Restrictive Cardiomyopathy

1. See Also
   1. Cardiomyopathy
2. Incidence
   1. Least common form of Primary Cardiomyopathy (2-5% of cases)
3. Definition
   1. Heart muscle dysfunction secondary to stiff ventricles that interfere with contraction
4. Pathophysiology
   1. Normal systolic function
   2. Decreased ventricular filling progresses to right-sided Heart Failure
5. Causes
   1. See Cardiomyopathy
   2. Infiltrative Cardiomyopathy
      1. Sarcoidosis
      2. Hemochromatosis
      3. Amyloidosis
      4. Scleroderma
      5. Radiation Therapy
   3. Structural and component abnormalities
      1. Desmin abnormality
      2. Troponin abnormality
6. Signs
   1. See Diastolic Heart Failure
7. Imaging
   1. Chest XRay
      1. Pulmonary vascular congestion
8. Diagnostics
   1. Electrocardiogram
      1. Decreased voltage
      2. Prolonged PR Interval (first degree AV Block)
   2. Echocardiogram
      1. Biatrial enlargement
      2. Diastolic Dysfunction
      3. Normal or reduced ventricular volume with impaired ventricular filling
      4. Normal left ventricular wall thickness and normal systolic function
9. Management
   1. General Measures
      1. See Diastolic Dysfunction
      2. Salt restriction
   2. Specific management based on underlying condition
      1. Chelation therapy or phlebotomy for infiltrative processes
   3. Devices
      1. Pacemaker (for higher grade AV Block)
      2. Implantable Defibrillator
   4. Cardiac Transplantation for children with recently diagnosed Restrictive Cardiomyopathy
      1. Fenton (2006) Heart 92(1): 85-9 [PubMed]
10. References
    1. Brieler (2017) Am Fam Physician 96(10):640-6 [PubMed]
    2. Kushawa (1997) N Engl J Med 336(4): 267-76 [PubMed]