Systemic amyloidosis of the immunoglobulin light-chain (AL) or transthyretin type (ATTR) is a multisystem protein deposition disease that often involves the heart. Delays in diagnosis are very common and can have detrimental consequences on patient outcomes. Because both major types can now be ...
"The heart is the most important abnormality in amyloidosis because if the heart is affected, that is the complete driver of how the patient will do and what their prognosis is," says Dr. Grogan. "That's why cardiac amyloidosis is so important to recognize and treat as early as possible....
Hereditary transthyretin-amyloid amyloidosis (ATTRv) is an underdiagnosed condition commonly manifesting as congestive heart failure. Recently, scintigraphy utilizing DPD as a tracer was shown to identify ATTRv and wild-type ATTR cardiomyopathy. The aim of this study was to determine the value of quan...
Aβ2M, β2-microglobulin amyloidosis; AA, amyloid A amyloidosis; ANP, atrial natriuretic peptide; BNP, brain natriuretic peptide; CA, cardiac amyloidosis; cTnI/T, cardiac troponin I/T; ECM, extracellular matrix; EMT, endothelial mesenchymal transition; FLC, free light chain; HF, heart failure; ...
Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis. BACKGROUND: Immunoglobulin amyloid light-chain (AL)-related cardiac amyloidosis (CA) has a worse prognosis than either wild-type (ATTRwt) or mutant (ATTRm)......
Heart failure from ATTRwt amyloid cardiomyopathy is associated with poor prognosis ESC Heart Fail., 7 (2020), pp. 3919-3928 CrossrefView in ScopusGoogle Scholar 7 S. Goland, I. Volodarsky, Y. Fabricant, et al. Wild-type TTR amyloidosis among patients with unexplained heart failure and systol...
Cardiac amyloidosis (CA) is increasingly recognized as an underlying cause of heart failure with preserved ejection fraction (HFpEF), associated with high morbidity and mortality. However, most studies, solely investigated the prevalence of CA in special subgroups including HFpEF and severe aortic valve...
cardiac amyloidosis and helps decide the treatment modality and determining disease prognosis; it further emphasizes the need for an interprofessional team to diagnose and manage cardiac amyloidosis, including cardiologists, cardiovascular imaging specialists, heart and transplant specialists, and hematologists...
(ATTR) amyloidosis is caused by the accumulation of either wild-type or mutated (“variant”) TTR (ATTRwt or ATTRv, respectively) [5]. The expansion of the extracellular space due to amyloid deposition leads to myocardial stiffness and diastolic dysfunction [6]. Amyloid may also accumulate in...
Prospective evaluation of the morbidity and mortality of wild-type and V122I mutant transthyretin amyloid cardiomyopathy: The Transthyretin Amyloidosis Cardiac Study (TRACS). Am Heart J 2012;164:e1. Google Scholar Falk RH, Alexander KM, Liao R, Dorbala S. AL (Light-Chain) cardiac amyloidosis...