| 1. |
- Gilljam, Thomas, et al.
(författare)
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First two decades of paediatric heart transplantation in Sweden - outcome of listing and post-transplant results.
- 2011
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Ingår i: Acta Pædiatrica. - : Wiley. - 1651-2227 .- 0803-5253. ; 100:11, s. 1442-1447
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Tidskriftsartikel (refereegranskat)abstract
- Aims: To evaluate outcome in the first generation of children with end-stage heart disease to whom heart transplantation was available. Methods: Retrospective review of all 135 Swedish children <18 years old listed for heart transplantation 1989-2009, followed to December 31, 2009, including 74 (55%) with cardiomyopathy and 61 (45%) with congenital heart disease; 34 (25%) were infants (<1 year). Cumulative risk of requiring heart transplantation was 1:17 300 (11 patients who improved were omitted from outcome analysis). Results: Waiting-list mortality was 31% (44% in infants). Median waiting time in 82 transplanted patients was 57 days (0-585 days). Post-transplant follow-up time was median 5.9 years (0.03-20.1 years), and actuarial survival was 92% at 1 year, 82% at 5 years, 76% at 10 years and 58% at 15 years. Survival after listing was 64% at 1 year, 58% at 5 years, 52% at 10 years and 40% at 15 years. Post-transplant complications included rejections (34%), malignancies (12%), renal failure (8%), coronary artery vasculopathy (6%) and re-transplantation (5%). Among 64 survivors, 84% were free of complications affecting prognosis. Conclusion: High waiting-list mortality and post-transplant attrition precluded 60% of this pioneer population from reaching adulthood. Functional status in survivors is generally good.
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| 2. |
- Hjortshøj, Cristel S., et al.
(författare)
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Outcome after heart-lung or lung transplantation in patients with Eisenmenger syndrome
- 2020
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Ingår i: Heart. - : BMJ. - 1355-6037 .- 1468-201X. ; 106:2, s. 127-132
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Tidskriftsartikel (refereegranskat)abstract
- Objective: The optimal timing for transplantation is unclear in patients with Eisenmenger syndrome (ES). We investigated post-transplantation survival and transplantation-specific morbidity after heart-lung transplantation (HLTx) or lung transplantation (LTx) in a cohort of Nordic patients with ES to aid decision-making for scheduling transplantation. Methods: We performed a retrospective, descriptive, population-based study of patients with ES who underwent transplantation from 1985 to 2012. Results: Among 714 patients with ES in the Nordic region, 63 (9%) underwent transplantation. The median age at transplantation was 31.9 (IQR 21.1-42.3) years. Within 30 days after transplantation, seven patients (11%) died. The median survival was 12.0 (95% CI 7.6 to 16.4) years and the overall 1-year, 5-year, 10-year and 15-year survival rates were 84.1%, 69.7%, 55.8% and 40.6%, respectively. For patients alive 1 year post-transplantation, the median conditional survival was 14.8 years (95% CI 8.0 to 21.8), with 5-year, 10-year and 15-year survival rates of 83.3%, 67.2% and 50.0%, respectively. There was no difference in median survival after HLTx (n=57) and LTx (n=6) (14.9 vs 10.6 years, p=0.718). Median cardiac allograft vasculopathy, bronchiolitis obliterans syndrome and dialysis/kidney transplantation-free survival rates were 11.2 (95% CI 7.8 to 14.6), 6.9 (95% CI 2.6 to 11.1) and 11.2 (95% CI 8.8 to 13.7) years, respectively. The leading causes of death after the perioperative period were infection (36.7%), bronchiolitis obliterans syndrome (23.3%) and heart failure (13.3%). Conclusions: This study shows that satisfactory post-transplantation survival, comparable with contemporary HTx and LTx data, without severe comorbidities such as cardiac allograft vasculopathy, bronchiolitis obliterans syndrome and dialysis, is achievable in patients with ES, with a conditional survival of nearly 15 years.
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| 3. |
- Ahmadi, Zainab, et al.
(författare)
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Smoking and home oxygen therapy : a review and consensus statement from a multidisciplinary Swedish taskforce
- 2024
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Ingår i: European Respiratory Review. - : European Respiratory Society. - 0905-9180 .- 1600-0617. ; 33:171
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Forskningsöversikt (refereegranskat)abstract
- Background: Home oxygen therapy (HOT) improves survival in patients with hypoxaemic chronic respiratory disease. Most patients evaluated for HOT are former or active smokers. Oxygen accelerates combustion and smoking may increase the risk of burn injuries and fire hazards; therefore, it is considered a contraindication for HOT in many countries. However, there is variability in the practices and policies regarding this matter. This multidisciplinary Swedish taskforce aimed to review the potential benefits and risks of smoking in relation to HOT, including medical, practical, legal and ethical considerations.Methods: The taskforce of the Swedish Respiratory Society comprises 15 members across respiratory medicine, nursing, medical law and ethics. HOT effectiveness and adverse risks related to smoking, as well as practical, legal and ethical considerations, were reviewed, resulting in five general questions and four PICO (population–intervention–comparator–outcome) questions. The strength of each recommendation was rated according to the GRADE (grading of recommendation assessment, development and evaluation) methodology.Results: General questions about the practical, legal and ethical aspects of HOT were discussed and summarised in the document. The PICO questions resulted in recommendations about assessment, management and follow-up of smoking when considering HOT, if HOT should be offered to people that meet the eligibility criteria but who continue to smoke, if a specific length of time of smoking cessation should be considered before assessing eligibility for HOT, and identification of areas for further research.Conclusions: Multiple factors need to be considered in the benefit/risk evaluation of HOT in active smokers. A systematic approach is suggested to guide healthcare professionals in evaluating HOT in relation to smoking.
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| 4. |
- Aldén, B, et al.
(författare)
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Long-term psychological outcome of children after surgery for transposition of the great arteries.
- 1998
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Ingår i: Acta Paediatrica (Oslo, Norway : 1992). - 0803-5253. ; 87:4, s. 405-410
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Tidskriftsartikel (refereegranskat)abstract
- To assess the psychological consequences of a single congenital heart defect, we tested intellectual function, self-perception, "body image", child psychiatric symptoms and the family climate in 21 boys and 10 girls, at a mean age of 13.2 y, on average 11.5 y after surgery for transposition of the great arteries. Where applicable, test norms were used for comparison. WISC-R IQ tended to be slightly lower than that of the general population. Self-perception, as reflected on the "I think I am" test, was normal. "Body image" as measured by the "Draw-a-man" test was poor in the boys, but did not show a relationship with any other test tapping mental health. Six children (19%) had clinically significant child psychiatric symptoms, which is slightly more than expected, and were overrepresented in patients with poorer cardiac function. Five of these represented "internalizing" disorders. The patients' families scored higher than expected on the family climate "chaos" subscale, which has been demonstrated to be associated with the development of psychiatric symptoms. Overall, however, the children and their families were regarded as socially and psychologically well-functioning.
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| 5. |
- Baturova, Maria A., et al.
(författare)
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Atrial fibrillation as a clinical characteristic of arrhythmogenic right ventricular cardiomyopathy : Experience from the Nordic ARVC Registry
- 2020
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273 .- 1874-1754. ; 298, s. 39-43
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Tidskriftsartikel (refereegranskat)abstract
- Background: Recent studies in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients have drawn attention to atrial fibrillation (AF) as an arrhythmic manifestation of ARVC and as an indicator of atrial involvement in the disease progression. We aimed to assess the prevalence of AF in the Scandinavian cohort of ARVC patients and to evaluate its association with disease clinical manifestations. Methods: Study sample comprised of 293 definite ARVC patients by 2010 Task Force criteria (TFC2010) and 141 genotype-positive family members (total n = 434, 43% females, median age at ARVC diagnosis 41 years [interquartile range (IQR) 28–52 years]). ARVC diagnostic score was calculated as the sum of major (2 points) and minor (1 point) criteria in all categories of the TFC2010. Results: AF was diagnosed in 42 patients (10%): in 41 patients with definite ARVC diagnosis (14%) vs in one genotype-positive family member (1%), p < 0.001. The median age at AF onset was 51 (IQR 38–58) years. The prevalence of AF was related to the ARVC diagnostic score: it significantly increased starting with the diagnostic score 4 (2% in those with score 3 vs 13% in those with score 4, p = 0.023) and increased further with increased diagnostic score (Somer's d value is 0.074, p < 0.001). Conclusion: AF is seen in 14% of definite ARVC patients and is related to the severity of disease phenotype thus suggesting AF being an arrhythmic manifestation of this cardiomyopathy indicating atrial myocardial involvement in the disease progression.
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| 6. |
- Borgquist, Rasmus, et al.
(författare)
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The diagnostic performance of imaging methods in ARVC using the 2010 Task Force criteria.
- 2014
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Ingår i: European heart journal cardiovascular Imaging. - : Oxford University Press (OUP). - 2047-2412 .- 2047-2404. ; 15:11, s. 1219-1225
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Tidskriftsartikel (refereegranskat)abstract
- This study evaluates the agreement between echocardiographic and cardiac magnetic resonance (CMR) imaging data, and the impact a discrepancy between the two may have on the clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC).
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| 7. |
- Börjesson, Mats, 1965, et al.
(författare)
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Hjärtstopp bland unga och idrottare särskiljer sig
- 2015
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Ingår i: Läkartidningen. - 0023-7205. ; 112:14-15
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- Dödsfall till följd av hjärtstopp av kardiella orsaker, s k plötslig hjärtdöd, hos unga och inom idrotten skiljer sig på flera sätt från plötslig hjärtdöd i högre åldrar. Drabbade kan sakna symtom, men de har vanligen en bakomliggande, ofta okänd, hjärtsjukdom. Flera olika hjärtsjukdomar har visats kunna ligga bakom plötsligt hjärtstopp bland unga och inom idrotten. Vanligen är de ärftliga och svårdiagnostiserade. Idrottande unga löper större risk att drabbas av plötsligt hjärtstopp än icke-idrottande. Screening av tävlingsidrottare rekommenderas internationellt, i Sverige också av Socialstyrelsen och Riksidrottsförbundet, för riskgrupper som elitidrottare från 16 års ålder. Vid hjärtstopp är tidig insats avgörande för utfallet. Det är därför viktigt att adekvat beredskap och kompetens finns på plats vid idrottsevenemang.
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| 8. |
- Börjesson, Mats, et al.
(författare)
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Hjärtstopp bland unga och idrottare särskiljer sig
- 2015
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Ingår i: Läkartidningen. - : Läkartidningen Förlag. - 0023-7205 .- 1652-7518. ; 112:14-15
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Tidskriftsartikel (refereegranskat)abstract
- Vi diskuterar i denna artikel hjärtstopp av kardiella orsaker, s k plötslig hjärtdöd, hos unga och inom idrotten. Sådana dödsfall röner alltid stor uppmärksamhet, såväl inom den närmaste kretsen som i medier. Detta är naturligt, eftersom det drabbar en ung individ och/eller en idrottare, som i samhället är en symbol för »friskhet«. På flera sätt skiljer sig dessa dödsfall från plötslig hjärtdöd i högre åldrar. Drabbade är vanligen till synes helt friska personer, dödsfallen är sällsynt förekommande och de orsakas av ett flertal olika hjärtsjukdomar, oftast ärftliga och svårdiagnostiserade.
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| 9. |
- Christiansen, Morten K, et al.
(författare)
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Incidence, Predictors, and Success of Ventricular Tachycardia Catheter Ablation in Arrhythmogenic Right Ventricular Cardiomyopathy (from the Nordic ARVC Registry).
- 2020
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Ingår i: The American journal of cardiology. - : Elsevier BV. - 1879-1913 .- 0002-9149. ; 125:5, s. 803-811
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Tidskriftsartikel (refereegranskat)abstract
- Catheter ablation may reduce ventricular tachycardia (VT) burden in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. However, little is known about factors predicting need for ablation. Therefore, we sought to investigate predictors and use of VT ablation and to evaluate the postprocedural outcome in ARVC patients. We studied 435 patients from the Nordic ARVC registry including 220 probands with definite ARVC according to the 2010 task force criteria and 215 mutation-carrying relatives identified through cascade screening. Patients were followed until first-time VT ablation, death, heart transplantation, or January 1st 2018. Additionally, patients undergoing VT ablation were further followed from the time of ablation for recurrent ventricular arrhythmias. The cumulative use of VT ablation was 4% (95% confidence interval [CI] 3% to 6%) and 11% (95% CI 8% to 15%) after 1 and 10 years. All procedures were performed in probands in whom cumulative use was 8% (95% CI 5% to 12%) and 20% (95% CI 15% to 26%). In adjusted analyses among probands, only young age predicted ablation. In patients undergoing ablation, risk of recurrent arrhythmias was 59% (95% CI 44% to 71%) and 74% (95% CI 59% to 84%) 1 and 5 years after the procedure. Despite high recurrence rates, the burden of ventricular arrhythmias was reduced after ablation (p = 0.0042). Young age, use of several antiarrhythmic drugs and inducibility to VT after ablation were associated with an unfavorable outcome. In conclusion, twenty percent of ARVC probands developed a clinical indication for VT ablation within 10 years whereas mutation-carrying relatives were without such need. Although the burden of ventricular arrhythmias decreased after ablation, risk of recurrence was substantial.
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| 10. |
- Dahlberg, Pia, et al.
(författare)
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QT correction using Bazett's formula remains preferable in long QT syndrome type 1 and 2
- 2021
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Ingår i: Annals of Noninvasive Electrocardiology. - : John Wiley & Sons. - 1082-720X .- 1542-474X. ; 26:1
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Tidskriftsartikel (refereegranskat)abstract
- Background: The heart rate (HR) corrected QT interval (QTc) is crucial for diagnosis and risk stratification in the long QT syndrome (LQTS). Although its use has been questioned in some contexts, Bazett's formula has been applied in most diagnostic and prognostic studies in LQTS patients. However, studies on which formula eliminates the inverse relation between QT and HR are lacking in LQTS patients. We therefore determined which QT correction formula is most appropriate in LQTS patients including the effect of beta blocker therapy and an evaluation of the agreement of the formulae when applying specific QTc limits for diagnostic and prognostic purposes.Methods: Automated measurements from routine 12-lead ECGs from 200 genetically confirmed LQTS patients from two Swedish regions were included (167 LQT1, 33 LQT2). QT correction was performed using the Bazett, Framingham, Fridericia, and Hodges formulae. Linear regression was used to compare the formulae in all patients, and before and after the initiation of beta blocking therapy in a subgroup (n = 44). Concordance analysis was performed for QTc >= 480 ms (diagnosis) and >= 500 ms (prognosis).Results: The median age was 32 years (range 0.1-78), 123 (62%) were female and 52 (26%) were children <= 16 years. Bazett's formula was the only method resulting in a QTc without relation with HR. Initiation of beta blocking therapy did not alter the result. Concordance analyses showed clinically significant differences (Cohen's kappa 0.629-0.469) for diagnosis and prognosis in individual patients.Conclusion: Bazett's formula remains preferable for diagnosis and prognosis in LQT1 and 2 patients.
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