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
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...