Dilated Cardiomyopathy

Systolic dysfunction HFrEF
1. Low LVEDV leads to High LVEDP leads to Dilitation of annulus of AV valve leads to Papillary dysfunction leaads to Valve incompetency leads to Atrial enlargement leads to Pulmonary congestion
  1. Heart
    1. * Tachy: due to lack of inotropy due to lots of catecholomines, * Enlarged apical impulse, laterally displaced, * Right Ventricular impulse along LSB, *S3, S4 * systolic murmur: regur murmurs because the heart is gigantic
  2. LUNGS
    1. Tachypnea, Clear, crackles, rales
Sx: dyspnea on exertion, orthopnea, PND, palpation
LABS: persistent elevated torponin T is indicative of poor prognosis
1. EKG
  1. Sinus tachy, Afib, Q waves, pseudo infarction due to fibrosis, ST-T waves abnormalities, QRSwidening, LVH, LBBB
2. CXR
  1. Cardiomegaly
    1. Pulmonary congestion
      1. pleural effusion
3. ECHO
  1. Hypokinesis
    1. Wall motion abnormalities
  2. Exercise ECHO
    1. Determines patient's functional status and whether arrhythmias may develop with exercise
4. Catheraterization
  1. identifies concurrent ischemic heart disease
Treatment
1. Goals
  1. Decrease cardiac workload
    1. Limit physical activity if decompensated. Cardiac rehab to increase exercise tolerance if able.
    2. Diet
      1. Na+ restriction. Increase calories v cachexia. Vitamins. No ETOH
  2. Improve sx
    1. Treat CHF
      1. Diuretics, ACEi/ARB, aldosterone antagonists, Hydralazine-nitrites, Digitalis, Antiarrhythmics
  3. Treat underlying dz
2. Surgical management
  1. Cardiac transplant
    1. 50% of transplants are for DCM
  2. ICD
  3. Assist Devices
CHF is the cause of death in 70% patients
20-50% annual mortality
Causes
1. Idiopathic 50%
  1. FAMILIAL
    1. All detectable causes have been excluded except genetic causes
    2. Autosomal Dominant
    3. Dx by screening family members
      1. 3-4 generation family Hx, clinical screening of first degree family members, Molecular genetic testing if indicated
        20-30% of relatives are found to have matching gene
        Unexplained HF < 60 yrs
        Sudden cardiac death in the absence of known ischemic heart sx
      2. Dx is made when 2 or more family members are also dx with IDCM
      3. HX, EKF, ECHO
      4. CK-MM
        First visit only to rule out muscular dystrophy
      5. Re-screen
        Every 3-5 yrs
        if asx of sx occur
        every year
        if abnormal screen suggestive or consistent with DCM
    4. pts present with IDCM
      1. LVEF</= 50%
        Evidence of dilation and imparied systolic function of one or both ventricles
    5. TX
      1. ICD in a LMNA + family member or family hx of sudden death
      2. Pre-sx medical tx with ACE II or Beta blocker will prolly not change course of genetic dz
2. Myocarditis 9%
3. Ischemic heart disease 7%

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Dilated Cardiomyopathy

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	Created by Kelly Ruiz about 9 years ago