ESC GUIDELINES

European Heart Journal (2019) 00, 110

doi:10.1093/eurheartj/ehz467

2019 ESC Guidelines for the management of

patients with supraventricular tachycardia:
supplementary data
The Task Force for the 2019 European Society of Cardiology (ESC)
Guidelines for the management of patients with supraventricular
tachycardia

Developed in collaboration with the Association for European

Paediatric and Congenital Cardiology (AEPC)

Authors/Task Force Members: Josep Brugada* (Chairperson) (Spain),

Demosthenes G. Katritsis* (Chairperson) (Greece), Elena Arbelo (Spain),
Fernando Arribas (Spain), Jeroen J. Bax (Netherlands),
Carina Blomström-Lundqvist (Sweden), Hugh Calkins (United States of America),
Andrew Coats (United Kingdom), Domenico Corrado (Italy), Spyridon G. Deftereos
(Greece), Gerhard-Paul Diller (Germany), Juan J. Gomez-Doblas (Spain),
Bulent Gorenek (Turkey), Andrew Grace (United Kingdom),
Siew Yen Ho (United Kingdom), Juan-Carlos Kaski (United Kingdom),

*Corresponding authors: Josep Brugada, Cardiology Department, Hospital Clinic, and Pediatric Arrhythmia Unit, Hospital Sant Joan de Déu, University of Barcelona, Spain.
Tel: þ34 3460 902 2351, Fax: þ34 3493 227 1777, Email: [email protected].
Demosthenes G. Katritsis, Deaprtment of Cardiology, Hygeia Hospital, E. Stavrou 4, 15123 Athens, Greece, Tel: þ30 6944 845 505, Fax: þ30 210 6722535, Email: dkatrits@
dgkatritsis.gr.
1
Representing the AEPC.
ESC entities having participated in the development of this document:
Associations: Acute Cardiovascular Care Association (ACCA), European Association of Cardiovascular Imaging (EACVI), European Association of Preventive Cardiology
(EAPC), European Heart Rhythm Association (EHRA), Heart Failure Association (HFA).
Councils: Council for Cardiology Practice.
Working Groups: Cardiac Cellular Electrophysiology, Cardiovascular Pharmacotherapy, Cardiovascular Surgery, Development Anatomy and Pathology, Grown-up Congenital
Heart Disease.
The content of these ESC Guidelines has been published for personal and educational use only. No commercial use is authorized. No part of the ESC Guidelines may be trans-
lated 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 ([email protected]).
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 avail-
able at the time of their publication. The ESC is not responsible in the event of any contradiction, discrepancy, and/or ambiguity between the ESC Guidelines and any other offi-
cial recommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of healthcare 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 pre-
ventive, 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 into full and careful consideration 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.
C The European Society of Cardiology and the European Atherosclerosis Association 2019. All rights reserved.
V
For permissions please email: [email protected].
2 ESC Guidelines

Karl-Heinz Kuck (Germany), Pier David Lambiase (United Kingdom),

Frédéric Sacher (France), Georgia Sarquella-Brugada1 (Spain), Piotr Suwalski
(Poland), Antonio Zaza (Italy)
Document Reviewers: Tom De Potter [Committee for Practice Guidelines (CPG) Review Co-ordinator]
(Belgium), Christian Sticherling (CPG Review Co-ordinator) (Switzerland), Victor Aboyans (France),
Cristina Basso (Italy), Mario Bocchiardo (Italy), Werner Budts (Belgium), Victoria Delgado (Netherlands),
Dobromir Dobrev (Germany), Donna Fitzsimons (United Kingdom), Sofie Gevaert (Belgium),
Hein Heidbuchel (Belgium), Gerhard Hindricks (Germany), Peter Hlivak (Slovakia), Prapa Kanagaratnam
(United Kingdom), Hugo Katus (Germany), Josef Kautzner (Czech Republic), Thomas Kriebel1 (Germany),
Patrizio Lancellotti (Belgium), Ulf Landmesser (Germany), Christophe Leclercq (France), Basil Lewis
(Israel), Yuri Lopatin (Russian Federation), Béla Merkely (Hungary), Thomas Paul (Germany),
Nikola Pavlovic (Croatia), Steffen Petersen (United Kingdom), Anna Sonia Petronio (Italy),
Tatjana Potpara (Serbia), Marco Roffi (Switzerland), Daniel Scherr (Austria), Evgeny Shlyakhto
(Russian Federation), Iain A. Simpson (United Kingdom), Katja Zeppenfeld (Netherlands)

The disclosure forms of all experts involved in the development of these Guidelines are available on the
ESC website www.escardio.org/guidelines

...................................................................................................................................................................................................
Keywords Guidelines • arrhythmia • tachycardia • supraventricular • flutter • atrioventricular • re-entrant • focal
• macrore-entrant • junctional • nodal • pre-excitation • ablation

..
Table of contents .. Supplementary Figure 4 Proposed circuit of atrioventricular
.. nodal re-entrant tachycardia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
..
1 Electrophysiological mechanisms of supraventricular tachycardia . . . . . 2 .. Supplementary Figure 5 Mahaim physiology . . . . . . . . . . . . . . . . . . . . . . . . . . 7
1.1 Mechanisms of non-re-entrant arrhythmias . . . . . . . . . . . . . . . . . . . . 2
.. Supplementary Figure 6 Anatomy of the right atrium . . . . . . . . . . . . . . . . . 8
..
1.2 Re-entry . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4 .. Supplementary Figure 7 Anatomy of the left atrium . . . . . . . . . . . . . . . . . . 9
1.3 Distinguishing re-entrant from non-re-entrant arrhythmias . . . . . 4
..
..
1.4 Specificities in anatomy and function of supraventricular ..
structures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
.. 1 Electrophysiological
..
1.5 Arrhythmia mechanism and response to therapy . . . . . . . . . . . . . . . 5 ..
2 Tachycardia circuits . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
.. mechanisms of supraventricular
..
3 Cardiac anatomy for the electrophysiologist . . . . . . . . . . . . . . . . . . . . . . . 6 .. tachycardia
..
3.1 Right atrium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6 ..
3.1.1 Non-invasive imaging of the right atrium . . . . . . . . . . . . . . . . . . 6 .. 1.1 Mechanisms of non-re-entrant
.. arrhythmias
3.1.1.1 Anatomical parameters . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6 ..
3.1.1.2 Functional parameters . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6 .. Automaticity is normally suppressed in non-pacemaking myocytes by
..
3.2 Left atrium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 .. hyperpolarizing membrane currents (IK1 or IKACh) that keep diastolic
3.2.1 Non-invasive imaging of the left atrium . . . . . . . . . . . . . . . . . . . 8 .. potential constant. In addition, non-pacemaking myocytes scantily
..
3.2.1.1 Anatomical parameters . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8 .. express a diastolic depolarizing current (If), a significant contribu-
3.2.1.2 Functional parameters . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 .. tor—along with ICaL (L-type Ca2þ current)—to the physiological
..
3.3 Conduction tissues . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 .. automaticity of the sinoatrial (SAN) and atrioventricular (AVN)
3.4 Accessory pathways . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 .. nodes. In non-pacemaking myocytes, an imbalance between hyper-
..
4 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 .. polarizing and depolarizing diastolic currents can occur under patho-
.. logical conditions, and lead to enhanced automaticity (EA).1 In this
..
.. case, the abnormality is intrinsic to the cell membrane, i.e. it resides in
List of figures .. (primary or secondary) dysfunction of sarcolemmal ion channels.
..
Supplementary Figure 1 Mechanisms of arrhythmogenesis . . . . . . . . . . . . 3 .. Albeit potentially modulated by excitation of surrounding tissue, fir-
Supplementary Figure 2 Re-entry ‘wavelength’ . . . . . . . . . . . . . . . . . . . . . . . 4
.. ing of automatic myocytes is independent and, therefore, automatic
..
Supplementary Figure 3 Tachycardia circuit in different types of .. rhythms are typically dissociated from the basic one and are charac-
narrow QRS tachycardia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
.. terized by variable coupling intervals. EA is facilitated by partial
ESC Guidelines 3

membrane depolarization (e.g. by ischaemia), hypokalaemia, and sym-

.. Ca2þ activates Ca2þ-sensitive membrane currents (mainly INCX)
..
pathetic activation, which disrupt the balance between hyperpolariz- .. resulting in membrane potential (Supplementary Figure 1). Ca2þ waves
ing and depolarizing diastolic currents.
.. occur when SR stability is compromised by excessive Ca2þ loading,
..
After-depolarizations may occur during the action potential [early .. or when opening of SR Ca2þ channels (RyRs) is enhanced.3 Thus,
after-depolarizations (EADs)], or during diastole [delayed after-depo-
.. DADs are facilitated by tachycardia and adrenergic stimulation.
..
larizations (DADs)] (Supplementary Figure 1). Arrhythmias caused by .. Under specific conditions, Ca2þ waves may occur during the action
..
after-depolarizations are named ‘triggered activity’ (TA) because, .. potential, thus inducing EADs instead.3 If large enough, phase 3 EADs
unlike abnormal automaticity, they are often related to a previous .. and DADs can directly excite the myocardium (TA). Even if they fail
..
excitation that sets the conditions for their occurrence. EADs are .. do so, EADs result in strong repolarization heterogeneity, thus pro-
classically due to partial ICaL recovery and reactivation during the .. moting ‘re-entry’.2
..
action potential.1 This is facilitated by repolarization slowing (long .. A further factor in the genesis of non-re-entrant arrhythmias is
QT), but also by a loss of a proper plateau phase (action potential ‘tri- .. electrical coupling between the focus of origin and the surrounding
..
angulation’), thus explaining why EADs may also occur in atria.2 ICaL .. tissue. Electrical coupling through ‘gap junctions’ guarantees silencing
reactivation causes a membrane potential oscillation, which, if arising .. of unstable tissue by the surrounding stable one; therefore, partial
..
at a sufficiently negative membrane potential (phase 3 EADs), may .. uncoupling (e.g. by fibrosis) may unveil membrane potential instabil-
activate surrounding myocytes or, in the case of phase 2 EADs, fur- .. ity. On the other hand, propagation of automatic/TA requires the
..
ther delay repolarization. Although promoted by bradycardia, EADs .. excitation of the adjacent tissue, which implies some degree of elec-
are also enhanced by adrenergic stimulation. DADs are generated by .. trical coupling and INa availability.
..
diastolic Ca2þ release from the sarcoplasmic reticulum (SR), its intra- .. Some system components are implicated in both EA and TA,
cellular store, to form intracellular ‘Ca2þ waves’.1,3 A rise in cytosolic .. thus making the two mechanisms similarly responsive to some
.

©ESC 2019

Supplementary Figure 1 Mechanisms of arrhythmogenesis. Abnormal impulse initiation: the cardiac impulse may be theoretically initiated by a sin-
gle myocyte (in yellow); this is the case for abnormal (enhanced) automaticity and triggered activity. Whereas abnormal automaticity involves progressive
membrane depolarization during the diastole, triggered activity is generated by membrane potential oscillations occurring during the action potential (early
after-depolarizations) or the diastolic interval (delayed after-depolarizations) (see text). Abnormal impulse propagation: ectopic self-activation of the heart
is caused by circulation of the impulse along a circuit (red line), formed by multiple connected myocytes under specific conditions (see text). The circuit
head and tail may be separated by a fully excitable gap if the propagation time is adequate for full recovery of excitability; when the circuit head impinges on
partially refractory tissue (a partially excitable gap), further propagation may be slowed, thus allowing circuit perpetuation. Because of conduction aniso-
tropy, fully and partially excitable gaps may coexist within a circuit.
DADs = delayed after-depolarizations; EADs = early after-depolarizations.
4 ESC Guidelines

interventions. A good example is ICaL, which directly contributes

to automaticity, EADs, and SR loading; accordingly, both EA and
TA are enhanced by adrenergic activation and suppressed by ICaL
blockade. Thus, in terms of response to drugs, EA and TA may not
be sharply discriminated; nonetheless, it would be useful to recog-
nize the contribution of SR instability to arrhythmogenesis
because it might assist in optimizing drug therapy. For instance,
while beta-blockade may be indicated in both EA- and EAD-based
TA, carvedilol is the only beta-blocker that seems to stabilize the
SR (by inhibiting RyR channels).3

1.2 Re-entry
Propagation in the myocardium can occur, albeit with different veloc-
ities (anisotropy), in all directions. Thus, given appropriate conditions,
regions activated later in propagation may re-excite regions that have

©ESC 2019
already recovered excitability. This phenomenon requires several
conditions to be present:1
(1) An area of unexcitable tissue (obstacle) that splits propa-
gation into two fronts. The obstacle can be an ‘anatomical’ struc-
ture [valve orifices, dual AVN or accessory pathways (APs), scar
Supplementary Figure 2 Re-entry ‘wavelength’. The activation
tissue, etc.] or even ‘functional’ (e.g. collision of centripetal fronts front travels around the obstacle (anatomical or functional). For re-
emanating from the circuit). entry to continue, the circuit length (set by anatomical obstacle size
(2) Conditions preventing mutual extinction of the two or tissue functional properties) must be larger than the wavelength. In
fronts by head-to-head or head-to-tail collision. ‘Unidirectional’ this case, an excitable gap separates the circuit head from its tail. If the
conduction, in at least part of the circuit, prevents head-to-head colli- circuit length is smaller than the wavelength, the circuit head collides
sion. Head-to-tail collision is prevented by a refractory period with the tail (no excitable gap) and propagation is extinguished. CV =
shorter than the time required by the front to circumnavigate the conduction velocity; EG = excitable gap; RP = absolute refractory
obstacle (Supplementary Figure 2). Accordingly, the minimum circuit period; WL = wavelength.
length theoretically achievable [commonly named wavelength (WL)]
depends on myocardial properties as follows, where CV is conduc-
tion velocity: .. ‘breakthrough’ of transmural propagation, even high-resolution
..
WL = CV  RP .. mapping may be inadequate to distinguish them from non-re-
..
.. entrant activity. However, an educated guess can be made from
The smaller the WL, the more likely re-entry is to occur; accord- .. the response to vagal manoeuvres, adenosine, adrenergic agonist/
ingly, either conduction slowing or decreased refractoriness facilitate ..
.. antagonists, and pacing protocols.1,5
re-entry. Albeit reality may be more complex, this simple concept is ..
pivotal in understanding antiarrhythmic therapy. ..
.. 1.4 Specificities in anatomy and function
The WL concept is based on the mode of re-entry first described .. of supraventricular structures
and named ‘leading circle’ (Supplementary Figure 2);1 nonetheless, ..
.. Structural specificities may contribute to atrial arrhythmogenesis.6
functional re-entry may also take the form of ‘spiral wave’.4 While ..
too complex to be described here, spiral waves have been implicated .. For instance, automatic/TA in venous sleeves is facilitated by very
.. thin and poorly coupled myocardium, dual AVN anatomy is pivotal in
in atrial fibrillation (AF), and their properties are best suited to ..
explain the high arrhythmia rate and the antiarrhythmic efficacy of .. the genesis of AVNRT, and strong anisotropy supports preferential
.. propagation pathways in atrial flutter. The action potential of atrial
Naþ channel blockers. ..
... ‘working’ myocytes has a prominent phase 1 repolarization, which
.. makes it more ‘triangular’ than the ventricular potential. Impulse
1.3 Distinguishing re-entrant from non- .. propagation is supported by INa in atrial ‘working’ myocytes, and by
..
re-entrant arrhythmias .. ICaL in SAN and AVN myocytes. Accordingly, a sharp response of
Supraventricular rhythms with specific electrocardiographic .. propagation to ICaL inhibition (via ICaL blockade, beta-blockade, or
..
(ECG) patterns [e.g. atrial flutter, atrioventricular nodal re- .. adenosine) is peculiar to nodal tissue, whereas effective interruption
entrant tachycardia (AVNRT), and atrioventricular re-entrant .. of a primarily nodal arrhythmia by INa blockade may suggest involve-
..
tachycardia (AVRT)] are caused by re-entry over well-defined .. ment of atrial tissue in the circuit. Several ionic currents are selec-
anatomical structures; therefore, once the rhythm diagnosis is .. tively (or predominantly) expressed in supraventricular myocytes.6
..
made, re-entry can be safely assumed as the mechanism. .. Ito and IKur, depolarization-activated Kþ currents, account for the tri-
However, this is not the case for focal atrial tachycardias (ATs), in
.. angular shape of the atrial action potential, and voltage-independent
..
which both re-entry and EA/TA may be involved.5 Because re- .. SK (Ca2þ-activated) and TASK (two-pore) Kþ channels also contrib-
entrant circuits may be microscopic or simulate foci by surface
.. ute to speed atrial repolarization.7,8 I , the atrially located
KACh
ESC Guidelines 5

..
analogue of the ventricular diastolic current IK1, is activated by acetyl- .. prevented (INa blockade or focus encircling). Re-entrant arrhythmias
choline or adenosine; it accounts for the suppression of automaticity .. are suppressed by discontinuing the circuit. This is achieved physically
..
and repolarization shortening by vagal activation. Notably, the pace- .. by ablation or pharmacologically with drugs that prolong refractori-
maker current If, typical of the SAN, is also weakly expressed in .. ness (Supplementary Figure 2). To this end, it should be stressed that
..
working atrial myocytes and may support EA when enhanced by sym- .. refractoriness may be prolonged beyond repolarization by Naþ
pathetic activation. Furthermore, features of atrial INa make it exqui- .. channel blockers characterized by slow ‘off kinetics’ (class Ic action,
..
sitely sensitive to some blockers (e.g. ranolazine).9 These differences .. e.g. flecainide).10 Disease-related membrane depolarization may slow
underpin the atrial selectivity of some drug effects. .. down the ‘off kinetics’ of intrinsically ‘faster’ drugs (e.g. lidocaine in
..
.. ischaemia).11 Therefore, refractoriness prolongation does not
1.5 Arrhythmia mechanism and response .. require prolonged repolarization, i.e. it is not an exclusive response
..
to therapy .. to Kþ channel blockade (class III action). If nodal structures are
.. involved, re-entry can be prevented by interventions inhibiting ICaL
Each arrhythmia type can be terminated by multiple mechanisms; ..
hence, there is no strict relationship between arrhythmia type and .. (ICaL blockade, beta-blockade, and adenosine).
.. Haemodynamically well-tolerated, myocardial activation at inap-
response to interventions. Furthermore, each drug has multiple rele- ..
vant actions. Thus, putatively effective ‘actions’, but not ‘drugs’, should .. propriately high rates maintained over long periods may lead to
.. changes in myocyte function and tissue structure, collectively named
be associated with each arrhythmia mechanism. ..
Automatic/triggered arrhythmias are logically suppressed by inhib- .. ‘remodelling’. Once remodelling has occurred, it may facilitate
..
iting their initiation (ICaL blockade, beta-blockade, drug actions stabi- .. arrhythmias and impair contractile function (tachycardiomyopathy).
lizing the SR,3 or focus ablation), but their propagation may also be ..
..
..
2 Tachycardia circuits

©ESC 2019

Supplementary Figure 3 Tachycardia circuit in different types of narrow QRS tachycardia. The sinus node, atrioventricular node, and right and left
bundle branches are depicted. Internodal tracts have not been anatomically demonstrated. The activation from the sinus node spreads through the atrial
myocardium to the atrioventricular node. AT = atrial tachycardia; AVNRT = atrioventricular nodal re-entrant tachycardia; AVRT = atrioventricular re-
entrant tachycardia; CTI = cavotricuspid isthmus.
6 ESC Guidelines

©ESC 2019
Supplementary Figure 4 Proposed circuit of atrioventricular nodal re-entrant tachycardia. During typical atrioventricular nodal re-entrant tachycar-
dia (slowfast), right- or left-sided circuits may occur with antegrade conduction through the inferior inputs, and retrograde conduction through the superior
inputs or the anisotropic atrionodal transitional area. In atypical atrioventricular nodal re-entrant tachycardia, conduction occurs antegradely through one of
the inferior inputs, left or right, and retrogradely through the other one. Depending on the orientation of the circuit, one may record the so-called fast-slow,
slow-slow, or indeterminate types. Reproduced with permission from Katritsis DG et al.12 AAT = anisotropic atrionodal transitional area; AVNRT = atrioven-
tricular nodal re-entrant tachycardia; CS = coronary sinus; FO = foramen ovale; LI = left input; RI = right input; S = superior input; TV = tricuspid valve.

..
3 Cardiac anatomy for the ..
..
3.1.1.1 Anatomical parameters
RA size, area, and volume are routinely assessed from standard two-
electrophysiologist ..
.. dimensional (2D) echocardiography at the end of left ventricular (LV)
.. systole, just before tricuspid valve opening, providing the maximum
3.1 Right atrium ..
.. size/volume. RA size is derived from the apical four-chamber view.18
A large triangular-shaped appendage dominates this chamber, occu- .. The major long-axis distance is measured at the end of systole, from
pying its anterior and lateral parts. Internally, the appendage wall is ..
.. the centre of the tricuspid annulus to the centre of the superior RA
lined by pectinate muscles that extend from the terminal crest to .. wall (normal range for men 2.4 ± 0.3 cm/m2 and 2.5 ± 0.3 cm/m2 for
reach the smooth vestibule surrounding the tricuspid valve orifice ..
.. women). The minor distance is measured at the mid-level of the RA,
(Supplementary Figure 6).14 ..
.. from the free wall to the interatrial septum (perpendicular to the RA
In the anterior wall, the larger pectinate muscles are arranged .. long axis) (normal range for men and women 1.9 ± 0.3 cm/m2).18
nearly in parallel fashion, with thinner branches in between, leaving .. The RA area can be derived by planimetry from the apical four-cham-
areas of very thin atrial wall.15 The terminal crest sweeps like a ..
.. ber view, and RA volumes are mostly reported from 2D echocar-
twisted C-shape, originating from the septal wall, marking the ante- .. diography (normal range 21 ± 6 mL/ m2 for women and 25 ± 6 mL/
rior border of the superior caval vein entrance before descending
..
.. m2 for men),18 but ideally RA volume is assessed from three-
posteriorly and laterally, and then turning anteriorly to skirt to the .. dimensional (3D) echocardiography with higher accuracy and repro-
right side of the Eustachian valve that guards the entrance of the infe-
..
.. ducibility than 2D echocardiography. Cardiac magnetic
rior caval vein. Its distal ramifications join with the vestibule toward ..
the orifice of the coronary sinus to form the cavotricuspid (flutter) ... resonance (CMR) is the current ‘gold standard’ for volume quantifica-

isthmus, lies between the entrance of the inferior caval vein and the
.. tion. Computed tomography (CT) has also been used recently for
.. this purpose. Intracardiac echocardiography also allows detailed
tricuspid annulus (Supplementary Figure 6). ..
.. exploration of the RA structures and can be very useful for the safe
The posterior portion of the isthmus is pouch-like in 10% of .. accomplishment of transseptal puncture.19
patients.16 The terminal crest marks the border between the smooth ..
..
intercaval posterior wall of the right atrium (RA) and the appendage. .. 3.1.1.2 Functional parameters
..
.. With tissue Doppler imaging (TDI), it is possible to directly measure
3.1.1 Non-invasive imaging of the right atrium .. the velocity of the atrial myocardium, where the a’ reflects the con-
..
In patients with supraventricular tachycardia, non-invasive .. traction of the atrium. More recently, 2D strain measurements per-
imaging can provide useful information when structural (anatomical)
.. mit assessment of RA strain (active deformation).20,21 These
..
and/or functional abnormalities are present in the atria, with more .. approaches have been used extensively for the LA, but limited data
experience available for the left atrium (LA) than the RA.17
.. are available for the RA.
ESC Guidelines 7

©ESC 2019

Supplementary Figure 5 Atypical pathway physiology. Change in QRS morphology from short to long ventriculoatrial atrioventricular re-entrant
tachycardia. (A) During short ventriculoatrial atrioventricular re-entrant tachycardia (tachycardia cycle length 300 ms), there is also antegrade activation
over the left anterior fascicle to produce a fused QRS complex with a normal axis. (B) With retrograde right bundle branch block, antegrade conduction
over the left anterior fascicle is no longer possible and conduction to the left ventricle proceeds only via the right free wall. Therefore, the long ventriculoa-
trial atrioventricular re-entrant tachycardia (tachycardia cycle length 350 ms) has a leftward axis. During the change from short to long ventriculoatrial
atrioventricular re-entrant tachycardia, the QRS width also increases from 120 to 150 ms. Reproduced with permission from Gandhavadi M et al.13 A =
atrial electrogram; AF = anterior fascicle; AVN = atrioventricular node; AVRT = atrioventricular re-entrant tachycardia; LBB = left bundle branch catheter;
M = Mahaim potential; PF = posterior fascicle; RB = right bundle potential; RBB = right bundle branch catheter.

3D echocardiography has been used recently for the sophisticated

.. vein and its continuation into the coronary sinus lie outside, along the
..
assessment of atrial function. This technique allows precise quantifi- .. posteroinferior wall of the LA, and have variable extents of muscular
cation of volume shifts from the atrium to the LV and has been
.. continuity with the atrial wall.
..
applied mainly for the LA.18,21 .. The atrial wall has significant regional variations in muscular thick-
..
.. ness. The superior wall (roof) is 3.56.5 mm thick while the antero-
.. superior wall augmented by Bachmann’s bundle (interauricular
..
3.2 Left atrium .. muscle band) is even thicker. However, other regions can be as thin
The LA is the most posteriorly situated heart chamber .. as 12 mm or nearly paper thin.22
..
(Supplementary Figure 7). Internally, its smooth venous part receives .. The LA appendage is a flattened tubular structure with consider-
the pulmonary veins (PVs) into the body of the chamber and its vesti-
.. able variability in shape and size. It has a narrow opening (ostium) to
..
bule surrounds the opening to the mitral valve.22 The great cardiac . the atrial body (Supplementary Figure 7). The tip of the appendage is
8 ESC Guidelines

©ESC 2019
Supplementary Figure 6 Anatomy of the right atrium. (A) This display of the inside of the right atrium with the lateral wall of the appendage
deflected posteriorly shows the atrial septum enface and the terminal crest with its array of pectinate muscles. The sinus and atrioventricular nodes are
superimposed. The dotted line marks the position of the tendon of Todaro and the white broken line marks the annulus of the septal leaflet of the tricuspid
valve, the borders of the triangle of Koch. (B) This four-chamber section shows three lines in the cavotricuspid isthmus corresponding to the paraseptal
isthmus (1), inferior/central isthmus (2), and inferolateral isthmus (3). The short arrow indicates a pouch-like depression crossed by line 2. Courtesy of Dr
Ho. AV = atrioventricular; CS = coronary sinus; ER = Eustachian ridge; EV = Eustachian valve; ICV = inferior caval vein; LA = left atrium; OF = oval fossa;
SCV = superior caval vein; SN = sinus node; TC = terminal crest; TV = tricuspid valve.

commonly directed anteriorly, overlying the pulmonary trunk or the .. The phrenic nerves track along the lateral mediastinum, coursing
..
LV summit, where its floor sits on the early branches of the left coro- .. along the outer surface of the fibrous pericardium. The right phrenic
nary artery and the interventricular vein.23
.. nerve has a close relationship with the superior caval vein and the ante-
..
Inside the atrium, a prominent left lateral ridge lies between the ori- .. rior border of the orifice of the right superior PV. In the majority of indi-
fices of the left PVs and the ostium of the appendage (Supplementary
.. viduals, the left phrenic nerve passes over the roof or the ostial wall of
..
Figure 7). Epicardially, the ridge is a fold containing the remnant of the .. the LA appendage.28 The oesophagus is located in close proximity to
oblique vein of Marshall with its accompanying autonomic nerves, and
.. the posterior wall and the vagal plexus descends on its anterior surface
..
in some hearts also the artery supplying the sinus node.22 .. (Supplementary Figure 7).22 Furthermore, pericardial access is limited by
Although there are usually four pulmonary venous orifices, two on
.. pericardial sinuses and recesses around the veins and great arteries.
..
each side of the venous component, variations in the number of orifi- ..
..
ces, lengths of conjoined veins, and atypical locations are not uncom- ..
mon.24 The pulmonary venous trunk is defined as the distance from .. 3.2.1 Non-invasive imaging of the left atrium
..
the orifice to the first-order branch. The superior PV orifices are .. Non-invasive imaging can provide useful information on structural
larger and have a longer trunk (1920 mm).25 .. (anatomical) and/or functional abnormalities in the LA.18,20,21,29
..
Complex muscular architecture is found at the venoatrial junctions ..
where the LA wall extends over the outer surface of the venous wall, .. 3.2.1.1 Anatomical parameters
..
creating muscular sleeves, longest along the upper veins.26 The .. LA size and shape are typically assessed with standard 2D echocar-
sleeves are thicker and circumferential at the venoatrial junctions, .. diography, at the end of LV systole, before mitral valve opening.
..
especially around the superior PVs, and thinner and more irregular .. Dilatation is assessed by measuring the LA anteroposterior diameter
towards the lung hilum. At the venoatrial junction, the veins connect .. (derived from the parasternal long-axis view); however, LA dilation in
..
through subepicardially and subendocardially located myocardial .. the anteroposterior direction is limited by the thorax, and dilation
bridges crossing the interpulmonary isthmus.22 The areas of the .. occurs mostly along the long axis of the LA. Therefore, assessment of
..
venoatrial junctions and adjoining PVs are also highly innervated by .. LA volumes is preferred, which can be performed with 2D echocar-
ganglionated nerves originating from the cardiac neural plexus.27 .. diography; the reported normal values are 16 - 34 mL/m2 for both
ESC Guidelines 9

..
.. From the colour-coded tissue Doppler images, the PA-TDI interval
.. [P-wave on the (ECG) to peak a’ on TDI] reflects the electromechani-
..
.. cal delay (reflecting LA fibrosis) and predicts future AF. Also, the use
.. of 2D echo strain imaging has been used to assess active deformation,
..
.. which provides further insights into LA function.20 Recently, 3D echo-
.. cardiography has been used for sophisticated assessment of LA func-
..
.. tion: reservoir function is related to increased LA fibrosis, indicating
.. less distensibility secondary to LA fibrosis.20,21,29
..
.. Delayed-enhancement CMR has also been introduced for the
.. assessment of atrial fibrosis.30
..
..
..
.. 3.3 Conduction tissues
.. The sinus node is located in the terminal crest at the anterolateral
..
.. border of the entrance of the superior caval vein (Supplementary
.. Figure 6). Tadpole-shaped, with a broad head portion and a tapering
..
.. tail, it extends superiorly from the subepicardial location and pene-
.. trates inferiorly into the terminal crest with its tail toward the
..
.. subendocardium.32
..
©ESC 2019
.. The triangle of Koch on the endocardial aspect of the RA is an
.. established guide to the location of the atrioventricular (AV) con-
.. duction tissues.14 Its posterior border is the tendon of Todaro
..
.. within the Eustachian ridge, while its anterior border is the annu-
.. lus of the septal leaflet of the tricuspid valve, and the orifice of the
..
Supplementary Figure 7 Anatomy of the left atrium. The left .. coronary sinus marks its base. The AVN and bundle lie at its apex,
atrium of this heart has been cut in a plane along the short axis of the .. while the orifice of the coronary sinus marks its base
..
thorax through the orifices of the right and left superior pulmonary veins. .. (Supplementary Figure 6). The inferior extensions of the compact
The double-headed arrow marks the ostium of the left atrial appendage, .. AVN are thought to account for the slow pathway in nodal re-
while the asterisk marks the ridge. The oesophagus and descending aorta
..
.. entry tachycardia, although the arrangement of the cardiomyo-
pass behind the left atrium. Courtesy of Dr Ho. DAo = descending aorta; ..
LA = left atrium; LAA = left atrial appendage; LSPV = left superior pulmo- .. cytes in this area may also play a role.33
.. As there are no histologically specialized conduction tissues
nary vein; RAA = right atrial appendage; RSPV = right superior pulmonary ..
vein; SCV = superior caval vein. .. between the sinus and AVNs, the sinus impulse is transmitted prefer-
.. entially toward the AVN via muscle bundles with a well-aligned
..
.. arrangement of cardiomyocytes, and thence into the ventricles
.. through the AV conduction system.14
men and women. Higher-resolution 3D imaging techniques to assess ..
..
LA volumes include 3D echocardiography, CMR, and CT. The .. 3.4 Accessory pathways
reported normal volume values according to 3D echocardiography ..
.. Atrial and ventricular myocardial masses are separated at the AV
are 1542 mL/m2 in men and 1539 mL/m2 in women (slightly ..
higher than 2D echocardiography).18 .. junction by a tissue plane of fibro-fatty tissues at the annulus of the
.. tricuspid and mitral valves, and at the septum. Breaches of this
LA fibrosis has been assessed with echocardiography using inte- ..
grated backscatter, where greater reflectivity indicates more fibro-
.. insulating tissue plane by myocardial bundles of varying sizes, mor-
.. phologies, and number constitute AV APs that allow the atrial
sis.21,29 Reduced LA strain has also been related to LA fibrosis, as well ..
as late gadolinium contrast-enhanced CMR; the contrast agent accu-
.. impulse to reach the ventricular mass, bypassing the AV conduc-
.. tion system.34 A detailed discussion can be found in section 11.3
mulates in the extracellular space (fibrosis) in the LA, appearing as ..
white regions on the CMR images.21,29 This might enable estimation of
.. of the main text.
..
the extent of fibrosis in the LA,30 which has been related to AF recur- ..
rence after ablation. However, this assessment depends on the method
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.