Ehz 467
Ehz 467
Ehz 467
Document Reviewers: Tom De Potter [Committee for Practice Guidelines (CPG) Review Coordinator]
(Belgium), Christian Sticherling (CPG Review Coordinator) (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),
The disclosure forms of all experts involved in the development of these Guidelines are available on the
ESC website www.escardio.org/guidelines
For the Supplementary Data which include background information and detailed discussion of the data
that have provided the basis for the Guidelines see https://academic.oup.com/eurheartj/article-lookup/doi/
10.1093/eurheartj/ehz467#supplementary-data
...................................................................................................................................................................................................
Keywords Guidelines • arrhythmia • tachycardia • supraventricular • flutter • atrioventricular • re-entrant • focal
• macrore-entrant • junctional • nodal • pre-excitation • ablation
..
Table of contents ..
..
9.2.1.3 QRS axis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
9.2.1.4 Chest lead concordance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
1 Preamble . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
.. 9.2.1.5 Right bundle branch block morphology . . . . . . . . . . . . . . . . . 15
..
2 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 .. 9.2.1.6 Left bundle branch block morphology . . . . . . . . . . . . . . . . . . 15
..
2.1 Evidence review . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 .. 9.2.2 Electrophysiology study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16
2.2 Relationships with industry and other conflicts of interest . . . . . . 7 .. 9.3 Irregular tachycardias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16
..
2.3 What is new in the 2019 Guidelines? . . . . . . . . . . . . . . . . . . . . . . . . . . 7 .. 10 Acute management in the absence of an established diagnosis . . . . 16
2.3.1 Change in recommendations from 2003 to 2019 . . . . . . . . . . 7 .. 10.1 Regular tachycardias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16
..
2.3.2 New recommendations in 2019 . . . . . . . . . . . . . . . . . . . . . . . . . . . 8 .. 10.1.1 Narrow QRS (=120 ms) tachycardias . . . . . . . . . . . . . . . . . . . 16
2.3.3 New revised concepts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8 .. 10.1.1.1 Haemodynamically unstable patients . . . . . . . . . . . . . . . . 16
..
3 Definitions and classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8 .. 10.1.1.2 Haemodynamically stable patients . . . . . . . . . . . . . . . . . . . 16
4 Electrophysiological mechanisms of supraventricular .. 10.1.2 Wide QRS (>120 ms) tachycardias . . . . . . . . . . . . . . . . . . . . . 18
..
tachycardia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 .. 10.1.2.1 Haemodynamically unstable patients . . . . . . . . . . . . . . . . 18
5 Cardiac anatomy for the electrophysiologist . . . . . . . . . . . . . . . . . . . . . . 10 .. 10.1.2.2 Haemodynamically stable patients . . . . . . . . . . . . . . . . . . . 18
..
6 Epidemiology of supraventricular tachycardia . . . . . . . . . . . . . . . . . . . . . 10 .. 10.2 Irregular tachycardias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
7 Clinical presentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10 .. 11 Specific types of supraventricular tachycardia . . . . . . . . . . . . . . . . . . . . 19
..
8 Initial evaluation of patients with supraventricular tachycardia . . . . . . 11 .. 11.1 Atrial arrhythmias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
9 Differential diagnosis of tachycardias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11 .. 11.1.1 Sinus tachycardia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
..
9.1 Narrow QRS (=120 ms) tachycardias . . . . . . . . . . . . . . . . . . . . . . . . 11 .. 11.1.1.1 Physiological sinus tachycardia . . . . . . . . . . . . . . . . . . . . . . . 19
9.1.1 Electrocardiographic differential diagnosis . . . . . . . . . . . . . . . . 11
.. 11.1.1.2 Inappropriate sinus tachycardia . . . . . . . . . . . . . . . . . . . . . . 19
..
9.1.1.1 Initiation and termination of the tachycardia . . . . . . . . . . . . 11 .. 11.1.1.2.1 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
9.1.1.2 Regularity of tachycardia cycle length . . . . . . . . . . . . . . . . . . 11
.. 11.1.1.2.2 Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
..
9.1.1.3 P/QRS relationship . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11 .. 11.1.1.3 Sinus node re-entrant tachycardia . . . . . . . . . . . . . . . . . . . . 20
9.1.2 Vagal manoeuvres and adenosine . . . . . . . . . . . . . . . . . . . . . . . . 13
.. 11.1.1.3.1 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
..
9.1.3 Electrophysiology study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14 .. 11.1.1.3.2 Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
9.2 Wide QRS (>120 ms) tachycardias . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
.. 11.1.1.4 Postural orthostatic tachycardia syndrome . . . . . . . . . . . . 20
..
9.2.1 Electrocardiographic differential diagnosis . . . . . . . . . . . . . . . . 14 .. 11.1.1.4.1 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
..
9.2.1.1 Atrioventricular dissociation . . . . . . . . . . . . . . . . . . . . . . . . . . . 14 .. 11.1.1.4.2 Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
9.2.1.2 QRS duration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14 .. 11.1.2 Focal atrial tachycardia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
..
ESC Guidelines 3
11.1.2.1 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
.. 12.2 Catheter and surgical ablation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
..
11.1.2.2 Acute therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 .. 12.3 Specific disease states . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
..
11.1.2.3 Catheter ablation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 .. 12.3.1 Atrial septal defect . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
11.1.2.4 Chronic therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 .. 12.3.2 Ebstein’s anomaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
..
11.1.3 Multifocal atrial tachycardia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24 .. 12.3.3 Transposition of the great arteries (dextro-transposition
11.1.3.1 Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24 .. of the great arteries) after atrial switch operation
..
..
HFrEF Heart failure with reduced ejection fraction .. responsible health professional(s) in consultation with the patient
HPS HisPurkinje system
.. and caregiver as appropriate.
..
HV Hisventrcular interval .. A great number of guidelines have been issued in recent years by
ICaL L-type Ca2þ current
.. the European Society of Cardiology (ESC), as well as by other soci-
..
ICD Implantable cardioverter defibrillator .. eties and organisations. Because of their impact on clinical practice,
IST Inappropriate sinus tachycardia
.. quality criteria for the development of guidelines have been estab-
..
Wording to use
Classes of recommendations
Class I Evidence and/or general agreement Is recommended or is indicated
that a given treatment or procedure is
© ESC 2019
given treatment or procedure is not
useful/effective, and in some cases
may be harmful.
slides, booklets with essential messages, summary cards for non- .. favourably influenced by the thorough application of clinical
..
specialists and an electronic version for digital applications .. recommendations.
(smartphones, etc.). These versions are abridged and thus, for more
.. Health professionals are encouraged to take the ESC Guidelines
..
detailed information, the user should always access to the full .. fully into account when exercising their clinical judgment, as well as in
text version of the Guidelines, which is freely available via the
.. the determination and the implementation of preventive, diagnostic
..
ESC website and hosted on the EHJ website. The National Societies .. or therapeutic medical strategies. However, the ESC Guidelines do
of the ESC are encouraged to endorse, translate and implement
.. not override in any way whatsoever the individual responsibility of
..
all ESC Guidelines. Implementation programmes are needed .. health professionals to make appropriate and accurate decisions in
because it has been shown that the outcome of disease may be
.. consideration of each patient’s health condition and in consultation
.
ESC Guidelines 7
Table 3 Continued i.v. amiodarone is not recommended for pre-excited AF. III
Performance of an EPS to risk-stratify individuals with
2003 2019 IIa
asymptomatic pre-excitation should be considered.
Verapamil and diltiazem I IIa
Catheter ablation is recommended in asymptomatic
Beta-blockers I IIa patients in whom electrophysiology testing with the
Digitalis is not mentioned in the 2019 Guidelines use of isoprenaline identifies high-risk properties, such I
Table 5 Conventional classification of supraventricular Table 6 Differential diagnosis of narrow and wide QRS
tachycardias tachycardias
..
5 Cardiac anatomy for the ..
..
various questionnaires are useful in the audit of ablation techniques.
Women are more often prescribed antiarrhythmic drugs before abla-
electrophysiologist ..
.. tion for SVT than men,28 and recurrence rates following AVNRT
.. ablation are higher in young women.29 However, overall, no signifi-
Knowledge of anatomical structures inside and outside the atrial ..
chambers is of clinical importance, especially when interventional .. cant differences in health-related quality of life or access to healthcare
.. resources between men and women have been reported.28
procedures are being considered. A detailed discussion is provided in ..
..
..
tend to last longer than AT episodes, which may occur in a series of .. activation of the His bundle can also occur in high septal VTs, thus
repetitive runs.32 Clear descriptions of pounding in the neck (the so- .. resulting in relatively narrow QRS complexes (110140 ms).44
..
called ‘frog sign’) or ‘shirt flapping’ would point to the possible com- ..
peting influences of atrial and ventricular contraction on the tricuspid ..
..
valve, and to AVNRT as a likely cause.15,38,39 .. 9.1.1 Electrocardiographic differential diagnosis
SVT may be unrecognized at initial medical evaluation and the clini- .. In the absence of an ECG recorded during the tachycardia, a 12 lead
..
cal characteristics can mimic panic disorder.40 In patients with possi-
Regular
Yes No
AF
Focal AT or flutter with variable AV conduction
No Visible Multifocal AT
P waves?
Yes
Atrial rate
greater than
ventricular rate?
Yes No
Yes No
High septal VT
JET Consider
AVNRT (rare) RP Interval
Nodoventricular/fascicular-nodal
re-entry (rare)
Typical AVNRT
AVRT Focal AT
©ESC 2019
Focal AT
Atypical AVNRT AVRT
JET
Focal AT Atypical AVNRT
AVRT (rare)
Figure 1 Differential diagnosis of narrow QRS tachycardia. Recording of a retrograde P wave should be sought by obtaining a 12 lead
Electrocardiogram and, if necessary, using the Lewis leads or even an oesophageal lead connected to a pre-cordial lead (V1) with use of alligator clamps.
The 90 ms cut-off is a rather arbitrary number used for surface electrocardiogram measurements if P waves are visible and is based on limited data. In the
electrophysiology laboratory, the cut-off of the ventriculoatrial interval is 70 ms. Junctional ectopic tachycardia may also present with atrioventricular dis-
sociation.
AF = atrial fibrillation; AT = atrial tachycardia; AV = atrioventricular; AVNRT = atrioventricular nodal re-entrant tachycardia; AVRT = atrioventricular re-
entrant tachycardia; JET = junctional ectopic tachycardia; RP = RP interval; VT = ventricular tachycardia.
ESC Guidelines 13
tachycardia with
injection may help in clinical diagnosis, particularly in situations in Micro-re-entrant
transient high-grade
focal AT
which the ECG during tachycardia is unclear. Possible responses to AV block
vagal manoeuvres and adenosine are shown in Table 8 and Figure 2.
Termination of the arrhythmia with a P wave after the last QRS Figure 2 Responses of narrow-complex tachycardias to adenosine.
complex is very unlikely in AT, and most common in AVRT and typi- AT = atrial tachycardia; AV = atrioventricular; AVNRT = atrioventricular
cal AVNRT. Termination with a QRS complex is often seen in AT, nodal re-entrant tachycardia; AVRT = atrioventricular re-entrant tachy-
and possibly in atypical AVNRT. Adenosine does not interrupt mac- cardia; DADs = delayed after-depolarizations; VT = ventricular
ro-re-entrant ATs (MRATs).61 Fascicular VTs, in particular, are tachycardia.
14 ESC Guidelines
..
9.2.1.5 Right bundle branch block morphology .. the S wave, and delayed nadir of the S wave are strong predictors
Lead V1: Typical RBBB aberrancy has a small initial r’, because in .. of VT.
..
RBBB the high septum is activated primarily from the left septal bun- .. Lead V6: In true LBBB, no Q wave is present in the lateral precor-
dle. Therefore, the following patterns are evident: rSR0 , rSr0 , or rR0 in .. dial leads. Therefore, the presence of any Q or QS wave in lead V6
..
lead V1. However, in VT, the activation wavefront progresses from .. favours VT, indicating that the activation wavefront is moving away
the left ventricle (LV) to the right precordial lead V1, in a way that a .. from the LV apical site.
..
prominent R wave (monophasic R, Rsr0 , biphasic qR complex, or .. These morphology criteria are not fulfilled in any lead in 4% of
broad R >40 ms) will be more commonly seen in lead V1. .. SVTs and 6% of VTs, and in one-third of cases when one lead (V1 or
..
Additionally, a double-peaked R wave (M pattern) in lead V1 favours .. V6) favours one diagnosis, the other favours the opposite diagnosis
VT if the left peak is taller than the right peak (the so-called ‘rabbit .. (VT in one lead and SVT in the other, and vice versa).69,70
..
ear’ sign). A taller right rabbit ear characterizes the RBBB aberrancy .. A number of algorithms have been developed to differentiate
but does not exclude VT. .. VT from SVT.69,71,72 Detailed presentation and comments are
..
Lead V6: A small amount of normal right ventricular voltage is .. beyond the scope of these Guidelines, and can be found in the
directed away from lead V6. As this is a small vector in RBBB aber-
.. 2018 European Heart Rhythm Association/Heart Rhythm
..
rancy, the R:S ratio is >1. In VT, all of the right ventricular voltage, and .. Society/Asia Pacific Heart Rhythm Society/Sociedad
some of the left, is directed away from V6, leading to an R:S ratio <1
.. Latinoamericana de Estimulaci on Cardıaca y Electrofisiologıa
..
(rS and QS patterns). An RBBB morphology with an R:S ratio in V6 of .. consensus document.3
<1 is seen rarely in SVT with aberrancy, mainly when the patient has
.. All of these criteria have limitations. Conditions such as bundle
..
a left axis deviation during sinus rhythm. .. branch re-entrant tachycardia, fascicular VT, VT with exit site close
Differentiating fascicular VT from SVT with bifascicular block
..
.. to the HisPurkinje system, and wide QRS tachycardia occurring
(RBBB and left anterior hemiblock) is very challenging. Features that .. during antiarrhythmic drug treatment are difficult to diagnose using
..
indicate SVT in this context include QRS >140 ms, r’ in V1, overall .. the mentioned morphological criteria. Differentiating VT from anti-
negative QRS in aVR, and an R/S ratio >1 in V6.44 .. dromic AVRT is extremely difficult for the very fact that the QRS
..
.. morphology in antidromic AVRT is similar to that of a VT, with its ori-
9.2.1.6 Left bundle branch block morphology .. gin at the insertion of the AP in the ventricular myocardium. An algo-
..
Lead V1: As stated above for RBBB, for the same reasons, the .. rithm has been derived for differential diagnosis, based on the analysis
presence of a broad R wave, slurred or notched-down stroke of .. of 267 wide QRS tachycardias, consisting of VT and antidromic
16 ESC Guidelines
AVRT. The derived criteria were found to offer sensitivity of 75% and Recommendations for the acute management of nar-
specificity of 100%,73 and the algorithm was also validated in another row QRS tachycardia in the absence of an established
study,74 but experience is still limited. diagnosis
In fact, several independent studies have found that various ECG-
based methods have specificities of 4080% and accuracies of Recommendation Classa Levelb
75%.44,68,7580 Indeed, a similar diagnostic accuracy of 75% would Haemodynamically unstable patients
..
.. than smaller distal veins.108 Dosing should then be incremental, start-
Narrow QRS .. ing at 6 mg in adults followed by 12 mg. An 18 mg dose should then
..
tachycardia .. be considered, also taking into account tolerability/side effects in the
.. individual patient. Adenosine has a very short plasma half-life due to
..
.. enzymatic deamination to inactive inosine being achieved in seconds,
.. with end-organ clinical effects complete within 2030 s.107 Thus,
Haemodynamic ..
repeat administration is safe within 1 min of the last dose.2,3 The dose
©ESC 2019
mended in haemodynamically unstable I B
patients.86,130
If ineffective
Haemodynamically stable patients
A 12 lead ECG during tachycardia is Figure 5 Acute therapy of wide complex tachycardia in the absence of
I C
recommended. an established diagnosis.
Vagal manoeuvres are recommended. I C AVRT = atrioventricular re-entrant tachycardia; i.v. = intravenous.
Adenosine should be considered if vagal
manoeuvres fail and there is no pre-excitation IIa C
on a resting ECG. .. 10.1.2.2 Haemodynamically stable patients
Procainamide (i.v.) should be considered if
..
IIa B .. In a patient with wide QRS tachycardia who is haemodynamically sta-
vagal manoeuvres and adenosine fail.132 .. ble, the response to vagal manoeuvres may provide insight into the
..
Amiodarone (i.v.) may be considered if vagal
IIb B .. mechanism responsible for the arrhythmia. SVT with aberrancy, if
manoeuvres and adenosine fail.132 .. definitively identified, may be treated in the same manner as narrow
..
Synchronized DC cardioversion is recom- .. complex SVT, with vagal manoeuvres or drugs (adenosine and other
mended if drug therapy fails to convert or I B ..
.. AVN-blocking agents such as beta-blockers or calcium channel
control the tachycardia.86,130 .. blockers).117119,129
..
Verapamil is not recommended in wide QRS- .. Some drugs used for the diagnosis or treatment of SVT (e.g. vera-
complex tachycardia of unknown III B .. pamil) can cause severe haemodynamic deterioration in patients with
..
aetiology.64,133,134 .. a previously stable VT.64,133,134 Thus, they should only be used for
.. the treatment of patients in whom the diagnosis of SVT is fully estab-
DC = direct current; ECG = electrocardiogram; i.v. = intravenous. ..
a
Class of recommendation. .. lished and secure. Adenosine may be helpful by means of allowing a
b
Level of evidence. .. diagnosis or interrupting an adenosine-sensitive VT, but it must be
..
.. avoided if pre-excitation on resting ECG suggests a pre-excited
.. tachycardia. There is a risk that in antidromic re-entry, adenosine
..
10.1.2.1 Haemodynamically unstable patients .. may precipitate cardiac arrest if there is induction of AF by adenosine,
Haemodynamic instability may occur with any wide QRS tachycardia, .. as may occasionally occur.135
..
regardless of the cause, but is more likely in patients with VT. .. For pharmacological termination of a haemodynamically stable
Synchronized cardioversion is recommended for any persistent wide .. wide QRS-complex tachycardia of unknown aetiology, i.v. procaina-
..
QRS tachycardia resulting in hypotension, acutely altered mental sta- .. mide or amiodarone can be used in-hospital.132,136138 In the
tus, chest pain, acute HF symptoms, or signs of shock.86,87
.. PROCAMIO trial132 in patients with well-tolerated wide QRS
..
.
ESC Guidelines 19
©ESC 2019
ers (verapamil or diltiazem) in the absence of diltiazem ablation
IIb C (IIb C) (IIa C)
HFrEF, may be considered in symptomatic If ineffective
163
patients.
Catheter ablation should be considered in Figure 6 Therapy of sinus tachycardias.
symptomatic patients who do not respond to IIa C
drug therapy.164166
Postural orthostatic tachycardia syndrome
A regular and progressive exercise pro-
.. 11.1.1.3.2 Therapy. Medical treatment is empirical, and no drugs have
IIa B ..
gramme should be considered.167169 .. been studied in controlled trials. Verapamil and amiodarone have
.. demonstrated variable success, whereas beta-blockers are often inef-
The consumption of <_23 L of water and ..
1012 g of sodium chloride daily may be IIb C .. fective.163 Sinus node re-entrant tachycardia may be effectively and
.. safely treated with catheter ablation targeting the site of earliest atrial
considered.170,171 ..
Midodrine, low-dose non-selective beta-
.. activation with respect to the P wave. This approach has been shown
.. to be feasible with a good long-term outcome.164166
blocker, or pyridostigmine may be IIb B ..
considered.167,170,172174
..
.. 11.1.1.4 Postural orthostatic tachycardia syndrome
Ivabradine may be considered.175 IIb C ..
.. POTS is defined as a clinical syndrome usually characterized by an
HFrEF = heart failure with reduced ejection fraction.
.. increase in heart rate of >_30 b.p.m. when standing for >30 s (or >_40
a
..
Class of recommendation. .. b.p.m. in individuals aged 12 - 19 years) and an absence of orthostatic
b
Level of evidence. ..
.. hypotension (>20 mmHg drop in systolic blood pressure).177,178
.. The prevalence of POTS is 0.2% and it represents the most com-
..
11.1.1.3 Sinus node re-entrant tachycardia .. mon cause of orthostatic intolerance in the young,178 with most
.. patients aged between 15 and 25 years, and >75% being female.
Sinus node re-entrant tachycardia arises from a re-entry circuit ..
involving the sinus node and, in contrast to IST, is characterized by .. Although the long-term prognosis of POTS has been poorly
.. explored, 50% of patients spontaneously recover within 1 - 3 years.
paroxysmal episodes of tachycardia.176 This uncommon arrhythmia ..
may be associated with paroxysmal symptoms of palpitation, dizzi- .. A number of mechanisms have been described in patients with
.. POTS, including autonomic nervous system dysfunction,
ness, and light-headedness. On the ECG, the polarity and configura- ..
tion of the P waves are similar to the configuration of sinus .. peripheral autonomic denervation, hypovolaemia, hyperadrenergic
.. stimulation, diabetic neuropathy, deconditioning, anxiety, and
P waves.163 ..
.. hypervigilance.178181
..
11.1.1.3.1 Diagnosis. The diagnosis of sinus node re-entrant tachycar- ..
dia is suspected on ECG and Holter ECG. It can be confirmed with .. 11.1.1.4.1 Diagnosis. POTS is diagnosed during a 10 min active stand
.. test or head-up tilt test with non-invasive haemodynamic
an EPS. ..
ESC Guidelines 21
..
monitoring. The evaluation of a patient suspected of having POTS .. sustained or incessant. Dynamic forms with recurrent interruptions
should eliminate other causes of sinus tachycardia such as hypovo- .. and reinitiations may be frequent.
..
laemia, anaemia, hyperthyroidism, pulmonary embolus, or pheo- .. In patients with PV-related AT, the focus is located at the ostium
chromocytoma.178 The clinical history should focus on defining .. of the vein (or within 1 cm of the designated ostium)184 rather than
..
the chronicity of the condition, possible causes of orthostatic .. further distally (24 cm).185
tachycardia, modifying factors, impact on daily activities, and ..
..
A B C
Acute therapy
Haemodynamically unstable patients
Synchronized DC cardioversion is recommended for haemodynamically unstable patients.8688 I B
Haemodynamically stable patients
Adenosine (618 mg i.v. bolus) should be considered.9294 IIa B
187,188
Beta-blockers (i.v. esmolol or metoprolol) should be considered in the absence of decompensated HF, if adenosine fails. IIa C
Verapamil or diltiazem (i.v.) should be considered for haemodynamically stable patients in the absence of hypotension or HFrEF,
IIa C
if adenosine fails.92,94
If the above measures fail, the following may be used:
• i.v. ibutilide191;
IIb C
• or i.v. flecainide or propafenone189,190;
• or i.v. amiodarone.192
Synchronized DC cardioversion is recommended when drug therapy fails to convert or control the tachycardia.87,88 I B
Chronic therapy
Catheter ablation is recommended for recurrent focal AT, especially if incessant or causing TCM.184,187,194197 I B
Beta-blockers or non-dihydropyridine calcium channel blockers (verapamil or diltiazem in the absence of HFrEF), or propafenone or fle-
IIa C
cainide in the absence of structural or ischaemic heart disease, should be considered if ablation is not desirable or feasible.188190,198
Ivabradine with a beta-blocker may be considered if the above measures fail.199,200 IIb C
Amiodarone may be considered if the above measures fail.201,202 IIb C
i.v. verapamil and diltiazem are contraindicated in the presence of hypotension or HFrEF.
i.v. beta-blockers are contraindicated in the presence of decompensated heart failure.
i.v. ibutilide is contraindicated in patients with prolonged QTc interval.
i.v. flecainide and propafenone are contraindicated in patients with ischaemic or structural heart disease. They also prolong the QTc interval but much less than class III agents.
i.v. amiodarone prolongs the QTc but torsades des pointes is rare.
AT = atrial tachycardia; DC = direct current; HF = heart failure; HFrEF = heart failure with reduced ejection fraction; i.v. = intravenous; TCM = tachycardiomyopathy.
a
Class of recommendation.
b
Level of evidence.
ESC Guidelines 23
..
the culprit PV along with other PVs may be preferred. Catheter .. blockers may be effective, and there is a low risk of side effects
ablation is reported to have a 75100% success rate.184,187,194197 .. (Figure 9).188 Class IC drugs may be effective if first-line therapy has
..
Table 11 presents a synopsis of success rates and complications of .. failed.189,190,198 Ivabradine may also be effective in focal AT,199,200 and
catheter ablation for the most common SVTs in the current .. ideally should be given with a beta-blocker. Amiodarone has been
..
era.1113,203208 .. tried in the young and paediatric populations,201,202 and theoretically
.. should be attractive in patients with impaired LV function. However,
..
..
Focal AT
Focal AT
Recurrent or
incessant
Haemodynamic
instability Yes No
No Yes
Catheter ablation Drug therapy
(I B) No desirable
Adenosine Synchronized
(IIa B) cardioversion
(I B) Yes
If ineffective If ineffective
Beta-blocker or
verapamil or diltiazem
or propafenone
or flecainide
i.v. verapamil i.v. (IIa C)
or diltiazem beta-blocker
(IIa C) (IIa C) If ineffective
©ESC 2019
©ESC 2019
or propafenone Amiodarone
or amiodarone (IIb C)
If ineffective
(IIb C)
or not tolerated
Figure 8 Acute therapy of focal atrial tachycardia. Figure 9 Chronic therapy of focal atrial tachycardia.
AT = atrial tachycardia; i.v. = intravenous. AT = atrial tachycardia.
Table 11 Average success and complication rates of catheter ablation for supraventricular tachycardia
Acute success (%) Recurrence (%) Complications (%) Mortality (%)
Focal AT 85 20 1.4a 0.1
Cavotricuspid-dependent atrial flutter 95 10 2b 0.3
AVNRT 97 2 0.3c 0.01
d
AVRT 92 8 1.5 0.1
Success rates, recurrence, and complications for focal atrial tachycardia and atrioventricular re-entrant tachycardia vary, being dependent on the location of the focus or path-
way, respectively.1113,203208
a
Vascular complications, AV block, and pericardial effusion.
b
Vascular complications, stroke, myocardial infarction, and pericardial effusion.
c
Vascular complications, AV block, and pericardial effusion.
d
Vascular complications, AV block, myocardial infarction, pulmonary thromboembolism, and pericardial effusion.
AT = atrial tachycardia; AV = atrioventricular; AVNRT = atrioventricular nodal re-entrant tachycardia; AVRT = atrioventricular re-entrant tachycardia.
24 ESC Guidelines
..
11.1.3 Multifocal atrial tachycardia .. dysfunction, or AV block. There has also been a case report on the
Multifocal AT is defined as a rapid, irregular rhythm with at least three .. successful use of ibutilide.215 In symptomatic and medically refractory
..
distinct morphologies of P waves on the surface ECG. Multifocal AT is .. cases in which LV deterioration is seen, AV nodal modification may
commonly associated with underlying conditions, including pulmonary
.. be used for the control of ventricular rate.216
..
disease, pulmonary hypertension, coronary disease, and valvular heart ..
disease, as well as hypomagnesaemia and theophylline therapy.209 It
..
..
..
Acute therapy
Treatment of an underlying condition is rec-
I C
A B
ommended as a first step, if feasible.209
i.v. beta-blockers, or i.v. non-dihydropyridine
calcium channel blockers (verapamil or diltia- IIa B
zem) should be considered.213,214
Chronic therapy
Oral verapamil or diltiazem should be consid-
ered for patients with recurrent symptomatic IIa B
multifocal AT in the absence of HFrEF.217,218
A selective beta-blocker should be considered
for patients with recurrent symptomatic multi- IIa B
focal AT.214,219
AV nodal ablation followed by pacing (prefera-
ble biventricular or His-bundle pacing) should
be considered for patients with LV dysfunction IIa C
due to recurrent multifocal AT refractory to
drug therapy.216
©ESC 2019
Anticoagulation, as in AF, is recommended for patients with atrial flutter and concomitant AF.4 I B
Patients with atrial flutter without AF should be considered for anticoagulation, but the threshold for initiation has not been
IIa C
established.241247
Acute therapy
Haemodynamically unstable patients
Synchronized DC cardioversion is recommended for haemodynamically unstable patients.248,249 I B
Haemodynamically stable patients
i.v. ibutilide or i.v. or oral (in-hospital) dofetilide are recommended for conversion to sinus rhythm.250257 I B
248,249
Low-energy (<_100 J biphasic) electrical cardioversion is recommended for conversion to sinus rhythm. I B
High-rate atrial pacing is recommended for termination of atrial flutter in the presence of an implanted pacemaker or
I B
defibrillator.258260
i.v. beta-blockers or non-dihydropyridine calcium channel blockers (verapamil or diltiazem) (i.v.), should be considered for control of
IIa B
rapid ventricular rate.235238
Invasive and non-invasive high-rate atrial pacing may be considered for termination of atrial flutter.258,261 IIb B
239,240
i.v. amiodarone may be tried if the above are not available or desirable. IIb C
Propafenone and flecainide are not recommended for conversion to sinus rhythm.250 III B
Chronic therapy
Catheter ablation should be considered after the first episode of symptomatic typical atrial flutter.262,263 IIa B
Catheter ablation is recommended for symptomatic, recurrent episodes of CTI-dependent flutter.262264 I A
Catheter ablation in experienced centres is recommended for symptomatic, recurrent episodes of non-CTI-dependent
I B
flutter.224,265269
Catheter ablation is recommended in patients with persistent atrial flutter or in the presence of depressed LV systolic function due
I B
to TCM.233,234
Beta-blockers or non-dihydropyridine calcium channel blockers (verapamil or diltiazem, in the absence HFrEF) should be considered
IIa C
if ablation is not desirable or feasible.237,270
Amiodarone may be considered to maintain sinus rhythm if the above measures fail.263 IIb C
AV nodal ablation with subsequent pacing (‘ablate and pace’), either biventricular or His-bundle pacing, should be considered if all the
IIa C
above fail and the patient has symptomatic persistent macro-re-entrant atrial arrhythmias with fast ventricular rates.
i.v. verapamil and diltiazem are contraindicated in the presence of hypotension or HFrEF.
i.v. beta-blockers are contraindicated in the presence of decompensated heart failure.
i.v. ibutilide, and i.v. and oral dofertilide are contraindicated in patients with prolonged QTc interval.
i.v. amiodarone prolongs the QTc but torsades des pointes is rare.
AF = atrial fibrillation; AV = atrioventricular; CTI = cavotricuspid isthmus; DC = direct current; HFrEF = heart failure with reduced ejection fraction; i.v. = intravenous; LV = left
ventricular; TCM = tachycardiomyopathy.
a
Class of recommendation.
b
Level of evidence.
26 ESC Guidelines
Atrial flutter/MRAT
No Yes
Synchronized
Rhythm control cardioversion
strategy (I B)
No Yes
i.v. beta-blocker
or Electrical
i.v. diltiazem or verapamil cardioversion
(IIa B) preferred
Yes No
Low-energy
synchronized cardioversion PPM/ICD
(I B) present?
No Yes
i.v. ibutilide
Ηigh-rate
or dofetilide i.v. or
atrial pacing
oral (in-hospital)
(I B)
(I B)
If not available
or contra-indicated
Invasive or non-invasive
i.v. amiodarone
©ESC 2019
..
flutter and even the combination of AVN-blocking drugs (digoxin, .. compared with those in AF.247,282 The thrombo-embolic risk of
beta-blockers, and calcium channel blockers)235238 may fail, mak- .. atrial flutter, although lower than that of AF,246 is still signifi-
..
ing cardioversion to sinus rhythm necessary. Dofetilide and ibuti- .. cant.241244 That, together with the association with AF, justifies
lide, pure class III antiarrhythmic drugs, are generally effective in .. thromboprophylaxis, and anticoagulation has been recom-
..
interrupting atrial flutter in i.v. administration (dofetilide may be .. mended as in AF.2,3 These recommendations extend to the acute
also given orally for this purpose), while class IA and IC drugs have .. setting for cardioversion when flutter lasts for >48 h.278
..
little or no effect.250257 Class IC antiarrhythmic drugs should not ..
Atrial flutter/MRAT
Symptomatic and
recurrent
Drug therapy
desirable
Yes No
Beta-blocker Catheter
or diltiazem
ablation
or verapamil (IIa B)
(IIa C)
If ineffective
©ESC 2019
Catheter Catheter ablation
ablation in experienced
(I A) centres (I B)
..
RA MRAT may also occur in the absence of previous interven- .. Owing to its widespread use, AF ablation is the procedure that fre-
tion. Most of these are sustained around areas of ‘electrical .. quently causes the lesions able to sustain re-entry circuits, usually after
..
silence’ in the RA free wall, probably due to fibrosis.224,264,266 .. linear ablation or extensive defragmentation. Pre-existing atrial disease
Atypical atrial flutter could also arise from upper loop re-entry in .. is also predictive of macro-re-entry.293 Localized segmental PV dis-
..
the right atrium with conduction through the gap in the crista .. connection may cause focal tachycardias,294 and circumferential antral
terminalis.269
.. ablation may also create MRAT due to gaps in the lines.295299 AT
..
Rate control is often difficult due to the regularity and usually .. due to a small re-entrant circuit after ablation of AF may possibly be
slow rate of the tachycardia. Antiarrhythmic drugs are often inef-
.. distinguished from macro-re-entry by a shorter P-wave duration. RA
..
fective, or their use is limited because of structural heart disease .. MRATs have a higher incidence of negative polarity in at least one pre-
and comorbidities. Radiofrequency ablation of often several crit-
.. cordial lead compared with LA macro-re-entry.300302
..
ical isthmuses is the most effective treatment. Circuits around .. Atrial circuits are also created after surgery for different condi-
scars of longitudinal atriotomy can be mapped and ablated with
.. tions, including mitral valve disease, and are related to incisions or
..
good long-term results.267,292 However, owing to the complexity .. cannulation.303 Surgery to treat AF may also result in macro-re-entry
..
of possible substrates and difficulty of reaching critical isthmuses, .. circuits and focal AT.304
ablation procedures for these patients should be restricted to .. Circuits causing atypical left MRAT may also occur in the LA with-
..
experienced operators and centres. .. out prior intervention, commonly, but not invariably, associated with
.. significant left heart disease.305 These are based on areas of electrical
..
11.1.4.2.2 Left atrium macro2re-entrant atrial tachycardia. Circuits .. silence, probably due to fibrosis, engaging anatomical obstacles such
sustaining LA atypical flutter/MRAT are most usually due to electri- .. as the ostia of the PVs or the mitral annulus, and may be ablated by
..
cally silent areas of abnormal tissue, following medical interventions .. interrupting critical isthmuses.265,306 Circuits may also happen in the
or progressive atrial degeneration/fibrosis.268 Anatomical obstacles .. LA septum due to slow conduction caused by atrial disease or antiar-
..
such as the ostia of PVs, and mitral annulus, are often involved. ... rhythmic drugs.307
ESC Guidelines 29
..
Peri-mitral flutter, sometimes incorporating silent areas at the .. circuit for all forms of AVNRT based on the concept of atrionodal
roof of the LA, is ablated in a similar way to peri-tricuspid circuits. .. inputs have been proposed.47,317
..
However, the deployment of a stable line of block at critical isth- .. Onset of AVNRT seems to occur bimodally over time. In
muses is more challenging.308310 Circuits around the PVs are
.. many patients, attacks indeed manifest early in life, whereas in a sub-
..
also frequently recognized and ablated.280,295,296 Intervention to .. stantial proportion of patients AVNRT starts later, e.g. in the
treat these tachycardias after the initial procedure should be
.. fourth or fifth decade of life.318 One-half of the patients with minimal
..
..
A B C
I
II
III
aVR
aVL
aVF
V1
V2
V3
V4
V5
V6
©ESC 2019
25 mm/sec
Figure 13 Atrioventricular nodal re-entrant tachycardia. (A) Typical atrioventricular nodal re-entrant tachycardia. (B) Atypical atrioventricular nodal re-
entrant tachycardia. (C) Atypical AVNRT with (unusual) left bundle branch block aberration. Retrograde P waves are indicated by arrows.
30 ESC Guidelines
11.2.1.1.2 Electrophysiology study. Heterogeneity of both fast and Recommendations for the management of atrioventric-
slow conduction patterns has been well described, and all forms of ular nodal re-entrant tachycardia (AVNRT)
AVNRT may display anterior, posterior, and middle, or even LA ret-
Recommendation Classa Levelb
rograde, activation patterns.322324 Thus, specific electrophysiologi-
cal manoeuvres may be required for differential diagnosis of typical Acute therapy
and, especially, atypical AVNRT from focal AT or AVRT due to a Haemodynamically unstable patients
concealed septal pathway.45 The rare form of verapamil-sensitive AT Synchronized DC cardioversion is recommended
is due to re-entry in the atrial tissue close to the AVN, but not the I B
for haemodynamically unstable patients.8688
AV nodal conducting system.325 Haemodynamically stable patients
Vagal manoeuvres, preferably in the supine posi-
11.2.1.1.3 Typical atrioventricular nodal re-entrant tachycardia. In I B
tion with leg elevation, are recommended.41,8991
the slowfast form of AVNRT, the onset of atrial activation appears
Adenosine (618 mg i.v. bolus) is recommended
before, at the onset of, or just after the QRS complex, thus maintain- I B
if vagal manoeuvres fail.9294
ing an AH/Hisatrial (HA) ratio >1. The VA interval measured from
Verapamil or diltiazem i.v. should be considered if
the onset of ventricular activation on surface ECG to the earliest IIa B
vagal manoeuvres and adenosine fail.92,9498
deflection of the atrial activation in the His bundle electrogram is <_60
ms. Although, the earliest retrograde atrial activation is typically Beta-blockers (i.v. esmolol or metoprolol) should
be considered if vagal manoeuvres and adenosine IIa C
recorded at the His bundle electrogram, careful mapping studies
have demonstrated that posterior or even left septal fast pathways fail.97,99,100
may occur in <_7.6% in patients with typical AVNRT.326328 Synchronized DC cardioversion is recommended
when drug therapy fails to convert or control the I B
11.2.1.1.4 Atypical atrioventricular nodal re-entrant tachycardia. tachycardia.87,88
Atypical AVNRT is seen in 6% of all AVNRT cases,317 and in some Continued
ESC Guidelines 31
..
Chronic therapy .. they are less successful in AVNRT than in AVRT.89,90,102 A single
.. dose of oral diltiazem (120 mg) plus a beta-blocker (i.e. proprano-
Catheter ablation is recommended for sympto-
I B
..
matic, recurrent AVNRT.208,336339 .. lol 80 mg) may convert <_94% of patients, but there is a risk of
.. hypotension, transient AV block, or—rarely—syncope.342,343
Diltiazem or verapamil, in patients without HFrEF, ..
or beta-blockers should be considered if ablation IIa B
.. Caution is needed in the elderly, and in patients with known sinus
.. or AV nodal conduction disturbances. A single dose of oral flecai-
is not desirable or feasible.340342 ..
nide (3 mg/kg) may also be effective, albeit at a lower rate.342,344
..
.. propranolol370 or, in the absence of ischaemic or structural
.. heart disease, flecainide380 and propafenone,381 may be tried.
AVNRT ..
.. Selective catheter ablation at the site of the earliest retrograde
.. atrial activation is feasible, but carries a lower success rate and
..
.. higher AV block risk compared with AVNRT (5 - 10%).369,382
.. Cryoablation is safer.375,383
Symptomatic and ..
..
whereas those that conduct in the retrograde direction only are .. QRS complex (>120 ms). In most cases, APs giving rise to the
more frequent (<_50%). When the AP conducts antegradely, ventric- .. WPW pattern are seen in structurally normal hearts. Rare, familial
..
ular pre-excitation is usually evident at rest during sinus rhythm and .. forms of pre-excitation associated with LV hypertrophy and multi-
the AP is referred to as ‘manifest’. Conversely, APs are referred to as .. system disease [mutations in the protein kinase adenosine
..
‘concealed’ if they exclusively conduct retrogradely. Concealed APs .. monophosphate-activated non-catalytic subunit gamma 2
may have decremental properties.395 The term ‘latent AP’ denotes .. (PRKAG2) gene, Danon and Fabry disease, and others] have also
..
been described.398
Figure 16 The St George’s algorithm for the localization of accessory pathways.399 þve = QRS complex-positive; ve = QRS complex-negative; þ/- =
QRS complex equiphasic; AP = accessory pathway; LAL = left anterolateral; LP = left posterior; LPL = left posterolateral; LPS = left posteroseptal; MS =
mid-septal; RAS = right anteroseptal; RL = right lateral; RP = right posterior; RPS = right posteroseptal.
34 ESC Guidelines
..
retrograde limb of the re-entrant circuit. Orthodromic .. difficult to assess as the retrograde P wave is usually inscribed
AVRT tends to be a rapid tachycardia, with frequencies .. within the ST-T segment.
..
ranging from 150 to, rarely, >220 b.p.m. During tachycardia ..
(Figure 18), the following ECG features can be present: (i) RP ..
.. 11.3.5 Accessory pathway as a bystander
interval constant and, usually but not invariably, up to one-half of .. In the presence of focal AT, atrial flutter, AF, or AVNRT, the QRS
the tachycardia CL; (ii) narrow QRS; (iii) functional BBB usually
..
.. complexes can be pre-excited when the AP acts as a bystander, and
associated with an AP ipsilateral to the blocked bundle, especially .. is not a critical part of the re-entry circuit.
in young patients (aged <40 years); and (iv) ST-segment
..
..
depression. ..
.. 11.3.6 Pre-excited atrial fibrillation
.. Paroxysmal AF has been found in 50% of patients with WPW, and
..
11.3.4 Antidromic atrioventricular re-entrant tachycardia .. may be the presenting arrhythmia in affected patients.405,406
.. These patients are typically young and have no structural heart
Antidromic AVRT occurs in 3 - 8% of patients with WPW syn- ..
drome.402404 The re-entrant impulse travels from the atrium to .. disease. High-rate AVRT may potentially initiate AF. AF with
.. fast ventricular response over an overt AP with a short anterog-
the ventricle through the AP with anterograde conduction; mean- ..
while, retrograde conduction occurs over the AVN or another .. rade refractory period is a potentially life-threatening arrhythmia
.. in patients with WPW syndrome, due to potential degeneration
AP, usually located in a contralateral position to ensure longer ..
travel distances, thus allowing for sufficient recovery of refractori- .. into VF.
..
ness of the respective elements of the re-entrant circuit. In ..
30 - 60% of patients with spontaneous antidromic AVRT, multiple .. 11.3.7 Concealed accessory pathways
..
APs (manifest or concealed), which could act or not as the retro- .. Concealed APs give rise only to orthodromic AVRT. Their true prev-
grade limb during the AVRT, may be detected. Antidromic AVRT
.. alence is unknown because they are not detectable on the resting
..
has the following ECG features, illustrated in Figure 18: (i) a wide .. surface ECG, but only at occurrence of AVRT, or during electro-
QRS complex (fully pre-excited) and (ii) an RP interval that is
.. physiology testing.45 No sex predilection is found and these pathways
ESC Guidelines 35
I
I
II
II III
III
aVR aVL
aVL aVF
aVF
V1
V1
V2
V2
V3
V3
V4
V4
V5
V5
©ESC 2019
V6 V6
Figure 18 Atrioventricular re-entrant tachycardia. Left: othodromic atrioventricular re-entrant tachycardia due to a concealed posteroseptal accessory
pathway. Retrograde P waves are negative during tachycardia in the inferior leads (arrows). Right: Antidromic atrioventricular re-entrant tachycardia due
to an atriofascicular accessory pathway. The axis during tachycardia due to atypical pathways depends on the way of insertion into the right bundle and
fusion over the left anterior fascicle.
..
tend to occur more frequently in younger patients than in those with .. Other potential causes of long RP tachycardias are sinus tachycar-
AVNRT; however, significant overlap exists.3 Concealed APs are pre- .. dia, AT, atypical AVRT, and JET with 1:1 retrograde conduction.
..
dominantly localized along the left free wall (64%), and less frequently ..
at septal (31%) and right free wall locations.395 Clinical presentation .. 11.3.9 Atypical forms of pre-excitation
..
is with AVRT. Concealed pathways are not associated with an .. Other APs are postulated to result in cardiac pre-excitation. Atypical
increased risk of sudden cardiac death. The management of AVRT
.. APs (also called Mahaim fibers) are connections between the right
..
due to a concealed AP is similar to that of an overt AP, but in this .. atrium or the AVN and the right ventricle, into or close to the right
case is related to symptoms without significant prognostic relevance
.. bundle branch.409414 Most of them are atriofascicular or nodoven-
..
in most cases. .. tricular (as initially described), but they can also be atriofascicular,
.. atrioventricular, nodofascicular, or nodoventricular, depending on
..
.. their variable proximal and distal insertions.413,414 Left-sided atypical
.. pathways have also been described but are extremely rare.415417
11.3.8 Permanent junctional reciprocating tachycardia ..
PJRT is a rare form of AV reciprocating tachycardia using a concealed .. Atypical pathways usually contain accessory nodal tissue, which
.. results in decremental properties, and connect the atrium to the fas-
AP. Usually these APs, originally described by Coumel, are located in ..
the posteroseptal region and are associated with retrograde decre- .. cicles by crossing the lateral aspect of the tricuspid annulus, but post-
.. eroseptal locations can also be found in rare cases. Conduction is
mental conduction properties.407 PJRT is a long RP tachycardia due ..
to the slow conduction properties of the AP, and is characterized by .. usually anterograde only, but concealed fibres have also been
.. described.412,418 The following properties define the behaviour of
deeply inverted retrograde P waves in leads II, III, and aVF due to the ..
retrograde nature of atrial activation. The incessant nature of PJRT .. atypical pathways:
..
may result in TCM that usually resolves after successful treatment by .. • Baseline normal QRS or different degrees of manifest pre-
radiofrequency catheter ablation, particularly in younger ..
.. excitation with LBBB morphology;
patients.407,408 Catheter ablation is strongly recommended in symp- .. • Programmed atrial pacing, leading to obvious manifest pre-
tomatic patients or in cases with impaired LV ejection fraction likely
.. excitation following an increase in AV interval along with short-
..
related to TCM. . ening of the HV interval at shorter pacing CLs;
36 ESC Guidelines
..
• Antidromic AVRT due to an atriofascicular pathway usually pro- .. conduction could also induce ventricular fibrillation, therefore electrical
duces a horizontal or superior QRS axis, but a normal axis may .. cardioversion should always be available. During orthodromic and anti-
also occur, depending on the way of insertion into the right bun- ..
.. dromic AVRT, drug therapy could be directed at one of the components
dle and fusion over the left anterior fascicle. .. of the circuit, the AVN (beta-blockers, diltiazem, verapamil, or etripa-
• Right bundle electrogram preceding His bundle activation during ..
.. mil),100,129,419,420 or the AP (ibutilide, procainamide, propafenone, or fle-
anterograde pre-excitation and SVT. .. cainide)421,422 (Figure 19). Antidromic AVRT is associated with malignant
..
WPW syndrome due to a very fast-conducting AP,403 and drugs acting
Recommendations for the therapy of atrioventricular re-entrant tachycardia due to manifest or concealed accessory
pathways
Acute therapy
Haemodynamically unstable patients
Synchronized DC cardioversion is recommended for haemodynamically unstable patients.8688 I B
Haemodynamically stable patients
Vagal manoeuvres, preferably in the supine position with leg elevation, are recommended.41,8991 I B
In orthodromic AVRT, adenosine (618 mg i.v. bolus) is recommended if vagal manoeuvres fail and the tachycardia is
I B
orthodromic.9294
In orthodromic AVRT, i.v. verapamil or diltiazem should be considered if vagal manoeuvres and adenosine fail.92,9498 IIa B
In orthodromic AVRT, i.v. beta-blockers (esmolol or metoprolol) should be considered in the absence of decompensated HF, if
IIa C
vagal manoeuvres and adenosine fail.97,99,100
In antidromic AVRT, i.v. ibutilide or procainamide or i.v. flecainide or profanenone or synchronized DC cardioversion should be
IIa B
considered if vagal manoeuvres and adenosine fail.421,422,429,437
In antidromic AVRT, i.v. amiodarone may be considered in refractory cases.423425,435 IIb B
87,88
Synchronized DC cardioversion is recommended when drug therapy fails to convert or control the tachycardia. I B
Chronic therapy
Catheter ablation of AP(s) is recommended in patients with symptomatic, recurrent AVRT.391393,438441 I B
Beta-blockers or non-dihydropyridine calcium-channel blockers (verapamil or diltiazem in the absence of HFrEF) should be consid-
IIa B
ered if no signs of pre-excitation are present on resting ECG, if ablation is not desirable or feasible.340,341,442,443
Propafenone or flecainide may be considered in patients with AVRT and without ischaemic or structural heart disease, if ablation
IIb B
is not desirable or feasible.429,444,445
Digoxin, beta-blockers, diltiazem, verapamil, and amiodarone are not recommended and are potentially harmful in patients with
III B
pre-excited AF.427,428,432434,446
i.v. verapamil and diltiazem are contraindicated in the presence of hypotension or HFrEF.
i.v. beta-blockers are contraindicated in the presence of decompensated heart failure.
i.v. ibutilide is contraindicated in patients with prolonged QTc interval.
i.v. procainamide prolongs the QTc interval but much less than class III agents.
i.v. flecainide and propafenone are contraindicated in patients with ischaemic or structural heart disease. They also prolong the QTc interval but much less than class III agents.
i.v. amiodarone prolongs the QTc but torsades des pointes is rare.
AF = atrial fibrillation; AP = accessory pathway; AVRT = atrioventricular re-entrant tachycardia; DC = direct-current; ECG = electrocardiogram; HFrEF = heart failure with
reduced ejection fraction; i.v. = intravenous.
a
Class of recommendation.
b
Level of evidence.
ESC Guidelines 37
AVRT
Patient out
Yes No
Vagal
manoeuvres Haemodynamic
(I B) instability
No Yes
Vagal Synchronized
manoeuvres cardioversion
(I B) (I B)
If ineffective
Orthodromic
Yes No
i.v. verapamil
i.v. beta-blocker
or diltiazem
(IIa C)
(IIa B)
©ESC 2019
If ineffective
Pre-excited AF AVRT
Orthodromic
i.v. ibutilide i.v. flecainide or AVRT
or procainamide propafenone
No Yes
(IIa B) (IIb B)
©ESC 2019
Diltiazem
Propafenone
©ESC 2019
If ineffective or verapamil
or flecainide
If or beta-blocker
If ineffective (IIb B)
ineffective (IIa B)
Figure 20 Acute therapy of pre-excited atrial fibrillation.
AF = atrial fibrillation; i.v. = intravenous.
Figure 21 Chronic therapy of atrioventricular re-entrant tachycardia.
AVRT = atrioventricular re-entrant tachycardia.
over the AP, may also be used, even if they may not restore sinus
rhythm.429431 However, class Ic drugs should be used with caution .. The 2015 American College of Cardiology/American Heart
..
as they do exert an effect on the AVN. In pre-excited AF, i.v. amio- .. Association/Heart Rhythm Society Guideline for the Management
darone may not be as safe as previously thought, because enhanced .. of Adult Patients With Supraventricular Tachycardia reported
..
pathway conduction and ventricular fibrillation have been reported, .. major complication rates after radiofrequency catheter ablation
and should not be considered.432435 Procainamide appears to be .. of 3.0 and 2.8% for AVNRT and AVRT, respectively.2 These rates
..
safer in this setting.436 .. are much higher than those reported by experienced electro-
..
.. physiologists in the current era, as summarized in Table 9, but the
11.3.10.2 Catheter ablation .. procedure still carries a very small, non-negligible, mortality
The treatment of choice for patients with symptomatic and recur- ..
.. risk.203,205
rent AVRT, or pre-excited AF, is catheter ablation (Figure 21). For ..
other patients with asymptomatic and infrequent episodes, thera- ..
.. 11.3.10.3 Chronic therapy
peutic decisions should be balanced between the overall risks and .. If ablation is not desirable or feasible in patients with pre-
benefits of the invasive nature of ablation vs. long-term commit-
..
.. excitation and symptomatic antidromic AVRT, and in whom struc-
ment to pharmacological therapy. Ablation of the AP has a high .. tural or ischaemic heart disease has been excluded, class IC antiar-
acute success rate and is associated with a low complication rate
..
.. rhythmic drugs act mainly on the AP and can be used in
depending on the pathway location (Table 9).391393,438440 Major .. antidromic tachycardia (Figure 21).429,437,444,445 In cases of pre-
complications include cardiac tamponade (0.131.1%) and com-
..
.. excited AF, caution should be taken not to transform it into atrial
plete AV block (0.172.7%) in patients in whom ablation of septal .. flutter and induce 1:1 conduction. Apart from class IC drugs, beta-
APs is attempted. With septal APs close to the AVN, the ECG typ-
..
.. blockers, diltiazem, or verapamil may also be considered in case of
ically displays a positive delta wave in leads avF and avL, and a nar- .. orthodromic tachycardias if no signs of pre-excitation are
..
row positive delta wave in lead V1 that has a prominently negative .. observed on the resting ECG.340,341,442,443
QRS complex.394 ..
..
When targeting septal pathways and applying cryoenergy, the inci- ..
dence of AV block is lower compared with radiofrequency energy.447 .. 11.3.11 The asymptomatic patient with pre-excitation
..
However, recurrence of previously blocked pathways has been .. Most patients with an asymptomatic WPW pattern will go
reported to be significantly higher when cryoenergy is applied.438 .. through life without any clinical events related to their ventricular
..
Two approaches are available for left-sided pathways: an antegrade .. pre-excitation. Approximately one in five patients will develop an
transseptal and a retrograde aortic approach. There is evidence that .. arrhythmia related to their AP during follow-up. The most com-
..
the transseptal approach, in experienced hands, results in reduced .. mon arrhythmia in patients with WPW syndrome is AVRT (80%),
radiation and procedure times.441,448 .. followed by a 2030% incidence of AF. Sudden cardiac death
ESC Guidelines 39
secondary to pre-excited AF that conducts rapidly to the ventricle Recommendations for the management of patients with
over the AP, resulting in ventricular fibrillation, is the most feared asymptomatic pre-excitation
manifestation of WPW syndrome. The risk of cardiac arrest/ven-
tricular fibrillation has been estimated at 2.4 per 1000 person- Recommendation Classa Levelb
years (95% confidence interval 1.33.9), but no deaths were
Performance of an EPS, with the use of isopre-
reported in a registry of 2169 patients over an 8 year follow-up
naline, is recommended to risk stratify individ-
period.439 However, in a Danish registry of 310 individuals with
time.22,405,439,449,450,454,456,470472 Among these studies, there has Catheter ablation should be considered in
been one prospective RCT of catheter ablation (37 patients) vs. patients with asymptomatic pre-excitation and
clinical follow-up without treatment (35 patients) of patients with IIa C
LV dysfunction due to electrical
asymptomatic pre-excitation.453 Catheter ablation reduced the dyssynchrony.478481
frequency of arrhythmic events (7 vs. 77%, P < 0.001) over 5 years. Catheter ablation may be considered in
One patient in the control group had an episode of cardioverted patients with low-risk asymptomatic pre-exci-
ventricular fibrillation. tation in appropriately experienced centres IIb C
Figure 22 summarizes the recommendations for the screening and according to patient
management of patients with asymptomatic pre-excitation. preferences.203,439,450,453,454,471,474,482
Asymptomatic
pre-excitation
Yes No
High-risk occupation
or competitive athlete?
EPS for
risk stratification
(I B)
Yes No
Catheter ablation No
Low-risk features
(I C)
Yes
(IIa C) (IIb C)
Figure 22 Risk stratification and therapy of patients with asymptomatic pre-excitation. High-risk features at electrophysiology study are shortest pre-
excited RR interval during atrial fibrillation <_250 ms, accessory pathway effective refractory period <_250 ms, multiple accessory pathways, and inducible
atrioventricular re-entrant tachycardia. Low-risk features at non-invasive risk stratification are induced or intermittent loss of pre-excitation on exercise or
drug testing, resting electrocardiogram, and ambulatory electrocardiogram monitoring.
EPS = electrophysiology study.
ESC Guidelines 41
Recommendations for the therapy of supraventricular tachycardia in congenital heart disease in adults
Anticoagulation for focal AT or atrial flutter should be similar to that for patients with AF.241,242,499 I C
Acute therapy
Haemodynamically unstable patients
i.v. verapamil and diltiazem are contraindicated in the presence of hypotension or HFrEF.
i.v. beta-blockers are contraindicated in the presence of decompensated heart failure.
AF = atrial fibrillation; AT = atrial tachycardia; DC = direct-current; HF = heart failure; HFrEF = heart failure with reduced ejection fraction; i.v. = intravenous; SVT = supraven-
tricular tachycardia.
a
Class of recommendation.
b
Level of evidence
..
sinus node dysfunction may occur as a consequence of the atrial redi- .. milk can substantially modify the absorption of the drug and, as feed-
rection procedure.495 As tachycardia is not well tolerated in patients .. ing schedules tend to be erratic, can affect effective drug availability.
..
with asystemic right ventricular and diastolic dysfunction, maintaining .. Furthermore, many drugs have to be prepared at specialized pharma-
long-term sinus rhythm is highly desirable in this setting. Use of antiar- .. cies, adding to the risk of incorrect dosing, and drug solutions may
..
rhythmic drugs is limited due to ventricular and sinus node dysfunction, .. also need to be stored under special conditions to maintain their
and pro-arrhythmic risk. Ablation procedures in patients with a .. stability. This can be inconvenient when long-term treatment is
..
Catheter ablation is recommended in symptomatic women with recurrent SVT who plan to become pregnant.538 I C
Acute therapy
Immediate electrical cardioversion is recommended for any tachycardia with haemodynamic instability.539,540 I C
Vagal manoeuvres and, if these fail, adenosine are recommended for acute conversion of SVT.541,542 I C
An i.v. beta-1 selective blocker (except atenolol) should be considered for acute conversion or rate control of SVT.542,543 IIa C
i.v. digoxin in the latest pocket Gls version should be considered for rate control of AT if beta-blockers fail.542,543 IIa C
544,545
i.v. ibutilide in the latest pocket Gls version may be considered for termination of atrial flutter. IIb C
Chronic therapy
During the first trimester of pregnancy, it is recommended that all antiarrhythmic drugs should be avoided, if possible. I C
Beta-1 selective (except atenolol) beta-blockers or verapamil, in order of preference, should be considered for prevention of
IIa C
SVT in patients without WPW syndrome.543,546548
Flecainide or propafenone should be considered for prevention of SVT in patients with WPW syndrome, and without ischaemic
IIa C
or structural heart disease.549
Flecainide or propafenone in patients without structural heart disease should be considered if AV nodal blocking agents fail to
IIa C
prevent SVT.533,543
Digoxin or verapamil should be considered for rate control of AT if beta-blockers fail in patients without WPW syndrome.543 IIa C
Amiodarone is not recommended in pregnant women.153,543 III C
Fluoroless catheter ablation should be considered in cases of drug-refractory or poorly tolerated SVT, in experienced
IIa C
centres.550552
higher risk of infants born small for their gestational age compared ..
.. 15.3 Diagnosis
with metoprolol and propranolol, consistent with this association .. TCM is one of the very few reversible causes of HF and dilated cardi-
not being a class effect.543,555 Diltiazem has been found to be terato- ..
.. omyopathy, and should be considered in any patient with new onset
genic in animals, with only limited human data, and its use is not gen- .. of LV dysfunction. In the presence of persistent or frequent tachycar-
erally recommended in pregnancy. Verapamil is considered safer ..
.. dia, or frequent premature ventricular contractions, a high index of
than diltiazem and could be used as a second-line drug.153,543 .. suspicion should be maintained. The diagnosis is established by
..
..
with either biventricular or His-bundle pacing is appropriate.569572 .. performed in those with high-risk features.577 Asymptomatic
Long-term medical therapy with beta-blockers, and angiotensin- .. patients stratified at low risk are allowed to practice competitive
..
converting enzyme inhibitors or angiotensin II receptor blockers, is .. sports.
indicated before and after successful ablation for the known beneficial .. AVNRT, orthodromic AVRT over a concealed AP, and AT are
..
effects of these drugs on the LV remodelling process. Given the risk .. not listed among the causes of sudden cardiac death during exer-
of recurrence of arrhythmias, long-term monitoring of patients is .. cise in patients with a structurally normal heart. However, their
..
Table 13 Recommendations for sports participation in athletes with ventricular pre-excitation and supraventricular
arrhythmias
Criteria for eligibility Eligibility
Premature atrial beats No symptoms, no cardiac disease All sports
AVRT or AF in the context of WPW syndrome Ablation is mandatory. Sports are allowed 1 month after All sports
ablation if there are no recurrences
Asymptomatic ventricular pre-excitation Ablation is mandatory in patients at high risk. Sports are All sports
allowed 1 month after ablation if there are no recurrences
Paroxysmal SVT (AVNRT, AVRT over Ablation is recommended. Sports are allowed 1 month All sports
a concealed AP, and AT) after ablation if there are no recurrences
Ablation undesirable or not feasible All sports, except those
with high intrinsic risk of
loss of consciousness
AF = atrial fibrillation; AP = accessory pathway; AT = atrial tachycardia; AVNRT = atrioventricular nodal re-entrant tachycardia; AVRT = atrioventricular re-entrant tachycardia;
SVT = supraventricular tachycardia; WPW = WolffParkinsonWhite
ESC Guidelines 47
Table 14 European Working Group 2013 report on driving and cardiovascular disease: driving in arrhythmias and con-
duction disorders: supraventricular tachycardia
Conduction disorder/ Group 1 Group 2
arrhythmia
AF/atrial flutter/focal AT Driving may continue provided no history of syncope. Driving may continue provided no history of syncope and
If history of syncope, driving must cease until the con- anticoagulation guidelines are adhered to.
AF = atrial fibrillation; AT = atrial tachycardia; AVNRT = atrioventricular nodal re-entrant tachycardia; AVRT = atrioventricular re-entrant tachycardia; WPW =
Wolff-Parkinson-White.
adrenergic activation and suppressed by L-type Ca2þ current .. • The proper management of asymptomatic pre-excitation and
..
blockade. .. strict catheter ablation indications have not been established.
• Re-entrant circuits may be microscopic or simulate foci by sur- .. • The genetics of SVT have not been adequately studied. There
face ‘breakthrough’ of transmural propagation. Thus, mapping
.. has been evidence for familial forms of AVNRT, AVRT, sinus
..
may be inadequate to distinguish them from automatic/triggered .. tachycardia, and AT, but data are scarce.
activity. .. • Novel electroanatomical mapping systems now allow the simulta-
..
• The exact circuit of AVNRT, the most common regular arrhyth- .. neous visualization of activation and voltage. The implications
20 ‘What to do’ and ‘what not to do’ messages from the Guidelines
Recommendations for the acute management of narrow QRS tachycardia in the absence of an established Classa Levelb
diagnosis
AF = atrial fibrillation; AP = accessory pathway; AT = atrial tachycardia; AVNRT = atrioventricular nodal re-entrant tachycardia; AVRT = atrioventricular re-entrant tachycardia;
CTI = cavotricuspid isthmus; DC = direct current; ECG = electrocardiogram; EPS = electrophysiology study; ERP = effective refractory period; HF = heart failure; i.v. = intrave-
nous; MRAT = macro-re-entrant atrial tachycardia; SPERRI = shortest pre-excited RR interval during atrial fibrillation; SVT = supraventricular tachycardia; TCM =
tachycardiomyopathy.
a
Class of recommendation.
b
Level of evidence.
..
21 Areas for further research .. robotic techniques, and sophisticated anatomical navigation systems
.. have been developed, and it is now possible to perform ablation
With the advent of catheter ablation in the 1990s, resulting in the
..
.. without exposing the operator to radiation and ergonomically unfav-
successful elimination of APs in symptomatic patients, AVRT now .. ourable positions.581 New materials for electrodes and other equip-
represents <20% of all SVTs.11,13 The frequency of AVNRT, which
..
used to account for 50% of all SVT cases,14 has changed to
... ment have allowed the concept of a radiation-free electrophysiology
.. laboratory with the use of CMR. The vision of a fully radiation-free,
30%,11,13 and the proliferation of AF ablation will unavoidably result .. magnetic laboratory in the future is not science fiction anymore.582
in more iatrogenic left atrial MRAT. Moreover, the prolonged survival
..
.. The revolution in computer technology offers not only improved
of paediatric and ACHD patients is expected to impose a further .. mapping and electrode-moving systems, but also the enhancement of
..
challenge on electrophysiologists, who will encounter even more .. specific SVT classification schemes with fully automated algorithms
complex MRATs. Several important advances in the field of anatomi- .. that may greatly assist emergency departments, ambulances, and
..
cal and electrical mapping, as well as our appreciation of scar tissue .. monitored patients.583 Mathematical modelling and numerical analy-
and the transmurality of ablation lesions, should improve our effi- .. ses have also been employed in the investigation of the circuit of
..
ciency in treating these patients. .. AVNRT.317,329 Further analysis of recorded ECGs using fast Fourier
The past decade has witnessed a rapid evolution of ablation equip- .. and Gaussian models may also provide useful diagnostic information
..
ment and electrode-guiding systems, which has resulted in more con- .. about the nature of the tachycardia. Novel electroanatomical map-
trollable and safer procedures. Intracardiac echocardiography, .. ping systems are being developed to assist the identification of the
50 ESC Guidelines
tachycardia mechanism and optimal ablation site of SVT, and, espe- .. electrophysiologist, along with the related supplementary references, are
..
cially, complex atrial macro-re-entrant tachycardias, with reduced flu- .. available on the European Heart Journal website and via the ESC website at
oroscopy times.584589 Systems are now available that allow the
.. www.escardio.org/guidelines.
..
simultaneous visualization of activation and voltage. The implications ..
..
that these may have on characterizing the tachycardia substrate, and .. 23 Appendix
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