Dilated Cardiomyopathy

1
Dilated Cardiomyopathy

• Clinical manifestations fatigue, dyspnea on
  exertion, edema, orthopnea
• Also sudden cardiac death, systemic embolism
• In the majority of cases, no identifiable cause
  is found (IDCM)
• 25 of patients have evidence for familial
  disease predominately autosomal dominant
  inheritance
• Screen family members

2
Diagnosis

• EKG can be normal. May show T wave
  abnormalities, conduction delays, or BBB. Sinus
  tach, SVT, nonsustained VT, afib are common.
• Echo confirms Dx.
• If poor windows obtained or suspicion for
  infiltrative disease is high, can obtain cardiac
  MRI.

3
Diagnosis

• Metabolic stress testing exercise testing
  combined with respiratory gas requirement. Use
  to diagnose primary metabolic abnormalities such
  as mitochondrial disease, or as transplant w/u.
• Viral serologies not very useful, because the
  most commonly implicated enteroviruses (coxsackie
  B and echovirus) are ubiquitous
• Endomyocardial biopsy useful only in guiding
  immunomodulatory treatment.

4
Rare Causes of Dilated Cardiomyopathy Young

• Myocarditis (viral, toxic, immune)
• Carnitine, Selenium, or calcium deficiency
• Anomalous coronary arteries
• AV malformations
• Kawasaki disease
• Endocardial fibroelastosis
• Non-compacted myocardium
• Familial IDCM
• Barth syndrome

5
Rare Causes of Dilated Cardiomyopathy Adult

• Familial IDCM, X linked ICDM
• Alcohol
• Myocarditis
• Tachycardiomyopathy
• Mitochondrial
• RV dysplasia
• Eosinophilic (Churg-Strauss)
• Drugs (chemo, etc)
• Peripartum
• Endocrine GH excess, hyper or hypothyroidism
• Infiltrative sarcoid, hemochromatosis
• Nutritional thiamine, carnitine, calcium, or
  phosphate deficiencies

6
Treatment

• Oral heart failure regimen ACEI, Beta blockers,
  spironolactone
• Anticoagulants if EF
• Antiarrhythmics two large scale trials of
  amiodorone in ICDM no benefit
• ICD a pt who has had bouts of documented
  sustained VT/VF or syncope with reduced EF.
• Biventricular pacing reduces ventricular
  dysynchrony, can undo remodeling
• LVAD, Cardiac Transplantation

7
Treatment

• Immunomodulation preliminary data to suggest
  that this may be of benefit in certain disease
  states
• acute lymphocytic myocarditis
• peripartum cardiomyopathy.
• IVIG, steroids, plasma exchange.