Cardiomyopathy

Last updated: January 26, 2023

Summarytoggle arrow icon

Cardiomyopathies are diseases of the muscle tissue of the heart. Types of cardiomyopathies include dilated (most common), hypertrophic, restrictive, and arrhythmogenic right ventricular cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) primarily affects the right ventricle and is characterized by fibrofatty replacement of myocardium, which causes myocardial thinning and subsequent ventricular dilation. Although the hallmark finding is arrhythmia, symptoms are highly variable. Because management depends greatly on individual factors, such as the extent of the disease, there is no single best course of treatment. All patients should avoid strenuous exercise.

Arrhythmia-induced cardiomyopathy is a very rare type of cardiomyopathy. It is caused by long-standing arrhythmia and typically affects the left ventricle. Features include palpitations, syncope, and signs of arrhythmia on ECG. Progression to left heart failure is possible in severe cases. Treatment involves antiarrhythmics such as beta blockers for rhythmic control.

Dilated cardiomyopathy”, “Hypertrophic cardiomyopathy,” “Restrictive cardiomyopathy,” and “Peripartum cardiomyopathy” are described in their respective articles in more detail.

Differential diagnosis of major cardiomyopathies
Types Dilated cardiomyopathy

Hypertrophic cardiomyopathy

Restrictive cardiomyopathy
Etiology

Pathophysiology

Distinctive clinical features
Echocardiography LV cavity size
  • Significantly increased
  • Decreased
  • Decreased
EF
  • Significantly decreased
  • Normal
  • Normal or increased
Wall thickness
  • Normal or decreased
  • Significantly increased
  • Usually increased
Additional findings
Other characteristics
  • Most common cardiomyopathy
  • Second most common cardiomyopathy
  • Most common cause of sudden heart failure in athletes and teenagers

Epidemiology

  • Most common in young adults (mean age at diagnosis: ∼ 30 years) [2]
  • Prevalence: 1:1,000–2,000 [3]

Etiology

Pathophysiology

Clinical features

Diagnostics [2][3][5]

Approach

ARVC is diagnosed based on the AHA criteria which include the following features:

  1. Dysfunction and structural abnormalities of RV (can be revealed by echocardiography, MRI, or RV angiography)
  2. Histological characteristics (require myocardial biopsy)
  3. Abnormal repolarization (diagnosed with ECG)
  4. Depolarization/conduction abnormalities (diagnosed with ECG)
  5. Arrhythmias (diagnosed with ECG)
  6. Family history (confirmation of ARVC in a relative either by criteria, pathological examination in surgery or autopsy, or by genetic testing)

Findings

Management [5]

Left ventricular noncompaction [7]

Arrhythmia-induced cardiomyopathy [8][9]

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  2. William J McKenna, MD. Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/arrhythmogenic-right-ventricular-cardiomyopathy-pathogenesis-and-genetics.Last updated: June 6, 2019. Accessed: July 5, 2019.
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