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Cardiomyopathy

Cardiomyopathy is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • Cardiomyopathy encompasses a large group of diseases of the myocardium that commonly result in mechanical pump dysfunction. The current classification scheme attempts to differentiate between myocardial diseases confined to the myocardium (primary) and those due to systemic disorders (secondary). Specific causes of myocardial dysfunction due to other cardiovascular disorders are considered a third, separate category (1).
  • Classification of cardiomyopathy
    • Primary
      • Genetic
        • Hypertrophic cardiomyopathy (HCM)
        • Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D)
        • Left ventricular (LV) noncompaction (LVNC)
        • Glycogen storage (Danon type, PRKAG2)
        • Conduction defects
        • Mitochondrial myopathies
        • Ion channel disorders: long QT syndrome (LQTS), Brugada syndrome, short QT syndrome, catecholaminergic polymorphic ventricular tachycardia (CPVT), Asian SUNDS
      • Mixed
        • Dilated cardiomyopathy (DCM) (genetic or nongenetic)
        • Restrictive
      • Acquired
        • Myocarditis, stress cardiomyopathy, peripartum, tachycardia-induced, infants of type 1 diabetic mothers
    • Secondary (see list below)
      • Specific
        • Ischemic
        • Valvular
        • Hypertensive
        • Congenital heart disease
  • Patients with end-stage cardiomyopathy have stage D heart failure or severe symptoms at rest refractory to standard medical therapy.
  • System(s) affected: cardiovascular; renal

Pediatric Considerations
Etiology: idiopathic, viral, congenital heart disease, and familial

Pregnancy Considerations
May occur in women postpartum

Epidemiology

Predominant age: Ischemic cardiomyopathy is the most common etiology; predominantly in patients aged >50 years. Consider uncommon causes in young.


Incidence
  • 60,000 patients <65 years old die each year from end-stage heart disease.
  • 35,000 to 70,000 people might benefit from cardiac transplant or chronic support.

Prevalence
Most rapidly growing form of heart disease

Etiology and Pathophysiology

  • Ischemic heart disease: most common etiology; up to 66% of patients
  • Hypertension
  • Valvular heart disease
  • Primary genetic causes
  • Congenital heart disease
  • Peripartum/postpartum
  • Endocrine
    • Diabetes mellitus
    • Hyperthyroidism
    • Hypothyroidism
    • Hyperparathyroidism
    • Pheochromocytoma
    • Acromegaly
  • Nutritional deficiencies
    • Beriberi, pellagra, scurvy, selenium, carnitine, kwashiorkor
  • Autoimmune/collagen
    • Systemic lupus erythematosus
    • Dermatomyositis
    • Rheumatoid arthritis
    • Scleroderma
    • Polyarteritis nodosa
  • Infectious causes
    • Viral (e.g., HIV, coxsackievirus, adenovirus)
    • Bacterial and mycobacterial (e.g., diphtheria, rheumatic fever)
    • Parasitic (e.g., toxoplasmosis, Trypanosoma cruzi)
  • Infiltrative (2)
    • Amyloidosis
    • Gaucher disease
    • Hurler disease
    • Hunter disease
    • Fabry disease
  • Storage
    • Hemochromatosis
    • Fabry disease
    • Glycogen storage disease (type II, Pompe)
    • Niemann-Pick disease
  • Neuromuscular/neurologic
    • Duchenne and Emery-Dreifuss muscular dystrophies
    • Friedreich ataxia
    • Myotonic dystrophy
    • Neurofibromatosis
    • Tuberous sclerosis
  • Toxic
    • Alcohol
    • Drugs and chemotherapy: anthracyclines, cyclophosphamide, Herceptin
    • Radiation
    • Heavy metal, chemical agents
  • Inflammatory (granulomatous):
    • Sarcoidosis
  • Idiopathic
  • Endomyocardial
    • Endomyocardial fibrosis
    • Hypereosinophilic syndrome (Loeffler endocarditis)

Genetics
Autosomal dominant HCM is the most common form of primary genetic cardiomyopathy (1/500 in the general population). Genetic causes of DCM are less common, accounting for 1/3 cases, with mostly autosomal dominant inheritance. LVNC and ARVC are also inherited in an autosomal dominant fashion in addition to LQTS and other ion-channel disorders.

Risk Factors

  • Hypertension
  • Hyperlipidemia
  • Obesity
  • Coronary artery disease
  • Diabetes mellitus
  • Smoking
  • Physical inactivity
  • Excessive alcohol intake
  • Dietary sodium
  • Obstructive sleep apnea
  • Chemotherapy

General Prevention

Reduce salt and water intake; home BP and daily weight measurement

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Citation

Stephens, Mark B., et al., editors. "Cardiomyopathy." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816714/all/Cardiomyopathy.
Cardiomyopathy. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816714/all/Cardiomyopathy. Accessed April 24, 2019.
Cardiomyopathy. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816714/all/Cardiomyopathy
Cardiomyopathy [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 24]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816714/all/Cardiomyopathy.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Cardiomyopathy ID - 816714 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816714/all/Cardiomyopathy PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -