2014 ESC Guidelines on diagnosis and management of ...

European Heart Journal

doi:10.1093/eurheartj/ehu284a

ESC GUIDELINES

2014 ESC Guidelines on diagnosis and

management of hypertrophic cardiomyopathy:

web addenda

The Task Force for the Diagnosis and Management of Hypertrophic

Cardiomyopathy of the European Society of Cardiology (ESC)

Authors/Task Force Members: Perry M. Elliott* (Chairperson) (UK), Aris Anastasakis

(Greece), Michael A. Borger (Germany), Martin Borggrefe (Germany), Franco Cecchi

(Italy), Philippe Charron (France), Albert Alain Hagege (France), Antoine Lafont

(France), Giuseppe Limongelli (Italy), Heiko Mahrholdt (Germany),

William J. McKenna (UK), Jens Mogensen (Denmark), Petros Nihoyannopoulos (UK),

Stefano Nistri (Italy), Petronella G. Pieper (Netherlands), Burkert Pieske (Austria),

Claudio Rapezzi (Italy), Frans H. Rutten (Netherlands), Christoph Tillmanns

(Germany), and Hugh Watkins (UK).

Additional Contributor: Constantinos O¡¯Mahony (UK).

ESC Committee for Practice Guidelines (CPG): Jose Luis Zamorano (Chairperson) (Spain), Stephan Achenbach

(Germany), Helmut Baumgartner (Germany), Jeroen J. Bax (Netherlands), He?ctor Bueno (Spain), Veronica Dean

(France), Christi Deaton (UK), ?etin Erol (Turkey), Robert Fagard (Belgium), Roberto Ferrari (Italy), David Hasdai

(Israel), Arno W. Hoes (Netherlands), Paulus Kirchhof (Germany/UK), Juhani Knuuti (Finland), Philippe Kolh

(Belgium), Patrizio Lancellotti (Belgium), Ales Linhart (Czech Republic), Petros Nihoyannopoulos (UK),

Massimo F. Piepoli (Italy), Piotr Ponikowski (Poland), Per Anton Sirnes (Norway), Juan Luis Tamargo (Spain),

Michal Tendera (Poland), Adam Torbicki (Poland), William Wijns (Belgium), and Stephan Windecker (Switzerland).

* Corresponding author: Perry M. Elliott, Cardiology Department, The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, United Kingdom, Tel: +44 203 456 7898,

Email: perry.elliott@ucl.ac.uk

?

Other ESC entities having participated in the development of this document:

Associations: European Association of Cardiovascular Imaging (EACVI), European Association of Percutaneous Cardiovascular Interventions (EAPCI), European Heart Rhythm Association (EHRA), Heart Failure Association of the ESC (HFA).

Working Groups: Cardiovascular Pharmacology and Drug Therapy, Working Group on Cardiovascular Surgery, Working Group on Developmental Anatomy and Pathology, Working

Group on Grown-up Congenital Heart Disease, Working Group on Myocardial and Pericardial Diseases.

Councils: Cardiology Practice, Cardiovascular Primary Care.

The content of these European Society of Cardiology (ESC) Guidelines has been published for personal and educational use only. No commercial use is authorized. No part of the ESC

Guidelines may be translated or reproduced in any form without written permission from the ESC. Permission can be obtained upon submission of a written request to Oxford University

Press, the publisher of the European Heart Journal and the party authorized to handle such permissions on behalf of the ESC.

Disclaimer: The ESC Guidelines represent the views of the ESC and were produced after careful consideration of the scientific and medical knowledge and the evidence available at the

time of their dating.

The ESC is not responsible in the event of any contradiction, discrepancy and/or ambiguity between the ESC Guidelines and any other official recommendations or guidelines issued by

the relevant public health authorities, in particular in relation to good use of health care or therapeutic strategies. Health professionals are encouraged to take the ESC Guidelines fully into

account when exercising their clinical judgment, as well as in the determination and the implementation of preventive, diagnostic or therapeutic medical strategies. However, the ESC

Guidelines do not override, in any way whatsoever, the individual responsibility of health professionals to make appropriate and accurate decisions in consideration of each patient¡¯s

health condition and in consultation with that patient and, where appropriate and/or necessary, the patient¡¯s caregiver. Nor do the ESC Guidelines exempt health professionals from

taking careful and full consideration of the relevant official updated recommendations or guidelines issued by the competent public health authorities in order to manage each patient¡¯s

case in light of the scientifically accepted data pursuant to their respective ethical and professional obligations. It is also the health professional¡¯s responsibility to verify the applicable rules

and regulations relating to drugs and medical devices at the time of prescription.

National Cardiac Societies document reviewers: listed in Appendix 1

& The European Society of Cardiology 2014. All rights reserved. For permissions please email: journals.permissions@.

Page 2 of 9

ESC Guidelines

Document Reviewers: David Hasdai (Israel) (CPG Review Coordinator), Piotr Ponikowski (Poland) (CPG Review

Coordinator), Stephan Achenbach (Germany), Fernando Alfonso (Spain), Cristina Basso (Italy), Nuno Miguel Cardim

(Portugal), Juan Ramo?n Gimeno (Spain), Stephane Heymans (Netherlands), Per Johan Holm (Sweden), Andre Keren

(Israel), Paulus Kirchhof (Germany/UK), Philippe Kolh (Belgium), Christos Lionis (Greece), Claudio Muneretto (Italy),

Silvia Priori (Italy), Maria Jesus Salvador (Spain), Christian Wolpert (Germany), Jose Luis Zamorano (Spain).

The disclosure forms of the authors and reviewers are available on the ESC website guidelines

- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - Guideline ? Diagnosis ? Cardiac imaging ? Genetics ? Symptoms ? Heart failure ? Arrhythmia ? Left

ventricular outflow tract obstruction ? Sudden cardiac death ? Implantable cardioverter defibrillators ?

Pregnancy ? Athletes ? Hypertension ? Valve disease

Keywords

Web addenda

Web Table 1:

Summary of studies reporting prevalence and incidence of hypertrophic cardiomyopathy

First author (year)

Study design

HCM prevalence %

or

Incidence/100 000

Mean age at diagnosis

(years)

Males

(%)

Hada et al 19873

Echocardiography after screening/clinical

evaluation

0.17%

47

91%

Codd et al 19894

Registry study

0.02%

59

60%

Maron et al 19955

Population screened by echocardiography

0.17%

30

71%

Corrado et al 19986

Echocardiography after screening/clinical

evaluation

0.07%

20

91%

Maron et al 19997

Echocardiography after screening/clinical

evaluation

0.19%a

57a

51%a

Nistri et al 20038

Echocardiography after screening/clinical

evaluation

0.05%

19

Only males were studied

Population screened by echocardiography

0.16%

52

69%

Maron et al 2004

Population screened by echocardiography

0.23%

64

50%

Maro et al 200611

Echocardiography after screening/clinical

evaluation

0.19%

55

68%

Ng et al 201112

Echocardiography after screening/clinical

evaluation

0.005%

19.5

Only males were studied

Registry study

0.47/100 000

5.9

N/A

Registry study

0.32/100 000

0.47

69

Zou et al 20049

10

Lipshultz et al 200313

Nugent et al 2005

a

14

Refers only to patients with de novo diagnosis.

N/A ? not available.

Page 3 of 9

ESC Guidelines

Web Table 2: Main genes associated with familial hypertrophic cardiomyopathy (Online Mendelian Inheritance in Man

OMIM phenotypic series, 192600)21

Protein

Gene

Location

MIM gene

Frequency

Myosin-7 (?-myosin heavy chain)

MYH7

14q11.2

160760

10¨C20%

Myosin-binding protein C, cardiac-type

MYBPC3

11p11.2

600958

15¨C30%

Troponin T, cardiac muscle

TNNT2

1q32.1

191045

3¨C5%

Troponin I, cardiac muscle

TNNI3

19q13.42

191044

30 mmHg

LGE is an independent MVA performed

predictor for NSVT

(>7 fold increased

relative risk)

Maron

et al 2008143

- n = 202

- 42 years

- 55%

- HCM patients presenting to Tufts

Medical Center and Minneapolis Heart

Institute Foundation (Incomplete data

Automated

counting of

pixels ¡Ý6 SD

of myocardial

mean

1) Composite:

SD, ICD

discharge +

NYHA ¡Ý1

(1.8 years post

CMR)

1) MWT ¡Ý30 mm

associated with

2) Rest LVOT

gradient >30 mmHg adverse outcomes

Study

method

Underpowered to

show predictive

ability of LGE for

CV death

A large part of the

difference in 1¡ã

outcome between

LGE+/? is driven

by differences in HF

admissions

participants)

participants)

MVA not

performed due to

low incidence of

events resulting

from shortest FU

duration

BP ? blood pressure; CMR ? cardiovascular magnetic resonance; CV ? cardiovascular; FU ? follow-up; FWHM ? full width half maximum; HCM ? hypertrophic

cardiomyopathy; HF ? heart failure; ICD ? implantable cardioverter defibrillator; LGE ? late gadolinium enhancement; LVOTO ? left ventricular outflow tract obstruction;

MVA ? multivariable analysis; MWT ? maximal wall thickness; NSVT ? non-sustained ventricular tachycardia; NYHA ? New York Heart Association functional class; PI ? primary

investigator; RF ? risk factor; SD ? standard deviation; SCD ? sudden cardiac death; VF ? ventricular fibrillation; VT ? ventricular tachycardia.

a

High risk due to presence of ¡Ý2 RF (or ¡®malignant¡¯ family history).

b

Low risk as .75% of patients did not have any recognized clinical RF for SCD; 1 RF in n? 43 (19.5%); 2 RF in n? 7; 3 RF in n? 3.

c

Intermediate risk as 31.8% had 1 RF and 15.2% ¡Ý2 RF.

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