Restrictive Cardiomyopathy

• Rare
• Less genetic

• Amyloidosis (most common), desmin mutation, hemochromatosis (Fe metabolism defect)
• Endomyocardial fibrosis and fibroelastosis in kids/infants
• Löffler syndrome = eosinophilic endomyocardial fibrosis in the tropics
• Radiation injury
• Glycogen storage diseases (type II Pompe’s), Fabry’s disease (lysosomal storage disease, defect of α galactosidase)

• Stiffening of heart muscle interferes with pumping action
• R and L sided failure Thick, waxy, opaque endocardium with normal sized chambers
• Thickened valves, patchy fibrosis throughout from hyaline deposition
• Mural and valvular thrombi
• Sometimes Eosinophilic infiltrate
• Clinically similar to hypertrophic cardiomyopathy, but without hyperdynamic state
• Fatigue, dyspnea, CP, late stage → CHF
• Fibrosis encroaches on conduction system → arrythmia
• Löffler – dilated atria, mural thrombi

Category: Medicine Notes