cardiomyopathy

1. Restrictive cardiomyopathy

2. Restrictive cardiomyopathy
• Disorder characterized by a
primary decrease in ventricular
compliance resulting in impaired
ventricular filling during diastole

4.  The myocardium is partially infiltrated
by noncontractile tissue or
extracellular material
 This infiltration of the myocardium
impairs the ability of the heart to dilate.

5. ETIOLOGY
• ■ Infiltrative causes: Amyloid, sarcoid
• ■ Noninfiltrative causes: Idiopathic,
scleroderma.
• ■ Other causes: Hemochromatosis ,
diffuse interstitial fibrosis,sarcoidosis,
postradiation fibrosis,metastatic
tumors,inborn errors of metabolism

7. Amyloidosis-heart

8. sarcoidosis

9. MORPHOLOGY
• Ventricles- normal/slightly enlarged
Cavities- not dilated
• Myocardium- firm and non
compliant
• Atria- biatreal dilation
• Microscopy-interstitial fibrosis

10. Clinical features
 Usually causes diastolic
dysfunction.
 Patients have signs and
symptoms of CHF with prominent
right-sided features.

12. Other restrictive conditions
Endomyocardial fibrosis
Loeffler endomyocarditis
Endocardial fibroelastosis

13. Endomyocardial fibrosis
Fibrosis of ventricular endocardium and
subendocardium
Extends from apex upward often invovling
tricuspid & mitral valves
Children & young adults
Africa & other tropical regions

14. Endomyocardial fibrosis
• Fibrous tissue markedly diminishes the volume &
compliance of affected chambers
• Induces a restrictive functional defect
• Ventricular mural thrombi & fibrous tissue results
from its organisation
• Etiology unknown