Hypertrophic cardiomyopathy (HCM) is a common genetic heart disease with an autosomal dominant inheritance pattern, presenting with varying degrees of left ventricular outflow tract obstruction and associated symptoms like syncope and heart failure. Common mechanisms include dynamic obstruction during exertion and mitral regurgitation due to systolic anterior motion of the mitral valve. The management of obstructive HCM may involve drug therapy, alcohol septal ablation, or surgical myectomy depending on symptom severity and anatomical considerations.

1. Hypertrophic
Cardiomyopathy
-Dr.Akif Baig

2. Epidemiology
 HCM is a common genetic heart disease reported in
populations globally
 Inherited in an autosomal dominant pattern
 The distribution of HCM is equal by sex, although women are
diagnosed less commonly than men
 The prevalence of unexplained asymptomatic hypertrophy in
young adults has been reported to range from 1:200 to 1:500

3.  Most common site involved is anterior basal septum
 Apical HCM was first reported in Japan and is more
prevalent in individuals of Japanese versus European descent
(13% to 25% vs. 1% to 2%)
 Biventricular hypertrophy was seen in 13% of patients
of HCM

4. Associated Syndromes
 Leopard Syndrome
 Noonan Syndrome

6. Leopard Syndrome
 Noonan syndrome plus multiple skin lentigenes
 (L)entigines - dark spots on the skin
 (E)lectrocardiographic conduction defects - abnormalities of the
electrical activity of the heart
 (O)cular hypertelorism - widely spaced eyes
 (P)ulmonary stenosis - obstruction of the normal outflow of blood
from the right ventricle of the heart
 (A)bnormalities of the genitalia
 (R)etarded (slowed) growth resulting in short stature
 (D)eafness

7. Definition
 2D echocardiography or cardiovascular magnetic resonance
(CMR) showing a maximal end-diastolic wall thickness of
≥15 mm anywhere in the left ventricle, in the absence of
another cause of hypertrophy in adults
 More limited hypertrophy (13–14 mm) can be diagnostic
when present in family members of a patient with HCM or in
conjunction with a positive genetic test
2020 AHA/ACC Guideline for the Diagnosis andTreatment of PatientsWith Hypertrophic Cardiomyopathy

8. Types
•3/4th
Obstructive
•1/4th
Non-
Obstructive

9. Pathophysiology

10. LVOTO
 LVOTO, either at rest or with provocation, is present in ~75% of
patients with HCM
 Two principal mechanisms are responsible for LVOTO
 Septal hypertrophy with narrowing of the LVOT, leading to
abnormal blood flow vectors that dynamically displace the mitral valve
leaflets anteriorly (Venturi Effect) and
 Anatomic alterations in the mitral valve and apparatus, including
longer leaflets as well as anterior displacement of the papillary muscles
and mitral valve apparatus, which makes the valve more susceptible to
the abnormal flow vectors

11.  Consequently, there is systolic anterior motion of the
mitral valve leaflets, which leads to LVOTO, high
intracavitary pressures, and MR from the loss of leaflet
coaptation

12.  Presence of a peak LVOT gradient of ≥30 mm Hg is
considered to be indicative of obstruction
 Resting or provoked gradients ≥50 mm Hg generally
considered to be the threshold for septal reduction
therapy (SRT) in those patients with drug refractory
symptoms

13.  LVOTO in HCM is dynamic and sensitive to ventricular
load and contractility
 Increased myocardial contractility, decreased preload, or
lower afterload will increase the LVOT gradient
Decreased Preload
Increased Myocardial
Contractility
Decreased
Afterload
Increase LVOT
Gradient

15.  Thus, provocative maneuvers may be necessary in
patients with low or absent peak resting gradients (i.e, <30
mm Hg) to elicit the presence of LVOTO, particularly in
patients with symptoms
 Such maneuvers include:
 Standing
 Valsalva strain
 Amyl nitrite inhalation, or
 Exercise (fasted or postprandial)

16. Mitral Regurgitation
 Mitral regurgitation (MR) can occur secondarily from LVOTO (70-
80%) or from primary leaflet abnormalities (20-30%) and
contributes to symptoms of dyspnea
 In MR caused by LVOTO, SAM of the mitral valve leads to loss
of leaflet coaptation, and the jet is predominantly mid to-late systolic
and posterior or lateral in orientation
 Primary abnormalities of the mitral valve and its apparatus are
also common, including excessive leaflet length, anomalous papillary
muscle insertion, and anteriorly displaced papillary muscles
- Molisana M, Selimi A, Gizzi G, D'Agostino S, Ianni U, ParatoVM. Different mechanisms of mitral
regurgitation in hypertrophic cardiomyopathy:

17. Diastolic Dysfunction
 Following factors to contribute to the presence of diastolic
dysfunction:
 Altered ventricular load with high intracavitary pressures
 Nonuniformity in ventricular contraction and relaxation
 Delayed inactivation from abnormal intracellular calcium
reuptake are common abnormalities in HCM, and

18. Myocardial Ischemia
 Patients with HCM are susceptible to myocardial ischemia
attributable to a mismatch between myocardial
oxygen supply and demand
 Myocardial hypertrophy, microvascular
dysfunction with impaired coronary flow reserve,
and medial hypertrophy of the intramural arterioles and their
reduced density are common findings

19. Myocardial Bridging in HCM
 Myocardial bridging (MB) is a congenital coronary artery anomaly in which
a band of myocardium overlies a segment of the normal epicardial
coronary artery
 While the average prevalence of MBs in adults is 25%, it is seen that this rate
is 41% in adults with hypertrophic cardiomyopathy (HCM)
 This high rate demonstrates the importance of examining coronary CT scans
of patients with HCM for MB
 Some reports have suggested an association between MB and increased
severe symptoms, such as ventricular arrhythmias and sudden death
in HCM
CelenkV, Celenk C. Myocardial bridging in adult with hypertrophic cardiomyopathy: Imaging findings with coronary
computed tomography angiography. Radiol Case Rep

20. Autonomic Dysfunction
 Patients with HCM can have autonomic dysfunction, with
impaired heart rate recovery and inappropriate vasodilatation
 The prevalence of autonomic dysfunction in HCM is
uncertain, although studies have described an abnormal
blood pressure response to exercise in ~25% of patients
Abnormal blood pressure response to exercise, defined as failure to increase
systolic blood pressure by at least 20 mm Hg, or a drop in systolic blood pressure
during exercise of >20 mm Hg from the peak value obtained, has been associated with
a poorer prognosis

21. Natural History of HOCM
 Presentation can be from Infancy to old age
 Annual mortality is 1% per year
 23% live atleast 75years
 Mean age of death from HCM is 56 years

22.  The major causes of death in HCM are SCD, heart failure, and
stroke
 SCD accounts for approximately half of total mortality in patients with
HCM
 Progressive heart failure is responsible for the death of one-third
of patients, usually after middle age (more than 55 years), and
 Atrial fibrillation (AF)-associated stroke is responsible for the death
of 13% of the patients at a higher age, usually older than 65 years

23.  Approximately 10% to 20% of individuals with HCM have a
lifetime-increased risk for SCD, most likely resulting from
VT and ventricular fibrillation (VF)
 Although SCD occurs most often in adolescents or adults
younger than 35 years, it can occur at any age
 However, SCD is uncommon in young children and
very uncommon in patients older than 60 years, even
among those with established risk factors.

24. Clinical Presentation

25. Heart Failure
 Variable degrees of heart failure occur in more than 50% of
HCM patients
 Shortness of breath, particularly exertional, is the most
common symptom of HCM
 The most common mechanism of heart failure is LVOT
obstruction, but diastolic LV dysfunction can precipitate
heart failure symptoms even in the absence of LVOT
obstruction

26.  Although left ventricular ejection fraction (LVEF) is typically
preserved or even hyperdynamic, a minority of
patients can progress to the end-stage phase of HCM, marked
by LV systolic dysfunction and occasionally progressive LV
dilatation and wall thinning

27. Burned Out HCM
 It refers to the end-stage of hypertrophic
cardiomyopathy and is characterized by
myocardial fibrosis, systolic dysfunction and left ventricular
wall thinning
 3-5% of patients with hypertrophic cardiomyopathy
 In patients with a known diagnosis of hypertrophic
cardiomyopathy the burned-out phase can be made by the
development of systolic dysfunction (
left ventricular ejection fraction <50%)

28.  These patients can develop advanced heart failure symptoms
of pulmonary and systemic venous congestion (orthopnea,
paroxysmal nocturnal dyspnea, edema), which can become
refractory to medical therapy and require cardiac
transplantation

29. Myocardial Ischemia
 Exertional chest discomfort occurs in 25% to 30% of patients
with HCM, usually in the setting of a normal coronary
arteriogram, and likely is related to microvascular angina
caused by supply/demand mismatch
 Some patients also experience atypical chest pain, frequently
precipitated or worsened by heavy meals
 A subset of patients can also have obstructive epicardial
coronary artery disease, which heralds an adverse outcome.

30. Syncope
 Syncope occurs in 15% to 25% of patients with HCM
 Another 20% complain of presyncope
 Different mechanisms can precipitate syncope in HCM
patients, including arrhythmias (VT or AF), neurocardiogenic
syncope, and hypotension during exercise caused by LVOT
obstruction or by abnormal vascular responses

31.  Changes in LV loading conditions during exercise, heavy
meals, and dehydration often provoke symptoms
 Syncope typically occurs in younger patients with small LV
end-diastolic volume
 Syncope during exertion is more common in patients with
LVOT obstruction than in patients without obstruction,
whereas unexplained syncope at rest and neurally mediated
syncope do not appear to be related to LVOT obstruction

32. Atrial Fibrillation
 AF is the most common arrhythmia observed in HCM, with an annual
incidence of approximately 1% to 2% and a prevalence of
approximately 20% to 25%
 The incidence of AF increases with age, occurring most frequently after
the age of 55 years (but approximately 10 years earlier than in the general
population), and is very uncommon in patients younger than 25 years
 The high incidence of AF in HCM is likely related to increased left
atrium (LA) atrial pressure and size, caused by LV diastolic
dysfunction
 In fact, LA size is one of the most important determinants ofAF occurrence

33. Ventricular Arrhythmias
 Ambulatory cardiac monitoring frequently reveals premature
ventricular complexes (PVCs) (in more than 80% of
patients) and nonsustainedVT (25% to 30%)
 NonsustainedVT is associated with severity of LV
hypertrophy and symptom class
 Stable sustained monomorphicVT is rare but has been
observed particularly in patients with LV apical aneurysms

34. Investigations

35. ECG in HOCM

36.  Classical (asymmetrical) Hypertrophic
Cardiomyopathy:
 Voltage criteria for LVH in precordial and limb leads
 Narrow,“dagger-like” Q waves in inferior and lateral leads

38. Q wave morphology
 Q waves seen in HCM can mimic prior myocardial
infarction, although the Q-wave morphology is different:
 Infarction Q waves are typically > 40 ms duration
 Septal Q waves in HCM are < 40 ms
 Lateral Q waves are more common than inferior Q waves in
HCM

39.  The
apical variant of HOCM, known as “Yamaguchi Syndrome,”
does not result in septal Q waves, as the septum is normal in
thickness in this conduction
 The cardiac apex is abnormally thickened, resulting in diffuse
T wave changes throughout the precordial leads
 This is sometimes referred to as “giantTWave Inversion

41. Other associated features:
 Left atrial enlargement (“P mitrale”) — left ventricular
diastolic dysfunction may lead to compensatory left atrial
hyertrophy
 Signs ofWPW (short PR, delta wave) — ECG features
of Wolff-Parkinson-White (WPW) were seen in 33% of
patients with HCM in one study
 Dysrhythmias: atrial fibrillation and supraventricular
tachycardias are common; PACs, PVCs,VT

42. Echocardiography

43.  The initial echocardiographic studies of hypertrophic
cardiomyopathy used M-mode echocardiography for
diagnosis
 With this technique, a septal to posterior wall-thickness
ratio of 1.3:1 or more was considered evidence of inappropriate
septal hypertrophy and was used to establish the diagnosis
 This was referred to as asymmetric septal hypertrophy
(ASH), a term which often understates the distribution of the
pathologic hypertrophy

44.  It should be emphasized that there are a number of other disease
states, such as pulmonary hypertension with right ventricular
hypertrophy, and inferior wall infarction in the presence of
left ventricular hypertrophy that result in a similar septal to
posterior wall-thickness ratio
 In the normal aging heart there is often hypertrophy and angulation
of the proximal anterior septum which may mimic hypertrophic
cardiomyopathy if only the septal to posterior wall ratio is considered
 Therefore, septal to posterior wall-thickness ratio alone should
not be used as a marker of hypertrophic cardiomyopathy.

45. Parasternal long-axis view recorded in a patient with classic
hypertrophic cardiomyopathy.
Note the marked thickening of the interventricular septum and the normal thickness
of the posterior wall

47. - Note the markedly thickened interventricular septum and the relatively normal
thickness posteriorWall
Also note the systolic anterior motion of the mitral valve (downward-pointing arrows) which
opposes the ventricular septum throughout the majority of ventricular systole suggesting obstructive
physiology

49. LVOT Gradient
 Left ventricular outflow tract gradient (LVOT) in
hypertrophic obstructive cardiomyopathy (HOCM) is
usually measured from the apical five chamber view
(apical 5C)

50.  The CW jet in HOCM is described as dagger
shaped or sickle shaped, unlike the
symmetrical tongue shaped jet in fixed obstruction of
aortic stenosis

51. HOCM

52. Aortic Stenosis

53.  The shape of the jet indicates the dynamic nature of
LVOT obstruction in hypertrophic cardiomyopathy
 The gradient progressively increases as the systole
progresses, to produce this characteristic appearance
 The delayed peaking in systole is quite evident

54. Brockenberg Phenomenon

55. Brockenbrough–Braunwald–Morrow sign
 Paradoxical haemodynamic response to post-extrasystolic potentiation (a
decrease in pulse pressure) in patients with HOCM
 An augmented contraction is seen during the post-extrasystolic beat ,
producing SAM and severe LVOT obstruction despite the prolonged
diastolic filling time
 Extracellular Ca2+
enters the cardiac myocytes during a premature beat and
stored in the sarcoplasmic reticulum
 During the post-extrasystolic beat, the increased contribution of the
sarcoplasmic reticulum to intracellular Ca2+
is the cause of the increased
contraction

58. Cardiac MRI

59.  Cardiac magnetic resonance imaging (CMR) plays a
valuable role in evaluating patients with known or suspected
hypertrophic cardiomyopathy
 Because of its intrinsic three-dimensional acquisition, CMR is
able to accurately evaluate the global extent and distribution
of left ventricular wall hypertrophy more accurately
than two- or three-dimensional echocardiography

60.  CMR can identify the abnormal architecture,
hypertrophy, and geometry of papillary muscles and also
characterize anatomical abnormalities of the mitral valve
which may contribute to mitral regurgitation and require
attention at the time of surgical myectomy
 CMR allows identification and quantification of
abnormal flow velocities within the cardiac chambers
and can identify mitral regurgitation, dynamic outflow tract
obstruction, and calculate dynamic obstructive gradients

65. Heart
Rhythm
Assessment

67. Cardiac Catheterisation
In HOCM

69. Spike and Dome Configuration
 Early Spike
 Rapid LV ejection by hypercontractile myocardium
 Pressure dip and doming
 Reflects Dynamic Outflow tract obstruction

70. Brockenbrough–Braunwald–Morrow sign

72. Management

74. Types of Beta Blockers
Vasodilating
• Acebutalol
• Carvedilol
• Labetolol
• Nebivolol
Non
Vasodilating
• Metoprolol
• Atenolol,
• Propranolol

83. Intervention in HOCM

84. Alcohol Septal Ablation(ASA)

85.  First performed by Sigwart in 1995
 Minimally invasive
 Lack of surgical incision and general anaesthesia
 Shorter recovery and hospital stay

86. Patient Selection
 Severe, drug-refractory cardiac symptoms (NewYork Heart Association functional
class III/IV dyspnea or Canadian Cardiac Society angina class III/IV) due to obstructive HCM
 Dynamic LVOT obstruction (gradient ≥30 mm Hg at rest or ≥50 mm Hg with
provocation) that is due to septal hypertrophy and systolic anterior motion of the mitral valve
 Ventricular septal thickness of 15 mm or greater; patients with markedly severe hypertrophy,
especially 30 mm or greater, should be avoided
 Absence of significant intrinsic mitral valve disease
 Absence of the need for concomitant cardiac surgical procedure (e.g., bypass grafting, valve
replacement)
 Age >40 years

87. Things to consider before choosing ASA
over Septal Myectomy (SM)
 Need for PPI post procedure is 4-5 times higher as compared with SM
 Clinical and hemodynamic effects are achieved immediately after SM
but may be delayed for upto 3 months in ASA
 Patients with severe septal hypertrophy (>30mm) derive limited or
no benefit from ASA
 Surgical myectomy is almost always predicatable whereas success of
ASA depends on distribution of targeted septal branch and blood
supply to the area of septum

88. Procedure Technique
 Conscious sedation
 Pain control at time of alcohol infusion into septal perforator

89. First step
 Perform a standard coronary angiography to define
 Coronary anatomy and
 concomitant atherosclerotic disease
 RAO Cranial or Posteroanterior cranial projections is
used to visualise septal artery coursing through basal
interventricular septum
 While in majority septal perforators arises from LAD
 It may also be seen in Left MainTrunk, Ramus intermedius. LCX or
RCA

90. 2nd
Step
 Temporary Pacemaker should be placed as prophylactic
measure in case of development of CHB
 Heparin in administered to keep ACT >300 to prevent
thrombosis in guiding catheters or wires

91. Temporary Pacemaker Placement
 The risk of pacemaker dependency from septal ablation varies according to
the baseline electrocardiographic abnormalities
 Septal ablation frequently results in right bundle branch block
(approximately 50% of cases)
 Thus, for those patients with left bundle branch block, severe left axis
deviation, or a very wide QRS complex, the rate of pacemaker dependency
approaches 50%
 However permanent pacemaker dependency from complete atrioventricular
block still occurs in 10%–15% of patients with a normal electrocardiogram

92. 3rd
step
 A guiding catheter (6F or 7F Catheter) is used to engage the left main
 A 0.014 inch guidewire with a soft tip is passed into the selected
septal perforator
 A short over-the-wire (OTW) angioplasty baloon usually 1.5-2mm
in diameter is passed over the guidewire into the selected septal
branch
 Baloon is inflated to completely occlude the septal branch to prevent
any reflux of injected alcohol

94. 4th
step
 Verify Myocardial territory being supplied by the selected
septal perforator, given a significant variation in septal
anatomy in HCM patients
 Both angiographic as well as echocardiographic confirmation
must be obtained prior to proceeding with alcohol injection

95. Angiographic confirmation
 1-2cc of contrast administered through OTW baloon
 Three things to be observed
 Ensure that selected perforator supplies the basal septum that is
responsible for LVOT Obstruction
 Contrast doesn’t reflux back into LAD
 Contrast shouldn’t reach RCA circulation through septal
collaterals

96. Echocardiographic confirmation
 After careful visualising the septum in A4CV and PLAX view
 1-2cc of Albumex (20% Albumin) is injected into the septal
perforator through the OTW Balloon
 Appearance of echo contrast should be visible at greatest extent of
septal-mitral contact
 Appearance of contrast in the distal septum, right ventricle or
other areas of myocardium is a contraindication to ethanol
infusion

97. Myocardial contrast echocardiography during ASA
Echocardiographic images in the apical four chamber view before (A) and after (B) injection of
echo contrast through the lumen of the OTW balloon. Increased echo signal is seen in the
correct position in the basal septum (circled).

98. Myocardial contrast echocardiography during ASA
Echocardiographic images in the parasternal long-axis view confirm the presence of increased
echo signal situated correctly exclusively on the left side of the interventricular septum
(circled).

99.  Final method of confirmation
>30% reduction in LVOT gradient on balloon
inflation in the selected septal perforator

100. 5th
step: Alcohol Injection
 1-2ml of 100% Ethanol (Absolute Alcohol)
 Over 1-5minute with the balloon remaining inflated
 In cases where there is rapid contrast washout due to
collateralization of the septal branch, the rate and volume of
ethanol

101. Final Step
 Angiography of Left Coronary Artery to document occlusion
of septal artery and to verify integrity of rest of coronary
circulation

102. Post-Procedural
 Monitoring in ICU for atleast 48hours
 TPI can be discontinued after 48hours if there is no
bradyarrhythmias or heart block

103. Results
 Reduction in LVOT gardient after ASA demonstrates a
triphasic response
1. Acute reduction in LVOT gradient
2. Increase in gradient to baseline level within 3days
3. Decrease in LVOT gradient to immediate post ablation
level within 3 months

104. Acute reduction in LVOT gradient
 Loss of septal contractility caused by ischemia, necrosis and
stunning of septal myocardium

105. Increase in gradient to baseline level within
3days
 Recovery of septal myocardium from stunning

106. Decrease in LVOT gradient to immediate
post ablation level within 3 months
 Thinning of infarcted septum and LVOT remodeling, leading
to sustained and more permanent reduction in gradients

107. Successful Alcohol Septal Ablation
- Reduction in LVOT gradient to <30mmHg in setting of resting
gradient > 50mmHg or
- >50% reduction of a provacable gradient

108. Complications
Ventricular Fibrillation
(2.2%)
LAD Dissection (1.8%)
Pericardial Effusion (0.6%)
CHB (12-15 %)
Large Anterior wall MI
30 day Mortality Rate
(1.5%)
Post 30 day Mortality Rate
(0.5 %)
Right Bundle Branch
Block (80 %)

109. Septal Myectomy

110.  Transaortic SM is the gold standard for majority of patients
with obstructive HCM and severe symptoms refractory to
medical therapy
 5-15g of myocardial tissue is resected from the base of aortic
valve to a region distal to mitral leaflet such that the area of
mitral septal contact that results in SAM is removed

111.  To correctly identify the involved portion of LV septum,
simultaneous Trans-Esophageal Echo is done to
 Assist with localization of desired region for resection and
 To monitor effects of resection of LVOT Gradient
intraoperatively

112. Patient Selection
 Severe, drug-refractory cardiac symptoms (NewYork Heart Association
functional class III/IV dyspnea or Canadian Cardiac Society angina class III/IV) due
to obstructive HCM
 Dynamic LVOT obstruction (gradient ≥30 mm Hg at rest or ≥50 mm Hg with
provocation) that is due to septal hypertrophy and systolic anterior motion of the
mitral valve
 Significant intrinsic mitral valve disease
 Need for concomitant cardiac surgical procedure (e.g., bypass grafting, valve
replacement)
 Younger Age (< 40years)

113. Complications
 Complete Heart Block (2%)
 Left bundle branch block
 VSD (<1%)
 Mortality <1%

114. Factors to consider while
chosing between
Alcohol septal ablation vs
Surgical Myectomy

115. Age & Arrhythmias
 Induction of transmural myocardial scar by ASA
 Increases future risk of Ventricular Arrhythmias
 Alcohol septal ablation should not be performed in patients
<21years old and should be discouraged in Age<40years old
unless there is contraindications to surgery

116. Pre-existing Bundle Branch block
 Myectomy produces LBBB
 Contraindicated in RBBB
 Alcohol Septal Ablation produces RBBB
 Contraindicated in LBBB

117. Anatomy of LVOT
 Anamolous insertion of Papillary muscle into the mitral valve
 Massive LV Hypertrophy (>30mm)
 Anatomically mitral valve with long leaflet
 All these favours Septal Myectomy

118. Concomitant Cardiac Problems
 Coronary Artery Disease
 MitralValve disease
 Favours Septal Myectomy

120. Pacemaker
In
HOCM

121.  Dual chamber pacing , as a less invasive alternative to the
Septal Myectomy was first introduced in 1990s

122. Mechanism
 Exact mechanism is unclear
 Activation of RV Apex
 Dyssynchronous contraction of IVS
 Reduction of LVOT Gradient in short term
 Positive ventricular remodelling in long term

123. Mean LVOT gradient reduction after DDD Pacing is
10mmHg
Mean LVOT gradient reduction after septal
reduction is 40-50 mmHg

124. Recommendations
ACC-2017
Medically refractory symptomatic HOCM who are suboptimal
candidates for septal reduction therapy
Class II b
Patients with HCM who have a dual chamber device implanted
for non HCM indiactions, it is reasonable to consider a trial of
dual chamber AV pacing (From the RV apex) for the relief of
symptoms attributable to LVOT Obstruction
Class IIa

125. ACC-2017
Non obstructive HCM Class III
Medically refractory patients who are candidates for septal
reduction therapy
Class III
Asymptomatic or medically controlled Class III

126. ICD
In
HOCM

131. HeartTransplantation

132. Recommendation
Advanced Heart Failure and Non-
obstructive HCM and EF<50% who are not
amenable to other intervention
Class I
Symptomatic children with HCM and
restrictive physiology who are not responsive to
or appropriate candidates for other thereupatic
interventions should also be considered for
heart transplantation
Class I
Heart transplantation should not be considered
or performed in mildly symptomatic
patients of any age with HCM
Class III

133. Screening in
Family Members

136. HCM vs Athlete Heart

139. EmergingTherpaies

141. Mavacamten

142.  Allosteric inhibitor of cardiac myosin ATPase

144.  Reduces excessive cardiac contractility
 Improves diastolic function
 Reduces Ejection fraction
 C.I in EF< 50%

147. Gene Therapy
 Gene-based therapy is also emerging as a potential strategy,
including genome editing, exon skipping, allele-specific
silencing, spliceosome-mediated RNA trans-splicing, and
gene replacement
 The therapeutic goal is to replace, remove, or mitigate the
effect of the germline genetic defect

148. References
 ACC 2020 HOCM Guidleines
 Braunwald’s Cardiovascular InterventionTextbook
 Grossman CatheterisationTextbook
 Braunwald’s CardiologyTextbook
 Daubert C, Gadler F, Mabo P, Linde C. Pacing for hypertrophic
obstructive cardiomyopathy: an update and future directions. Europace.
2018 Jun 1;20(6):908-920. doi: 10.1093/europace/eux131. PMID:
29106577.