Restrictive cardiomyopathy is characterized by impaired ventricular filling during diastole due to a primary decrease in ventricular compliance. The myocardium is partially infiltrated by noncontractile tissue or extracellular material, impairing the heart's ability to dilate. Causes include amyloidosis, sarcoidosis, and idiopathic restrictive cardiomyopathy. On morphology, the ventricles are normally or slightly enlarged while the cavities are not dilated. The myocardium is firm and noncompliant, and the atria show biatrial dilation. Patients experience signs and symptoms of congestive heart failure with prominent right-sided features.

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