cardiomyopathy
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Introduction
Primary heart muscle disease, often of obscure or unknown etiology, not associated with coronary disease, hypertension, valve disease, etc.
Classification
- dilated cardiomyopathy (D)
- left &/or right ventricular enlargement
- ventricular systolic dysfunction
- congestive heart failure
- arrhythmias
- emboli
- ischemic cardiomyopathy
- restrictive cardiomyopathy (R)
- endomyocardial scarring or myocardial infiltration
- restriction to left &/or right ventricular filling
- hypertrophic cardiomyopathy (H)
- disporportional left ventricular hypertrophy
- generally involves septum more than free wall
- with or without intraventricular systolic pressure gradient
- left ventricular cavity generally non dilated
Etiology
- primary
- idiopathic (D, R, H)
- familial (D, H)
- eosinophilic endomyocardial disease (R)
- endomyocardial fibrosis (R)
- secondary
- infective (D)
- viral myocarditis
- bacterial myocarditis
- fungal myocarditis
- protozoal myocarditis
- metazoal myocarditis
- spirochete-induced myocarditis
- rickettsial-induced myocarditis
- metabolic (D) - LV may not be dilated
- familial storage disease (D,R)
- deficiency (D)
- electrolytes
- nutritional (thiamine)
- connective tissue disorder (D)
- infiltrations & granulomas (R, D)
- neuromuscular (D)
- hypersensitivity & toxic reactions (D)
- peripartum heart disease (D)
- endocardial fibroelastosis (R)
- infective (D)
* D = dilated cardiomyopathy R = restricted cardiomyopathy H = hypertrophic cardiomyopathy
Laboratory
* no need in premenopausal women with normal CBC
Diagnostic procedures
- electrocardiogram
- dilated: ST segment & T wave abnormalities
- restrictive: low voltage, conduction defects
- hypertrophic: ST segment & T wave abnormalities, LVH, Q waves
- echocardiogram
- dilated: LV dilatation & systolic dysfunction
- restrictive:
- increased LV wall thickness
- normal or mildly reduced LV systolic function
- hypertrophic:
- asymmetric ventricular septal hypertrophy
- systolic anterior motion of mitral valve
- cardiac catherization
- dilated:
- LV dilatation & dysfunction
- elevated left & often right-sided filling pressures
- diminished cardiac output
- restrictive:
- hypertrophic:
- vigorous systolic function
- dynamic left ventricular outflow obstruction
- elevated left & right-sided filling pressures
- dilated:
Radiology
- chest X-ray
- cardiac enlargement
- greatest with dilated cardiomyopathy
- least with restictive cardiomyopathy
- cardiac enlargement
- radionucliide studies
- evaluate systolic function
- asymmetric interventricular septal hypertrophy
Complications
Management
- treat heart failure
- treat underlying disorder
- observation for asymptomatic non-sustained ventricular tachycardia
- patients with non-ischemic cardiomyopathy[10]
- observation for asymptomatic non-sustained ventricular tachycardia
- ICD improves survival in ischemic & non-ischemic cardiomyopathy[8]
More general terms
More specific terms
- dilated cardiomyopathy
- endocardial fibroelastosis
- histiocytoid cardiomyopathy
- hypertrophic cardiomyopathy (HCM), including idiopathic hypertrophic subaortic stenosis (IHSS)
- myocardial fibrosis
- peripartum cardiomyopathy
- rate-related cardiomyopathy
- restrictive cardiomyopathy
- stress cardiomyopathy; Takotsubo cardiomyopathy; broken heart syndrome; transient apical ballooning syndrome; stress-induced cardiomyopathy; Gebrochenes-Herz-Syndrome; myocardial infarction with nonobstructive coronary arteries; MINOCA
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Companion Handbook, Isselbacher et al (eds), McGraw-Hill Inc. NY, 1995, pg 829-39
- ↑ Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 252-255
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1089
- ↑ Seward JB, Casaclang-Verzosa G. Infiltrative cardiovascular diseases: cardiomyopathies that look alike. J Am Coll Cardiol. 2010 Apr 27;55(17):1769-79. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20413025
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 16, 18 American College of Physicians, Philadelphia 2012, 2018.
- ↑ Watkins H, Ashrafian H, Redwood C. Inherited cardiomyopathies. N Engl J Med. 2011 Apr 28;364(17):1643-56. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21524215
- ↑ Geiger S, Lange V, Suhl P, Heinemann V, Stemmler HJ. Anticancer therapy induced cardiotoxicity: review of the literature. Anticancer Drugs. 2010 Jul;21(6):578-90 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20375725
- ↑ 8.0 8.1 Kolodziejczak M, Andreotti F, Kowalewski M Implantable Cardioverter-Defibrillators for Primary Prevention in Patients With Ischemic or Nonischemic Cardiomyopathy: A Systematic Review and Meta-analysis. Ann Intern Med. June 27, 2017. <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28632280 <Internet> http://annals.org/aim/article/2633844/implantable-cardioverter-defibrillators-primary-prevention-patients-ischemic-nonischemic-cardiomyopathy-systematic
- ↑ ARUP Consult: Cardiomyopathy and Arrhythmia Panel, Sequencing and Deletion/Duplication. https://arupconsult.com/ati/Cardiomyopathy-Arrhythmia-Panel
- ↑ 10.0 10.1 NEJM Knowledge+