Hypertrophic cardiomyopathy

Background

• Genetically-linked (AD) hypertrophy of cardiac muscle - can but does not always cause outflow obstruction
• "HOCM"
• Abnormal LV diastolic function due to decr compliance

Diagnosis

• Sx: Syncope or sudden death most common
  • Also exertional dyspnea, chest pain, syncope, dizzyness, palpitations, or CHF
• Systolic murmur that increases w/ valsalva
• 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

Differential Diagnosis

Cardiomyopathy

• Dilated cardiomyopathy
• Hypertrophic cardiomyopathy
• Restrictive cardiomyopathy
• Peripartum cardiomyopathy
• Takotsubo cardiomyopathy
• Arrhythmogenic right ventricular dysplasia

Treatment

Definitive = Myomectomy

Decompensated

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