Contents

Search

cardiomyopathy

Primary heart muscle disease, often of obscure or unknown etiology, not associated with coronary disease, hypertension, valve disease, etc. Classification: 1) dilated cardiomyopathy (D) a) left &/or right ventricular enlargement b) ventricular systolic dysfunction c) congestive heart failure d) arrhythmias e) emboli f) ischemic cardiomyopathy 2) restrictive cardiomyopathy (R) a) endomyocardial scarring or myocardial infiltration b) restriction to left &/or right ventricular filling 3) hypertrophic cardiomyopathy (H) a) disporportional left ventricular hypertrophy b) generally involves septum more than free wall c) with or without intraventricular systolic pressure gradient d) left ventricular cavity generally non dilated Etiology: 1) primary - idiopathic (D, R, H) - familial (D, H) - eosinophilic endomyocardial disease (R) - endomyocardial fibrosis (R) 2) 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 - hyperthyroidism - myocardial ischemia - familial storage disease (D,R) - glycogen storage disease - mucopolysaccharidosis - deficiency (D) - electrolytes - nutritional (thiamine) - connective tissue disorder (D) - systemic lupus erythematosus (SLE) - polyarteritis nodosa - rheumatoid arthritis - progressive systemic sclerosis - dermatomyositis - infiltrations & granulomas (R, D) - amyloidosis - sarcoidosis - malignancy - hemochromatosis - neuromuscular (D) - muscular dystrophy - myotonic dystrophy - Friedreich's ataxia (H,D) - Refsum's disease - hypersensitivity & toxic reactions (D) - alcohol - radiation - pharmaceutical agents - adriamycin, daunorubicin, doxorubicin - emetine - lithium - phenothiazines - sulfonamides - sympathomimetics - peripartum heart disease (D) - endocardial fibroelastosis (R) * D = dilated cardiomyopathy R = restricted cardiomyopathy H = hypertrophic cardiomyopathy Special laboratory: 1) electrocardiogram a) dilated: ST segment & T wave abnormalities b) restrictive: low voltage, conduction defects c) hypertrophic: ST segment & T wave abnormalities, LVH, Q waves 2) echocardiogram a) dilated: LV dilatation & systolic dysfunction b) restrictive: 1] increased LV wall thickness 2] normal or mildly reduced LV systolic function c) hypertrophic: 1] asymmetric ventricular septal hypertrophy 2] systolic anterior motion of mitral valve 3) cardiac catherization a) dilated: 1] LV dilatation & dysfunction 2] elevated left & often right-sided filling pressures 3] diminished cardiac output b) restrictive: 1] normal or mildly reduced systolic function 2] elevated left & right-sided filling pressures c) hypertrophic: 1] vigorous systolic function 2] dynamic left ventricular outflow obstruction 3] elevated left & right-sided filling pressures Laboratory: - TSH in serum - iron studies* * no need in premenopausal women with normal CBC Radiology: 1) chest X-ray -> cardiac enlargement 1] greatest with dilated cardiomyopathy 2] least with restictive cardiomyopathy 2) radionucliide studies a) evaluate systolic function b) asymmetric interventricular septal hypertrophy Complications: - heart failure Management: - treat heart failure - treat underlying disorder - observation for asymptomatic non-sustained ventricular tachycardia - patients with non-ischemic cardiomyopathy [10] - ICD improves survival in ischemic & non-ischemic cardiomyopathy [8]

Interactions

disease interactions

Specific

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

General

heart disease (cardiac disease)

References

  1. Harrison's Principles of Internal Medicine, 13th ed. Companion Handbook, Isselbacher et al (eds), McGraw-Hill Inc. NY, 1995, pg 829-39
  2. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 252-255
  3. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1089
  4. Seward JB, Casaclang-Verzosa G. Infiltrative cardiovascular diseases: cardiomyopathies that look alike. J Am Coll Cardiol. 2010 Apr 27;55(17):1769-79. PMID: 20413025
  5. Medical Knowledge Self Assessment Program (MKSAP) 16, 18 American College of Physicians, Philadelphia 2012, 2018.
  6. Watkins H, Ashrafian H, Redwood C. Inherited cardiomyopathies. N Engl J Med. 2011 Apr 28;364(17):1643-56. PMID: 21524215
  7. 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: 20375725
  8. 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. PMID: 28632280 http://annals.org/aim/article/2633844/implantable-cardioverter-defibrillators-primary-prevention-patients-ischemic-nonischemic-cardiomyopathy-systematic
  9. ARUP Consult: Cardiomyopathy and Arrhythmia Panel, Sequencing and Deletion/Duplication. https://arupconsult.com/ati/Cardiomyopathy-Arrhythmia-Panel
  10. NEJM Knowledge+