 R40: 30 yo man found unconscious, on ambo arrival VT. ROSC after single DC shock  In ED conscious, mildly intoxicated  Normal bloods, CXR, alcohol 44  12 Lead ECG:

 Admitted under medicine, monitored  Several codes for non sustained VT’s

 Assymetrical septal hypertrophy  1% of all cardiology FUP clinics  Most common genetic cardiac disease  Prevalence in adults 0.2%  Prim myocardial abnormality w sarcomeric disarray and assym LV hypertrophy

 50% AD- > 450 mutations  Acquired ( athletes, on/off exercise)  Unknown  ???? environmental

Dynamic LVOTO, ant motion of MV  septum ( SAM- systolic anterior motion) hypertrophied septum - subaortic obstruction  Pressure overload of LV, diastolic Dysfunction  MR  Arrhythmia  MI  Sudden cardiac death

 Widely variable  Subaortic obstr, dynamic,  contractility and loading, location  LVOTO assoc. with incr. wall stress, fibrosis  VT/VF  SAM worse with inc. contractility, reduced pre or afterload  MI - ? Small CA, partially obliterated by hypertrophy/ too much muscle for small vessells

 Athletes  Concentric, regresses w deconditioning  Wall thickness <15 mm  LA <40mm  LVEDD >45mm  2% of elite athletes  HCM ? Asymmetric  >15mm  LA<45mm  LVEDD < 45mm  Abn diast function

 Dyspnoea on exertion (90%)  CCF- orthopnea, PND  Angina ( 70-80%)  Syncope (20%)  Palpitations  Sudden cardiac death

 Diagnostic features usually present by 21 years  < 12 years: morphological features unlikely

 Evidence of CCF  Jerky pulse  Paradoxically split S2 ( if high LVOT gradient)  Prominent A wave of JVP (red. RV compliance)

 ESM betw. apex and sternum  SS notch, NOT carotid, quiet-squatting, loud with less pre/after load  MR  Holosystolic murmur at apex+ axilla  AR  diastolic decrescendo murmur

 LVH, LAD, deep ant/lat TWI,  Dagger like Q waves inf/lat  CXR- left atrial enlargement ( or normal)  ECHO- septal hypertrophy, SAM, early aortic closure  Cardiac catheter( DD CAD, severe MR

 Pharmacology B blockers. –ve inotrope, low HR, low O2 demand, longer diastolic filing, less exercise intolerance and dyspnoea  disopyramide  -ve inotrope

 Surgical- indic: subaortic gradient >50mmHg  Septal myotomy/ myectomy  Complication: 2% perforation or CHB, 3% mortality  Non surgical ablation (10% CHB)  ICD- Arrhythmia ( SCD, FH of, VT/VF, age <30)  transplant

 31 y/o man BIB ambulance, celebrated his 31 st birthday, fell under influence of alcohol. Ankle fracture dislocation with compromise of foot circulation  History: previous VT arrest because of HCM. Missed appointment for ICD insertion….

 Avoid worsening of subaortic gradient, tachycardia, vasodilatation or inotropes  Aim to increase pre/after load, reduce contractility

 Volume  Induction: narcotics, propofol  Avoid tachycardia, Inotropes, Calcium, B agonist