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
• In children
• Diagnosis of HCM requires an LV wall thickness more than two standard
deviations greater than the predicted mean
3. Aetiology
• Sarcomeric HCM (by mutations in cardiac sarcomere protein
genes):40–60%
• Unknown :25–30%
• Genetic or non-genetic causes:5–10%
• Metabolic disorders
• Mitochondrial cardiomyopathies
• Neuromuscular disease
• Malformation syndromes
• Infiltrative disease, endocrine disorders, heart disease and chronic use of
drugs [anabolic steroids and hydroxychloroquine
4. 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.
5. Syncope evaluation
• ECG
• Upright exercise test
• 48-hour ambulatory ECG monitoring
• Recurrent episodes of unexplained syncope: implantable loop
recorder
• Palpitations :48-hour Holter ECG
• Electrophysiological testing :Not recommended for risk stratification
or for the exploration of syncope; testing is indicated for the
assessment of persistent or recurrent supraventricular tachycardia,
for ventricular pre-excitation or for sustained monomorphic
ventricular tachycardia.
6. Genetic counselling
• The relations of proband
• Post-mortem for deceased Proband
• If no causative mutation was characterised in the proband with HCM,
then family screening is solely based on cardiac screening with ECG
and echocardiography, which should be considered in first-degree
relatives aged ≥ 10years, and should be repeated every 1 or 2 years
between 10—20 years of age and every 2 or 5 years thereafter, as
delayed cardiac expression of HCM is observed quite often, even in
adults
• Mutation carrier without cardiac expression (preclinical phase of
HCM), the guidelines mention that sporting activity may be allowed,
taken into account the type of sport, the underlying mutated gene
and the results of regular and repeated cardiac examinations.
7. Symptomatic
• Still high-dose beta-blockers
• verapamil if does not Beta blocker
• Disopyramide maybe combined with beta-blockers to reduce the
gradient in symptomatic
8. 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
9. 7 risk of sudden death
• Age
• family history of sudden cardiac death at a young age
• maximum LV wall thickness
• left atrial diameter
• LVOT obstruction
• non-sustained ventricular tachycardia
• unexplained syncope.
10. 5 Year risk of SCD for primary prevention using 7 risk
factors
• low risk (< 4%)-No ICD
• intermediate risk (4—6%)-May
be ICD
• high risk (> 6%)-ICD
1. Age
2. Family history of sudden
cardiac death at a young age
3. Maximum LV wall thickness
4. Left atrial diameter
5. LVOT obstruction
6. Non-sustained ventricular
tachycardia
7. Unexplained syncope
• http://www.doc2do.com/hcm
/webHCM.html
11. Routine follow-up
• 12-lead ECG and ECH every 12—24 months
• Close F/U of symptomatic
• A 48-hourambulatory ECG is recommended every 12—24 months in
stable patients, every 6—12 months in patients in sinus rhythm with
left atrial dimension ≥ 45 mm and whenever patients complain of
new palpitations
• CMR may be considered systematically every 5 years in clinically
stable patients and every 2—3 years in patients with progressive
disease
• Exercise testing may be considered every 2—3 years in stable
patients and every year in case of progressive symptoms
12. Reproduction
• No gradient or mild gradient are low risk
• Significant gradient across the LVOT posses risk and should be on
Beta blocker with fetal monitoring
13. Lifestyle
Competitive sports activities are contraindicated
Watch on weight , dehydration and excess alcohol
Sexual activity
Normal
PDE-5 inhibitors avoided in LVOT obstruction
Close watch on Rx and side effects
Eligible for an ordinary driving licence
With ICD, follow EHRA and local recommendations
Except for heavy manual jobs with strenuous activity
Life insurance for children of the patients
17. 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
22. 1st
ALCOHOL SEPTAL
ABALATION[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
23. Map before ASA
• Inject Levovist and map the hypertrophy topology using TTE
24. ASA in cath Lab
Pig tail catheter in the LV
ATW angioplasty guide in the first septal artery
OTW 1.5-20 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
29. Advantages of ASA
1. No chest opening
2. No CPB
3. Myomectomy is contra indicated
30. Eligibility criteria for 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;
31. Complications of ASA
1. Mortality -1-4%
2.Conduction abnormalities
are relatively common complications of
PTSMA, with permanentright bundle
branch block and transitory heart
block in about50% and high-gradeAV
block requiring permanent
pacemakers in 5% to 20%.
1.completeheart block
- monitoring for 4 to 5 days.
1.AWMMI due to ethanol reflux
32. Procedural success
• 50% reduction in the peakLVOT gradient observed at rest or, after
provocation with a final residualresting gradient of <20 mm Hg in the
absence of death orneed for emergency surgery up to 3 months
33. Risk factors for CHB
1. Rapidadministration, large volumes of ethanol.
2. Smaller doses of ethanol (1 to2 ml) over longer time periods (5 to
10 min) has decreased theincidence of CHB
3. Risk factors for CHB
• LBBB
• first-degree atrioventricular block
• female
• volume of alcohol
• number of septal perforators treated
36. Bad days of myomectomy
In the early 1990s, a time when myectomy was associated with
relatively high mortality (up to 8%), dual-chamber pacing with short
A-V delay was promoted as a surgical alternative for gradient
and symptom relief
37. Survival benefit
Myectomy also promoteslong-term survival. Operated patients
experience enhanced longevityindistinguishable from that
expected in the general populationand superior to that of non
operated patients with obstruction
After Myectomy, survival free from all-cause mortalityis 98%,
96%, and 83% at 1, 5, and 10 years, and survival freefrom HCM-
related mortality (heart failure and sudden death)is 99%, 98%, and
95%, respectively.Therefore, surgicalseptal myectomy favorably
alters the natural course of HCM,providing a reasonable
expectation for normal or nearly normallife expectancy
38. Surgical Myectomy
0.5
0.6
0.7
0.8
0.9
1.0
0 2 4 6 8 10
I 1 30
II 2 24
III 48 7
IV 14 0
NYHA Pre PostNYHA Pre Post
ObstructiveObstructive
Obstructive Post-myectomyObstructive Post-myectomy
Operative mortality 0.8%
Ommen S et al. J Am Coll Cardiol 2005
39. Operative mortality 0.8%
Gradient reduction 67.3%
Post-op NYHA 1-2 94%
Myectomy for severely symptomaticMyectomy for severely symptomatic
HOCM is safe and effectiveHOCM is safe and effective
Ommen S et al. J Am Coll Cardiol 2005
40. Ablation vs. Myectomy
4 Comparison Studies - 279 patients
Procedural Mortality (%)
0.9
1.3
0
0.2
0.4
0.6
0.8
1
1.2
1.4
Myectomy Ablation Nagueh et al.Nagueh et al. JACCJACC 2001 38(6)2001 38(6)
Qin et al.Qin et al. JACCJACC 2001 38(7)2001 38(7)
Firoozi et al.Firoozi et al. Eur Heart JEur Heart J 2002 23(20)2002 23(20)
Ralph-Edward et al.Ralph-Edward et al. CircCirc 20052005
41. Ablation vs. Myectomy
4 Comparison Studies - 279 patients
Gradient reduction
71
7
75
15
0
10
20
30
40
50
60
70
80
Myectomy Ablation
Nagueh et al.Nagueh et al. JACCJACC 2001 38(6)2001 38(6)
Qin et al.Qin et al. JACCJACC 2001 38(7)2001 38(7)
Firoozi et al.Firoozi et al. Eur Heart JEur Heart J 2002 23(20)2002 23(20)
Ralph-Edward et al.Ralph-Edward et al. CircCirc 20052005
42. Ablation vs. Myectomy
4 Comparison Studies - 279 patients
Symptom Improvement
2.97
1.31
3.17
1.55
0
1
2
3
4
Myectomy Ablation Nagueh et al.Nagueh et al. JACCJACC 2001 38(6)2001 38(6)
Qin et al.Qin et al. JACCJACC 2001 38(7)2001 38(7)
Firoozi et al.Firoozi et al. Eur Heart JEur Heart J 2002 23(20)2002 23(20)
Ralph-Edward et al.Ralph-Edward et al. CircCirc 20052005
50. Conclusion
• Myomectomy for persistent
LVOT obstruction is gold
Standard care for HOCM when
symptoms persist after optimal
medical management to further
improve in the quality of life
and symptom and consistent
reduction in left ventricular
outflow tract gradient
• ASA is an alternative treatment