Hypertrophic cardiomyopathy European society of cardiology guidelines,2014 Prevention of sudden cardiac death Left ventricular outflow tract obstruction

1. Cardio Egypt 2015 23rd - 26th February 2015,Cairo – Egypt.

4. The guideline is fairly long and detailed
(55 pages, with 506 references, and
36 tables listing recommendations).
0Data derived from multiple randomized
clinical trials or meta-analyses.
Level of
Evidence A
36
(27%)
Data derived from a single randomized
clinical trial or large non-randomized studies.
Level of
Evidence B
96
(73%)
Consensus of opinion of the experts and/ or
small studies, retrospective studies, registries.
Level of
Evidence C
132 recommendations in the guideline

5. 1) Etiologic diagnosis.
2) Diagnostic criteria.
3) Diagnostic work-up.
4) Genetic testing and family screening.
5) Management of left ventricular outflow tract obstruction.
6) Management of symptoms in the absence of obstruction.
7) Atrial arrhythmias.
8) Prevention of SCD.
9) Management of pregnancy.
10)Special issues.
The most important or novel points
What do the guidelines say ?

6. Hypertrophic cardiomyopathy can be caused by
many genetic and non-genetic disorders.
In up to 60% of
patients with HCM,
the disease is an
autosomal
dominant trait
caused by
mutations in
cardiac sarcomere
protein genes.
5-10% of adult cases are caused by other genetic disorders
including inherited metabolic and neuromuscular diseases,
chromosome abnormalities and genetic syndromes.
Etiologic Diagnosis

7. Diagnostic Criteria
Hypertrophic cardiomyopathy is defined by the
presence of increased LV wall thickness that is not
solely explained by abnormal loading conditions.
In an adult, this represents a wall thickness ≥15 mm
in one or more LV myocardial segments(or ≥13 mm
in a first degree relative of someone with HCM)
measured by any imaging technique(echo,CMR or CT)
Patient ≥ 15 mm
FDR ≥ 13 mm
In children : wall thickness ≥ 2 standard deviations above the predicted mean.

8. Diagnostic Work-up
Standard 12-lead ECG and 48-hour ambulatory ECG monitoring
are routinely recommended
Class of recommendation : I
Level of evidence : B
Class of recommendation : I
Level of evidence : B
NSVT

9. Transthoracic echocardiographic
In all patients with HCM at initial evaluation , transthoracic 2D and
Doppler echocardiography are recommended, at rest and during
Valsalva manoeuvre in the sitting and semi-supine positions—and
then on standing if no gradient is provoked.
( Class of recommendation : I Level of evidence : B )

10. In symptomatic patients with a resting or provoked peak
LVOTgradient <50 mm Hg , echo during exercise in the standing,
sitting or semi-supine position is recommended to detect provocable
LVOTO and exercise-induced mitral regurgitation. ( Class I – B )
Exercise stress echocardiography

11. In the absence of contraindications,
CMR with LGE is recommended
in patients with suspected HCM
who have inadequate echo
windows, in order to confirm the
diagnosis.
Class of recommendation : I
Level of evidence : B
Cardiovascular magnetic resonance imaging
With Late Gadolinium Enhancement
HCM with predominately
ventricular septal hypertrophy
(maximal wall thickness, 24 mm)
should be considered in patients who have inadequate
echocardiographic imaging and contraindications for CMR.(IIa - C)

12. Genetic Testing and Family Screening
Genetic testing is recommended in patients fulfilling
diagnostic criteria for HCM, when it enables cascade
genetic screening of their relatives.
Class of recommendation : I Level of evidence : B

13. Management of Left Ventricular Outflow
Tract Obstruction : Medical Treatment
ß-blocker
(Class I-B)
Verapamil
(Class I-B)
Add Disopyramide
(Class I-B)
Diltiazem
(Class IIa-C)
Intolerance or contraindications
Intolerance or contraindications
Digoxin is contraindicated for
the treatment of AF in patients
with obstruction (Class III-C)

14. Management of Left Ventricular Outflow
Tract Obstruction :Invasive treatment
Septal myectomy
Septal alcohol ablation
Dual chamber pacing
Septal
Reduction
Therapy

15. The indication for septal reduction therapy remains
unchanged in patients with significant obstruction
(baseline or provoked gradient > 50 mmHg) and
moderate-to-severe symptoms (NYHA functional class III-
IV) or recurrent exertional syncope despite maximum
tolerated medical therapy (I-B and IIa-C , respectively).
Septal myectomySeptal alcohol ablation

16. Surgery vs. alcohol ablation
 For the first time, septal alcohol ablation is assigned the same
class of recommendation (I-B) as myectomy in expert centers.
 The 2 procedures have similar efficacy and complications rates.
Septal alcohol ablation has a higher rate of atrioventricular block
than surgery (12% vs 5%).
Septal myectomy, rather than septal
alcohol ablation, is recommended in patients
with an indication for septal reduction therapy
and other lesions requiring surgical intervention
(e.g. mitral valve repair/replacement, papillary
muscle intervention). (Class I-C)

17. Dual chamber pacing to reduce LVOTO
Permanent AV sequential pacing with optimal AV
interval may be considered in symptomatic adult
patients who are unsuitable for—or unwilling to
consider—other invasive septal reduction
therapies,and in patients who require an ICD (IIb-C)
LVOTO is abolished70 mmHg

18. Management of symptoms in the absence of
obstruction
Heart failure NYHA functional Class II–IV
and preserved LV ejection fraction (≥50%)
& no evidence for resting or provocable LVOTO
ß-blockers, verapamil or Diltiazem
( class IIa- C )
Low-dose loop and thiazide Diuretics
( class IIa-C )

19. Heart failure and reduced LV ejection
fraction (< 50%) & no evidence for
resting or provocable LVOTO
ß-blocker + ACEI (or ARB if ACEI not tolerated) ( Class IIa - C )
Low-dose loop diuretics ( Class IIa -C )
Mineralocorticoid receptor antagonist ( Class IIa - C )
Digoxin for Heart Rate Control in Patients With Atrial Fib (Class IIb- C )
Management of symptoms in the absence of
obstruction
is recommended in patients in NYHA functional class II-IV, LV
ejection fraction < 50%, a QRS duration > 120 ms, and LBBB (ClassIIa-C).

20.  Atrial fibrillation is the most common arrhythmia in patients
with HCM.
 New-onset AF is frequently associated with heart failure
symptoms and so should be treated promptly in accordance
with ESC guidelines.
Atrial tachyarrhythmia

21. Atrial fibrillation
 Patients with HCM and paroxysmal,
persistent or permanent AF should receive
treatment with vitamin K antagonists.
(Class I-B)
 Lifelong therapy with oral anticoagulants is
recommended, even when sinus rhythm is
restored. (Class I-C)
Patients in sinus rhythm with LA diameter
≥45 mm should undergo 6–12 monthly
48-hour ambulatory ECG monitoring to
detect AF. (Class IIa-C)
CHA2DS2-VASc
score is not
recommended
Warfarin for all

22. Prevention of sudden cardiac death
Model for estimating sudden cardiac death risk
The HCM Risk-SCD formula is as follows:
Probability = 1 – 0.998
where = [0.15939858 x maximal wall thickness
(mm)] - [0.00294271 x maximal wall thickness² (mm²)] +
[0.0259082 x left atrial diameter (mm)] + [0.00446131 x maximal
(rest/Valsalva) left ventricular outflow tract gradient (mm Hg)] +
[0.4583082 x family history SCD] + [0.82639195 x NSVT] +
[0.71650361 x unexplained syncope] - [0.01799934 x age at clinical
evaluation (years)].
SCD at 5 years
exp (Prognostic index)
The formula is available online
European Society of Cardiology. HCM Risk-SCD Calculator. 2014.
Available at:www.doc2do.com/hcm/webHCM.html

24. Prevention of sudden cardiac death : ICD
Secondary Prevention :
ICD implantation is recommended in patients who have
survived a cardiac arrest due to VT or VF (Class I-B)
Primary Prevention :
Estimate the 5-year risk of SCD using the HCM Risk-SCD model
HIGH RISK
5 – year risk ≥6%
INTERMEDIATE RISK
5 – year risk ≥4 - <6%
LOW RISK
5–year risk <4%
ICD should be considered
( Class IIa – B )
ICD may be considered
( Class IIb-B )
ICD generally not indicated
(Class III-B)

25. Management of pregnancy
HCM Women : Ideally, risk assessment should be
performed before conception,using the modified
WHO classification.

26. Modified WHO classification of maternal cardiovascular risk
Application to HCMRisk of pregnancyRisk
class
-of
maternal mortality and no/mild
risk of morbidity
I
Most women with HCM:
mild to moderate LVOTO;
asymptomatic with or without
medication, well-controlled
arrhythmia, normal systolic LV
function or mild LV dysfunction
of maternal
mortality or moderate increase
in morbidity
II
Severe LVOTO, symptoms or
arrhythmias despite optimal
medication, moderate systolic LV
dysfunction
of
maternal mortality or severe
morbidity
III
Severe systolic LV dysfunction, severe
symptomatic LVOTO
of maternal
mortality or severe morbidity;
pregnancy contraindicated
IV
Yes
No

27. Recommendations on reproductive issues in women with HCM
β-Blockers (preferably metoprolol) should be started in women
who develop symptoms during pregnancy. ( Class I - C )
β-Blockers (preferably metoprolol) should be continued in women
who used them before pregnancy. (Class IIa - C )
Therapeutic anticoagulation with LMWH or vitamin K
antagonists depending on the stage of pregnancy is
recommended for atrial fibrillation. (Class I - C )
Cardioversion should be considered for persistent atrial
fibrillation. (Class IIa - C)

28. SPECIAL ISSUES
 The last section of the guideline discusses several
scenarios requiring special considerations in the
diagnosis and management of HCM.
 This section discusses the differential diagnosis between
HCM and the normal training effect in athletes or
hypertensive heart disease

29. Clinical features that assist in the differential diagnosis of
hypertensive heart disease and hypertrophic cardiomyopathy
Clinical features favouring hypertension only
 Normal 12 lead ECG or isolated increased voltage
without repolarisation abnormality
 Regression of LVH over 6–12 months tight systolic
blood pressure control (<130 mm Hg)
Clinical features favouring HCM
 Family history of HCM
 Right ventricular hypertrophy
 Late gadolinium enhancement at the RV insertion points
or localized to segments of maximum LV thickening on CMR
 Maximum LV wall thickness ≥15 mm (Caucasian); ≥20 mm (black)
 Severe diastolic dysfunction
 Marked repolarisation abnormalities, conduction disease
or Q-waves on 12 lead ECG

30. Web Table 7:
Clinical features that favour the diagnosis of hypertrophic cardiomyopathy in
elite athletes with maximal left ventricular wall thickness 12–15 mm
(Level of Evidence B or C )
Hypertrophic
CardiomyopathyAthlete’s Heart
The challenge : “Grey Zone” of left ventricular wall thickness 12–15 mm
How to differentiate athlete’s heart from HCM ?

31.  ASH (septal to posterior wall thickness ≥1.5)
 Complete SAM of mitral valve
 LV end diastolic diameter <45 mm
 Late gadolinium enhancement on CMR
 Resting intraventricular gradient
 Incomplete SAM of mitral valve
 LVH of the anterior septum or the posterior wall ≥12 mm
 Left atrium >45 mm
 RVH (right ventricular subcostal thickness >5 mm)
 Myocardial crypts identified with CMR
 Mitral inflow pattern E < A(20 years old)
 Tissue Doppler Imaging: Ea <9 cm/sec
 Tissue Doppler Imaging: Sa <9 cm/sec
 Increased BNP
 Ea 10–13 cm/sec
 Diastolic radial strain <7 cm/sec
 VO2max <50ml/kg/min or <120% of predicted VO2max
 Increased left ventricular torsion
Structural
Functional
Features favouring HCM in Athlete’s

32.  Abnormal Q waves in at least two leads from II, III, aVF (absence of left
anterior hemiblock), V1–V4, I, aVL, V5–V6
 Inverted T –waves in two or more leads from lead groups II, III, aVF or/and
I, aVL,V5-V6
 Inverted T–waves V2-V4 (>16 years old)
 Giant negative T–waves in two contiguous leads (> 5mm)
 Inverted T–waves in leads V2–V4 (<16 years old)
 Complex ventricular arrhythmias at 24 h Holter rhythm recording or >2000
PVCs/24 h
 Family history of HCM in first degree relative(s)
 Female gender
 Family history of SCD in first degree relative(s) ≤40 years
 Cardiovascular symptoms (unexplained syncope,
disproportionate dyspnoea on exertion, chest pain, palpitations)
 No response to detraining for 3 months
 Disease causing sarcomere mutationGenetics
Detraining
ECG
Demographics
Features favouring HCM in Athlete’s

33. The ESC guideline on the
diagnosis and management
of HCM is the most recent
update on the topic.
The most important points of
the guideline refer to the
etiologic diagnosis of HCM,
genetic testing, structured
management of LVOTO, atrial
arrhythmias, and SCD
prevention.
Conclusion