Work

Features and Prognosis of Exertional Syncope in Light-Chain Associated AL Cardiac Amyloidosis

Year: 1997

Type: article

Abstract: Primary (AL) amyloidosis is a multisystem disease caused by plasma cell dyscrasia and characterized by the extracellular deposition of immunoglobulin light chains within various organs. Cardiac dysfunction secondary to amyloid infiltration is a well-recognized feature occurring in up to 50% of patients with primary systemic amyloidosis. Clinically, cardiac amyloidosis frequently presents with a restrictive pathophysiology secondary to the deposition of the amyloid fibrillar protein within the myocardium,[1]resulting in a “stiff heart syndrome.”[2]Conduction disturbances and coronary insufficiency due to amyloid deposition may also be part of the clinical picture of cardiac AL.[3]Common presenting symptoms of cardiac AL include fatigue, weakness, edema, dyspnea, light-headedness, and syncope.[4]This study details an undescribed type of syncope in a group of patients with severe cardiac AL and contrasts the potential mechanism with other cases of syncope.

Source: The American Journal of Cardiology

DOI: 10.1016/s0002-9149(97)00653-x

Authors: Bindu Chamarthi, Simon W Dubrey, Karen Cha, Martha Skinner, Rodney H Falk

Institution: Boston University

Language: English

Topic: Amyloidosis: Diagnosis, Treatment, Outcomes

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $3,380