Abstract:
:Transthyretin amyloidosis (ATTR) is a rare, yet underdiagnosed disease characterized by progressive impairment of neurologic and cardiac function due to deposition of misfolded transthyretin. Despite great efforts, such as the introduction of orthotopic liver transplant, the devastating prognosis for both variant and wild-type ATTR patients remained unchanged over the last decades, mainly due to a lack of specific therapies. Fortunately, recent years saw the introduction of promising targeted therapies, which aim to interfere with the deposition of misfolded transthyretin (TTR) at various stages of the cascade underlying ATTR progression. These include TTR tetramer stabilizers (tafamidis, diflunisal, epigallocatechin-3-gallate), TTR silencers (inotersen, patisiran) and fibril disruptors (monoclonal antibodies, doxycycline and tauroursodeoxycholic acid). In the context of this review we explain their mechanisms of action, analyse their efficacy on neurologic and cardiac function based on all clinical trials conducted to date and discuss their clinical applicability. Eventually suggestions for future clinical research into the field are provided.
journal_name
Eur J Heart Failjournal_title
European journal of heart failureauthors
Müller ML,Butler J,Heidecker Bdoi
10.1002/ejhf.1695subject
Has Abstractpub_date
2020-01-01 00:00:00pages
39-53issue
1eissn
1388-9842issn
1879-0844journal_volume
22pub_type
杂志文章,评审abstract:BACKGROUND AND AIMS:Sleep disordered breathing (SDB), especially Cheyne-Stokes respiration (CSR) is common in patients with chronic heart failure (CHF). Adaptive servoventilation (ASV) was recently introduced to treat CSR in CHF. The aim of this study was to investigate the effects of ASV on CSR and CHF parameters. ME...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/j.ejheart.2008.04.007
更新日期:2008-06-01 00:00:00
abstract:AIMS:Right ventricular (RV) dysfunction and pulmonary hypertension (PH) are increasingly recognized in heart failure with preserved ejection fraction (HFpEF). The prevalence and prognostic value of RV dysfunction in HFpEF have been widely but variably reported. We therefore conducted a systematic review and meta-analys...
journal_title:European journal of heart failure
pub_type: 杂志文章,meta分析,评审
doi:10.1002/ejhf.630
更新日期:2016-12-01 00:00:00
abstract:AIMS:Chronic heart failure (CHF) due to left ventricular systolic dysfunction is associated with poor quality of life (QoL). This study aimed to assess factors affecting health-related QoL in CHF patients and their carers and the impact of QoL on clinical outcomes. METHODS AND RESULTS:Demographic, social, and clinical...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1093/eurjhf/hfq114
更新日期:2010-09-01 00:00:00
abstract:AIMS:To characterise the prevalence, in-hospital complications, management, and long-term outcome of patients with congestive heart failure but preserved left ventricular systolic function after acute myocardial infarction. METHODS:3166 consecutive patients screened for entry in the Bucindolol Evaluation in Acute Myoc...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/s1388-9842(03)00159-4
更新日期:2003-12-01 00:00:00
abstract:AIMS:Supervised exercise can benefit selected patients with heart failure, however the effectiveness of home-based exercise remains uncertain. We aimed to assess the effectiveness of a home-based exercise programme in addition to specialist heart failure nurse care. METHODS AND RESULTS:This was a randomized controlled...
journal_title:European journal of heart failure
pub_type: 杂志文章,随机对照试验
doi:10.1093/eurjhf/hfn029
更新日期:2009-02-01 00:00:00
abstract:BACKGROUND:Cardiac angiotensin converting enzyme (ACE) is activated by an increase in wall stress and is involved in remodeling processes. Heart failure is often treated with ACE inhibitors and diuretics although diuretic treatment could activate the renin-angiotensin system (RAS). AIMS:To examine the effects of diure...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/s1388-9842(02)00242-8
更新日期:2003-06-01 00:00:00
abstract:BACKGROUND:Although discontinuation of chronic ACE inhibitor (ACEi) therapy after myocardial infarction (MI) is common in clinical practice, some clinical studies reported an increased incidence of ischemia-related events after withdrawal. To further address this issue, we assessed hemodynamic, neurohormonal and vascul...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/j.ejheart.2005.04.014
更新日期:2006-03-01 00:00:00
abstract:BACKGROUND:The rate constant for global fatty acid influx (k(1)) was studied in 12 male patients with dilated cardiomyopathy (DCM). METHOD:10 normal subjects served as controls. 201-Thallium (201TI) and [123I]-phenyl-pentadecanoic acid (IPPA) were administered during bicycle exercise under fasting conditions. RESULTS...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/s1388-9842(02)00024-7
更新日期:2002-08-01 00:00:00
abstract:INTRODUCTION:Atrial fibrillation (AF) is common in patients with heart failure (HF) due to left ventricular systolic dysfunction (LVSD), with conflicting prognostic data. The aim of our study was to assess the prevalence and incidence of AF in patients with HF and to determine the prognostic impact of baseline AF and t...
journal_title:European journal of heart failure
pub_type: 杂志文章,多中心研究
doi:10.1016/j.ejheart.2006.08.004
更新日期:2007-03-01 00:00:00
abstract::Heart failure (HF) and chronic obstructive pulmonary disease (COPD) are global epidemics incurring significant morbidity and mortality. The combination presents many diagnostic challenges. Clinical symptoms and signs frequently overlap. Evaluation of cardiac and pulmonary function is often problematic and occasionally...
journal_title:European journal of heart failure
pub_type: 杂志文章,评审
doi:10.1093/eurjhf/hfn013
更新日期:2009-02-01 00:00:00
abstract:AIMS:Heart failure (HF) with normal or preserved left ventricular (LV) ejection fraction (HFPEF) has been reported to be associated with similar outcome as HF with reduced EF (HFREF) in registry-based and epidemiological analyses, but many of these studies excluded patients who did not have EF measurements. Conversely,...
journal_title:European journal of heart failure
pub_type: 杂志文章,meta分析
doi:10.1093/eurjhf/hfp103
更新日期:2009-09-01 00:00:00
abstract::We have documented a pre-junctional beta-2 adrenoceptor mediated reduction in cardiac norepinephrine spillover (CNES) in heart failure patients receiving chronic beta-blockade. Our present objective was to ascertain the consequence of this decrease for vagal heart rate (HR) regulation by determining CNES, arterial bar...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/j.ejheart.2004.10.012
更新日期:2005-08-01 00:00:00
abstract:AIMS:Cardiogenic shock is the leading cause of in-hospital mortality after acute myocardial infarction (MI). This study investigates the importance of age and preexisting diabetes mellitus on the incidence and prognosis of cardiogenic shock in a large group of consecutive patients with MI. METHODS AND RESULTS:Baseline...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/j.ejheart.2004.09.007
更新日期:2005-08-01 00:00:00
abstract:AIMS:The aim of this review is to assess the effects of exercise training on the symptoms of depression in heart failure (HF) patients. METHODS AND RESULTS:Randomized controlled trials of exercise training in patients with HF and symptoms of depression were identified. The depression data were pooled using meta-analys...
journal_title:European journal of heart failure
pub_type: 杂志文章,meta分析,评审
doi:10.1002/ejhf.101
更新日期:2014-07-01 00:00:00
abstract::Despite the availability of a number of different classes of therapeutic agents with proven efficacy in heart failure, the clinical course of heart failure patients is characterized by a reduction in life expectancy, a progressive decline in health-related quality of life and functional status, as well as a high risk ...
journal_title:European journal of heart failure
pub_type: 杂志文章,评审
doi:10.1002/ejhf.809
更新日期:2017-06-01 00:00:00
abstract::We investigated the hypothesis that increased intracellular [Na+]i in heart failure contributes to preservation of SR Ca2+ load which may become particularly evident at slow heart rates. [Na+]i in SBFI-loaded myocytes from rabbits with pacing-induced heart failure (PHF) was significantly higher at each frequency as co...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/j.ejheart.2006.01.013
更新日期:2006-11-01 00:00:00
abstract:AIMS:Left ventricular contractile reserve is a prognostic indicator for adverse outcome in patients with severe chronic heart failure with reduced ejection fraction (HFrEF). We investigated the dobutamine-induced changes of LV multidimensional deformation and their predictive value for cardiac mortality of patients wit...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1002/ejhf.787
更新日期:2017-07-01 00:00:00
abstract:BACKGROUND:Hereditary hemochromatosis (HH), a common autosomal recessive disease, leads to excessive iron accumulation in some organs, including the heart. It is therefore not surprising that cardiomyopathy is one of the most severe complications of HH. The HFE gene defects have been thought to contribute to idiopathic...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/j.ejheart.2004.03.007
更新日期:2005-01-01 00:00:00
abstract:AIM:To investigate the role of MMP-2 and MMP-9 in cardiac remodelling induced by tobacco smoke exposure in rats. METHODS:Rats were allocated into two groups: C (n=9): control animals; ETS (n=9): exposed to tobacco smoke. After 4months, the animals underwent echocardiography, morphometric study and determination of MMP...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/j.ejheart.2007.09.004
更新日期:2007-11-01 00:00:00
abstract:AIMS:Right ventricular (RV) dysfunction has been associated with a worse outcome in heart failure patients undergoing CRT. However, evidence on the RV response to CRT is controversial and there are no data regarding the early effects of CRT on RV function (RVF). We sought to investigate whether a CRT device favourably ...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1002/ejhf.450
更新日期:2016-02-01 00:00:00
abstract:AIMS:ACE-inhibitors, β-blockers, implantable cardioverter-defibrillator (ICD) and cardiac resynchronization therapy (CRT) improved prognosis of heart failure. We sought to analyse the long-term prognostic impact of evidence-based integrated therapeutic strategies in patients with idiopathic dilated cardiomyopathy (IDCM...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1002/ejhf.16
更新日期:2014-03-01 00:00:00
abstract:AIMS:As pulmonary arterial hypertension (PAH) is associated with significant morbidity and mortality, particularly among patients with right ventricular (RV) dysfunction, we aimed to determine the impact of therapy to reduce pulmonary vascular resistance (PVR) on RV and LV deformation in PAH. METHODS AND RESULTS:Right...
journal_title:European journal of heart failure
pub_type: 杂志文章,随机对照试验
doi:10.1002/ejhf.177
更新日期:2015-01-01 00:00:00
abstract:AIMS:To examine the current status of care and needs of adult congenital heart disease (ACHD) services in the Central and South Eastern European (CESEE) region. METHODS AND RESULTS:We obtained data regarding the national ACHD status for 19 CESEE countries from their ACHD representative based on an extensive survey for...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1002/ejhf.2040
更新日期:2020-11-02 00:00:00
abstract:AIMS:The Exercise Joins Education: Combined Therapy to Improve Outcomes in Newly-discharged Heart Failure (EJECTION-HF) study will evaluate the impact of a supervised exercise training programme (ETP) on clinical outcomes in recently hospitalized heart failure patients attending a disease management programme (DMP). Me...
journal_title:European journal of heart failure
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1093/eurjhf/hfr139
更新日期:2011-12-01 00:00:00
abstract:BACKGROUND:Most patients suspected of having heart failure (HF) will get a 12-lead electrocardiogram (ECG) but its utility for excluding HF or assisting in its management has rarely been investigated. METHODS:The EuroHeart Failure survey identified 11,327 patients hospitalised with a suspected diagnosis of HF from 115...
journal_title:European journal of heart failure
pub_type: 杂志文章,多中心研究
doi:10.1016/j.ejheart.2006.11.003
更新日期:2007-05-01 00:00:00
abstract:AIMS:The primary objective of this systematic review was to estimate the prevalence and temporal changes in chronic comorbid conditions reported in heart failure (HF) clinical trials. METHODS AND RESULTS:We searched MEDLINE for HF trials enrolling more than 400 patients published between 2001 and 2016.Trials were divi...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1002/ejhf.1818
更新日期:2020-06-01 00:00:00
abstract:AIMS:Guidelines recommend down-titration of loop diuretics (LD) once euvolaemia is achieved. In outpatients with heart failure (HF), we investigated LD dose changes in daily cardiology practice, agreement with guideline recommendations, predictors of successful LD down-titration and association between dose changes and...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1002/ejhf.1796
更新日期:2020-08-01 00:00:00
abstract::This article updates the Heart Failure Association of the European Society of Cardiology (ESC) 2007 classification of advanced heart failure and describes new diagnostic and treatment options for these patients. Recognizing the patient with advanced heart failure is critical to facilitate timely referral to advanced h...
journal_title:European journal of heart failure
pub_type: 杂志文章,评审
doi:10.1002/ejhf.1236
更新日期:2018-11-01 00:00:00
abstract:BACKGROUND:Chronic heart failure (CHF) is a complex syndrome, in which reactive oxygen species and inflammatory cytokines are important stressors that contribute to the pathogenesis. AIM:We have studied physiological stress response parameters in CHF, in particular the redox-active regulator thioredoxin. SUBJECTS:A c...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1016/j.ejheart.2004.03.003
更新日期:2004-12-01 00:00:00
abstract:AIMS:Current guidelines for the diagnosis of heart failure with normal or preserved ejection fraction (HFpEF) are based on measurements at rest. However, in HFpEF ventricular dysfunction is more apparent on exercise. We hypothesized that Mitral annular plane systolic excursion (MAPSE) which is easy to acquire on exerci...
journal_title:European journal of heart failure
pub_type: 杂志文章
doi:10.1093/eurjhf/hfr081
更新日期:2011-09-01 00:00:00