2014 esc hcm

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

The guideline is fairly long and detailed

(55 pages, with 506 references, and

36 tables listing recommendations).

0Data derived from multiple randomizedclinical trials or meta-analyses.

Level ofEvidence A

36 (27%)

Data derived from a single randomizedclinical trial or large non-randomized studies.

Level ofEvidence B

96 (73%)

Consensus of opinion of the experts and/ or small studies, retrospective studies, registries.

Level ofEvidence C

132 recommendations in the guideline

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 ?

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

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.

Diagnostic Work-up

Standard 12-lead ECG and 48-hour ambulatory ECG monitoring are routinely recommended

Class of recommendation : ILevel of evidence : B

Class of recommendation : ILevel of evidence : B

NSVT

Transthoracic echocardiographic

In all patients with HCM at initial evaluation , transthoracic 2D andDoppler 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 )

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 provocableLVOTO and exercise-induced mitral regurgitation. ( Class I – B )

Exercise stress echocardiography

In the absence of contraindications,CMR with LGE is recommendedin patients with suspected HCMwho have inadequate echo windows, in order to confirm the diagnosis.

Class of recommendation : ILevel of evidence : B

Cardiovascular magnetic resonance imagingWith 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)

Genetic Testing and Family Screening

Genetic testing is recommended in patients fulfilling diagnostic criteria for HCM, when it enables cascadegenetic screening of their relatives.

Class of recommendation : I Level of evidence : B

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)

Management of Left Ventricular Outflow Tract Obstruction :Invasive treatment

Septal myectomy

Septal alcohol ablation

Dual chamber pacing

Septal Reduction Therapy

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

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)

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

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 )

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).

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

Atrial fibrillation

Patients with HCM and paroxysmal, persistent or permanent AF should receivetreatment 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

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 clinicalevaluation (years)].

SCD at 5 years

exp (Prognostic index)

The formula is available onlineEuropean Society of Cardiology. HCM Risk-SCD Calculator. 2014.

Available at:www.doc2do.com/hcm/webHCM.html

Prevention of sudden cardiac death : ICDSecondary 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 RISK5 – year risk ≥6%

INTERMEDIATE RISK5 – year risk ≥4 - <6%

LOW RISK5–year risk <4%

ICD should be considered( Class IIa – B )

ICD may be considered( Class IIb-B )

ICD generally not indicated(Class III-B)

Management of pregnancy

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

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 increasein 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

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)

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

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

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 ?

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

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

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 SCDprevention.

Conclusion