Abstract:
AIMS:Transthyretin amyloidosis (ATTR amyloidosis) is a heterogeneous disorder with cardiac, neurologic, and mixed phenotypes. We describe the phenotypic and genotypic profiles of this disease in continental Western Europe as it appears from the Transthyretin Amyloidosis Survey (THAOS). METHODS AND RESULTS:THAOS is an ongoing, worldwide, longitudinal, observational survey established to study differences in presentation, diagnosis, and natural history in ATTR amyloidosis subjects. At data cut-off, 1411 symptomatic subjects from nine continental Western European countries were enrolled in THAOS [1286 hereditary (ATTRm) amyloidosis; 125 wild-type ATTR (ATTRwt) amyloidosis]. Genotypes and phenotypes varied notably by country. Four mutations (Val122Ile, Leu111Met, Thr60Ala, and Ile68Leu), and ATTRwt, were associated with a mainly cardiac phenotype showing symmetric left ventricular (LV) hypertrophy, normal diastolic LV dimensions and volume, and mildly depressed LV ejection fraction (LVEF). Morphologic and functional abnormalities on echocardiogram were significantly more severe in subjects with cardiac (n = 210), compared with a mixed (n = 298), phenotype: higher median (Q1-Q3) interventricular septal thickness [18 (16-21) vs. 16 (13-20) mm; P = 0.0006]; and more frequent incidence of LVEF <50% (38.1 vs. 17.5%; P = 0.0008). Subjects with cardiac mutations or ATTRwt (or cardiac or mixed phenotype) had a lower survival rate than subjects in other genotype (or the neurologic phenotype) categories (P < 0.0001, for both). CONCLUSION:ATTR amyloidosis genotypes and phenotypes are highly heterogeneous in continental Western Europe. A geographic map of the different disease profiles and awareness that a subset of subjects have a dominant cardiac phenotype, mimicking hypertrophic cardiomyopathy, at presentation can facilitate the clinical recognition of this underdiagnosed disease. TRIAL REGISTRATION:ClinicalTrials.gov: NCT00628745.
journal_name
Eur Heart Jjournal_title
European heart journalauthors
Damy T,Kristen AV,Suhr OB,Maurer MS,Planté-Bordeneuve V,Yu CR,Ong ML,Coelho T,Rapezzi C,THAOS Investigators.doi
10.1093/eurheartj/ehz173subject
Has Abstractpub_date
2019-04-01 00:00:00eissn
0195-668Xissn
1522-9645pii
5425268pub_type
杂志文章abstract::The prognostic significance of angina pectoris and the effect of intervention with verapamil on the incidence of angina pectoris were studied in patients recovering from myocardial infarction and included in the Danish Verapamil Infarction Trial II. During the second week after admission patients were doubly-blindly r...
journal_title:European heart journal
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1093/oxfordjournals.eurheartj.a060486
更新日期:1994-02-01 00:00:00
abstract::Percutaneous excimer laser coronary angioplasty (ELCA) was performed in a first group of 20 patients with stable angina pectoris caused by significant coronary stenosis, and long-term follow-up was evaluated. Prototype 4 to 5.5 French multifibre catheters with 18-20 quartz fibres of 100 microns diameter, concentricall...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a060172
更新日期:1992-03-01 00:00:00
abstract::Large-scale trials have demonstrated a consistent mortality reduction from angiotensin converting enzyme (ACE) inhibitor therapy for chronic heart failure. Nonetheless, the pharmacologic and physiologic mechanisms of this favourable affect and the appropriate target population remain controversial. ACE inhibitors exer...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/16.suppl_o.133
更新日期:1995-12-01 00:00:00
abstract:AIMS:This study compared plasma levels of established risk markers for atherosclerosis and indices of inflammation in 50 patients with severe periodontitis to those in 46 healthy cases. METHODS AND RESULTS:Full blood counts were performed and levels of high density lipoproteins (HDL), total cholesterol, haptoglobin, e...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1016/j.ehj.2003.09.016
更新日期:2003-12-01 00:00:00
abstract:AIMS:Circulating progenitor cells (PC) may contribute to myocardial recovery following infarction. Growth factors including VEGF are produced during ischaemia and stimulate PC release and activation. In this study, we focused on the functional chemotactic response of PC to VEGF in subjects early after myocardial ischae...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehm542
更新日期:2008-01-01 00:00:00
abstract:AIMS:Characteristics of sudden cardiac arrest (SCA) during sports offers a novel (and unexplored) setting to assess factors associated with disparities in outcomes across regions. METHODS AND RESULTS:From a prospective 5-year community-based French registry concerning SCA during sports in 10-75 year-olds, we evaluated...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/eht282
更新日期:2013-12-01 00:00:00
abstract:AIM:To compare patients treated for heart failure in relation to the management in general practices versus hospital admission. METHODS AND RESULTS:Twelve randomly selected general practices (GP) were screened for patients receiving ACE-inhibitor, digoxin, or loop diuretic treatment. The first 500 volunteers of 959 po...
journal_title:European heart journal
pub_type: 杂志文章,多中心研究
doi:10.1016/j.ehj.2004.06.033
更新日期:2004-10-01 00:00:00
abstract::Exercise-induced U-wave inversion on chest wall mapping was compared with coronary arteriographic findings in 160 consecutive patients who presented with chest pain suggestive of ischaemic heart disease. ECG recordings were made from 16 points on the chest wall before, during and after exercise. None of the 27 patient...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a061883
更新日期:1985-05-01 00:00:00
abstract:AIMS:Short-QT syndrome 1 (SQT1) is an inherited channelopathy with accelerated repolarization due to gain-of-function in HERG/IKr. Patients develop atrial fibrillation, ventricular tachycardia (VT), and sudden cardiac death with pronounced inter-individual variability in phenotype. We generated and characterized transg...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehy761
更新日期:2019-03-07 00:00:00
abstract:AIMS:Phenotypic heterogeneity and incomplete penetrance are common in patients with hypertrophic cardiomyopathy (HCM). We aim to improve the understanding in genotype-phenotype correlations in HCM, particularly the contribution of an MYL2 founder mutation and risk factors to left ventricular hypertrophic remodelling. ...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehv522
更新日期:2016-06-14 00:00:00
abstract::Echocardiographic parameters of left ventricular (LV) function were compared with the electrocardiographic findings in 40 patients with chronic severe aortic regurgitation. Total 12-lead QRS amplitude was linearly correlated with LV mass and its 'relative reduction' (less than 250 mm) indicated LV hypertrophy. Patient...
journal_title:European heart journal
pub_type: 杂志文章
doi:
更新日期:1988-01-01 00:00:00
abstract:AIMS:It is reported that one third of patients with heart failure have normal left ventricular systolic function, and may or may not have left ventricular diastolic dysfunction. We sought to define the prevalence of left ventricular diastolic filling abnormalities in a large unselected group of patients, unlike the dia...
journal_title:European heart journal
pub_type: 临床试验,杂志文章
doi:10.1093/oxfordjournals.eurheartj.a015387
更新日期:1997-06-01 00:00:00
abstract:AIMS:The utilization and timing of revascularization in unstable coronary artery disease varies, which could have important consequences for patients and for treatment costs. The FRISC II invasive trial compared an early invasive strategy vs a non-invasive strategy with respect to the composite end-point of death and m...
journal_title:European heart journal
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1053/euhj.2001.2695
更新日期:2002-01-01 00:00:00
abstract::A series of 42 patients with chest pain but normal coronary angiograms and normal haemodynamics at rest were prospectively classified as typical angina (group 1, N = 9) or atypical angina (group 2, N = 16) or non-anginal chest pain (group 3, N = 17). All patients underwent radionuclide ventriculography and measurement...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a062119
更新日期:1986-08-01 00:00:00
abstract::A 35-year-old man developed giant cell myocarditis resulting in severe congestive cardiac failure. He needed urgent orthotopic cardiac transplantation despite maximal doses of inotropes and augmentation with an intra-aortic balloon pump. The patient presented with rhythm disturbances and echocardiographically diminish...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a059938
更新日期:1991-04-01 00:00:00
abstract:AIMS:To evaluate the efficacy of atrial pacing in the suppression of early reinitiation of atrial fibrillation after successful internal cardioversion. METHODS AND RESULTS:The efficacy of atrial pacing in suppressing early reinitiation of atrial fibrillation was studied in 12 of 45 (29%) patients with early reinitiati...
journal_title:European heart journal
pub_type: 临床试验,杂志文章
doi:10.1053/euhj.1999.1991
更新日期:2000-07-01 00:00:00
abstract:AIMS:microRNA (miRNA) is reported to be present in the blood of humans and has been increasingly suggested as a biomarker for diseases. We aim to determine the potential of cardiac-specific miRNAs in circulation to serve as biomarkers for acute myocardial infarction (AMI). METHODS AND RESULTS:By verifying their tissue...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehq013
更新日期:2010-03-01 00:00:00
abstract::The scientific assessment of diagnostic tests should not be based upon a small series of results published by the best academic institutions, but ought to require large scale, multicentre validation founded on grass roots institutions with real doctors, real patients and real problems. To this purpose, an internationa...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/16.suppl_j.2
更新日期:1995-10-01 00:00:00
abstract:BACKGROUND:Stent thrombosis (ST) is a rare but serious complication following percutaneous coronary intervention. Analysis of thrombus composition from patients undergoing catheter thrombectomy may provide important insights into the pathological processes leading to thrombus formation. We performed a large-scale multi...
journal_title:European heart journal
pub_type: 杂志文章,多中心研究
doi:10.1093/eurheartj/ehv419
更新日期:2016-05-14 00:00:00
abstract::This overview provides a Europe-wide update on the current burden of cardiovascular disease, and specifically of coronary heart disease and stroke. Cardiovascular disease continues to cause a large proportion of deaths and disability in Europe, and places a substantial burden on the health care systems and economies o...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/eht356
更新日期:2013-10-01 00:00:00
abstract:AIMS:Adults who engage in leisure-time physical activity (LTPA) have a reduced risk of developing heart failure. We hypothesized that high levels of LTPA are associated with diminished adverse age-related changes in cardiac structure and function. METHODS AND RESULTS:We studied 4342 Atherosclerosis Risk in Communities...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehw121
更新日期:2016-08-21 00:00:00
abstract:Aims:This study aimed to investigate the association between smoking cessation, post-cessation body mass index (BMI) change and risk of myocardial infarction (MI) and stroke in men. Methods and results:A prospective cohort study using the National Health Insurance Service (NHIS) data set collected from 2002 to 2013 wa...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehx761
更新日期:2018-05-01 00:00:00
abstract::To study the haemodynamic and neurohumoral effects of nisoldipine (2 X 10 mg) vs captopril (3 X 25 mg), 24 patients with heart failure (New York Heart Association class II and III) due to coronary artery disease were treated in a randomized double-blind trial over 3 months. Both drugs were well tolerated. Clinical sta...
journal_title:European heart journal
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1093/oxfordjournals.eurheartj.a059786
更新日期:1990-08-01 00:00:00
abstract::Patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions but to date only one official position paper related to left circulation thromboembolism has been published. This interdisciplinary paper, prepared with the involvement of eight European scientific societies, reviews the ava...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehaa1070
更新日期:2021-01-28 00:00:00
abstract::Promising approaches towards new therapeutic strategies in coronary heart disease might emerge from an improved pathophysiological understanding of the role of certain peptides, which require a localizing event for their accumulation or expression in the coronary vasculature. Temporal myocardial ischaemia or hypoperfu...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/12.suppl_f.112
更新日期:1991-12-01 00:00:00
abstract::Five, ten and fifteen min after sublingual administration of 10mg isosorbide dinitrate (ISDN) in chewing capsules, in 10 patients with coronary artery disease, ISDN plasma levels were correlated with the dilation of epicardial coronary arteries as well as with changes in aortic blood pressure and heart rate. Due to th...
journal_title:European heart journal
pub_type: 杂志文章
doi:
更新日期:1989-11-01 00:00:00
abstract::Electrophysiological properties were studied in a heterotopic heart transplant model developed in 44 dogs (Group I). Cycle length was 359.5 +/- 55.2 ms in the recipient heart and 500.9 +/- 77.9 ms in the donor heart (P less than 0.001). Sinoatrial conduction time was 38.6 +/- 13.6 ms in the recipient heart and 38.6 +/...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a059744
更新日期:1990-06-01 00:00:00
abstract:Aims:To appraise the clinical and genetic evidence that low-density lipoproteins (LDLs) cause atherosclerotic cardiovascular disease (ASCVD). Methods and results:We assessed whether the association between LDL and ASCVD fulfils the criteria for causality by evaluating the totality of evidence from genetic studies, pro...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/ehx144
更新日期:2017-08-21 00:00:00
abstract:BACKGROUND:Antiarrhythmic drugs are still used for the treatment of ventricular tachyarrhythmias, in combination with implantable cardioverter-defibrillators or without them. AIM OF THE STUDY:In a double-blind randomized crossover design, the short- and long-term efficacy and safety of oral dofetilide or oral sotalol ...
journal_title:European heart journal
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1053/euhj.2001.2679
更新日期:2001-12-01 00:00:00
abstract:AIMS:Although a recent expert consensus statement has recommended periprocedural uninterrupted (UI) non-vitamin K antagonist oral anticoagulants (NOACs) during catheter ablation of atrial fibrillation (AF) as a Class I indication, there have been no clear randomized trials. We investigated the safety and efficacy of UI...
journal_title:European heart journal
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1093/eurheartj/ehy870
更新日期:2019-05-14 00:00:00