Related Experiment Video
Updated: Jun 4, 2026

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Behind heart failure syndrome: remember AL amyloidosis. Two case-reports
Doroteia Silva1, Luís Sargento, Manuel Gato Varela
1Serviço de Cardiologia I, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, EPE, Lisboa, Portugal. dojreis@hotmail.com
Abstract:
Amyloidosis is a systemic disease that is a consequence of extracellular deposition of insoluble fibrils composed of subunits of low molecular weight (5-25 kD) derived from a variety of plasma proteins. Identification of the amyloidogenic protein determines the type of amyloidosis. In primary systemic amyloidosis (classically called AL amyloidosis), the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia. Cardiac manifestations are the most common clinical presentation of this type of amyloidosis, occurring in 50% of patients. The authors describe two cases in which hospitalization was due to decompensated heart failure, which were similar in their etiology (multiple myeloma/amyloid cardiomyopathy) and evolution (sudden death). The authors wish to draw attention to an entity that is rarely encountered in clinical practice and that requires a high index of suspicion.
Related Concept Videos
Cardiomyopathy II: Dilated Cardiomyopathy
Cardiomyopathy V: Interprofessional Care
Heart Failure II: Pathophysiology
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Pathophysiology of Heart Failure
Heart Failure I: Introduction

