Tuesday, 16 November 2010

MRCP revision battle 51.6: Hypertrophic cardiomyopathy

Hypertrophic obstructive cardiomyopathy is a condition in which there is left ventricular outflow tract obstruction due to asymmetric septal hypertrophy.

It is inherited in an autosomal dominant fashion but 50% of cases are sporadic.
Presentation may be with:
  • angina
  • syncope
  • shortness of breath
  • sudden death
  • palpitations

Signs include:
  • jerky pulse
  • ESM radiating to axilla
    • increases with valsalva, decreases with squatting
  • large a waves
  • double apical beat
  • ?MR
  • ?split S2

On ECG look for:
  • LVH
  • TWI
  • Q waves
  • possibly AF

Associations include:
  • WPW
  • phaechromocytoma
  • Friedreichs ataxia

Poorer prognosis if:
  • younger
  • family history of sudden death
  • syncope
No correlation between degree of LVOT obstruction and prognosis.

On angio look for a difference between the LV and aortic systolic pressures - in a normal patient they should be the same, in HCOM aortic pressure will be lower than LV pressure.

Management is:
  • avoid 
    • nitrates
    • ACE-i
    • inotropes
    • atropin
  • give beta blockers
  • ?amiodarone
  • ?ICD
  • ?surgery

Annual mortality is 2.5% in adults, 6% in children.

Lets finish on the smelly topic of bacterial vaginosis...