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Common Congenital Heart Lesions - page 93 / 126

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THE CARDIOMYOPATHIES

(from readings on Tuesday, Sept 28th

  • pgs 237-252)

Objectives:

Recognize common clinical presentations, including symptoms, physical findings, electrocardiographic changes and diagnostic imaging abnormalities in patients with primary myocardial disease

General Info on the Cardiomyopathies:

  • Group of heart disorders where major abnormality is with myocardium… often results in symptoms of heart failure

  • Often etiology is unknown

  • Classification into 3 types: (based on anatomic appearance and abnormal phys of the LV)

    • o

      Dilated cardiomyopathies Æ big ventricle and impaired systolic contraction

      • o

        Hypertrophic cardiomyopathies Æ thick ventricle wall and abnormal diastolic relaxation but intact systolic fxn

      • o

        Restrictive cardiomyopathies Æ very stiff myocardium and impaired diastolic relaxation but normal/near normal systolic fxn

Dilated Cardiomyopathy (DCM):

ETIOLOGY

  • Myocyte damage causes ventricular dilation w/ only a little hypertrophy

    • o

      While the majority of the causes for myocyte damage are idiopathic, commonly recognized causes are viral myocarditis, alcohol toxicity and gene mutations

      • o

        DCM from Acute Viral Myocarditis Æ in youngsters, from Coxsackie group B or echo viruses, usually self limiting with full recovery… however some progress to DCM… (Hypothesis that myocardial destruction is immune-mediated, but not sure)

      • o

        Alcoholic cardiomyopathy Æ only in small number of alcoholics, EtOH impairs cellular fxn, important b/c it’s one of the few reversible causes of DCM… stop EtOH and you get dramatic improvement of ventricular fxn

      • o

        Several familial forms of DCM recently been discovered

PATHOLOGY

  • Marked enlargement of all 4 chambers (though sometimes limited to R or L side)

  • Chamber dilatation is out of proportion to hypertrophy

  • Microscopically Æ myocyte degeneration w/ irreg hypertrophy and atrophy of myofibrs…

  • Interstitial and perivascular fibrosis often extensive

  • pic on pg 239 (fig 10.2) of the dilation of both ventricles

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