Author name: mozarb31@gmail.com

BACKGROUNDTransthyretin amyloidosis with cardiomyopathy (ATTR-CM) is a progressive, fataldisease. Vutrisiran, a subcutaneously administered RNA interference therapeuticagent, inhibits the production of hepatic transthyretin.METHODSIn this double-blind, randomized trial, we assigned patients with ATTR-CM in a 1:1ratio to receive vutrisiran (25 mg) or placebo every 12 weeks for up to 36 months.The primary end point was a composite […]

Background—Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal cardiomyopathy for which severalpromising therapies are in development. The diagnosis is frequently delayed or missed because of the limited specificityof echocardiography and the traditional requirement for histological confirmation. It has long been recognized thattechnetium-labeled bone scintigraphy tracers can localize to myocardial amyloid deposits, and use of

Guidelines on the Diagnosis and Treatment of Hypertrophic Cardiomyopathy – 2024Realização: Departamento de Insuficiência Cardíaca (DEIC) e Grupo de Estudos de Miocardiopatias (GEMIC)da Sociedade Brasileira de Cardiologia (SBC)Coordenadores: Fábio Fernandes e Marcus Vinicius SimõesAutores da Diretriz: Fabio Fernandes,1 Marcus V. Simões,2 Edileide de Barros Correia,3 Fabiana GoulartMarcondes-Braga,1 Otavio Rizzi Coelho-Filho,4 Cláudio Tinoco Mesquita,5 Wilson Mathias

Cardiac amyloidosis is a serious and progressive infiltrative disease that is caused by the deposition of amyloidfibrils at the cardiac level. It can be due to rare genetic variants in the hereditary forms or as a consequence ofacquired conditions. Thanks to advances in imaging techniques and the possibility of achieving a non-invasivediagnosis, we now know

This article provides a critical review of the diagnosticvalue of several echocardiographic findings in cardiacamyloidosis (CA). The importance of early andaccurate detection of CA is emphasized, considering itschallenging diagnosis and the need for a high index ofsuspicion by clinicians. Echocardiography is often thefirst choice for imaging assessment of cardiac structureand function when CA is suspected.

Recent-onset cardiomyopathy represents a clinically dynamic and potentially reversible clinical framework of non-ischaemiccardiomyopathy, characterized by high variability in left ventricular (LV) function and arrhythmic risk. This clinical consensusstatement provides a structured diagnostic and therapeutic approach based on two prognostic axes: the potential for LV

Background: Cardiac amyloidosis (CA), characterized by a progressive infiltration of amyloid fibrils in cardiac tissue, presents a diagnosticchallenge due to its resemblance to other cardiomyopathies by echocardiography. Although reduced left ventricular (LV) global longitudinalstrain associated with a relative apical sparing pattern (RELAPS) has been associated with CA, its diagnostic accuracy has varied acrossstudies.Purpose: The aim

A Report of the American College of Cardiology Solution Set Oversight CommitteeEndorsed by the American Association of Neuromuscular & Electrodiagnostic Medicine, Heart FailureSociety of America, and International Society of Amyloidosis. The American Academy of Neurologyaffirms the value of this statement.

Cardiac amyloidosis represents a unique disease process characterized by amyloid fibril deposition within the myocardialextracellular space. Advances in multimodality cardiac imaging enable accurate diagnosis and facilitate prompt initiationof disease-modifying therapies. Furthermore, rapid advances in multimodality imaging have enriched understanding ofthe underlying pathogenesis, enhanced prognostication, and resulted in the development of imaging-based markers thatreflect the amyloid

Transthyretin amyloid cardiomyopathy is caused by the deposition of transthyretin amyloid fibrils in the myocardium. The deposition occurs when wild-type or variant transthyretin becomes unstable and misfolds. Tafamidis binds to transthyretin, preventingtetramer dissociation and amyloidogenesis.