View clinical trials related to Amyloidosis Cardiac.
Filter by:Heart failure with preserved ejection fraction (HFpEF) accounts for half of heart failure cases with heterogenous cause and variable presentations. The diagnosis of HFpEF required clinical signs and symptoms of HF, normal left ventricular ejection fraction (LVEF) and evidence of diastolic dysfunction. No treatment has been shown in recent major clinical trials having benefits in these patients. One major reason of the poor response to medical treatment is the heterogeneity of HFpEF, which contains many different underline causes. To identify the underlying causes of HFpEF may improve the diagnosis and treatment in these patients. Age-related amyloid deposition has first been reported in 1876 and the following autopsy studies showed the prevalence of senile cardiac amyloid is up to 25%. Recently, it has been recognized that the deposits in senile cardiac amyloid are derived from wild-type transthyretin (TTR). Transthyretin amyloidosis cardiac amyloidosis (ATTR CA) is caused by myocardial deposition of misfolded transthyretin protein. There are 2 types of ATTR classified by genetic mutation including wild-type ATTR (ATTRwt) and familial cardiac amyloid caused by TTR mutation (ATTRm). Multimodality techniques have been developed to assist in the diagnosis of the diagnosis of TTR. Among them, 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) scintigraphy is a non-invasive test and it can diagnose TTR from other cause diverse form of cardiac amyloidosis and cardiomyopathy. In the study of Gonzalez-Lopez et al, in 120 HFpEF patients, 16 (13.3%) had positive 99mTc-DPD scan. Four patients with positive 99mTc-DPD scan received endomyocardial biopsy and confirmed cardiac amyloid deposition. ATTRwt could be an important cause of HFpEF and it was often under diagnosed. A recent study in Spain reported that 13% of patents over age of 60 years with HFpEF and left ventricular wall thickness of 12mm or more had ATTRwt. However, the prevalence of ATTRwt among patients with HFpEF is not well-established in Taiwan and Asia. The aim of this study is to determine the prevalence, clinical characteristics, risk factors and outcomes of ATTRwt related HFpEF patients in Taiwan.
This phase II clinical trial aimed at influencing the improvement of major organ functions, especially the objective response rate, in Amyloid light-chain amyloidosis involving myocardium.
Severe aortic stenosis is defined with a mean transvalvular pressure gradient (MTPG) > 40mmHg and a calculated aortic valve area of < 1cm2. However, a considerable proportion of patients do have a MTPG < 40mmHg due to a reduced stroke volume (stroke volume indexed to body surface area ≤ 35ml/m2) despite a normal left ventricular ejection fraction (LVEF > 50%). This entity is termed paradoxical low flow low gradient aortic stenosis (PLFLG AS) and is associated with a worse prognosis. ATTR amyloidosis is a disease of the elderly and might coexist in patients with severe aortic stenosis. Case reports and small observational studies suggest that senile ATTR amyloidosis could be frequent but underdiagnosed in patients with aortic stenosis. There is significant overlap between PLFLG AS and cardiac amyloidosis with regard to symptoms, increasing prevalence with age, concentric hypertrophy, impaired diastolic filling of the left ventricle (LV), as well as longitudinal LV dysfunction despite preserved ejection fraction - all features, which lead to a reduction in stroke volume, the underlying mechanism of the low flow condition as observed in PLFLG AS patients.
Wild-type transthyretin cardiac amyloidosis is an underdiagnosed depository disease in which fibril monomers of misfolded amyloid protein accumulates in various tissues, including the heart, and cause tissue dysfunction. Before onset of cardiac symptoms, many patients will have undergone surgery for idiopathic carpal tunnel syndrome since the protein also deposits in the transversal carpal ligament of the hand. This study investigates patients previously operated for idiopathic carpal tunnel syndrome to determine if they display signs and symptoms of cardiac amyloidosis.
To analyze the prevalence and impact on the prognosis of amyloidosis due to transthyretin in patients with severe aortic stenosis who undergo percutaneous aortic valve implantation.