Myocardium

Restrictive Cardiomyopathy

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Restrictive Cardiomyopathy

  • See Also
  1. Cardiomyopathy
  2. Diastolic Heart Failure
  3. Dilated Cardiomyopathy
  • Incidence
  1. Least common form of Primary Cardiomyopathy (2-5% of cases)
  • Pathophysiology
  1. Normal systolic function
  2. Decreased ventricular filling progresses to right-sided Heart Failure
  3. Restrictive Cardiomyopathy Definition
    1. Heart Muscle dysfunction secondary to stiff ventricles that interfere with contraction
  • Causes
  1. See Cardiomyopathy
  2. Infiltrative Cardiomyopathy
    1. Sarcoidosis
    2. Hemochromatosis
    3. Cardiac Amyloidosis
    4. Scleroderma
    5. Radiation Therapy
  3. Structural and component abnormalities
    1. Desmin abnormality
    2. Troponin abnormality
  • Signs
  1. See Diastolic Heart Failure
  2. Ascites
  3. Edema
  4. Hepatomegaly
  • Imaging
  1. Chest XRay
    1. Pulmonary vascular congestion
  • Diagnostics
  1. Electrocardiogram
    1. Decreased voltage
    2. Prolonged PR Interval (first degree AV Block)
    3. Biatrial enlargement
  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
  3. Cardiac MRI (CMR)
    1. May identify underlying cause (infiltrative or storage disease cause)
  4. Other testing in unclear cases
    1. Endomyocardial biopsy
    2. Genetic analysis
  • Management
  1. General Measures
    1. See Diastolic Dysfunction
    2. Salt Restriction
    3. Diuretics for volume overload
    4. Resulting Hypotension may limit the use of Beta Blockers, ACE Inhibitors and ARBs in Restrictive Cardiomyopathy
  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]
  • Prevention
  1. Screening for first degree relatives of those with Dilated Cardiomyopathy (e.g. ekg, Echocardiogram, exam)
    1. Screen every 3-5 years
  • References
  1. Brieler (2017) Am Fam Physician 96(10):640-6 [PubMed]
  2. Coppiano (2026) Am Fam Physician 113(2): 166-73 [PubMed]
  3. Kushawa (1997) N Engl J Med 336(4): 267-76 [PubMed]