AHA HCM 2020

Question 1

what is 30 day mortality for septal reduction and myomectomy?

Answer 1

1%

Question 2

What is major adverse effect rate for Myomectomy and ETOH reduction?

Answer 2

10%

Question 3

Rate of PPM for CHB for Myomectomy and ETOH reduction?

Answer 3

5% myomectomy

10% ETOH

Question 4

Name 4 phenocopies of HCM?

Answer 4

• Infiltrative (Amyloid)
• Fabry’s disease
• Glycogen Storage disorders
• Freidrich Ataxia

Question 5

How to screen FDRs for HCM and how often?

Answer 5

Adults:
-At time of diagnosis in proband and then every 3-5 years.

Children and Adolescents:

• 1-2y: If Genotype + or early disease in proband
• 2-3y: All others

Question 6

How often to repeat echo in HCM?

Answer 6

Every 1-2 years

Question 7

Who to perform stress echo in?

Answer 7

if resting and echo with provocative manouvre echo is still less than LVOT < 50mmhg

Question 8

What is recommended for imaging during ETOH ablation?

Answer 8

-TEE with US enhancing contrast injection of the candidate’s septal perforators

Question 9

4 reasons for CMR in HCM?

Answer 9

• If echo inconclusive for diagnosis
• Alternative causes (Fabrys, Amyloid, Athletes heart)
• Scar burden if no other reason for ICD
• To plan for selection of SRT

Question 10

How extensive should family history be on initial assessment?

Answer 10

3 generations

Question 11

Class 1 indication for HCM?

Answer 11

SCD/VT/VF

Question 12

5 Class 2a recommendations for ICD?

Answer 12

• FH SCD
• Massive LVH (> 30)
• Unexplained Syncope
• Apical Aneurysm
• EF < 50%

Question 13

2 Class 2b for ICD?

Answer 13

NSVT

LGE on CMR

Question 14

Indications for septal reduction therapy (3 things)

Answer 14

Severely symptomatic despite OMT at experienced centers

Question 15

When is myomectomy preferred over ETOH ablation?

Answer 15

Associated disease needing surgery

Question 16

How long to have SCAF to anticoagulation?

Answer 16

> 24 hours duration

Question 17

3 indications for Cath in HCM?

Answer 17

• Prior to SRT
• Symptoms of angina/evidence of ischemia
• To quantify LVOT gradient when uncertainty in degree of obstruction

Question 18

What to do if genotype positive identified in HCM case? What if not identified?

Answer 18

+ Test FDRs for that gene

• Clinical surveillance in FDRs (1-2 years in children, 3-5 years in adults)

Question 19

After beta blockers/CCB’s, what are 4 AADs that can be used in HCM?

Answer 19

• Amiodarone
• Sotalol
• Dofetilide
• Mexiletene

Question 20

When to initiate GDMT for HCM with LV dysfunction?

Answer 20

-LVEF < 50% (Also DC Disopyramide, CCB and consider ICD (2a))

Question 21

When to order CPET in patients with HCM?

Answer 21

NYHA III/IV or refractory arrhythmias -> transplant considerations

Question 22

Name 4 meds that can be used for rhythm control of AF in HoCM

Answer 22

• Disopyarmide (First line)
• Amiodarone (Reasonable)
• Sotalol (Reasonable)
• Dofetilide (Reasonable)

Question 23

What are three AADs that can be used for Ventricular Arrhythmias?

Answer 23

Amiodarone

Sotalol

Mexiletene

Question 24

What are three things that weight loss in HoCM can result in improvement?

Answer 24

• AF
• LVOTO
• HF symptoms

Question 25

At what LVEF in HCM would you consider CRT when they have an ICD indication already?

Answer 25

LBBB and LVEF < 50%

Question 26

Name 4 scenarios where you could offer SRT in patients with mild (NYHA II symptoms) ?

Answer 26

Severe and Progressive Pulmonary hypertension thought to be due to LVOT-O or MR

LAE and 1 or more episode of AF

Poor functional capacity attributable to LVOT-O on functional testing

Children and young adults with LVOT-O > 100mmhg

Question 27

When is it class 1 indication for Myectomy > ETOH ablation ? Vise Versa?

Answer 27

• Myectomy: When other needs for OHS

- ETOH: When unacceptable candidate for Myectomy

Question 28

Name 4 things to do for HCM patient with LVEF < 50%

Answer 28

• Start on GDMT
• Discontinue Negative Inotropes (Non DHP CCB, Disopyramide)
• Evaluate for ICD (Class 2a) (or CRT if concomitant symptoms and LBBB)
• Evaluate for other causes of LV dysfunction

Question 29

When should echo be done in pregnant patients with HoCM?

Answer 29

2nd/3rd trimester when hemodynamic load is highest, or if symptoms change