The diagnosis and treatment of the most common form of restrictive cardiomyopathy: Cardiac amyloidosis
Abstract
The most common restrictive cardiomyopathy is light-chain (AL) amyloidosis with an estimated prevalence of 8-10/1 mil. pop. The clinical picture is a combination of non-specific general symptoms with presentations of heart failure.
The principal diagnostic method is echocardiography showing myocardial wall thickening and increased stiffness leading to left ventricular diastolic dysfunction. The gold standard is histologic evidence of amyloid with its immunohistochemical or immuno-electron microscope typing.
Treatment is performed in close collaboration with a department of hematology, or even there, and is designed to inhibit the formation of amyloidogenic protein. If initiated early, treatment of AL amyloidosis may improve the hitherto very grim prognosis of these patients.