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Cardiomyopathy, Idiopathic

  • • 2 most common causes: dilated (idiopathic) and ischemic

    • Idiopathic

    • -Unknown etiology

      -Often multifactorial resulting in large, dilated heart with poor ventricular function

    • Ischemic

Epidemiology

  • • 60% of patients with NYHA Class IV disease will die within 1 year

    • Medical therapy for cardiomyopathy carries dismal prognosis

Symptoms and Signs

  • • Heart failure symptoms

Laboratory Findings

  • • ECG: Q waves, widened QRS, conduction defects

Imaging Findings

  • • Chest film: Pulmonary congestion, enlarged heart

  • • Echocardiography

    • Catheterization

Rule Out

  • • Other causes of heart failure

  • • Physical exam

    • ECG

    • Echocardiography

  • • Idiopathic

    • -Cardiac transplantation may have best long-term results

      -Mitral valve annuloplasty if enlarged annular size from LV enlargement

      -Batista procedure: ventricular reduction; limited use

      -Biventricular pacing: Restores normal depolarization to ventricles, increasing ejection fraction significantly

    • Ischemic

    • -Consider coronary artery bypass grafting (CABG) if suitable target vessels and evidence of "hibernating" mycoardium

Surgery

Indications

  • • CABG: If suitable target vessels, hibernating myocardium on PET scan or stress thallium

    • Idiopathic: Cardiac transplantation in suitable candidates

Prognosis

  • • Batista procedure: Limited survival, high mortality

    • Medical therapy carries dismal prognosis

    • See Cardiomyopathy, Ischemic.

References

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Braile DM et al. Dynamic cardiomyoplasty: long-term clinical results in patients with dilated cardiomyopathy. Ann Thorac Surg. 2000;69:1445.  [PubMed: 10881820]
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Bardy GH et al: Amiodarone or an implantable cardioverter-defibrillator for congestive heart failure. N Engl J Med 2005;352:225.  [PubMed: 15659722]
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Cleland JG et al: The effect of cardiac resynchronization on morbidity and mortality in heart failure. N Engl J Med 2005;352:1539.  [PubMed: 15753115]
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Dargie HJ: Effect of carvedilol on outcome after myocardial infarction in patients with left-ventricular dysfunction: The CAPRICORN randomised trial. Lancet 2001;357:1385.  [PubMed: 11356434]
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Hunt SA et al: ACC/AHA 2005 guideline update for the diagnosis and management of chronic heart failure in the adult—summary article. Circulation 2005;112:1825.

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