Abstract:
AIMS:Cardiomyopathies are a heterogeneous group of disorders associated with premature death due to ventricular arrhythmia or heart failure. The purpose of this study was to examine the characteristics of patients enrolled in the pilot phase of the EURObservational Research Programme (EORP) cardiomyopathy registry. METHODS AND RESULTS:Between 1 December 2012 and 30 November 2013, four cardiomyopathy phenotypes were studied: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). Twenty-seven centres in 12 countries participated; 1115 patients were enrolled. The commonest cardiomyopathy was HCM (n = 681), followed by DCM (n = 346), ARVC (n = 59), and RCM (n = 29); 423 patients (46.4% of those reported) had familial disease; and 56 (5.0%) had rare disease phenocopies. Median age at enrolment and diagnosis was 54 [interquartile range (IQR), 42-64] and 46 years (IQR, 32-58), respectively; fewer patients with ARVC and more with RCM were diagnosed in the upper age quartile (P < 0.0001). There was a male predominance for all cardiomyopathies except RCM (P = 0.0023). Most patients were in New York Heart Association functional class I (n = 813) at enrolment; 139 (12.5%) reported syncope, most frequently in ARVC (P = 0.0009). Five hundred and seven (45.5%) patients underwent cardiac magnetic resonance imaging, 117 (10.6%) endomyocardial biopsy, and 462 (41.4%) genetic testing with a causative mutation reported in 236 individuals (51.1%). 1026 patients (92.0%) were receiving drug therapy; 316 (28.3%) had received an implantable cardioverter defibrillator (highest proportion in ARVC, P < 0.0001). CONCLUSION:This pilot study shows that services for patients with cardiomyopathy are complex, requiring access to a large range of invasive and non-invasive investigations and involvement of multidisciplinary teams. Treatment regimens are equally multifaceted and show that patients are likely to need long-term follow-up in close liaison with expert centres.
journal_name
Eur Heart Jjournal_title
European heart journalauthors
Elliott P,Charron P,Blanes JR,Tavazzi L,Tendera M,Konté M,Laroche C,Maggioni AP,EORP Cardiomyopathy Registry Pilot Investigators.doi
10.1093/eurheartj/ehv497subject
Has Abstractpub_date
2016-01-07 00:00:00pages
164-73issue
2eissn
0195-668Xissn
1522-9645pii
ehv497journal_volume
37pub_type
杂志文章,多中心研究abstract::Left ventricular flow patterns were studied at rest by colour flow Doppler echocardiography in 33 patients with mitral tilting-disc prostheses (group 1), in 38 patients with mitral tissue prostheses (group 2) and in 18 healthy volunteers (controls). A 'crossed' flow pattern was seen in 14 patients with mechanical (42%...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a060494
更新日期:1994-03-01 00:00:00
abstract::This report concerns a patient with cardiac manifestation of Whipple's disease. For the first time, the gene that encodes the 16s rRNA of Tropheryma whippelii was identified in a native aortic valve by means of a polymerase chain reaction technique. DNA amplification gives evidence of Tropheryma whippelii as a causati...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a060928
更新日期:1995-03-01 00:00:00
abstract:AIMS:An abnormal left ventricular volume response during dobutamine echocardiography identified patients with severe coronary artery disease. The aim of the study was to assess the prognostic value of left ventricular volume changes during dobutamine stress echocardiography in 136 patients. METHODS AND RESULTS:Endpoin...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a015139
更新日期:1997-10-01 00:00:00
abstract::An exercise test was performed in 455 patients in the third week after acute myocardial infarction (AMI). One hundred and seventeen (26%) of them were considered as having a complicated AMI. During a follow-up of 4.5 years their mortality was 49% vs 23% in the remaining patients with uncomplicated AMI. The survival of...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a062329
更新日期:1987-06-01 00:00:00
abstract::The endothelins are a family of three widely expressed 21 amino acid peptides. Apart from the ability to constrict vascular smooth muscle, they have numerous other potent effects, from enhancing mitogenesis to influencing nervous function. The pathway of their production involves the initial synthesis of a predominant...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:
更新日期:1993-11-01 00:00:00
abstract::Pulmonary disease is common in patients with heart failure, through shared risk factors and pathophysiological mechanisms. Adverse pulmonary vascular remodelling and chronic systemic inflammation characterize both diseases. Concurrent chronic obstructive pulmonary disease presents diagnostic and therapeutic challenges...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/eht192
更新日期:2013-09-01 00:00:00
abstract:AIMS:Long-term randomized comparisons of percutaneous coronary intervention (PCI) to coronary artery bypass grafting (CABG) in left main coronary (LM) disease and/or three-vessel disease (3VD) patients have been limited. This analysis compares 3-year outcomes in LM and/or 3VD patients treated with CABG or PCI with TAXU...
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/ehr213
更新日期:2011-09-01 00:00:00
abstract::Left ventricular volume and ejection fraction were measured by 2-dimensional echocardiography from 2 orthogonal apical long axis views in 90 patients admitted with acute transmural myocardial infarction. Results were correlated with worst Killip class during hospital stay, enzymatic infarct size (peak CK-MB) and morta...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a061638
更新日期:1984-03-01 00:00:00
abstract::Nucleic acid-based therapeutics are currently developed at large scale for prevention and management of cardiovascular diseases (CVDs), since: (i) genetic studies have highlighted novel therapeutic targets suggested to be causal for CVD; (ii) there is a substantial recent progress in delivery, efficacy, and safety of ...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehaa229
更新日期:2020-10-21 00:00:00
abstract:AIMS:Collagen degradation in atherosclerotic plaques with thin fibrous caps renders them more prone to rupture. Fibroblast activation protein (FAP) plays a role in arthritis and tumour formation through its collagenase activity. However, the significance of FAP in thin-cap human fibroatheromata remains unknown. METHOD...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehq519
更新日期:2011-11-01 00:00:00
abstract:AIMS:Emerging evidence indicates that nuclear receptors play a critical regulatory role in cardiovascular physiology/pathology. Recently, farnesoid-X-receptor (FXR), a member of the metabolic nuclear receptor superfamily, has been demonstrated to be expressed in vascular cells, with important roles in vascular physiolo...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehs011
更新日期:2013-06-01 00:00:00
abstract::Left ventricular (LV) filling as assessed by Doppler transmitral flow velocity measurements was studied in 20 male patients with coronary artery disease (CAD) and in 18 normal individuals. Stroke volume, blood pressure and heart rate in the two groups were not significantly different. Compared to normals, the ratio be...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a062425
更新日期:1988-11-01 00:00:00
abstract:BACKGROUND:Myocardial ischaemia occurs principally in the subendocardial layer, whereas conventional myocardial perfusion imaging provides no information on the transmural myocardial blood flow (MBF) distribution. Subendocardial perfusion measurements and quantification of the transmural perfusion gradient (TPG) could ...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehu170
更新日期:2014-08-14 00:00:00
abstract:AIMS:To assess the safety and effects of combined treatment with vascular endothelial growth factor-A(165) plasmid (VEGF-A(165)) and granulocyte- colony stimulating factor (G-CSF) mobilization of bone marrow stem cells in patients with severe chronic ischaemic heart disease (IHD). METHODS AND RESULTS:Sixteen patients ...
journal_title:European heart journal
pub_type: 杂志文章,随机对照试验
doi:10.1093/eurheartj/ehl117
更新日期:2006-08-01 00:00:00
abstract:AIMS:Patients undergoing catheter ablation for atrial fibrillation (AF) often experience recurrent arrhythmias within the first few months post-ablation. We aimed to investigate whether short-term use of amiodarone to prevent early arrhythmias following radiofrequency ablation for AF could reduce later recurrence. MET...
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/ehu354
更新日期:2014-12-14 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::Non-thrombotic PE does not represent a distinct clinical syndrome. It may be due to a variety of embolic materials and result in a wide spectrum of clinical presentations, making the diagnosis difficult. With the exception of severe air and fat embolism, the haemodynamic consequences of non-thrombotic emboli are usual...
journal_title:European heart journal
pub_type: 杂志文章,实务指引
doi:10.1093/eurheartj/ehn310
更新日期:2008-09-01 00:00:00
abstract::Forty patients with prosthetic valve endocarditis (PVE) presenting during a 12-year period at a single hospital were studied. Twenty-six were male and 14 female and ages ranged from 19 to 67 years. During the first six post-operative months, most episodes were caused by staphylococci (38%) and Gram negative bacilli (2...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/5.suppl_c.133
更新日期:1984-10-01 00:00:00
abstract:AIMS:Pulmonary hypertension (PH) represents an important phenotype among the broader spectrum of patients with heart failure with preserved ejection fraction (HFpEF), but its mechanistic basis remains unclear. We hypothesized that activation of endothelin and adrenomedullin, two counterregulatory pathways important in ...
journal_title:European heart journal
pub_type: 临床试验,杂志文章
doi:10.1093/eurheartj/ehz626
更新日期:2019-12-01 00:00:00
abstract::This report describes the results of right ventricle endomyocardial biopsies from 26 subjects (mean age 27 +/- 10 years) with premature ventricular beats and normal cardiac anatomy and mechanical function. Light microscopy examination revealed normal myocardium in 10 subjects (38%), acute myocarditis in two (7%), bord...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a060248
更新日期:1992-06-01 00:00:00
abstract:BACKGROUND:Recent experimental and clinical data indicate that abnormal central and peripheral immune reactions contribute to the progression of chronic heart failure, and that immunomodulation may be an important therapeutic approach in this syndrome. Aims We sought to study the effects of growth hormone (GH) administ...
journal_title:European heart journal
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1016/s0195-668x(03)00480-9
更新日期:2003-12-01 00:00:00
abstract::Experience accumulated from several large trials strongly suggests that beta-blockers should be used for the management of chronic heart failure[87]. It is appropriate to add beta-blockade to conventional therapy such as diuretics, ACE inhibitors and digoxin, as this was the approach used in the major trials. It is ap...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/oxfordjournals.eurheartj.a014745
更新日期:1996-11-01 00:00:00
abstract:AIMS:The role of aspirin in the primary prevention setting is continuously evolving. Recent randomized trials have challenged the role of aspirin in the primary prevention setting. METHODS AND RESULTS:Electronic databases were searched for randomized trials that compared aspirin vs. placebo (or control) in subjects wi...
journal_title:European heart journal
pub_type: 杂志文章,meta分析
doi:10.1093/eurheartj/ehy813
更新日期:2019-02-14 00:00:00
abstract:AIM:To investigate fatalities from myocardial infarction at 28 days and one-year among patients aged 35-64 years in the Nordic and Lithuanian centres participating in the World Health Organization MONICA (Monitoring of Trends and Determinants of Cardiovascular Disease) Project. METHODS AND RESULTS:Altogether 9100 myoc...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a015124
更新日期:1997-01-01 00:00:00
abstract:AIMS:LV reverse remodelling has been shown to be a favourable response after cardiac resynchronization therapy (CRT) in many clinical trials. This study investigated whether left ventricular (LV) reverse remodelling after CRT has any structural benefit, which include the improvement of LV mass or regional wall thicknes...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/eurheartj/ehi885
更新日期:2006-06-01 00:00:00
abstract::The purpose of this study was to evaluate various methods of diagnosis of left atrial thrombi (LAT) in patients (pts) with mitral stenosis (MS). From 1980 to 1990, 581 pts with MS have undergone open mitral commissurotomy (n = 169) or valve replacement (n = 412). All pts had transthoracic 2D echocardiography (TTE), 10...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/12.suppl_b.70
更新日期:1991-07-01 00:00:00
abstract:AIMS:Angiographic studies on the natural course of both focal and diffuse coronary atherosclerosis have not been performed before, but can both be assessed by quantitative coronary angiography. The objective of this study was to describe the natural course of focal and diffuse coronary atherosclerosis over time. METHO...
journal_title:European heart journal
pub_type: 杂志文章,多中心研究
doi:10.1093/oxfordjournals.eurheartj.a015401
更新日期:1997-07-01 00:00:00
abstract:AIM:To describe the relative impact on survival of the delay from estimated time of collapse to call for an ambulance among patients who suffer from a bystander witnessed out of hospital cardiac arrest of a cardiac aetiology. METHODS:A majority of all ambulance organizations in Sweden (covering 85% of Sweden inhabitan...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1016/s0195-668x(03)00475-5
更新日期:2003-10-01 00:00:00
abstract::Assessment of percutaneous transluminal coronary angioplasty (PTCA) by early radionuclide exercise test was evaluated for 11 arteries undergoing a successful procedure. Exercise thallium-201 (Tl-201) and radionuclide ventriculography (RNV) were performed within 3 days before and after PTCA and compared to % diameter s...
journal_title:European heart journal
pub_type: 杂志文章
doi:10.1093/oxfordjournals.eurheartj.a062282
更新日期:1987-04-01 00:00:00
abstract::Increased arteriolar tone is the pathophysiological hallmark of essential hypertension and is determined by the intracellular free calcium concentration in the vascular smooth muscle cell. Calcium influx is an important determinant of vasoconstriction and excess calcium influx-dependent vasoconstriction has been shown...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/8.suppl_k.49
更新日期:1987-11-01 00:00:00