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Restrictive Cardiomyopathy
Aka: Restrictive Cardiomyopathy
- See Also
- Cardiomyopathy
-
Incidence
- Least common form of Primary Cardiomyopathy (2-5% of cases)
- Definition
- Heart muscle dysfunction secondary to stiff ventricles that interfere with contraction
- Pathophysiology
- Normal systolic function
- Decreased ventricular filling progresses to right-sided Heart Failure
- Causes
- See Cardiomyopathy
- Infiltrative Cardiomyopathy
- Sarcoidosis
- Hemochromatosis
- Amyloidosis
- Scleroderma
- Radiation Therapy
- Structural and component abnormalities
- Desmin abnormality
- Troponin abnormality
- Signs
- See Diastolic Heart Failure
- Imaging
- Chest XRay
- Pulmonary vascular congestion
- 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
- Management
- General Measures
- See Diastolic Dysfunction
- Salt restriction
- 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
- Fenton (2006) Heart 92(1): 85-9 [PubMed]
- References
- Brieler (2017) Am Fam Physician 96(10):640-6 [PubMed]
- Kushawa (1997) N Engl J Med 336(4): 267-76 [PubMed]