Dilated cardiomyopathy

Last updated: October 3, 2022

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Dilated cardiomyopathy (DCM) is the occurrence of ventricular dilatation and systolic dysfunction despite normal filling pressures, in the absence of coronary artery disease, abnormal loading pressures (e.g., valvular heart disease, hypertension), or congenital heart disease. It is the most common type of cardiomyopathy. Although most cases are idiopathic or inherited, DCM can also be caused by a number of conditions (e.g., endocrine disorders, autoimmune disease) and infections (e.g., Coxsackie B virus, Chagas disease) and by the use of certain substances (e.g., heavy drinking, cocaine). In DCM, decreased left ventricular contractility leads to left heart failure and eventually right heart failure with decreased ventricular output. Isolated dilation and subsequent decrease in right ventricular contractility is rare. Diagnosis is confirmed with echocardiography. Studies to evaluate the underlying etiology should be guided by clinical suspicion and include laboratory studies, genetic testing, advanced cardiac imaging, and, rarely, endomyocardial biopsy. Management involves treatment of the underlying condition and associated complications, e.g., congestive heart failure and arrhythmias. Severe or refractory disease may be managed with device implantation or cardiac transplantation.

Left or biventricular dilatation with structural and/or functional systolic dysfunction in the absence of coronary artery disease, abnormal loading pressures (e.g., valvular heart disease, hypertension), or congenital heart disease [1][2]

  • Incidence: ∼ 6/100,000 per year (most common cardiomyopathy) [3][4]
  • Sex: > (∼ 1.5:1) [5]
  • Age at presentation: most commonly between 30 and 40 years of age, but can occur at any age [2]
  • Ethnicity: more common in individuals of African descent [2]

Epidemiological data refers to the US, unless otherwise specified.

Primary causes

Secondary causes [8]

Volume and pressure overload secondary to conditions such as hypertension and valvular heart disease can cause dilation of the myocardium; however, these are not considered forms of DCM, as filling pressures are abnormal. [1]

To remember some high-yield secondary causes of dilated cardiomyopathy, think ABCCCDD: Alcohol use, Beriberi, Cocaine, Coxsackie B virus, Chagas, Doxorubicin/Daunorubicin

Symptoms [1]

Physical examination [11]

DCM is typically diagnosed during the workup for associated cardiac conditions (e.g., heart failure) or through screening of family members of patients with known or suspected familial DCM. [13]

Approach [8][14]

Echocardiography [14]

  • Indications
    • To confirm the diagnosis of suspected DCM
    • To screen first-degree relatives of patients with familial DCM [1][8][14]
  • Characteristic findings

Offer screening with a physical examination, ECG, and echocardiography (with assessment of left ventricular size and function) to first-degree relatives of patients with familial DCM. [7][14]

Initial diagnostic workup

Laboratory studies [8][14]

Chest x-ray

ECG [8][18]

Holter monitoring

Genetic testing [14]

Refer patients for genetic counseling prior to ordering genetic testing for DCM. [7]

Advanced studies [8][14]

Cardiac MRI

Endomyocardial biopsy

  • Indication: suspected underlying etiology that requires specific management (e.g., amyloidosis) and cannot be confirmed by other diagnostic methods
  • Findings vary based on the underlying etiology.

Overview [8][14]

Management of the underlying etiology [14]

Management of underlying disease in dilated cardiomyopathy [14]
Treatment
Substance use disorder
Endocrine disorders Hyperthyroidism
Hypothyroidism
  • Start levothyroxine replacement.
  • For older patients and those with known or suspected CAD: [14]
    • Start thyroid hormone replacement at 25–50% of the expected required dose.
    • Titrate up as tolerated every 6–8 weeks.
Acromegaly

Infections

Myocarditis
HIV
Chagas disease
Chemotherapy-related cardiomyopathy
  • All patients: Monitor cardiac function before, during, and after treatment with cardiotoxic chemotherapy agents.
  • For patients undergoing anthracycline-based chemotherapy, consider:
    • Selecting less cardiotoxic derivatives (e.g., epirubicin or idarubicin)
    • Using cardioprotective agents (e.g., dexrazoxane)
  • For patients with cardiac damage: Weigh the risks and benefits of continued treatment with cardiotoxic agents.
Peripartum cardiomyopathy
Autoimmune diseases

Avoid immunosuppressants with potential cardiotoxicity (e.g., etanercept, infliximab) in patients with DCM. [14]

Management of complications

Severe or refractory DCM [17][21]

DCM is the leading indication for heart transplantation. [25]

Dilated cardiomyopathy in pregnancy [15]

Planning pregnancy

During pregnancy

Avoid ACEIs, ARBs, and mineralocorticoid receptor antagonists during the preconception period and pregnancy. [15]

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