and in infants with TGA regardless of surgical age. This finding, along with the similar postoperative outcome regardless the distance between Lund and the referral clinic lend further support to the concept of centralization of pediatric cardiac surgery.
|
|
| 41. |
- Björk Werner, Josefin, et al.
(författare)
-
Factors Influencing Need for Late ASD Closure after Neonatal Repair of Severe Pulmonary Valve Obstruction and Intact Ventricular Septum
- 2018
-
Ingår i: Cardiology in the Young. - 1467-1107. ; 28:S1, s. 151-151
-
Konferensbidrag (refereegranskat)abstract
- Background: In neonates with critical pulmonary stenosis (CPS) or pulmonary atresia with intact ventricular septum (PAIVS), a nonrestrictive atrial septal defect (ASD) has been speculated to improve the initial clinical course after pulmonary valvotomy (PV) but some concerns exist in terms of its potentially longterm adverse effect on the right ventricle`s (RV) growth and persistent desaturation due to right-to-left (R-L) shunt. Objective: to assess the relationship between the size of ASD and the need for post-valvotomy reinterventions. Method: Patients with PAIVS and CPS treated at our center during 2001- 2015 were reviewed. Exclusion criteria were associated cardiac malformations and hypoplastic RV deemed unsuitable for biventricular circulation. Clinical and echocardiographic data were retrieved from the hospital ́s databases. Results: In total, 48 patients (18 with PAIVS and 30 CPS) were included. The median follow-up was 5 and 8 years, respectively. One patient with PAIVS died on day 3 after surgical valvotomy and Blalock- Taussig shunt (BTs). The majority (89%) of patients with PAIVS had surgical valvotomy whereas transcatheter valvotomy was used in the majority (87%) of patients with CPS. Palliation with BTs or PDA stenting was used in 13 (72%) patients with PAIVS and in 4 (13%) patients with CPS. Reintervention within 1 month after initial repair was needed in 4 (22%) patients with PAIVS and in 4 (13%) patients with CPS. Later reinterventions were performed in in 11(61%) patients with PAIVS and in 10(33%) patients with CPS. Of these, 7 (39%) patients with PAIVS and 5 (17%) with CPS underwent ASD closure due to persistent resting desaturation. The latter did not correlate with ASD size after valvotomy (p>0.1). Initial palliation with BTs was the only variable associated with ASD device closure (p=0.04). No patient required univentricular conversion. Conclusion: Neonatal biventricular repair for severe pulmonary valve obstruction has low mortality but significant need for late reinterventions, mostly consisting of ASD closure due to clinically significant desaturation secondary to R-L shunt. The use of aorto-pulmonary shunt, probably illustrating a more severe form of RV hypoplasia, but not the size of the ASD, predicts the need for later ASD closure due to desaturation.
|
|
| 42. |
|
|
| 43. |
|
|
| 44. |
|
|
| 45. |
|
|
| 46. |
|
|
| 47. |
- Cortez, Daniel, et al.
(författare)
-
Lower spatial QRS-T angle rules out sustained ventricular arrhythmias in children with hypertrophic cardiomyopathy
- 2017
-
Ingår i: Cardiology in the Young. - 1047-9511. ; 27:2, s. 354-358
-
Tidskriftsartikel (refereegranskat)abstract
- Introduction: The spatial peaks QRS-T angle accurately distinguishes children with hypertrophic cardiomyopathy from their healthy counterparts. The spatial peaks QRS-T angle is also useful in risk stratification for ventricular arrhythmias. We hypothesised that the spatial peaks QRS-T angle would be useful for the prediction of ventricular arrhythmias in hypertrophic cardiomyopathy patients under 23 years of age. Methods: Corrected QT interval and spatial peaks QRS-T angles were retrospectively assessed in 133 paediatric hypertrophic cardiomyopathy patients (12.4±6.6 years) with versus without ventricular arrhythmias of 30 seconds or longer. Significance, positive/negative predictive values, and odds ratios were calculated based on receiver operating characteristic curve cut-off values. Results: In total, 10 patients with ventricular arrhythmias were identified. Although the corrected QT interval did not differentiate those with versus without ventricular arrhythmias, the spatial peaks QRS-T angle did (151.4±19.0 versus 116.8±42.6 degrees, respectively, p<0.001). At an optimal cut-off value (124.1 degrees), the positive and negative predictive values of the spatial peaks QRS-T angle were 15.4 and 100.0%, respectively, with an odds ratio of 25.9 (95% CI 1.5–452.2). Conclusion: In children with hypertrophic cardiomyopathy, the spatial peaks QRS-T angle is associated with ventricular arrhythmia burden with high negative predictive value and odds ratio.
|
|
| 48. |
- Ekman-Joelssin, Britt-Marie, et al.
(författare)
-
Quality of life in children with pulmonary atresia and intact ventricular septum.
- 2004
-
Ingår i: CARDIOLOGY IN THE YOUNG. - 1047-9511. ; 14:6, s. 615-621
-
Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: To measure quality of life in children with pulmonary atresia and intact ventricular septum, and make comparisons with a healthy group of children from the general Swedish population. An additional aim was to compare quality of life in two subgroups, children undergoing biventricular and univentricular repair, the latter by establishment of the Fontan circulation. METHODS: Quality of life was measured using a mailed questionnaire, taking into consideration the three spheres of personal, interpersonal, and external living conditions. In all, 52 children received the questionnaire, and the answers were compared with those of a random sample of 1856 healthy Swedish children. RESULTS: We received responses from 42 children and parents. The overall differences between groups were small, and no difference was found when comparing the children undergoing univentricular as opposed to biventricular surgical repair. In terms of personal quality of life, we found a higher level of psychosomatic complaints, and lower satisfaction with their own and parent-child activities, in those with the congenital anomaly than in their normal peers. In terms of the interpersonal sphere, more time was available for those with malformed hearts, but the experience of support from the relatives was significantly lower. CONCLUSIONS: Overall, quality of life was equal between children with pulmonary atresia and intact ventricular septum and the children in the reference group. The personal psychological conditions were less favourable for children with pulmonary atresia and intact ventricular septum. The perceived lack of support from relatives was also higher in these families. No difference was found when comparing the children undergoing univentricular and biventricular surgical repair.
|
|
| 49. |
|
|
| 50. |
- Ekman-Joelsson, Britt-Marie, 1956, et al.
(författare)
-
Post-transplant lymphoproliferative disease is associated with early sternotomy and left ventricular hypoplasia during infancy: a population-based retrospective review.
- 2017
-
Ingår i: Cardiology in the young. - 1467-1107. ; 27:9, s. 1823-1831
-
Tidskriftsartikel (refereegranskat)abstract
- Heart transplantation has been an option for children in Sweden since 1989. As our unit faced an increased rate of post-transplant lymphoproliferative disorder, the objective of the study was to identify possible risk factors.This is a retrospective study of all children aged 0-18 years who underwent heart transplantation in Gothenburg from 1989 to 2014.A total of 71 children underwent heart transplantation. The overall incidence of post-transplant lymphoproliferative disorder was 14% (10/71); however, 17% (6/36) of those undergoing transplantation after 2007 developed lymphoma, compared with only 10% (4/35) of transplantation cases before 2007 (p=0.85). The mean age at transplantation was 9 years (0-17). The mean post-transplant follow-up time was 5.5 years (0.5-21.9) in the group that developed post-transplant lymphoproliferative disorder, compared with 10.2 years (0.02-25.2) in those who did not. In our study group, risk factors for post-transplant lymphoproliferative disorder were surgically palliated CHD (p=0.0005), sternotomy during infancy (p⩽0.0001), hypoplastic left ventricle (p=0.0001), number of surgical events (p=0.0022), mismatch concerning Epstein-Barr virus infection - that is, a positive donor-negative recipient (p⩽0.0001) - and immunosuppressive treatment with tacrolimus compared with ciclosporine (p=0.028). Discussion This study has three major findings. First, post-transplant lymphoproliferative disorder only developed in subjects born with CHD. Second, the vast majority (9/10) of the subjects developing the disorder had undergone sternotomy as infants. Third, the number of surgical events correlated with a higher risk for developing post-transplant lymphoproliferative disorder.
|
|
