Cardiomyopathy

Aka: Cardiomyopathy, Myocardial Disease, Primary Cardiomyopathy, Secondary Cardiomyopathy, Cardiomyopathy Causes

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II. Images

  1. Cardiomyopathy Types
    1. cvCardiomyopathy.png

III. Causes: Primary Cardiomyopathy (intrinsic, isolated cardiac effects)

  1. Acquired Primary Cardiomyopathy
    1. See Myocarditis
    2. Tachycardia induced Cardiomyopathy (Arrhythmia-induced)
      1. Atrial Fibrillation with persistent rapid ventricular rate
      2. Grogan's Syndrome
    3. Takotsubo Cardiomyopathy (Stress Cardiomyopathy)
    4. Peripartum Cardiomyopathy (or Postpartum Cardiomyopathy)
      1. Associated with Obesity, age over 30 years, Multiparity, and Preeclampsia
      2. Onset from third trimester to five months postpartum
  2. Genetic Primary Cardiomyopathy
    1. Hypertrophic Cardiomyopathy
      1. Autosomal Dominant condition with Incidence of 1 in 500
      2. Leading cause of Sudden Death in Athletes
    2. Arrhythmogenic Right Ventricular Cardiomyopathy (rare, hereditary)
    3. Left Ventricular Noncompaction
    4. Other genetic disorders
      1. Ion channel disorder
      2. Mitochondrial Myopathy
  3. Mixed Primary Cardiomyopathy
    1. Restrictive Cardiomyopathy (rare)
    2. Dilated Cardiomyopathy
      1. Most common Cardiomyopathy
      2. Ischemic Cardiomyopathy is considered a subset of Dilated Cardiomyopathy
      3. Third leading cause of Heart Failure behind Coronary Artery Disease and Hypertension

IV. Causes: Secondary Cardiomyopathy (extrinsic or systemic condition related)

  1. See Myocarditis
  2. Autoimmune, Collagen Vascular Disease or inflammatory conditions
    1. Dermatomyositis
    2. Polyarteritis Nodosa
    3. Rheumatoid Arthritis
    4. Sarcoidosis
    5. Scleroderma
    6. Systemic Lupus Erythematosus
  3. Endocrine conditions
    1. Acromegaly
    2. Diabetes Mellitus
      1. Multi-factorial (LVH, micro/macro coronary disease, endothelial dysfunction, Autonomic Dysfunction)
    3. Hyperparathyroidism
    4. Hyperthyroidism (high output Heart Failure)
    5. Hypothyroidism
    6. Obesity
    7. Pheochromocytoma
    8. Paget's Disease (high output Heart Failure)
  4. Infectious conditions
    1. Parasitic Infections
      1. Chagas Disease (Trypanosoma cruzi, most common Myocarditis cause wordwide)
      2. Trichinella species (TrichinosisRoundworm)
      3. Taenia Solium (Cysticercosis)
      4. Entamoeba histolytica (Amoebiasis)
    2. Viral Infections
      1. Parvovirus B19 (most common Myocarditis cause in U.S. in 2017)
      2. Human Herpes Virus 6 (HH6)
      3. Coxsackie Virus B (previously most common in U.S.)
      4. Covid19
      5. Human Immunodeficiency Virus (HIV)
      6. Hepatitis C
      7. Adenovirus
      8. Epstein-Barr Virus (EBV, Mononucleosis)
      9. Influenza
      10. Cytomegalovirus (CMV)
    3. Bacterial Infections
      1. Rickettsia
      2. Mycobacteria
      3. Streptococcal Pharyngitis (with secondary Acute Rheumatic Fever, Myocarditis)
      4. Lyme Disease (strongly consider in endemic areas as cause of Myocarditis)
      5. Diphtheria (toxin-induced Myocarditis)
  5. Infiltrative conditions
    1. Amyloidosis
    2. Gaucher Disease
    3. Hunter Syndrome
    4. Hurler Syndrome
  6. Neuromuscular conditions and Glycogen Storage Disease
    1. Glycogen Storage Disease
    2. Neurofibromatosis
    3. Barth Syndrome
    4. Muscular Dystrophy
      1. Duchenne Muscular Dystrophy
      2. Becker Muscular Dystrophy
      3. Myotonic Dystrophy
  7. Nutritional deficiency
    1. Protein Malnutrition (Kwashiorkor)
    2. Thiamine deficiency (Wet Beriberi)
    3. Niacin Deficiency (Pellagra)
    4. Vitamin C Deficiency (Scurvy)
    5. L-Carnitine Deficiency
    6. Selenium Deficiency
    7. Severe Anemia (high output Heart Failure)
  8. Toxic-metabolic conditions
    1. Anabolic Steroids
    2. Chloroquine (Aralen)
    3. Hemochromatosis
    4. Radiation Exposure including Radiation Therapy
    5. Carbon Monoxide Poisoning (Myocarditis)
    6. Chronic Stimulant Abuse
      1. Cocaine and other Catecholamines
      2. Methylphenidate
    7. Substance Abuse
      1. Cocaine
      2. Methamphetamine
      3. Alcoholic Cardiomyopathy
        1. See Alcohol Related Cardiac Effects
    8. AnthracyclineChemotherapy
      1. Doxorubicin (Adriamycin, Doxil)
      2. Daunorubicin (Cerubidine)
      3. Epirubicin (Ellence)
      4. Idarubicin
      5. Mitoxantrone (Novantrone)
      6. Valrubicin
    9. Other Chemotherapeutic agents
      1. Cyclophosphamide
      2. Immune Checkpoint Inhibitors (e.g. Pembrolizumab, Nivolumab)
    10. Heavy Metal Poisoning
      1. Arsenic Poisoning
      2. Cobalt Poisoning
      3. Lead Poisoning
      4. Mercury Poisoning
    11. Other medications
      1. Clozapine and other Antipsychotics (Myocarditis)
  9. Miscellaneous conditions
    1. Arteriovenous Fistula (high output Heart Failure)

V. HIstory

  1. Cardiopulmonary symptoms
  2. Past medical history
  3. Regular Exercise performed (and recent Exercise tolerance)
  4. Family History of cardiac conditions (including Sudden Cardiac Death, known Genetic Syndromes)

VI. Findings

  1. See Congestive Heart Failure

VII. Diagnostics

  1. Electrocardiogram
  2. Echocardiogram
  3. Genetic Counseling and testing indications
    1. Consider when heritable cause is suspected
  4. Cardiac MRI
    1. May be indicated in some Cardiomyopathy cases (typically cardiology driven)

VIII. Management

  1. See Congestive Heart Failure
  2. See causative condition for specific management
  3. Screening indications first degree relatives for Cardiomyopathy (e.g. EKG, Echo, Genetic Testing)
    1. Hypertrophic Cardiomyopathy
    2. Dilated Cardiomyopathy
  4. Exercise restrictions
    1. Based on specific Cardiomyopathy and associated risks of Arrhythmia and Sudden Cardiac Death
    2. Shared Decision Making with patient in light of cardiology assessment

IX. References

  1. (1995) Circulation 92:2764-84 [PubMed]
  2. (1999) J Card Fail 5:357-82 [PubMed]
  3. Brieler (2017) Am Fam Physician 96(10):640-6 [PubMed]
  4. Coppiano (2026) Am Fam Physician 113(2): 166-73 [PubMed]
  5. Hunt (2009) Circulation 119(14):e391-479 [PubMed]
  6. King (2012) Am Fam Physician 85(12): 1161-8 [PubMed]
  7. Luk (2009) J Clin Pathol 62(3): 219-25 [PubMed]
  8. Maron (2006) Circulation 113(14): 1807-16 [PubMed]
  9. Senni (1997) Mayo Clin Proc 72:453-60 [PubMed]
  10. Wexler (2009) Am Fam Physician 79(9): 778-84 [PubMed]

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