Abstract
Idiopathic cardiomyopathies are divided into three forms: primary restrictive, infiltrative, and storage disorders. Primary restrictive cardiomyopathies are usually genetically inherited. They are characterized by bi-atrial enlargement with non-dilated ventricles in addition to a characteristic histopathological morphology. Cardiac transplantation is the ideal treatment. Cardiac amyloidosis is the major form of infiltrative restrictive cardiomyopathies. Its prognosis is poor, but several therapies are being investigated and implemented. Storage cardiomyopathies are the consequence of accumulation of substances due to genetic defects affecting metabolic pathways; we review Fabry disease, glycogen storage diseases and hemochromatosis.
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