Hypertrophic cardiomyopathy

Background

Genetically-linked (AD) hypertrophy of cardiac muscle - can but does not always cause outflow obstruction

Diagnosis

1. Sx: Syncope or sudden death most common. Also CP, SOB, dizzyness, palpitations, or CHF
2. PE: if + syst murmur, will increase with valsalva
3. EKG: Nonspecific/normal. Or, high voltage/LVH, deep narrow Q waves in 1, avL, V5, V6 = "daggers of death"

Work-Up

1. EKG
2. CXR
3. ECHO

Treatment

Myomectomy

Decompensated!

Presents as hypotensive CHF

1. Preserve preload
   1. careful hydration
   2. avoid high airway pressures with mechanical ventilation (small TV with high RR)
2. Limit tachycardia
   1. beta blockers
3. Avoid vasodilators (no nitrates)
4. Maintain sinus rythm (i.e. cardiovert A. fib)
5. Increase afterload (hypotenisve only)
   1. phenlephrine

Source

Adapted from ....Rosen, Mattu (lecture)

Background

• "HOCM"
• Abnormal LV diastolic function due to decr compliance

Diagnosis

• Exertional dyspnea, chest pain, syncope
• Systolic murmur that increases w/ valsalva
• ECG
  • LV hypertrophy, deep Qs in 1, avL, V5-6 (daggers of death)

Treatment

• If decompensated presents as hypotensive CHF
  • Preserve preload
    • Careful hydration
    • Avoid high airway pressure if intubate
  • Limit tachycardia
    • Beta blockers
  • Avoid vasodilators (no nitrates)
  • Maintain sinus rythm (i.e. cardiovert A. fib)
  • Increase afterload (hypotensive only)
    • Phenylephrine

See Also

• Cardiomyopathy

Source

Tintinalli