Abstract: 2D
: two-dimensional
99mTc-DPD
: 99mTechnetium-3,3-diphosphono- 1,2-propanodi-carboxylic acid
ACE
: angiotensin-converting enzyme
AF
: atrial fibrillation
AL
: amyloid light chain
AR
: aortic regurgitation
ARB
: angiotensin receptor blocker
ATTR
: amyloidosis-transthyretin type
AV
: atrioventricular
BiVAD
: biventricular assist device
BNP
: brain natriuretic peptide
BPM
: Beats per minute
CCS
: Canadian Cardiovascular Society
CFC
: cardiofacialcutaneous
CHA2DS2-VASc
: Congestive Heart failure, hypertension, Age ≥75 (doubled), Diabetes, Stroke (doubled), Vascular disease, Age 65–74, and Sex (female)
CMR
: cardiac magnetic resonance
CRT
: cardiac resynchronization therapy
CRT-D
: cardiac resynchronization therapy-defibrillator
CRT-P
: Cardiac resynchronization therapy with a pacemaker
CT
: computed tomography
DC
: direct current
DNA
: deoxyribonucleic acid
E/A
: ratio of mitral peak velocity of early filling (E) to mitral peak velocity of late filling (A)
E/e’
: ratio of early transmitral flow velocity (E) to early mitral annulus velocity (e’)
EACTS
: European Association for Cardio-Thoracic Surgery
ECG
: electrocardiogram
EF
: ejection fraction
EPS
: electrophysiological study
ESC
: European Society of Cardiology
FDA
: (US) Food and Drug Administration
FHL1
: four and a half LIM domains 1
HAS-BLED
: hypertension, abnormal renal/liver function, stroke, bleeding history or predisposition, labile INR, elderly (>65 years), drugs/alcohol concomitantly
HCM
: hypertrophic cardiomyopathy
hs-cTnT
: high sensitivity cardiac troponin T
HTS
: high throughput sequencing
ICD
: implantable cardioverter defibrillator
ILR
: implantable loop recorder
INR
: international normalized ratio
IUD
: intrauterine device
LA
: left atrium
LAMP-2
: lysosome-associated membrane protein 2
LBBB
: left bundle branch block
LEOPARD
: Lentigines, ECG abnormalities, Ocular hypertelorism, Pulmonary stenosis, Abnormal genitalia, Retardation of growth, and sensory-neural Deafness
LGE
: late gadolinium enhancement
LV
: left ventricular
LVAD
: left ventricular assist device
LVH
: left ventricular hypertrophy
LVOTO
: left ventricular outlow tract obstruction
MADIT-RIT
: Multicenter Automatic Defibrillator Implantation Trial—Reduce Inappropriate Therapy
MAPK
: mitogen activated protein kinase
MELAS
: mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes
MERFF
: myoclonic epilepsy with ragged red fibres
MRA
: mineralocorticoid receptor antagonist
MYBPC3
: myosin-binding protein C, cardiac-type
MYH7
: myosin-7 (s-myosin heavy chain)
MYL3
: myosin light chain 3
NOAC
: new oral anticoagulants
NSVT
: non-sustained ventricular tachycardia
NT-proBNP
: N-terminal pro brain natriuretic peptide
NYHA
: New York Heart Association
OAC
: oral anticoagulants
o.d.
: omni die (every day)
PC-CMR
: phase contrast cardiac magnetic resonance
PDE5
: phosphodiesterase type 5
PET
: positron emission tomography
PRKAG2
: gamma-2 sub-unit of the adenosine monophosphate-activated protein kinase
RAAS
: renin angiotensin aldosterone system
RV
: right ventricular
SAM
: systolic anterior motion
SCD
: sudden cardiac death
SAA
: septal alcohol ablation
S-ICD™
: Subcutaneous lead implantable cardioverter defibrillator
SPECT
: single photon emission computed tomography
SSFP
: steady-state free precession
SVT
: supraventricular tachycardia
TOE
: transoesophageal echocardiography
TNNI3
: troponin I, cardiac muscle
TNNT2
: troponin T, cardiac muscle
TPM1
: tropomyosin alpha-1 chain
TTE
: transthoracic echocardiography
TTR
: transthyretin
VF
: ventricular fibrillation
VKA
: vitamin K antagonist
VT
: ventricular tachycardia
WHO
: World Health Organization
Guidelines summarize and evaluate all available evidence at the time of the writing process, on a particular issue with the aim of assisting health professionals in selecting the best management strategies for an individual patient, with a given condition, taking into account the impact on outcome, as well as the risk-benefit-ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help the health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.
A great number of Guidelines have been issued in recent years by the European Society of Cardiology (ESC) as well as by other societies and organisations. Because of the impact on clinical practice, quality criteria for the development of guidelines have been established in order to make all decisions transparent to the user. The recommendations for formulating and issuing ESC Guidelines can be found on the ESC website (http://www.escardio.org/guidelines-surveys/esc-guidelines/about/Pages/rules-writing.aspx). ESC Guidelines represent the official position of the ESC on a given topic and are regularly updated.
Members of this Task Force were selected by the ESC to represent professionals involved with the medical care of patients with this pathology. Selected experts in the field undertook a comprehensive review of the published evidence for management (including diagnosis, treatment, prevention and rehabilitation) of a given condition according to ESC Committee for Practice Guidelines (CPG) policy. A critical evaluation of diagnostic and therapeutic procedures was performed including assessment of the risk-benefit-ratio. Estimates of expected health outcomes for larger populations were included, where data exist. The level of evidence and the strength of recommendation of particular management options were weighed and graded according to predefined scales, as outlined in Tables 1 and 2 .
The experts of …