View clinical trials related to Cardiac Amyloidosis.
Filter by:Cardiac amyloidosis is a condition where the heart muscle, amongst other tissues, is infiltrated by the abnormal build-up of proteins called amyloid. This stiffens and thickens the heart muscle over time which makes it less efficient and puts further stress and strain on the other chambers of the heart, leading to heart failure. The commonest form, that affects predominantly the elderly, is called 'wild-type' ATTR amyloid (TTR is the protein that accumulates). In this condition a patient has a 60% chance of admission to hospital each year after diagnosis. There is no current treatment for ATTR amyloid other than using water tablets to reduce excess fluid and prevent more serious fluid build up in lungs and other tissues. Increasing body weight is the most reliable clinical sign of this fluid build up. Tele-monitoring is the practice of monitoring patients from a distance and has been shown to reduce heart failure admissions and death in patients with heart failure from any cause. Due to reduced access to primary and secondary care during COVID-19 the investigators instigated tele-monitoring of heart failure in ATTR amyloid patients. This appeared to be an effective intervention in the pilot study. The investigators propose to monitor the weight of patients with cardiac amyloidosis at home and intervene where a build up of fluid is observed by telephone discussion with a doctor. The investigators propose to evidence this in a prospective clinical trial. The investigators will evaluate the effect fairly by comparing tele-monitoring with usual care.
The study team will generate preliminary data on whether patients with cardiac amyloidosis feel better when their beta-blocker is stopped. To achieve this objective, 20 N-of-1 trials (on vs. off) will be conducted, and the study team will subsequently interview participants to better understand their outcomes. Each subject will participate in 2 periods lasting between up to 6 weeks each based on each patient's health profile. We will also engage stakeholders to understand the acceptability and feasibility of deprescribing N-of-1 trials. The N-of-1 trials will be iteratively refined in real-time based on feedback.
Amyloid heart disease is an accumulation of fibrillar proteins in the extracellular sector of the heart. Identified on echocardiography as Ventricular hypertrophy. The investigation of a Left Ventricular hypertrophy (LVH) is the most frequent discovery circumstance of amyloid heart disease. Pathophysiological mechanisms poorly understood, resulting in late diagnosis. Transthyretin amyloid heart disease (CATTR) is the most common form of cardiac amyloidosis in the West Indies due to an abnormally high frequency of the Val122Ile and Val107Ile mutations of the transthyretin gene in this population. Val122Ile and Val107Ile mutated-transthyretin are the substitution of valine for isoleucine at codon 122 of the TTR gene ( V122I) and at codon 107 of the TTR gene (V107I). Complications of CATTR are functional changes in heart cells or even death due to mechanical abnormalities (loss of contractility and increased wall stiffness cardiac arousal and conduction disturbances). These disorders result from an electrical abnormality of the heart the reason why the cardiologist performs preventive performance of electrophysiological explorations with EnSite Precision™. It's a registration system used to detect foci of necrosis within the myocardium. Amyloid deposits are areas devoid of electrical activity. Do they detectable by the EnSite Precision™ recording system ?
Patients with left ventricular hypertrophy are further examined according to an algorithm to check if they have a cardiac amyloidosis
The study aims to test the diagnostic accuracy of native T1 mapping for the diagnosis of cardiac amyloidosis prospectively. The hypothesis is that native T1 mapping with a cut-off value of 1341ms (3 tesla CMR) in older patients with symptomatic heart failure, increased LV wall thickness and elevated cardiac biomarkers is non-inferior to the reference method to diagnose cardiac amyloidosis (CA). As secondary measure, a web-based ATTR probability estimator for the diagnosis of CA will be evaluated.
The overall aim of this study is to improve our understanding of the effects of the build-up of amyloid deposits in the heart, in particular, our understanding of the risk of abnormal heart beats, or rhythms, associated with people with cardiac (heart) amyloidosis. Symptoms such as palpitations (fast, strong or irregular heart beat) and blackouts are common in people with cardiac amyloidosis, but there is not enough information on what causes this. At present, there is also not enough information on when they occur, how often they happen, and which patients are at risk of having serious, life-threatening types of abnormal heart rhythms. Some of these abnormal heart rhythms can be treated with medicine; others need electronic devices (e.g. pacemakers) implanted or inserted in the heart to prevent serious harm. The information on when is the best time to implant these life-saving devices remains limited. In this study, a small device known as an implantable loop recorder (ILR) will be implanted under the skin on the chest wall to continuously monitor participants' heart rhythm. This will help us answer some of the questions about what causes the abnormal heart rhythms, when they happen, and which patients are particularly likely to have them. Furthermore, it may help us to identify earlier, rather than later, those who are at risk of developing abnormal heart rhythms. This may lead to improvements in the care of people with cardiac amyloidosis in the future. Participants may not directly benefit from taking part in this study; however, there is a chance that the ILR may reveal heart rhythm abnormalities in some participants which might not be picked up otherwise, and so may lead to a change in their treatment.
Recently, treatment with tafamidis in patients with cardiac ATTR lead to a significant reduction in mortality. The Perugini score is commonly used on planar bone scans to differentiate cardiac ATTR from other amyloidosis or normal patients but fails to evaluate amyloid burden and patient prognosis. Although semi-quantitative methods have been suggested to evaluate the amyloid burden, there a need for quantitative methods for longitudinal assessment of the disease.
There is existing data in the literature that suggests an additional predictive value of three dimensional ECG with respect to the presence of electrical abnormalities and for an existing cardiac disease. Especially regarding patients who suffered from a myocardial infarction in the past (post MI patients), evidence has been provided for a potential association of 3D repolarisation abnormalities and incidence of sudden cardiac death (SCD). In addition, there is some vague evidence of so called 3D ECG and prediction of coronary artery disease. This 3D ECG device is using the technology of 3D ECG vector loops and is assessing the variability of these ECG vector loops in the 3-dimensional space. Based on these data, the parameters of 3D ECG are suggested to carry certain value to predict or to identify individuals already suffering from a cardiac disease or being at risk experiencing a cardiac event in the future. In this context we performed a preliminary study with 3D-ECG device in healthy volunteers evaluating the robustness of this method with respect to reproducibility, intra- and intra-observer variability which could be confirmed. We thus postulate that the 3D ECG technology might bear the potential to serve as a sufficient screening method for diagnosing cardiomyopathy in patients with an unknown heart failure etiology.
Amyloidosis is a disease caused by the continuous accumulation of fibrillary proteins in the extracellular matrix causing the architecture of different organs to be disrupted. The prevalence of the disease increases with age. The two most common forms are light chain amyloidosis (AL) and transthyretin (TTR). TTR amyloidosis may be hereditary (m-TTR, or mutated) or age-related (WT-TTR, or wild). The latter is also called senile amyloidosis. In all these forms, cardiac impairment is common and leads the patient to consult/or be referred to a cardiological center unfortunately often too late when the prognosis is directly related to the severity of the heart attack. The description/discovery of clinical signs prior to heart disease is important to improve the detection and diagnosis of early forms of cardiac amyloidosis (CA). For example, an infiltration of the carpal tunnel synovial by amyloid deposits is observed in some patients, 5 years before the onset of signs of heart failure and is the only warning sign of the disease known to date. We also showed in a previous study that patients had more severe and earlier impairment of hearing function than expected by age and gender. Objective The main objective is to define the prevalence and severity of smell and taste disorders in a population of patients with cardiac amyloidosis (3 types of mutated or wild AL amyloidosis and TTR). The main endpoint is to determine the number of patients with impaired smell and taste's functions in a population of patients diagnosed with cardiac amyloidosis (3 types of mutated (hereditary) or senile wild amyloidosis (3 types of AL amyloidosis and TTR). Method Successive monocentric cross-sectional study on the screening of smell and taste disorders carried out as part of a cardiology hospitalization programmed for the cardiology follow-up of his pathology in a population of patients diagnosed with AC.
Background: A significant portion of cardiac amyloidosis patients have a 5 to 10 years prior history of spinal canal stenosis, reflecting a diagnostic red flag that should raise suspicion for amyloidosis presence. Mild troponin release and NT-proBNP elevation, both serum cardiac biomarkers, often coincide with cardiac amyloidosis. Early cardiac amyloidosis treatment improves survival, warranting timely diagnosis. Study aim: to test a prospective screening strategy, based on serum cardiac biomarkers, to increase early detection of cardiac amyloidosis in patients with spinal canal stenosis. Design: Single-centre prospective observational non-interventional diagnostic study. Methods: Consecutive patients during a one-year period in AZ Sint-Jan Bruges, without known cardiac amyloidosis history and scheduled for spinal canal stenosis surgery, will have cardiac evaluation including serum cardiac biomarker (high-sensitive troponin T and NT-proBNP) assessment, electrocardiography and transthoracic echocardiography. During surgery, all patients will undergo ligamentum flavum biopsy to evaluate presence and burden of transthyretin amyloid deposition (Congo-red staining and immune histochemistry). All patients with suspicion for cardiac amyloidosis will undergo further diagnostic testing (including laboratory test and bone scintigraphy). A chronologic cascade screening process will be used starting with abnormal serum cardiac biomarkers (high-sensitive troponin T ≥ 14 ng/ml and/or NT-proBNP > 125 pg/ml), followed by electrocardiography, transthoracic echocardiography and finally ligamentum flavum biopsy results. The diagnostic performance of this biomarker-based strategy will be compared to electrocardiography, echocardiography and ligamentum flavum biopsy. Conclusion: It is hypothesised that serum cardiac biomarker testing in patients undergoing spinal canal stenosis surgery represents a simple and valuable prospective screening strategy for early detection of cardiac amyloid(osis).