This document discusses the management of hypertrophic cardiomyopathy (HCM). It covers the natural history of HCM, risk stratification including the role of implantable cardioverter defibrillators, pharmacological treatments, and invasive treatments such as alcohol septal ablation and surgical myectomy. Key points discussed include the use of beta blockers as first-line pharmacological therapy, guidelines for ICD implantation, the technique and outcomes of alcohol septal ablation versus surgical myectomy, and recommendations for experienced centers to perform these invasive procedures.

1. MANAGEMENT OF HCM
Dr Mohd Iqbal Dar

2. BROAD HEADINGS
• NATURAL HISTORY
• RISK STRATIFICATION & ROLE OF ICD
• PHARMACOLOGICAL RX , INCLUDING
AF TREATMENT , PREGNANCY ISSUES
• INVASIVE TREATMENT
ALCOHLIC SEPTAL ABLATION
Sx MYOMECTOMY
ROLE OF PACING

3. NATURAL HISTORY
• Clinical presentation
All phases of life effected –from birth to >90 yrs age.
• Overall HCM-related mortality rates of about 1%/yr ,
• Somewhat higher in children -2%/yr.
• Older literature.-Annual mortality rates of 4% to 6%

4. MANY PATIENTS DESERVE LARGE
MEASURE OF REASSURANCE …….

5. BUT SUBGROUPS AT HIGH RISK OF
PREMATURE DEATH & DISEASE
COMPLICATIONS DOES EXIST
• With history of Sudden & unexpected death in
family
• Progressive heart failure –LVOTO, ,Diastolic &
micro vascular dysfunction, LVEF<50%
• Atrial fibrillation, with risk for embolic stroke

7. DEFINITION - LVOTO

8. Probability of hypertrophic cardiomyopathy (HCM)–related death among 273 patients
with a left ventricular outflow gradient of at least 30 mm Hg under basal conditions and
828 patients without obstruction at entry. (Effect of Left Ventricular Outflow Tract
Obstruction on Clinical Outcome in Hypertrophic Cardiomyopathy N Engl J Med
2003;348:295-303).

9. Risk Stratification and Sudden
Death

10. Risk Stratification and Sudden
Death

11. ICD RECOMONDATIOS IN HCM

12. ACC Recommendations
Class I
1. The decision to place an ICD in patients with HCM
should include application of individual clinical
judgment, as well as a thorough discussion of
strength of evidence, benefits,& risks to allow
informed patient’s active participation in decision
making Level of Evidence: C)
2. ICD placement is recommended for patients with
HCM with prior documented cardiac arrest,
ventricular fibrillation, or hemodynamically significant
VT(Level of Evidence: B)

13. Selection of ICD Device
Type—Recommendations
Class IIa
1. In patients with HCM who meet indications for ICD ,
single-chamber devices are reasonable in younger
patients without a need for atrial or ventricular pacing.
2. In patients with HCM who meet indications for ICD,
dual-chamber ICDs are reasonable for patients with
sinus bradycardia and/or paroxysma AF.
3. In patients with HCM who meet indications for ICD ,
dual-chamber ICDs are reasonable for patients with
elevated resting outflow gradients greater than 50 mm
Hg & significant heart failure symptoms who may
benefit from RV pacing (most commonly, but not
limited to, patients >65 years of age).

14. ICD IN HCM……2 INTERNATIONAL
MULTICENTRE REGISTRY…..
• Among patients who received a device for secondary
prevention , annualized rate of subsequent appropriate
ICD discharge was 10% per yr.
• Patients with primary prevention ICDs placed on basis
of 1 or more of conventional risk markers experienced
appropriate ICD therapy at a rate of 4% per yr.
• Number of risk markers present did not predict
subsequent device discharge
Maron BJ, Spirito P, Shen WK, et al. Implantable cardioverterdefibrillators and prevention of sudden cardiac
death in hypertrophic cardiomyopathy. JAMA. 2007;298:405–12.
Maron BJ, Shen WK, Link MS, et al. Efficacy of ICDfor the prevention of sudden death in patientswith hypertrophic
cardiomyopathy. N Engl J Med. 2000;342:365–73.

15. Risks of ICD implantation
Risks of ICD implantation may be thought of,
& communicated to patients, as 4 ‘‘I’s,’’
• Implantation risk,
• Infection,
• Inappropriate shock,
• Insurance risk & never using device (you
buy the policy but don’t die).

16. Asymptomatic Patients
• Large proportion are asymptomatic, &
most will achieve a normal life
expectancy.
• Educate patient
• Screening of 1st -degree relatives
• Avoiding strenuous activity .
• Risk stratification for SCD
Watchful waiting is often appropriate

17. SCREENING ….

18. Symptomatic Patients
Major goal is to alleviate exertional dyspnea,
palpitations & chest discomfort, which may
reflect pathophysiologic mechanisms such as
LVOT obstruction, reduced supply of
myocardial O2 , MR and impaired LV
diastolic relaxation & compliance
Pharmacologic therapy of symptoms in
obstructive HCM is successful in more
than two-thirds of patients

19. Pharmacologic therapy…
Beta blockers first choice(Class I)
• Negative inotropic effect
↓ejection accleration , ↓flow velocity in early systole
, ↓ early drag forces on mitral valve , ↓ mitral septal
contact ,↓SAM & gradient ,decreased adrenegic
induced tachycardia,increased diastolic filling period .
• If low dose ineffective then increases dose to maintain
resting HR <60-65 , generally max
recommended/tolerable doses can be used

20. Titration of beta blockers

21. Verapamil (class I)
• Add on therapy to beta blockers if high doses of beta
blockers are not tolerated
• First choice when beta blockers are contraindicated
• Maximal doses of 280 mg/day
• AVOID in NYHA class IV dyspnoea and hypotension
• When used as add-on therapyto look for high grade
AV block
Diltiazem
• improves diastolic performance .
• Cautiously to be used in those with severe LVOTO,
increased PCWP, low BP

22. MANAGEMENT OF HCM
DISOPYRAMIDE (class II A)
• NOT USED AS A SOLO THERAPY
• ALWAYS AS AN ADD ON THERAPY TO BB AND
VERAPAMIL
• INCREASED AV CONDUCTION, PROLONG QT
INTERVAL, ANTICHOLINERGIC SIDE EFFECTS

23. ROLE OF DIURETICS
• USEFUL DRUGS WHEN WE KNOW
WHEN TO USE
• AVOIDED IN HOCM WITH PRESERVED
SYSTOLIC FUNCTION (class II B)
• CAN BE USED IN HOCM WITH
SYSTOLIC DYSFUNCTION (class II B),
NONOBSTRUCTIVE HCM (ONLY AS
ADD ON THERAPY-class II A)

24. MANAGEMENT OF ACUTE HYPOTENSION
IN HOCM
• IV FLUIDS (class I)
• PHENYLEPHRINE (class I)-vasopressor
• Positive inotropes can cause harm (class III)
• MANAGEMENT OF HOCM WITH
“DEPRESSED SYSTOLIC FUNCTION” IS A
SEPERATE ENTITY

25. MANAGEMENT OF HCM WITH LV SYSTOLIC
DYSFUNCTION
• ACEi/ARBs, Diuretics-STANDARD HF TREATMENT
(class I)
• DISCONTINUE VERAPAMIL, DILTIAZEM,
DISOPYRAMIDE (class III)
• CAD, VALVULAR HEART DISEASE, METABOLIC
DISORDERS TO BE RULED OUT
• ICDs TO BE CONSIDERED (class IIB)
• ANTICOAGULATION IN PRESENCE OF AF/LV APICAL
ANEURYSMS
• HEART TRANSPLANTATION FOR REFRACTORY NYHA
CLASS III/IV SYMPTOMS

27. ACC Recommendations…
Class III(HARM)
• Nifedipine or other dihydropyridine CCB in
patients with LVOT obstruction.
• Verapamil -obstructive HCM in setting of
systemic hypotension or severe dyspnea at rest.
• Digitalis in the absence of AF.
• Disopyramide alone without beta blockers or
verapamil in patients with HCM with AF .
• Dopamine, dobutamine, norepinephrine, & other
intravenous positive inotropic drugs in acute
hypotension in patients with obstructive HCM

28. Pharmacotherapy in Non
Obstructive HCM
• Limited options
• Verapramil, beta blockers may be used ,
not that helpful as in Obstructive cases
• Disopyramide not recommended
• Diuretics if HF +

30. INVASIVE THERAPIES

31. Alcohol Septal Ablation (ASA)

32. Alcohol Septal Ablation (ASA)
• A 68-year-old lady, unresponsive to DDD pacemaker & optimal medical
therapy for HOCM, agreed to become first patient for ASA for Dr
Sigwart 1994 who initially noticed that significant reduction in LVOT
gradient when angioplasty balloon was inflated in 1st septal artery,
• This was further supported by disappearance of typical auscultatory
findings, & echo manifestations of obstruction of HOCM following MI
• However it took a decade for ethical clearance for this revolutionary
idea of instilling alcohol & producing a controlled infarction.

33. Patient selection ….
• Clinical: NYHA III or IV DOE , AOE or occasionally
other exertional symptoms (such as syncope or near
syncope) that interfere with everyday activity or quality of
life despite optimal medical therapy.
• Hemodynamic: Dynamic LVOT gradient at rest or with
physiologic provocation 50 mm Hg associated with
septal hypertrophy & SAM of mitral valve.
• Anatomic: Targeted anterior septal thickness sufficient
to perform the procedure safely and effectively in the
judgment of individual operator.,Avoided if septal
thickness <18 mm

34. ASA…..Experienced operators
• Individual operator with a cumulative case
volume of at least 20 procedures
• Individual operator who is working in a
dedicated HCM program with a cumulative
total of at least 50 procedures

35. Alcohol Septal Ablation (ASA)…Technique
• Contrast angiography of septal perforator through
balloon central lumen with simultaneous echo Guidance
confirms delivery to only target myocardium.
• A short (∼10mm) OTW balloon is advanced into septal
artery, balloon material should not disintegrate on
exposure, should be at least equal or slightly bigger than
septal artery (2- 2.5 mm)
• About 1 -3mL of alcohol is infused in controlled fashion.
• It is important that balloon be inflated & that a contrast
injection also show that there is no extravasation of dye
into distal LAD.
• Contrast enhancement of other regions (papillary
muscles, free wall) indicates collateral circulation from
septal perforator artery, & alcohol should not be infused .

36. LVOTO gradient shows a triphasic response
following ASA.
• Stage 1: Stunning phase: There is immediate
decrease in gradient following ASA. stunning of
septum.
• Stage 2: Edema phase: There might be some
increase in LVOTO due to peri-infarction edema. This
is reason for recurrence of gradient during
discharge time. Lasts for 5–10 days after procedure .
• Stage 3: Scar phase:LVOTO gradually decreases, as
scar forms over weeks -months & septum becomes
thinner gradually. This stage lasts for 3–12 months
after ASA. Thus, accurate success of ASA can only be
determined after 3 months.

37. Alcohol septal ablation :
Advantages
Greater patient satisfaction :
• Absence of a surgical incision & GA
• Less overall discomfort
• Much shorter recovery time.
Selective advantage in older patients.

38. Complications
• Temporary CHB 50% occurs during
procedure.
• Persistent CHB prompting permanent
pacemaker occurs in 10% - 20%

39. Complications
• 5% of patients have sustained VT during
hospitalization.
• In-hospital mortality rate is up to 2%.(0-
4%)
• Because of potential for creating a VSD ,
septal ablation should not be performed if
target septal thickness is < 18 mm.
• LAD dissection, remote infarction,
ventricular fibrillation, stroke, pericardial
effusion, are relatively uncommon
complications

40. FABERS SCORING SYSTEM TO PREDICT
PPI NEED
• Upto 8, Low risk, discharge from monitoring;
• 8–12, intermediate risk,prolonged monitoring;
• >12, high risk, prepare for early pacemaker implantation.

41. PREDICTORS OF CHB AFTER
ASA…CHANG etal

42. ACC……..Class II a
• When surgery is contraindicated or risk is
considered unacceptable because of
serious comorbidities or advanced
age,ASA, when performed in experienced
centers, can be beneficial in eligible adult
patients with HCM with LVOT obstruction
& severe drug-refractory symptoms
(usually NYHA functional classes III or IV).
(Level of Evidence: B)

43. ACC……..Class II b
• ASA, when performed in experienced centers, may be
considered as an alternative to surgical myectomy for
eligible adult patients with HCM with severe drug-
refractory symptoms & LVOT obstruction when, after
a balanced and thorough discussion, the patient
expresses a preference for septal ablation. (Level of
Evidence: B)
• Effectiveness of ASA is uncertain in patients with
HCM with marked (i.e., >30 mm) septal hypertrophy,
and therefore the procedure is generally discouraged
in such patients. (Level of Evidence: C)

44. Class III : HARM
• Adult patients with HCM who are asymptomatic with normal
exercise tolerance or whose symptoms are controlled or
minimized on optimal medical therapy. (Level of evidence: C)
• Septal reduction therapy should not be done unless
performed as part of a program dedicated to the longitudinal
and multidisciplinary care of patients with HCM. (Level of
Evidence: C)
• Mitral valve replacement for relief of LVOT obstruction should
not be performed in patients with HCM in whom septal
reduction therapy is an option. (Level of Evidence: C)
• Alcohol septal ablation should not be done in patients with
HCM with concomitant disease that independently warrants
surgical correction , in whom surgical myectomy can be
performed as part of the operation. (Level of Evidence: C)

45. Clinical outcome of ASA……
• In a pooled analysis of 42 published studies between 1996
and 2005, Alam et al. have analyzed the outcome of ASA in
2959 patients. After a mean follow-up of 12.7 months,
• LVOTO gradients Resting from 65.3 to 15.8 mmHg
• Provocable from 125.4 to 31.5 mmHg,
• Basal septal diameter decreased from 20.9 to 13.9 mm.
• Mean NYHA functional class from 2.9 to 1.2.
• Mean exercise capacity ↑34%
• Peak oxygen consumption ↑ 33%,
• Procedural success rate was 89%.
• 6.6% of patients required multiple ablations
• 1.9% went on to septal myectomy.
• Mean 30-day mortality was 1.5%.
• Late all-cause mortality was 0.5%.

46. Surgical Therapy

47. Surgical Therapy
• Transaortic septal myectomy is currently
considered most appropriate treatment for
majority of patients with obstructive HCM & severe
symptoms unresponsive to medical therapy .
• Traditional myectomy (Morrow procedure) with
about a 3-cm long resection(Tips of MV)
• Extended myectomy (a resection of about 7 cm)(
upto apex) are currently used.
• RPRrepair- (R) resection of septum, (P) plication
of anterior leaflet of mitral valve, & (R) release of
abnormal papillary muscle attachments.

48. Mechanism…
• LVOT gradient reduction with myectomy
results from basal septal thinning with
resultant enlargement of LVOT area (and
redirection of forward flow with loss of
the drag & Venturi effects on mitral
valve)& consequently abolition of SAM &
mitral-septal contact.
• MR is also usually eliminated without need
for additional MV surgery.
• With myectomy, LA size,risk for AF is
reduced, & LV pressures & wall stress are
normalized.

50. Complications
• Complications following myectomy are rare
when performed in experienced centers.
• The risk of CHB is approximately 2% with
myectomy (higher with preexisting RBBB),
but in myectomy patients who have had
previous ASA, risk is much higher (50% to
85%)
• Iatrogenic VSD occurs in 1% of patients
• Aortic valve or mitral valve injury - 1%
• Operative mortality - < 1%

51. ACC …… Class II a
• Surgical septal myectomy, when
performed in experienced centers, can be
beneficial and is the first consideration for
majority of eligible patients with HCM with
severe drug-refractory symptoms and
LVOT obstruction. (Level of Evidence: B)

54. ROLE OF MYOMECTOMY
• Surgery is clearly indicated for the relief of
symptoms in medically refractory obstructive
HCM.
• However, till date, there is no evidence to
suggest that septal myectomy improves survival
in patients who achieve relief of gradient and
symptoms through pharmacologic therapy or
those who have only mild symptoms.

57. DDD Pacing
• Implantation of a dual-chamber pacemaker with short
AV delay was proposed as alternative treatment for
patients with severe symptomatic HOCM.
Mechanism ??
• Paradoxical septal movement widening LVOT
• Dyssynchrony—RV pacing, Short AV delay,
decreased LV ejection acceleration &decreases early
forces on mitral valve
• However, 3 randomized crossover trials showed
that although symptomatic improvement was reported
by majority of patients following continuous DDD
pacing, a similar frequency of improvement was
reported by patients during AAI mode (control mode
without pacing).Suggesting a placebo effect

58. DDD Pacing
• There are no data that dual-chamber pacing
either reduces the risk of SCD in patients with
HCM, alters the underlying progression of
disease, or is of benefit to patients with non
obstructive HCM.
• A trial of dual chamber pacing may be
considered for symptomatic patients with
obstruction in whom an ICD has already been
implanted for high-risk status. (Class IIa )

59. ACC …. Recommendations .
Class IIb:
• Permanent pacing may be considered in
medically refractory symptomatic patients
with obstructive HCM who are suboptimal
candidates for septal reduction therapy.
Class III:
• Asymptomatic, medically controlled & as
first line therapy for those eligible for septal
reduction .

60. ACC…. AF GUIDELINES
CLASS I :
• Anticoagulation , INR 2-3 …
• Rate control with Beta blockers, Non DiHydropyridine CCBs
Class IIa :
• Disopyramide( with rate controlling drugs), Amidarone ----
Antiarrythmics..
• RFA ---Refractory, unable to take medicines
• Maze procedure—during myomectomy or in selected patients
Class IIb
• Sotalol, Dofetilide, Dronaderone

61. ACC…..Pregnancy/Delivery—
Recommendations
Class I
In women with HCM who are asymptomatic or whose
symptoms are controlled with beta-blocking drugs, the
drugs should be continued during pregnancy, but
increased surveillance for fetal bradycardia or other
complications is warranted.
For patients (mother or father) with HCM, genetic
counseling is indicated before planned conception.

62. ACC…..Pregnancy/Delivery—
Recommendations
• In women with HCM & resting or provocable LVOT
obstruction greater than or equal to 50 mm Hg and/or
cardiac symptoms not controlled by medical therapy
alone, pregnancy is associated with increased risk, and
these patients should be referred to a high-risk
obstetrician. (Level of Evidence: C)
• The diagnosis of HCM among asymptomatic women is
not considered a contraindication for pregnancy, but
patients should be carefully evaluated in regarding to
the risk of pregnancy.

63. ACC…..Pregnancy/Delivery—
Recommendations
Class IIa
• For women with HCM whose symptoms are
controlled (mild to moderate), pregnancy is
reasonable but expert maternal/fetal medical
specialist care including cardiovascular and
prenatal monitoring, is advised. (Level of
Evidence: C)
Class III
• Harm For women with advanced heart failure
symptoms and HCM, pregnancy is associated with
excess morbidity/ mortality. (Level of Evidence: C)

64. HCM & ATHELETES
Recommendation to Avoid Competition
• HCM is most common structural heart disease
found at autopsy in those young atheletes who
have SCD in playing field.
It is recommended
• Patients with HCM should avoid competition &
extremes of exertion.
• To avoid activities where syncope would have
disastrous effect such as scuba diving or surfing.
• Patients not lift more than 40 lb.

65. ACC …. Guidelines
Class III
Patients with HCM should not participate in
intense competitive sports regardless of age,
sex, race, presence or absence of LVOT
obstruction, prior septal reduction therapy,
or implantation of a cardioverter-defibrillator
for high-risk status

66. CONCLUSION
• There is a need for basic understanding of
mechanisms that transform a genotype-positive
individual into a patient with HCM.
• If these were understood, means to prevent or
modify pathologic hypertrophy might be found
• More medications are needed for this condition for
both palliation of symptoms & preventing disease
progression. In particular, to prevent fibrosis
and improve chamber compliance.
• For obstruction, long-term study of benefits &
risks of alcohol ablation must be compiled &
compared to myectomy.
• Current paradigms for sudden death risk
stratification lack predictive accuracy

67. THANK YOU