HOCM 2024

Question 1

Management of HOCM

Answer 1

1. Avoid hypovolémia
2. Avoid Valsalva maneuver
3. 1st line treatment : BB or BCC-non-DHP
4. 2nd line drug treatment : Disopyramide or a myosin inhibitor (Mavacamten)
5. 2nd line non-drug treatment : Alcohol septal ablation or surgical myectomy

Question 2

Gestion mavacamten

Answer 2

Mecanisme : Myosin inhibitor
- decrease the excess availability of myosin heads to form cross bridges with actin molecules, thereby reducing the excessive force of contraction and imparired relaxation that are hallmarks of HCM

Do not use if LVEF < 55%
Interrupt treatment if LVEF < 50%

Question 3

How to provoke gradient

Answer 3

Valsalva
Standing
Exercise
Amyl nitrite
postprandial exercise cho
pharmacological stress testing
CT

Question 4

Effets secondaires dis-pyramide

Answer 4

Anticholinergic effets :
Dry eyes
Xerostomie
Constripation
Urinary retention
QT prolongation

Question 5

Explorer HCM

Answer 5

Explorer HCM
- Mavacamten 2.5-15mg DIE

Inclusion
- HCM
- NYHA 2-3
- LVOTO > 50 mmHg (rest, valvalva or exercise)
- LVEF > 55%

Outcom
- increase pVOx by 3 ml/kg/min
- increase pVO2 by 1.5 and improvement in NYHA classification by at least 1

Question 6

Valor - HCM

Answer 6

Valor HCM :

Inclusion
- HOCM referred for SRT

Question 7

ICD HOCM

Answer 7

• WT > 3 cm
• Anevrysme VG
• Dysfunction VG < 50%
• TVNS
• Hx familiale mort subite < 50 ans
• Syncope suspecte < 6 mois
• Fibrose > 15% VG à iRM

Question 8

Alcohol septal ablation vs surgical myectomy

Answer 8

Alcohol septal ablation
- Older with comorbidities
- Focal basal septal hypertrophy
- Patient preference
- BBD préexistante car ablation à l’alcool causera BBD

Surgical myectomy
- Young + low surgical risk
- Marked diffuse septal hypertrophy +/- mid ventricular
- Need for other cardiac surgery (including intrinsic mitral disease)
- Pre-excisting LBBB because surgical myectomie will cause LBBB

Question 9

Risk factors of developing HCM with systolic dysfunction