Abstract:
AIMS:Increased left ventricular wall thickness (LVWT) is a common finding in cardiology. It is not known how often hereditary transthyretin-related familial amyloid cardiomyopathy (mTTR-FAC) is responsible for LVWT. Several therapeutic modalities for mTTR-FAC are currently in clinical trials; thus, it is important to establish the prevalence of TTR mutations (mTTR) and the clinical characteristics of the patients with mTTR-FAC. METHODS AND RESULTS:In a prospective multicentre, cross-sectional study, the TTR gene was sequenced in 298 consecutive patients diagnosed with increased LVWT in primary cardiology clinics in France. Among the included patients, median (25-75th percentiles) age was 62 [50;74]; 74% were men; 23% were of African origin; and 36% were in NYHA Class III-IV. Median LVWT was 18 (16-21) mm. Seventeen (5.7%; 95% confidence interval [CI]: [3.4;9.0]) patients had mTTR of whom 15 (5.0%; 95% CI [2.9;8.2]) had mTTR-FAC. The most frequent mutations were V142I (n = 8), V50M (n = 2), and I127V (n = 2). All mTTR-FAC patients were older than 63 years with a median age of 74 [69;79]. Of the 15 patients with mTTR-FAC, 8 were of African descent while 7 were of European descent. In the African descendants, mTTR-FAC median age was 74 [72;79] vs. 55 [46;65] years in non-mTTR-FAC (P < 0.001). In an adjusted multivariate model, African origin, neuropathy, carpal tunnel syndrome, electrocardiogram (ECG) low voltage, and late gadolinium enhancement (LGE) at cardiac-magnetic resonance imaging were all independently associated with mTTR-FAC. CONCLUSION:Five per cent of patients diagnosed with hypertrophic cardiomyopathy have mTTR-FAC. Mutated transthyretin genetic screening is warranted in elderly subjects with increased LVWT, particularly, those of African descent with neuropathy, carpal tunnel syndrome, ECG low voltage, or LGE.
journal_name
Eur Heart Jjournal_title
European heart journalauthors
Damy T,Costes B,Hagège AA,Donal E,Eicher JC,Slama M,Guellich A,Rappeneau S,Gueffet JP,Logeart D,Planté-Bordeneuve V,Bouvaist H,Huttin O,Mulak G,Dubois-Randé JL,Goossens M,Canoui-Poitrine F,Buxbaum JNdoi
10.1093/eurheartj/ehv583subject
Has Abstractpub_date
2016-06-14 00:00:00pages
1826-34issue
23eissn
0195-668Xissn
1522-9645pii
ehv583journal_volume
37pub_type
临床试验,杂志文章,多中心研究abstract::The treatment of hypertensive aircrew should also include changes in dietary habits, and advice against smoking. Relaxation exercises and changes in life style may prove of benefit. Increasing age, hypertension and smoking multiply to give a risk of cardiovascular event which merits careful assessment. ...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/5.suppl_a.33
更新日期:1984-03-01 00:00:00
abstract::The long-term efficacy of transdermal nitrate therapy, in particular the ability of a single patch to provide 24 h prophylaxis against angina, has been questioned. Two mechanisms have been suggested for this loss of effect: the development of pharmacological tolerance, and premature patch exhaustion. This study was de...
journal_title:European heart journal
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1093/eurheartj/12.12.1266
更新日期:1991-12-01 00:00:00
abstract::On the occasion of a routine medical check-up 256 out of 1100 individuals with an accumulation of coronary risk factors were screened for silent myocardial ischaemia by exercise testing and Holter monitoring. Of these individuals 5.5% had a pathological exercise test, 7.4% had ischaemia-like events on the Holter ECG, ...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/9.suppl_n.65
更新日期:1988-12-01 00:00:00
abstract::Chronic increases in haemodynamic load modify the expression of cardiac genes, leading to a new phenotype better adapted to the new functional demand. Several mechanisms such as the global activation of genes, the modulation of single genes and/or differential expression of multigene families may account for these mod...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/11.suppl_g.1
更新日期:1990-11-01 00:00:00
abstract:AIMS:Nuclear receptors and their cofactors regulate key pathophysiological processes in atherosclerosis development. The transcriptional activity of these nuclear receptors is controlled by the nuclear receptor corepressors (NCOR), scaffolding proteins that form the basis of large corepressor complexes. Studies with pr...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehz667
更新日期:2020-03-01 00:00:00
abstract:OBJECTIVES:The Cholesterol Lowering Atherosclerosis PTCA Trial (CLAPT) is a prospective, randomized trial with blinded angiographic end-points to assess the effect of 2-year's treatment with lovastatin initiated 4 weeks prior to PTCA, compared to usual care on non-dilated coronary segments and on dilated coronary lesio...
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.1999.1483
更新日期:1999-10-01 00:00:00
abstract:AIMS:The vast majority of acute heart failure (AHF) trials to date have targeted dyspnoea. However, they enrolled patients relatively late and did not standardize their methods of dyspnoea measurement. URGENT Dyspnoea was designed to determine changes in dyspnoea in response to initial, standard therapy in patients pre...
journal_title:European heart journal
pub_type: 杂志文章,多中心研究
doi:10.1093/eurheartj/ehp458
更新日期:2010-04-01 00:00:00
abstract:AIMS:The aim of this study was to assess the risk of intra-aortic balloon counterpulsation and to identify clinical and procedural variables that would predict complications. METHODS AND RESULTS:We analysed 381 consecutive patients who were treated between 1977 and 1995 at our catheterization laboratory and/or medical...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1053/euhj.1997.0802
更新日期:1998-03-01 00:00:00
abstract::Clinical studies suggest that neutrophil activation during acute myocardial infarction (MI) aggravates tissue injury. Activated neutrophils are an important source of oxygen free radicals (OFR), the injurious effects of which are counteracted by endogenous antioxidants. We have previously shown in healthy subjects tha...
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.a061045
更新日期:1995-08-01 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:AIMS:Mitral regurgitation (MR) following an acute myocardial infarction (AMI) confers an adverse prognosis during long-term follow-up. There are no studies evaluating the influence of pre-AMI MR in the short- and long-term prognosis of such patients. Our aim was to assess the prognostic value of pre-AMI MR in the short...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehi065
更新日期:2005-02-01 00:00:00
abstract::Hemodynamic variables were measured in 34 patients with isolated systolic hypertension before and after acute administration of propranolol. The patients were separated into two groups, those younger than and those older than 45 years of age, respectively. After drug administration, systolic pressure decreased signifi...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/4.suppl_g.51
更新日期:1983-11-01 00:00:00
abstract::Out of 84,609 Europeans in the ERICA sample (EHJ 9, Suppl I) 18,931 had adequate 6-year follow-up data so that coronary heart disease (CHD) mortality could be assessed in relation to five risk factors (age, cholesterol, systolic blood pressure, body mass index and cigarette smoking) measured at baseline. In view of kn...
journal_title:European heart journal
pub_type: 杂志文章
doi:
更新日期:1991-03-01 00:00:00
abstract::The effects of xamoterol 0.2 mg kg-1 i.v. and placebo on left ventricular function at rest and on exercise were compared in patients with left ventricular dysfunction due to ischaemic heart disease. Improvements were seen in systolic and diastolic function at rest, and at up to 50% of maximal exercise, without evidenc...
journal_title:European heart journal
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1093/eurheartj/11.suppl_a.48
更新日期:1990-04-01 00:00:00
abstract:AIMS:In animal models of obesity and diabetes mellitus, myocardial TG accumulation is associated with decreased myocardial function. In the physiologically ageing heart, myocardial triglyceride (TG) accumulation may also occur due to reduced myocardial fatty acid oxidation. The role of myocardial TG in the ageing human...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehn207
更新日期:2008-06-01 00:00:00
abstract:AIMS:The prognostic role of time-to-treatment in primary angioplasty is still a matter of debate. The aim of our study was to evaluate the relationship between time-to-treatment and myocardial perfusion in patients with ST-segment-elevation myocardial infarction (STEMI) treated by primary angioplasty. METHODS AND RESU...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1016/j.ehj.2004.03.021
更新日期:2004-06-01 00:00:00
abstract:AIMS:To assess EuroSCORE performance in predicting in-hospital mortality in on-pump coronary artery bypass grafting (CABG) and off-pump coronary artery bypass grafting (OPCAB). METHODS AND RESULTS:Additive and logistic EuroSCORE were computed for consecutive patients undergoing CABG (n = 3440, 75%) or OPCAB (n = 1140,...
journal_title:European heart journal
pub_type: 杂志文章,meta分析,评审
doi:10.1093/eurheartj/ehn581
更新日期:2009-02-01 00:00:00
abstract:AIMS:Patients with atrial fibrillation (AF) present with symptoms of myocardial ischaemia despite exclusion of coronary artery disease. A small vessel disease has been suggested. We quantified myocardial perfusion, perfusion reserve, and coronary vascular resistance (CVR) in AF patients using positron emission tomograp...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehm246
更新日期:2007-09-01 00:00:00
abstract:Aims:Vascular ageing is characterized by arterial stiffening, dilation, and arterial wall thickening. We investigated the extent to which these changes are related and their heritability during 5 year follow-up in the Twins UK cohort. Methods and results:Carotid-femoral pulse wave velocity (PWVcf), carotid diameter, c...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehy165
更新日期:2018-06-21 00:00:00
abstract::The importance of fibrinogen has been identified in two prospective observational studies. Reactive elevations in fibrinogen levels that occur within hours of a major stroke invalidate most cross-sectional case-control studies evaluating fibrinogen as a risk factor. However, as no elevation is seen following fresh epi...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/16.suppl_a.42
更新日期:1995-03-01 00:00:00
abstract:AIMS:To evaluate outcomes for left main coronary artery (LMCA) stenting and compare results between protected (left coronary grafted) and unprotected LMCA stenting in the current bare-metal stent era. METHODS:We reviewed outcomes among 142 consecutive patients who underwent protected or unprotected LMCA stenting since...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1016/s0195-668x(03)00314-2
更新日期:2003-09-01 00:00:00
abstract:AIMS:It has been proposed that competitive sport increases the risk of ventricular tachyarrhythmias (VTA) and death in patients with arrhythmogenic right-ventricular cardiomyopathy (ARVC). However, it is unknown whether this only applies to competitive sport or if recreational sports activity also increases the risk of...
journal_title:European heart journal
pub_type: 临床试验,杂志文章
doi:10.1093/eurheartj/ehv110
更新日期:2015-07-14 00:00:00
abstract::In this study, the acute haemodynamic effects of angiotensin converting enzyme (ACE) inhibition with intravenous enalaprilat alone or in combination with preload restoration were determined in patients with severe heart failure complicating acute myocardial infarction. Ten patients with raised pulmonary arterial wedge...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a060537
更新日期:1994-04-01 00:00:00
abstract::Degenerative mitral valve disease has become the major cause of mitral regurgitation in North America; it is incumbent on surgeons to know repair techniques and to document their results. Fortunately, improvements in myocardial protection have been concurrent with the evolution of mitral valve repair in our country. T...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/12.suppl_b.30
更新日期:1991-07-01 00:00:00
abstract:AIMS:Through a 4-year follow-up of the abstracts submitted to the European Society of Cardiology Congress in 2006, we aimed at identifying factors predicting high-quality research, appraising the quality of the peer review and editorial processes, and thereby revealing potential ways to improve future research, peer re...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehs113
更新日期:2012-12-01 00:00:00
abstract::This study describes the clinical profile and prognosis of elderly patients not eligible for predischarge exercise testing. The database consisted of 133 patients 55-64 years of age, and 111 patients older than 64 years of age who survived an acute myocardial infarction. Follow-up was one year. In the younger age grou...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/5.suppl_e.97
更新日期:1984-11-01 00:00:00
abstract::Supraventricular tachycardia is due to altered automaticity, triggered activity or most commonly, reentry. Atrial flutter is most frequently due to reentry with counterclockwise activation in the right atrium with the left atrium acting as a bystander. The small wavefronts demonstrated during atrial fibrillation are l...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/14.suppl_e.2
更新日期:1993-09-01 00:00:00
abstract:BACKGROUND:Sixteen cases of sudden unexpected cardiac death, 15 males and one female, are known to have occurred among young Swedish orienteers from 1979 to 1992, of which seven cases occurred between 1989 and 1992. This is considered to be indicative of an increased death rate. RESULTS:Histopathological evaluation sh...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a014972
更新日期:1996-06-01 00:00:00
abstract::Recently published epidemiological and genetic studies strongly suggest a causal relationship of elevated concentrations of lipoprotein (a) [Lp(a)] with cardiovascular disease (CVD), independent of low-density lipoproteins (LDLs), reduced high density lipoproteins (HDL), and other traditional CVD risk factors. The ath...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/eht053
更新日期:2013-11-01 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