Critical Management Strategies for Cardiovascular Involvementin Light Chain Amyloidosis / 罕见病研究

ABSTRACT

Light chain amyloidosis (AL) is a kind of rare disease. The misfolding of the light chain of monoclonal immunoglobulin forms amyloid substances and deposit in different tissues and organs, resulting in organ dysfunction. The heart involvement is represented as light chain cardiac amyloidosis (AL-CA), leading to heart failure, arrhythmia, myocardial infarction, valvular and pericardial diseases. Cardiac involvement is crucial for the risk stratification and prognosis prediction of patients with AL. The median survival time of AL-CA patients is 24 months and only 6 months for the heart failure patients. In recent years, the prognosis of patients with AL-CA has been significantly improved, thanks to the advancement in diagnosis & treatment strategies of cardiac amyloidosis, the milestone in the breakthroughs in targeted therapy of AL, and the improvement of coordinated multidisciplinary treatment. This paper focuses on the cardiovascular involvement of AL and briefly illustrates the pathogenesis, clinical manifestations, and the diagnosis and treatment of AL-CA.