Cardiomyopathies and Related Conditions

29 Cardiomyopathies and Related Conditions


With Kevin S. Roman


Definition and Classification



image

Fig. 29.1 Classification of cardiomyopathies.


Pathophysiology



  • image Hypertrophic cardiomyopathy

    • • Rare form of cardiomyopathy in children
    • • May be obstructive or nonobstructive depending on the pattern of myocardial hypertrophy
    • • Myocardial hypertrophy develops in the absence of a hemodynamic afterload stress.
    • • Hypertrophy causes decreased ventricular compliance and elevates end-diastolic pressure, resulting in diastolic dysfunction.
    • • Ejection fraction and the rate of ejection are increased.

  • image Dilated cardiomyopathy

    • • Both end-systolic and end-diastolic volumes are increased, with decreased stroke volume and ejection fraction.
    • • Mild to moderate atrioventricular valve regurgitation associated with ventricular dilation
    • • Myocarditis refers to inflammation of the myocardium and may be due to either infectious organisms, most commonly viral, or noninfectious inflammatory conditions, such as systemic lupus erythematous, polyarteritis nodosa, and Kawasaki disease.

  • image Restrictive cardiomyopathy

    • • Poor ventricular compliance with reduced diastolic filling
    • • End-diastolic volume is usually normal or slightly reduced, and the ejection fraction is less than normal.
    • • Loeffler’s disease is due to an immunologic reaction to the eosinophil, resulting in infiltration of the myocardium, thrombi in the ventricular cavities, and characteristic scarring of the posterior mitral valve leaflet.

  • image Arrhythmogenic right ventricular cardiomyopathy

    • • Desmosomal mutations causing detachment of myocytes and untimate; replacement of right ventricular myocardium with fibrous or adipose tissue causing arrhythmia of right ventricular origin
    • • Right ventricular global or regional dysfunction













































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Jan 14, 2016 | Posted by in RESPIRATORY IMAGING | Comments Off on Cardiomyopathies and Related Conditions
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Table 29.1 Examples of Different Diseases that Cause Cardiomyopathies

Familial Nonfamilial
HCM Familial, unknown genetics Obesity

Sarcomeric protein mutations Infants of diabetic mothers

Glycogen storage diseases Athletic training

Lysosomal storage diseases Amyloid (AL/prealbumin)

Disorders of fatty acid metabolism

Carnitine deficiency

Phosphorylase B kinase deficiency

Mitochondrial cytopathies

Syndromic HCM

– Noonan’s syndrome

– LEOPARD syndrome