2. Define
• In adults
• HCM is defined by a wall thickness ≥ 15 mm(>13 mm in first-degree
relatives) in one or more left ventricular (LV) myocardial segments,
whatever the imaging technique (echocardiography, cardiac magnetic
resonance[CMR] or computed tomography), without any explained
loading conditions
3. Assessment by ECHO
• The presence or absence of a left ventricular outflow
tract(LVOT) obstruction must be assessed at rest and during
physiological provocation, such as the Valsalva manoeuvre.
• The threshold remains at 30 mmHg for the instantaneous peak
Doppler LV outflow tract pressure gradient at rest, and exercise
echocardiography is not recommended in asymptomatic
patients with a gradient > 50 mmHg at rest.
4. Symptomatic
• Septal reduction
• NYHA III—IV despite maximum-tolerated medical Rx and with a gradient > 50
mmHg;
• Unexplained recurrent syncope with < 50 mmHg LOVT gradient
• Morrow procedure in young with septal hypertrophy ≥ 17 mm
• In case of indication for ICD, a dual-chamber ICD may be
considered in patients with LVOT obstruction ≥ 50 mmHg, NSR
and drug-refractory symptoms
• Rx CHF
• Rx for Afib
5. HCM vs. HOCM
• HCM
No gradient at rest or at
exercise
• HOCM
Outflow gradients are
common in HCM, present
in 70% of patients at rest
or with physiological
exercise
6. Risk
• Annual mortality rate in obstructive HCM is ~ 4 %
• High risk
• Onset of age
• frequent unstained VT
• syncope
• resuscitated sudden death
• Family history of sudden death
• Effort angina
• Effort dyspnea
7. ACC/ESC Consensus-2003
1. Cardiac arrest (ventricular fibrillation)
2. Spontaneous sustained VT
3. family history of premature sudden death
4. Unexplained syncope
5. LV thickness ≥ 30mm
6. Abnormal exercise blood pressure
7. Non-sustained ventricular tachycardia (on Holter
monitoring)
10. 1st ALCOHOL SEPTAL ABLATION
[ASA]
• Nonsurgical
• Professor Ulrich Sigwart
• Royal Brompton Hospital
• 1994
• Injection of 1 to 4 mL of 96% ethanol into the first septal
perforator
branch of the left anterior descending coronary artery to produce a
basal septal myocardial infarction and ultimately remodeling of the
LV outflow tract
11. Map before ASA
• Inject Levovist and map the hypertrophy topology using
TTE
12. ASA in cath Lab
Pig tail catheter in the LV
Angioplasty guide in the first septal artery
OTW 1.5-2.0 mm balloon into 1st septal artery
Injecting two boluses of 1-4 ml of 96% into septal artery
distal to the balloon, 0.5 to 1.0 mL aliquots at 1 mL/min .
Assess for
Chest pain
Cardiac enzymes
RBBB
Gradient reduction
VSD
17. Eligibility criteriafor ASA
1. Symptomatic even with optimum MM
2. Dynamic LVOT obstructioncaused by systolic anterior motion
of the mitral valve (gradient30 mm Hg at rest or 50 mm Hg
with provocation)
3. ventricular septal thickness > 15 mm but <25 mm
4. the absence of significant intrinsicmitral valve disease;
18. Complications of ASA
1. Mortality -1-4%
2. Conduction abnormalities are relatively
common complications of PTSMA, with
permanent right bundle branch block and
transitory heart block in about 50% and
high-gradeAV block requiring permanent
pacemakers in 5% to 20%.
3. completeheart block
- monitoring for 2 to 5 days.
19. Procedural success
• 50% reduction in the peak LVOT gradient observed at
rest or, after provocation with a final residual resting
gradient of <20 mm Hg in the absence of death or need
for emergency surgery up to 3 months
20. Risk factors for CHB
1. Rapid administration, large volumes of ethanol.
2. Smaller doses of ethanol (1 to 2 ml) over longer time
periods (5 to 10 min) has decreased the incidence of
CHB
3. Risk factors for CHB
• LBBB
• first-degree atrioventricular block
• female
• volume of alcohol
• number of septal perforators treated