Cardiomyopathy

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Cardiomyopathy
Idiopathic cardiomyopathy, gross pathology 20G0018 lores.jpg
Opened left ventricle of heart shows a thickened, dilated left ventricle with subendocardial fibrosis manifested as increased whiteness of endocardium {Autopsy findings}.
Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Cardiomyopathy Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Cardiomyopathy from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

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CT

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Echocardiography

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Medical Therapy

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Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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Risk calculators and risk factors for Cardiomyopathy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2], Raviteja Guddeti, M.B.B.S. [3]

Synonyms and Keywords: Myocardiopathy; cardiac muscle disease; heart muscle disease.

Overview

Historical Perspective

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

  • The 2009 Heart Failure Society of America (HFSA) guidelines note that the finding of a specific mutation does not generally govern therapy, although certain clinical characteristics associated with some genes may influence screening, education, and counseling of family members, and the threshold for primary prevention (eg, implantable cardioverter-defibrillator use) or presymptomatic therapy (eg, beta-blocker or angiotensin converting enzyme inhibitor administration).
  • Medical therapy is recommended based on cardiac phenotype as outlined in the general guidelines. Early initiation of therapy (eg, with angiotensin converting enzyme inhibitors) has been proposed to slow progression in patients with LV enlargement, even in the absence of a reduced LVEF [1]. However, the efficacy of treating asymptomatic LV dysfunction has only been demonstrated in patients with a LVEF ≤35 to 40 percent.
  • Device therapies for conduction system disease and arrhythmia (pacemaker therapy and implantable cardioverter-defibrillator) are recommended based on cardiac phenotype as recommended in major society guidelines.
  • In addition, in patients with dilated cardiomyopathy (DCM) and significant arrhythmia or known risk of arrhythmia, an implantable cardioverter-defibrillator may be considered before the LV ejection fraction (LVEF) falls to ≤35 percent (the usual LVEF threshold for prophylactic implantable cardioverter-defibrillator placement). Specifically, an implantable cardioverter-defibrillator may be considered in patients with DCM with EF >35 percent with family history of sudden cardiac death OR with LMNA mutation (associated with high risk of sudden death).
  • When a new diagnosis of idiopathic DCM is made, a three to four generation family history and clinical screening of first-degree relatives is indicated. Clinical screening (history, exam, electrocardiogram, and echocardiogram) has been recommended for first-degree relatives to identify asymptomatic or undetected disease.
  • Genetic testing with appropriate genetic counseling is indicated for known familial DCM to facilitate risk assessment of family members. Genetic testing for nonfamilial but a well-established DCM diagnosis without other obvious cause is reasonable in many cases.
  • The finding of a specific mutation does not generally govern therapy, although certain clinical characteristics associated with some genes may influence screening, education, and counseling of family members, and the threshold for primary prevention (eg, implantable cardiac defibrillator use) or presymptomatic therapy (eg, beta-blocker or angiotensin converting enzyme inhibitor administration).

Case Studies

Case #1

de:Kardiomyopathie nl:Cardiomyopathie no:Kardiomyopati simple:Cardiomyopathy sr:Кардиомиопатија sv:Hjärtmuskelsjukdom


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