Cardiomyopathy: Difference between revisions

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[[Cardiomyopathy medical therapy|Medical Therapy]] | [[Cardiomyopathy surgery|Surgery]] | [[Cardiomyopathy primary prevention|Primary Prevention]] | [[Cardiomyopathy secondary prevention|Secondary Prevention]] | [[Cardiomyopathy cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Cardiomyopathy future or investigational therapies|Future or Investigational Therapies]]
[[Cardiomyopathy medical therapy|Medical Therapy]] | [[Cardiomyopathy surgery|Surgery]] | [[Cardiomyopathy primary prevention|Primary Prevention]] | [[Cardiomyopathy secondary prevention|Secondary Prevention]] | [[Cardiomyopathy cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Cardiomyopathy future or investigational therapies|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 Studies ==

Revision as of 18:43, 15 April 2019


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Cardiomyopathy
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

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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.

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