- Implantable cardioverter defibrillators (ICDs) are recommended for patients with hypertrophic cardiomyopathy (HCM) who have survived sudden cardiac arrest, have spontaneous sustained ventricular tachycardia, or meet certain high-risk criteria. - Risk stratification should be performed at initial evaluation and periodically to determine ICD need based on factors like family history of sudden cardiac death and abnormal blood pressure response. - For left ventricular outflow tract obstruction, beta blockers and calcium channel blockers are first-line medical therapies while septal myectomy or alcohol septal ablation are invasive options that provide long-term reduction in outflow gradient.

1. HOCM MANAGEMENT
-DR. ROHITWALSE
SENIOR RESIDENT
DM CARDIOLOGY
SCTIMST

2. SCOPE OF DISCUSSION
• INTRODUCTION
• RISK STRATIFICATION & ROLE OF ICD
• PHARMACOLOGICAL RX, INCLUDING AF AND
PREGNANCY ISSUES
• INVASIVETREATMENT
ALCOHLIC SEPTAL ABLATION
Sx MYOMECTOMY

3. INTRODUCTION
• HISTORY: 1ST Patient – Mr. Claude Brady (1959)
• Age of presentation- Birth to 90 yrs
• Sex predilection: M~F (M>F)
• Older Annual mortality rates- 4% to 6%
• Overall HCM-related mortality rates-1%/yr
• Somewhat higher in children -2%/yr.

4. • HCM is an inherited disease of heart muscle disease with
variable clinical expression and natural history.
• Characterised by hypertrophy of left ventricular walls.
• Sudden cardiac death –common cause of mortality.
• Can be effectively treated with implantable cardioverter
defibrillator.

5. European Heart Journal (2014):doi:10.1093/eurheartj/ehu284
HCM: Definitions
Increased left ventricular wall thickness not solely explained by
abnormal loading conditions
ADULTS:
● LV wall thickness ≥15 mm in one or more LV myocardial segments
measured by any imaging technique
CHILDREN:
● LV wall thickness more than two standard deviations above the predicted
mean (z-score >2)

6. Hypertrophic Cardiomyopathy

8. JACCVol. 58, No. 25, 2011 Gersh et al. December 13/20, 2011:e212–60
ACCF/AHA Hypertrophic Cardiomyopathy Guideline

9. Dai-Yin et al, Clinical Outcomes in Patients With Non obstructive, Labile,
and Obstructive Hypertrophic Cardiomyopathy, Journal of the American
Heart Association March 6, 2018,Vol 7, Issue 5

11. RISK STRATIFICATION
• Survival from a cardiac arrest due toVT orVF
• Spontaneous sustainedVT causing syncope or hemodynamic
compromise
• F/H/O SCD associated with HCM
• LV wall thickness > 30 mm
• Unexplained syncope within 6 months
• NSVT > 3 beats
• Abnormal BP response during exercise
Established risk factors:
Heart Rhythm,Vol 15, No 10, October 2018

12. RISK STRATIFICATION
• <30 years
• Delayed hyperenhancement on cardiac MRI
• LVOT obstruction
• Syncope >5 y ago
• LV aneurysm
• LVEF <50%
Potential risk modifiers:
High risk subsets:
Heart Rhythm,Vol 15, No 10, October 2018

13. CLASS I RECOMMENDATIONS
• To perform risk stratification at the time of initial
evaluation and periodically thereafter.
• ICD is recommended for those who have survived a SCA
due toVT orVF, or have spontaneous sustainedVT
causing syncope or hemodynamic compromise, an if
meaningful survival > 1 year is expected
Heart Rhythm,Vol 15, No 10, October 2018

14. CLASS II a RECOMMENDATIONS
• In patients with HCM who have spontaneous NSVT or an
abnormal blood pressure response with exercise, who also have
additional SCD risk modifiers or high-risk features, an ICD is
reasonable if meaningful survival greater than 1 year is expected
• Inpatients with HCM and 1 or more of the following risk factors,
an ICD is reasonable if meaningful survival of greater than 1 year
is expected:
• Maximum LV wall thickness ‡30 mm
• SCD in 1 or more first-degree relatives presumably caused by HCM
• 1 or more episodes of unexplained syncope within the preceding 6 months
Heart Rhythm,Vol 15, No 10, October 2018

15. CLASS II b RECOMMENDATIONS
• In patients with HCM who have NSVT or an abnormal
blood pressure response with exercise but do not have
any other SCD risk modifiers, an ICD may be considered,
but its benefit is uncertain.
• In patients with HCM and a history of sustainedVT orVF,
amiodarone may be considered when an ICD is not
feasible or not preferred by the patient .
Heart Rhythm,Vol 15, No 10, October 2018

16. SUMMARY
Heart Rhythm,Vol 15, No 10, October 2018

17. European Heart Journal (2014):doi:10.1093/eurheartj/ehu284

18. European Heart Journal (2014):doi:10.1093/eurheartj/ehu284
Prevention of Sudden Cardiac
Death
Recommendations for ICD in
each risk category take into
account not only the absolute
statistical risk, but also the
age and general health of the
patient, socio-economic
factors and the psychological
impact of therapy.

19. European Heart Journal (2014):doi:10.1093/eurheartj/ehu284
CMR and Sudden Death Risk
“On balance, the extent of
LGE on CMR has some
utility in predicting
cardiovascular mortality, but
current data do not support
the use of LGE
in prediction of SCD risk.”

20. • Clear benefit to patients being treated for secondary
prevention.
• However, the appropriate therapy rate for primary
prevention was lower than previously reported.
• No single risk factor appeared to have stronger
association with appropriate ICD therapy than others.

22. European Heart Journal (2014):doi:10.1093/eurheartj/ehu284
Atrial Fibrillation:
● Use of the CHA DS -VASc score to calculate stroke risk
is NOT recommended.
2
2
● In general, lifelong therapy with oral anticoagulants is
recommended, even when sinus rhythm is restored.
● As left atrial size is a consistent predictor for AF and
stroke in patients with HCM, patients in sinus rhythm
with LA diameter ≥45mm should undergo 6–12 monthly
48-hour ambulatory ECG monitoring to detect AF.

23. European Heart Journal (2014):doi:10.1093/eurheartj/ehu284
Maternal Risk

24. European Heart Journal (2014):doi:10.1093/eurheartj/ehu284
Reproduction & Contraception

25. 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

26. SCREENING ….

27. European Heart Journal (2014):doi:10.1093/eurheartj/ehu284
Treatment of Left Ventricular Outflow
Tract Obstruction
 By convention, LVOTO is defined as a peak
instantaneous Doppler LV outflow tract gradient of
≥30 mm Hg, but the threshold for invasive treatment
is usually considered to be ≥50 mm Hg.
 There are no data to support the use of invasive
procedures to reduce LV outflow obstruction in
asymptomatic patients, regardless of its severity.

28. European Heart Journal (2014):doi:10.1093/eurheartj/ehu284
Treatment of LV Outflow Tract Obstruction

29. European Heart Journal (2014):doi:10.1093/eurheartj/ehu284
Drug treatment of LVOTO
ß -blockers
Verapamil
(Diltiazem)
Disopyramide
Diuretics
FIRST LINE
SECOND LINE

30. MedicalTherapy
• Beta-blockers
• Increase ventricular diastolic filling/relaxation
• Decrease myocardial oxygen consumption
• Have not been shown to reduce the incidence of SCD
• Verapamil
• Augments ventricular diastolic filling/relaxation
• Disopyramide
• Used in combination with beta-blocker
• Negative inotrope
• Diuretics

32. RANOLAZINE
• Late sodium channel current inhibitor
• Reduces diastolic dysfunction, microvascular ischemia,
ventricular arrhythmias, or severity of left ventricular
outflow obstruction.

33. • Clinically significant
improvement in angina
and HF symptoms.
• Improved quality of life.

34. • 80 adult patients with non-obstructive HCM
• No overall effect on exercise performance, plasma pro-BNP
levels, diastolic function, or quality of life
• Excellent safety profile and reduced premature ventricular
complex burden

36. • The totality of the data did not support continuation of
the Eleclazine development program.
• So, this study was terminated in March 2018 prior to the
end of the double-blind phase.

37. INVASIVE THERAPIES

38. Surgical Septal Myectomy
Nishimura RA et al. NEJM. 2004. 350(13):1320.

39. SurgicalTherapy
• 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).
• RPRrepair- (R) resection of septum, (P) plication of anterior
leaflet of mitral valve, & (R) release of abnormal papillary
muscle attachments.

41. 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 forAF is reduced, & LV
pressures & wall stress are normalized.

44. 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.

45. Patient selection ….
• Clinical: NYHA III or IV-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 sufficient anterior septal thickness
Avoided if septal thickness <18 mm

46. 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 -3 mL 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.

47. Alcohol Septal Ablation
Braunwald. Atlas of Heart Diseases: Cardiomyopathies, Myocarditis, and Pericardial Disease. 1998.

48. Alcohol Septal Ablation
Before After

49. LVOTO gradient shows a triphasic response
followingASA.
• 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 wks -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.

50. 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.

51. Complications
• Temporary CHB 50% occurs during procedure.
• Persistent CHB prompting permanent pacemaker occurs in
10% - 20%
• 5% of patients have sustainedVT during hospitalization.
• In-hospital mortality rate is up to 2%.(0-4%)
• Because of potential for creating aVSD, 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.

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53. JOURNAL OFTHE AMERICAN COLLEGE OF CARDIOLOGYVOL. 72, NO. 24, 2018

54. Ablation vs Myectomy

55. • Long-term mortality and (aborted) SCD rates after ASA
and myectomy are similarly low.
• Patients who undergoASA have more than twice the risk
of permanent pacemaker implantation and a 5 times
higher risk of the need for additional septal reduction
therapy compared with those who undergo myectomy.

58. • There were no differences in survival of patients
undergoing myectomy or alcohol septal ablation, but
freedom from reintervention and early and late
reduction of left ventricular outflow tract gradient are
superior in patients undergoing septal myectomy.

61. • THANKYOU