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Low birth prevalence and referral bias constitute significant obstacles to elucidating the natural history of Ebstein anomaly (EA). An extensive 2-country register-based collaboration was performed to investigate the mortality in patients with EA. Patients born from 1970 to 2017 and diagnosed with EA were identified in Danish and Swedish nationwide medical registries. Each patient was matched by birth year and sex with 10 control subjects from the general population. Cumulative mortality and HR of mortality were computed using Kaplan-Meier failure function and Cox proportional regression model. The study included 530 patients with EA and 5,300 matched control subjects with a median follow-up of 11 years. In the EA cohort, 43% (228) underwent cardiac surgery. Cumulative mortality was lower for patients diagnosed in the modern era (the year 2000 and later) than for those diagnosed in the prior era (P < 0.001). Patients with isolated lesion displayed lower cumulative mortality than patients with complex lesions did (P < 0.001). Patients with a presumed mild EA anatomy displayed a 35-year cumulative mortality of 11% (vs 4% for the matched control subjects; P < 0.001), yielding an HR for mortality of 6.0 (95% CI: 2.7-13.6), whereas patients with presumed severe EA demonstrated an HR of 36.2 (95% CI: 15.5-84.4) compared with control subjects and a cumulative mortality of 18% 35 years following diagnosis. Mortality in patients with EA is high irrespective of presence of concomitant congenital cardiac malformations and time of diagnosis compared with the general population, but overall mortality has improved in the contemporary era. [Display omitted] [ABSTRACT FROM AUTHOR]

Coarctation of the aorta (CoA), a congenital narrowing of the proximal descending thoracic aorta, is a relatively common form of congenital heart disease. Untreated significant CoA has a major impact on morbidity and mortality. In the past 3 decades, transcatheter intervention (TCI) for CoA has evolved as an alternative to surgery. The authors report on all TCIs for CoA performed from 2000 to 2016 in 4 countries covering 25 million inhabitants, with a mean follow-up duration of 6.9 years. During the study period, 683 interventions were performed on 542 patients. The procedural success rate was 88%, with 9% considered partly successful. Complications at the intervention site occurred in 3.5% of interventions and at the access site in 3.5%. There was no in-hospital mortality. During follow-up, TCI for CoA reduced the presence of hypertension significantly from 73% to 34%, but despite this, many patients remained hypertensive and in need of continuous antihypertensive treatment. Moreover, 8% to 9% of patients needed aortic and/or aortic valve surgery during follow-up. TCI for CoA can be performed with a low risk for complications. Lifetime follow-up after TCI for CoA seems warranted. [Display omitted] [ABSTRACT FROM AUTHOR]

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2010.05.008 Byline: Mikael Aasa (a), Martin Henriksson (b), Mikael Dellborg (c), Lars Grip (d), Johan Herlitz (d), Lars-Ake Levin (b), Leif Svensson (e), Magnus Janzon (f) Abstract: In ST-elevation myocardial infarction, primary percutaneous coronary intervention (PCI) has a superior clinical outcome, but it may increase costs in comparison to thrombolysis. The aim of the study was to compare costs, clinical outcome, and quality-adjusted survival between primary PCI and thrombolysis. Author Affiliation: (a) Department of Clinical Science and Education/Department of Cardiology, Karolinska Institutet, Sodersjukhuset, Stockholm, Sweden (b) Centre for Medical Technology Assessment, Department of Medical and Health Sciences, Linkoping University, Linkoping, Sweden (c) Department of Emergency and Cardiovascular Medicine/Department of Internal Medicine, Gothenburg University/Sahlgrenska University Hospital, Goteborg, Sweden (d) Department of Molecular and Clinical Medicine/Department of Cardiology, Gothenburg University/Sahlgrenska University Hospital, Goteborg, Sweden (e) Stockholm Prehospital Centre, Sodersjukhuset, Stockholm, Sweden (f) Department of Cardiology, Heart Centre, University Hospital of Linkoping, Linkoping University, Linkoping, Sweden Article History: Received 14 December 2008; Accepted 8 May 2010 Article Note: (footnote) RCT no. NCT00806403.

Background: Atrial arrhythmias (ie, intra-atrial reentrant tachycardia and atrial fibrillation) are a leading cause of morbidity and hospitalization in adults with congenital heart disease (CHD). Little is known about their effect on quality of life and other patient-reported outcomes (PROs) in adults with CHD.Objective: The purpose of this study was to assess the impact of atrial arrhythmias on PROs in adults with CHD and explore geographic variations.Methods: Associations between atrial arrhythmias and PROs were assessed in a cross-sectional study of adults with CHD from 15 countries spanning 5 continents. A propensity-based matching weight analysis was performed to compare quality of life, perceived health status, psychological distress, sense of coherence, and illness perception in patients with and those without atrial arrhythmias.Results: A total of 4028 adults with CHD were enrolled, 707 (17.6%) of whom had atrial arrhythmias. After applying matching weights, patients with and those without atrial arrhythmias were comparable with regard to age (mean 40.1 vs 40.2 years), demographic variables (52.5% vs 52.2% women), and complexity of CHD (15.9% simple, 44.8% moderate, and 39.2% complex in both groups). Patients with atrial arrhythmias had significantly worse PRO scores with respect to quality of life, perceived health status, psychological distress (ie, depression), and illness perception. A summary score that combines all PRO measures was significantly lower in patients with atrial arrhythmias (-3.3%; P = .0006). Differences in PROs were consistent across geographic regions.Conclusion: Atrial arrhythmias in adults with CHD are associated with an adverse impact on a broad range of PROs consistently across various geographic regions. [ABSTRACT FROM AUTHOR]

Background: Implantable cardioverter-defibrillators (ICDs) are increasingly being used to prevent sudden death in the growing population of adults with congenital heart disease (CHD). However, little is known about their impact on patient-reported outcomes (PROs).Objective: The purpose of this study was to assess and compare PROs in adults with CHD with and without ICDs.Methods: A propensity-based matching weight analysis was conducted to evaluate PROs in an international cross-sectional study of adults with CHD from 15 countries across 5 continents.Results: A total of 3188 patients were included: 107 with ICDs and 3081 weight-matched controls without ICDs. ICD recipients were an average age of 40.1 ± 12.4 years, and >95% had moderate or complex CHD. Defibrillators were implanted for primary and secondary prevention in 38.3% and 61.7%, respectively. Perceived health status, psychological distress, sense of coherence, and health behaviors did not differ significantly among patients with and without ICDs. However, ICD recipients had a more threatening view of their illness (relative % difference 8.56; P = .011). Those with secondary compared to primary prevention indications had a significantly lower quality-of-life score (Linear Analogue Scale 72.0 ± 23.1 vs 79.2 ± 13.0; P = .047). Marked geographic variations were observed. Overall sense of well-being, assessed by a summary score that combines various PROs, was significantly lower in ICD recipients (vs controls) from Switzerland, Argentina, Taiwan, and the United States.Conclusion: In an international cohort of adults with CHD, ICDs were associated with a more threatening illness perception, with a lower quality of life in those with secondary compared to primary prevention indications. However, marked geographic variability in PROs was observed. [ABSTRACT FROM AUTHOR]

• Transcatheter closure of an atrial shunt after a stroke is not a panacea. • The absolute risk of recurrent stroke is mitigated after closure of an atrial shunt. • The relative risk though of recurrent stroke is much higher compared to controls. We aimed to investigate the risk of recurrent stroke in patients with transcatheter closure of an atrial shunt (ASCIos), compared to patients with an atrial shunt and cerebrovascular event (CVE) but only medical treated (ASMed), and to age- and sex-matched control individuals without a previous CVE. In total, 663 ASCIos patients were identified in the Swedish National Patient Register from 1997 to 2016 and matched by using propensity score with 663 ASMed patients. Nine age- and sex-matched controls to ASCIos patients (n = 6,302) without a diagnosis of atrial shunt or history of CVE were randomly selected from the general population. At a mean follow-up of 6.5 years, the incidence rate of recurrent stroke in the ASCIos group vs ASMed group was 0.9 vs 0.7 per 100 patient-years. The hazard ratio of recurrent stroke in the ASCIos group compared with index stroke in the control group was 9.9 (95% confidence interval, 5.5–17.9). The incidence of atrial fibrillation was similar in the ASCIos and the ASMed group, however four times higher in the ASCIos than in the control group. Our large nationwide, register-based cohort study showed that, unexpectedly, the risk of recurrent stroke in the ASCos group was as high as in the ASMed group and almost ten times higher than the risk of an index stroke in matched controls without previous stroke. [ABSTRACT FROM AUTHOR]

Background: Impaired quality of life (QOL) is associated with congenital heart disease (CHD) and country of residence; however, few studies have compared QOL in patients with differing complexities of CHD across regional populations. The current study examined regional variation in QOL outcomes in a large multinational sample of patients with a Fontan relative to patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs).Methods: From the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study (APPROACH-IS), 405 patients (163 Fontan and 242 ASD/VSD) across Asia, Europe, and North America provided consent for access to their medical records and completed a survey evaluating QOL (0 to 100 linear analog scale). Primary CHD diagnosis, disease complexity, surgical history, and documented history of mood and anxiety disorders were recorded. Differences in QOL, medical complications, and mood and anxiety disorders between Fontan and ASD/VSD patients, and across geographic regions, were examined using analysis of covariance. Hierarchical regression analyses were conducted to identify variables associated with the QOL ratings.Results: Patients with a Fontan reported significantly lower QOL, and greater medical complications and mood and anxiety disorders relative to patients with ASD/VSD. Inpatient cardiac admissions, mood disorders, and anxiety disorders were associated with lower QOL among patients with a Fontan, and mood disorders were associated with lower QOL among patients with ASD/VSD. Regional differences for QOL were not observed in patients with a Fontan; however, significant differences were identified in patients with ASD/VSD.Conclusions: Regional variation of QOL is commonplace in adults with CHD; however, it appears affected by greater disease burden. Among patients with a Fontan, regional variation of QOL is lost. Specific attempts to screen for QOL and mood and anxiety disorders among CHD patients may improve the care of patients with the greatest disease burden. [ABSTRACT FROM AUTHOR]

Aims: In adults with coarctation of the aorta (CoA), hypertension (HTN) is a common long-term complication. We investigated the prevalence of HTN and analyzed factors associated with HTN.Methods and Results: In the national register for congenital heart disease, 653 adults with repaired CoA were identified (mean age 36.9±14.4years); 344 (52.7%) of them had HTN, defined as either an existing diagnosis or blood pressure (BP) ≥140/90mmHg at the clinical visit. In a multivariable model, age (years) (odds ratio [OR] 1.07, CI 1.05-1.10), sex (male) (OR 3.35, CI 1.98-5.68), and body mass index (kilograms per square meter) (OR 1.09, CI 1.03-1.16) were independently associated with having HTN, and so was systolic arm-leg BP gradient where an association with HTN was found at the ranges of (10, 20] and >20mmHg, in comparison to the interval ≤10mmHg (OR 3.58, CI 1.70-7.55, and OR 11.38, CI 4.03-32.11). This model remained valid when all patients who had increased BP (≥140/90mmHg) without having been diagnosed with HTN were excluded from the analyses.Conclusions: Hypertension is common in patients with previously repaired CoA and is associated with increasing age, male sex, and elevated body mass index. There is also an association with arm-leg BP gradient, starting at relatively low levels that are usually not considered for intervention. [ABSTRACT FROM AUTHOR]

Primary percutaneous coronary intervention (PPCI) for ST-elevation myocardial infarction compares favorably to thrombolysis. In previous studies the benefit has been restricted to the early postinfarction period with no additional risk decrease beyond this period. Long-term outcome after use of third-generation thrombolytics and modern adjunctive pharmaceutics in the 2 treatment arms has not been investigated. This study was conducted to compare 5-year outcome after updated regimens of PPCI or thrombolysis. Patients with ST-elevation myocardial infarction were randomized to enoxaparin and abciximab followed by PPCI (n = 101) or enoxaparin followed by reteplase (n = 104), with prehospital initiation of therapy in 42% of patients. Data on survival and major cardiac events were obtained from Swedish national registries after 5.3 years. PPCI resulted in a better outcome with respect to the composite of death or recurrent myocardial infarction (hazard ratio 0.54, confidence interval 0.31 to 0.95) compared to thrombolysis. This was attributed to a significant decrease in cardiac deaths (hazard ratio 0.16, confidence interval 0.04 to 0.74). The difference evolved continuously over the 5-year follow-up. After adjustment for covariates, a significant benefit remained with respect to cardiac death or recurrent infarction but not for the composite of total survival or recurrent myocardial infarction (p = 0.07). The observed differences were not seen in patients in whom therapy was initiated in the prehospital phase. In conclusion, PPCI in combination with enoxaparin and abciximab compares favorably to thrombolysis in combination with enoxaparin with a risk decrease that stretches beyond the early postinfarction period. Prehospital thrombolysis may, however, match PPCI in long-term outcome. [ABSTRACT FROM AUTHOR]

Abstract: Background: Myeloperoxidase (MPO) has been listed as a potentially useful risk marker in acute coronary syndrome. However, its clinical utility in patients with acute chest pain is not yet defined. Design and methods: MPO (Architect, Abbott Diagnostics) was measured in 120 healthy controls and 303 chest pain patients who had been admitted to the coronary care units of three Swedish hospitals. Results: Chest pain patents had significantly higher median MPO levels compared to healthy controls (120.6 vs. 78. 9 pmol/L; p <0.001). However, MPO was not useful for the diagnosis of myocardial infarction (c-statistics 0.61 [95% CI 0.54–0.67]), and Cox regression analysis revealed no independent association between MPO and mortality (adjusted hazard ratio 1.3 [95% CI 0.8–2.0]) or the composite endpoint (adjusted hazard ratio 1.1 [95% CI 0.8–1.5]) after a median follow-up of 4.9 years. Conclusions: MPO provided no clinically relevant information in the present population of chest pain patients. [Copyright &y& Elsevier]

Abstract: Aim: To describe treatment and outcome in all patients in a community with acute myocardial infarction (AMI) in relation to gender. Methods: All patients discharged from hospital between 2001 and 2002 in Göteborg, Sweden, with a diagnosis of AMI underwent a survey to find possible gender differences. All p-values are age adjusted. Results: Among 1423 admissions, women comprised 41% and were older than men (mean 79 versus mean 72 years). Women were admitted to a coronary care unit less frequently than men (49% versus 67%; p =0.005). Women underwent coronary angiography less frequently (21% versus 40%; p =0.02). Percutaneous coronary intervention (PCI) was performed in 10% of the women and 18% of the men (p =0.36). Coronary artery bypass grafting (CABG) was performed in 2% of the women and in 9% of the men (p <0.0001). Female gender was associated with a lower risk of reinfarction during first year after hospital discharge (12% versus 16%; p =0.003). The cumulative three-year mortality was 49% in women and 41% in men. However, when adjusting for age, admittance to CCU, coronary angiography and coronary revascularisation, risk of death during 3 years was lower in women than men (odds ratio 0.72; 95% confidence interval 0.60–0.85; p =0.0001). Conclusion: In the community of Göteborg women (mean age 79 years) with AMI are prioritised differently than men (mean age 72 years), prior to admission to a CCU. This results in a less invasive strategy in women, particularly with regard to CABG. When adjusting for difference in age, admittance to CCU and coronary revascularisation female gender was associated with a low risk of death during the subsequent 3 years. [Copyright &y& Elsevier]

Abstract: Objectives: To describe the characteristics and outcome of patients with acute myocardial infarction (AMI) in a community, with particular emphasis on those who never reached a Coronary Care Unit (CCU) and those in whom the primary diagnosis was something other than a heart attack. Methods: Patients hospitalised in the city of Göteborg, Sweden, and discharged (dead or alive) with a diagnosis of AMI. Results: Among 1423 patient admissions the mean overall age was 75 years (81 years and 79 years in the two subsets). Among all patients, 33% had a history of heart failure and 20% had a history of cerebrovascular disease. The figures were even higher in the two subsets which were evaluated. In overall terms, an invasive strategy (coronary angiography) was used in 32% (in 5% and 9% in the two subsets respectively). The overall one-year and three-year mortality rate was 30% and 44% respectively. The three-year mortality rate among patients not admitted to a CCU was 65% and, among patients with no suspicion of a heart attack on admission, it was 68%. Conclusion: Even in the 21st century, patients with AMI who reach hospital alive run a high risk of death and nearly half are dead within the first three years. In overall terms, patients are characterised by high age and high co-morbidity. Among patients who do not reach a CCU and among patients with no suspicion of AMI on admission, approximately two thirds are dead within the subsequent three years. [Copyright &y& Elsevier]

Abstract: Background: Early risk stratification of patients with chest pain may be improved by combining cardiac Troponin I (cTnI) results and ECG findings with markers of left-ventricular dysfunction, inflammation or renal function. Methods: Serial measurements of cTnI were prospectively performed in 452 chest pain patients with a non-diagnostic ECG for AMI and admitted to the coronary care unit. NT-pro BNP, CRP, cystatin C and creatinine-clearance were retrospectively analyzed in admission samples. The prognostic value of these markers alone and in different combinations together with ECG findings was evaluated by multivariate logistic regression models. Results: During follow-up, 14 deaths and 21 myocardial (re)-infarctions occurred. Independent predictors for the combined endpoint of death or (re)-infarction were peak cTnI ≥0.1 μg/L within 24 h (OR 3.9; 95% confidence interval [CI]1.5–10.4), cystatin C ≥1.28 mg/L (OR 5.6; 95% CI 1.9–16.3) and NT-pro BNP ≥550 ng/L (OR 2.7; 95% CI 1.0–7.3). At 2 h from admission, a combination of cTnI ≥0.1 μg/L, an abnormal ECG and NT-pro BNP or cystatin C as a third variable resulted in a similar stratification of patients to different risk groups. Conclusion: cTnI, NT-pro BNP and cystatin C are strong risk predictors in patients with chest pain. For pragmatic reasons, a combination of cTnI ≥0.1 μg/L, ECG findings and a marker of renal function, preferably cystatin C, appears to be most appropriate for early risk stratification of these patients. [Copyright &y& Elsevier]

Abstract: Aims: The aim of this study was to investigate the influence of diabetes on treatment and outcome in acute myocardial infarction (AMI), during two time periods, in two countries, and to assess whether this influence has changed over the past decades. Methods: Patients, aged 30 to 74, with a diagnosis of AMI in two urban areas–Göteborg, Sweden and Minneapolis-St. Paul, Minnesota, USA–hospitalized during 1990–1991 and 1995–1996 were included. The primary endpoint was 7-year all-cause mortality. Results: The study included 3824 patients, 734 (19%) had diabetes. Age-adjusted in-hospital mortality of diabetic patients was nearly twofold higher compared with non-diabetic patients (9.8% vs. 5.0%, p <0.05). Between 1990–1991 and 1995–1996 in-hospital mortality declined for both diabetic (11.9% vs. 7.6%, p =0.07) and non-diabetic (6.3% vs. 3.6%, p =0.002) patients. A history of diabetes was associated with nearly twofold higher long-term mortality rate (48.5% vs. 26%, p <0.05). Seven-year mortality was reduced between 1990–1991 and 1995–1996 in both diabetic (51.6% vs. 45.2%, p =0.13) and non-diabetic patients (29.3% vs. 22.1%, p <0.0001) (The results did not reach statistical significance for diabetic patients, due to smaller sample size.) During their hospital stay, diabetic patients received significantly less aspirin, beta-blockers and thrombolysis. After adjustment, a history of diabetes remained significantly associated with 7-year mortality following AMI, doubling the hazard of death (hazard ratio (HR)=2.11; 95% confidence interval (CI): 1.80–2.46). Conclusion: A history of diabetes is associated with nearly twofold higher long-term mortality rate and is independently associated with 7-year mortality following AMI. Short- and long-term mortality decreased from 1990 to 1995 in both non-diabetic and diabetic patients. Underutilization of evidence-based treatments contributes to the remaining increased mortality in diabetic patients with acute coronary disease. [Copyright &y& Elsevier]

Abstract: Background: Advances in care have improved the longevity and quality of life for children with congenital heart disease; however, many of them need lifelong highly qualified specialist care. The cardiac lesion involved may not always be the patient''s main problem; issues related to quality of life may dominate. Aim: To explore and gain a deeper understanding of young adults´ experiences of living with congenital heart disease in order to enhance the quality of care provided by the health care system. Method: Transcribed in-depth interviews were analysed using a phenomenological–hermeneutic method. Six adults with congenital heart disease, aged 22–39 years old, were included in the study. Results: Analyses yielded two themes, having the disease and living with illness, both originating from the main theme of ambivalence. The interviewees were ambivalent in how they viewed themselves, how they faced their daily life and how they dealt with their encounters with the health care system. They had to strike a balance between being different and not being different; being sick and being healthy; revealing their congenital heart disease or hiding it and living with a hidden handicap. They also had to cope with the disease and with the health care system. Conclusion: Young adults with congenital heart disease are ambivalent. They have a strong wish to be healthy and they might hide their symptoms from the healthcare personnel and sometimes even from themselves. A psychosocial preparedness when meeting these patients is necessary. [Copyright &y& Elsevier]

Focuses on methods for identifying high-risk patients with unstable coronary artery disease (UCAD). Clinical classification of patients with UCAD; Clinical value of the 12-lead electrocardiogram and vectorcardiography; Investigation on short-term prognostic information obtained from continuous vectorcardiography and frequent measurement of troponin T levels.

Background: Recent published trials have shown the efficacy of transcatheter closure of patent foramen ovale (PFO) for preventing recurrent cryptogenic cerebrovascular events. However, the risk of recurrent cerebrovascular events (rCVEs) is up to 5.7%, and the etiology is unclear.Objective: This study aimed to investigate the risk factors for rCVEs after closure of PFO during long-term follow-up.Methods: In our center, 282 consecutive patients underwent PFO closure because of a cryptogenic cerebrovascular event between 2006 and 2014. Their Risk of Paradoxical Embolism (RoPE) score was calculated retrospectively. We followed up with the patients by telephone, using hospital records to identify those who suffered from rCVEs. Patients with rCVEs were matched with two control patients of the same sex and RoPE score without rCVEs who underwent PFO closure at approximately the same time. The patients with rCVEs and controls participated in a clinical examination, including contrast transthoracic echocardiography (TTE) and Holter electrocardiography, to investigate the possible cause of rCVEs compared with controls.Results: Fourteen (5%) out of the 282 consecutive patients who underwent PFO closure suffered from rCVEs during a mean follow-up of 8.4 years (1.7 rCVEs per 100 patient-years). The median RoPE score of the patients was 7. Recurrent CVE occurred in 3.2 patients per 100 patient-years in patients with residual shunting compared with 0.8 patients per 100 patient-years in those without residual shunt. These patients were on antiplatelet treatment or without any effective anticoagulant treatment at the time rCVE occurred. The risk ratio of rCVEs in patients with residual shunting was 2.9-times higher than in patients without residual shunting (95% CI: 1.4-6.1) at follow-up visit. Four patients who had the BioSTAR device implanted suffered from an rCVE despite lack of residual shunting.Conclusions: This study indicates that residual shunting and choice of the device may be the major reasons for rCVEs. [ABSTRACT FROM AUTHOR]