transthyretin amyloidosisprognosisdiagnostic delayheart failure stageTo apply the American College of Cardiology (ACC) and American Heart Association (AHA) heart failure (HF) staging system to patients with transthyretin cardiac amyloidosis (TTR-CA) in order to assess diagnostic delay and evaluate ...
Amyloidosis is caused by extracellular deposition of abnormal protein fibrils, resulting in destruction of tissue architecture and impairment of organ function. The most common forms of systemic amyloidosis are light-chain and transthyretin-related (ATTR). ATTR can result from an autosomal dominant heredi...
Cardiac amyloidosis is a progressive infiltrative disease and an important cause of atrial arrhythmias, stroke and heart failure. Abnormal amyloid fibril deposition throughout the heart leads to a host of clinical manifestations and complications. Althou
Cardiac amyloidosisTransthyretin amyloidosisLightヽhain amyloidosisTricuspid regurgitationPrognosisAims Tricuspid regurgitation (TR) is a common finding and has been associated with poorer outcome in patients with heart failure. This study sought to investigate the prognostic value of TR in patients with ...
Cardiac amyloidosis is a form of restrictive cardiomyopathy resulting in heart failure and potential risk on arrhythmia, due to amyloid infiltration of the
Ruberg, MD2 Since the identification of a valine-to-isoleucine substitution at posi- tion 122 (TTR V122I; pV142I) in the transthyretin (TTR)-derived fibrils extracted from the heart of a patient with late-onset cardiac amyloidosis, it has become clear that the amyloidogenic mutation ...
(n = 260) with advanced AL-CA (Mayo stage IV) treated with birtamimab plus standard therapy compared to control group (survival rate of 74% vs. 49%, at 9 months) [94]. Based on these results, the phase 3 AFFIRM-AL trial is enrolling patients with AL amyloidosis at Mayo stage...
Oubari S, Hegenbart U, Schoder R, Steinhardt M, Papathanasiou M, Rassaf T, Thimm A, Hagenacker T, Naser E, Duhrsen U, et al. Daratumumab in first-line treatment of patients with light chain amyloidosis and Mayo stage IIIb improves treatment response and overall survival. Haematologica. ...
Cardiac amyloidosis (CA) is caused by the deposition of abnormal amyloid fibrils in the myocardium, including the ventricular walls, valves, and conduction system. This infiltrative process typically causes heart failure with preserved ejection fraction (HFpEF) and, in more advanced stages, degenerates...
Cardiac involvement is present in about 70% of patients with AL amyloidosis at the time of diagnosis, and the severity of cardiac involvement is the main determinant of overall survival in this patient population.1 Measurement of B-type natriuretic peptide (NT-proBNP) and troponin T or I, ...