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Updated: Mar 29 2017

Restrictive / Obliterative Cardiomyopathy

Snapshot 
  • MRI A 44-year-old female presents to the emergency department complaining of shortness of breath. For the past several years she has suffered from chronic, progressive, congestive heart failure due to an inflammatory infiltrative process.
Introduction
  • Restrictive cardiomyopathy usually presents
    • progressively worsening over time
  • Caused by chronic, progressive, infiltrative or metabolic diseases including
    • amyloidosis
    • sarcoidosis
    • hemochromatosis 
    • cancer
    • endocardial fibroelastosis
    • Loffler's endocardial fibrosis
    • glycogen storage diseases
Presentation
  • Symptoms
    • chronic, progressively worsening symptoms
    • cough
    • dyspnea
    • fatigue
    • edema
  • Physical exam
    • JVD
    • hepatomegaly
    • crackles of auscultation
    • distant heart sounds
    • pale skin
    • weak pulses
Evaluation
  • Echocardiography is diagnostic
    • shows LVH
  • Tissue biopsy is required
    • to determine underlying cause
  • EKG demonstrates
    • low voltage in all leads
Differential
  • Other cardiomyopathies, and other causes of CHF
Treatment
  • Directed at underlying infiltrative or metabolic process(es)
    • Hemochromatosis is only reversible cause
      • treat with phlebotomy 
  • Diuretics for CHF symptoms
Prognosis, Prevention, and Complications
  • Prognosis and prevention depend on underlying infiltrative or metabolic process
  • Complications include
    • arrhythmias
    • mitral regurgitation
    • tricuspid regurgitation
    • progression to severe CHF
Question
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