II. Incidence
- Least common form of Primary Cardiomyopathy (2-5% of cases)
III. Definition
- Heart Muscle dysfunction secondary to stiff ventricles that interfere with contraction
IV. Pathophysiology
- Normal systolic function
- Decreased ventricular filling progresses to right-sided Heart Failure
V. Causes
- See Cardiomyopathy
- Infiltrative Cardiomyopathy
- Structural and component abnormalities
- Desmin abnormality
- Troponin abnormality
VI. Signs
VII. Imaging
-
Chest XRay
- Pulmonary vascular congestion
VIII. Diagnostics
-
Electrocardiogram
- Decreased voltage
- Prolonged PR Interval (first degree AV Block)
-
Echocardiogram
- Biatrial enlargement
- Diastolic Dysfunction
- Normal or reduced ventricular volume with impaired ventricular filling
- Normal left ventricular wall thickness and normal systolic function
IX. Management
- General Measures
- Specific management based on underlying condition
- Chelation therapy or phlebotomy for infiltrative processes
- Devices
- Pacemaker (for higher grade AV Block)
- Implantable Defibrillator
- Cardiac Transplantation for children with recently diagnosed Restrictive Cardiomyopathy