Europace (2020) 22, 1768–1780 REVIEW SERIES

doi:10.1093/europace/euaa232

Management of ventricular electrical storm: a

contemporary appraisal
Gurukripa N. Kowlgi and Yong-Mei Cha *
Department of Cardiovascular Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA

Downloaded from https://academic.oup.com/europace/article/22/12/1768/5912220 by guest on 21 January 2023

Received 10 May 2020; editorial decision 5 July 2020 accepted 15 July 2020; online publish-ahead-of-print 27 September 2020

Abstract Ventricular electrical storm (VES) is a clinical scenario characterized by the clustering of multiple episodes of sus-
tained ventricular arrhythmias (VA) over a short duration. Patients with VES are prone to psychological disorders,
heart failure decompensation, and increased mortality. Studies have shown that 10–28% of the patients with sec-
ondary prevention ICDs can sustain VES. The triad of a susceptible electrophysiologic substrate, triggers, and auto-
nomic dysregulation govern the pathogenesis of VES. The rate of VA, underlying ventricular function, and the pres-
ence of implantable cardioverter-defibrillator (ICD) determine the clinical presentation. A multi-faceted approach
is often required for management consisting of acute hemodynamic stabilization, ICD reprogramming when appro-
priate, antiarrhythmic drug therapy, and sedation. Some patients may be eligible for catheter ablation, and auto-
nomic modulation with thoracic epidural anesthesia, stellate ganglion block, or cardiac sympathetic denervation.
Hemodynamically unstable patients may benefit from the use of left ventricular assist devices, and extracorporeal
membrane oxygenation. Special scenarios such as idiopathic ventricular fibrillation, Brugada syndrome, Long and
short QT syndrome, early repolarization syndrome, catecholaminergic polymorphic ventricular tachycardia,
arrhythmogenic right ventricular cardiomyopathy, and cardiac sarcoidosis have been described as well. VES is a car-
diac emergency that requires swift intervention. It is associated with poor short and long-term outcomes. A struc-
tured team-based management approach is paramount for the safe and effective treatment of this sick cohort.
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Keywords Ventricular storm • Electrical storm • Ventricular tachycardia • Implantable cardioverter-

defibrillator • Catheter ablation
...........................................................................................................................................................................................

thorough clinical evaluation, resuscitation skills, critical care manage-

Introduction ment with sedation, ICD reprogramming, medical therapies, ablation,
Ventricular electrical storm (VES) is a clinical scenario characterized and sympathetic modulation procedures.9,10 Not surprisingly, VES
by the clustering of multiple episodes of sustained ventricular has a tremendous impact on healthcare resources. Prompt recogni-
arrhythmias (VAs) over a short duration. Several definitions of VES tion of VES and implementation of rapid treatment can be the differ-
have been proposed over the years (Supplementary material online, ence between life and death. With this comprehensive review, we
Table S1), but the most widely accepted one is three or more epi- attempt to provide an up-to-date contemporary understanding and
sodes of sustained VA occurring within 24 h, requiring either anti- management of VES.
tachycardia pacing (ATP) or implantable cardioverter-defibrillators
(ICDs) shocks, with each event separated by at least 5 min.1–5 In
patients without ICDs, VES is typified by three or more discrete Epidemiology
occurrences of sustained VA.1,6
Patients with VES are prone to psychological disorders, heart fail- Ventricular electrical storm is incompletely understood from large
ure decompensation, and increased mortality.7,8 The management of trials studying ventricular tachycardia (VT) ablation. This is because
VES is genuinely multidisciplinary, including but not limited to: patients with VES either form a small proportion of enrollees or are

*Corresponding author. Tel: þ1 5072843585; fax: þ1 5072552550. E-mail address: ycha@mayo.edu

C The Author(s) 2020. For permissions, please email: journals.permissions@oup.com.
Published on behalf of the European Society of Cardiology. All rights reserved. V
Ventricular storm
SUBSTRATE
- Myocardial scar
- Arrhythmia syndromes
ELECTRICAL
STORM
TRIAD
TRIGGERS
- Ischemia
- Electrolyte/Metabolic derangements
- Toxins/Drugs
- Hypoxia
Figure 1 The triad of ventricular electrical storm consists of a complex interplay between an arrhythmogenic substrate, autonomic imbalances,
and acute triggers. Some examples of each are enlisted in this figure.
excluded from most of the prominent VT studies.11 Furthermore,
depending on the defining criteria, the incidence may vary among dif-
ferent reports. Male sex, advanced age, lower left ventricular (LV)
ejection fraction, and the presence of medical comorbidities increase
the susceptibility of developing VES.11 Ventricular electrical storm
prevalence in ischaemic cardiomyopathy (ICM) and non-ischaemic di-
lated cardiomyopathy (NICM) is roughly estimated to be comparable
with high recurrence rates in both subsets.8,11,12 Studies have shown
that 10–28% of the patients with secondary prevention ICDs can sus-
tain VES3,13,14 with over a three-fold increase in mortality as com-
pared to controls.8 Monomorphic VT as a triggering arrhythmia, was
found to have a higher association with VES as compared to polymor-
phic VT and ventricular fibrillation (VF).8,15 (Supplementary material
online, Table S1) summarizes the clinical trials of VES. The mean fre-
quency of VES was 2–55 episodes. Prior treatment with Vaughan
Williams class I antiarrhythmic drugs is associated with a higher inci-
dence of VES.8,11
Pathophysiology
Patients with structural heart disease, predictably form the bulk of
the cases of VES. However, individuals with structurally normal
hearts can also be predisposed to VES owing to genetic arrhythmia
syndromes such as catecholaminergic polymorphic VT (CPVT),
Brugada syndrome, long QT syndrome (LQTS).16–18 The term VES is
quite apt in a way that it mandates a perfect storm of several factors.
1769
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AUTONOMICS
- Adrenal hypertonia
- Suppressed parasympathec system
Ventricular electrical storm is governed by a complex interplay of the
following elements (Figure 1):
A. Presence of a susceptible electrophysiologic substrate:
In ICM, NICM or other forms of cardiomyopathy, scarred myocar-
dium provides the anatomical basis for re-entrant VA.19
B. Triggers:
Inciting factors may include ischaemia, decompensated heart failure,
infections, endocrine emergencies, electrolyte derangements, and
antiarrhythmic drug non-compliance.20 At the cellular level, calcium
mishandling and disorders of protein phosphorylation have also
been proposed as potential mechanisms for arrhythmia-induced
cardiomyopathy.21–23 While addressing a reversible aetiology is the
prudent approach, per prior studies, a reversible cause cannot be
determined in the majority of cases.24
C. Autonomic dysregulation:
Certain chronic cardiac conditions may portend a neural remodel-
ling, which involves a decrease in parasympathetic input, and even-
tual sympathetic hyperinnervation.25 A temporal increase in
sympathetic activity may drive the initiation of the arrhythmic epi-
sode. Furthermore, ICD shocks increase the adrenergic tone, creat-
ing a vicious cycle that may lead to VES.
D. Additionally, genetic arrhythmia syndromes may provide the neces-
sary substrate for VES, even in the absence of structural heart dis-
ease.26 Fascinatingly, causative genes of genetic conditions such as
Brugada syndrome, and early repolarization have been discovered
in patients developing VES from other causes such as ischaemia.
This association begs the intriguing question: Is VES just a phenotypic
culmination of several disease processes, or is it a separate entity by
itself? The answer remains elusive.
1770
Clinical presentations and
diagnosis
Ventricular electrical storm can have diverse clinical presentations,
depending on multiple factors. The rate of VA, underlying LV func-
tion, and the presence of ICD determine how patients present.
Patients with significant LV dysfunction usually are unable to tolerate
VES and may present with syncope and sudden cardiac death as the
first symptom. Patients who are relatively more compensated, espe-
cially those with slower VA, may present with symptoms of palpita-
tions, and lightheadedness before developing syncope. Patients with
incessant VA with slower rates (100–120 beats/min), may also de-
velop symptoms representative of worsening congestive heart
failure.27
Individuals with ICDs can have a wide range of presentations—
from being completely asymptomatic with VA episodes treated with
ATP to recurrent ICD shocks.28 Patients with frequent ICD shocks
are at risk for developing psychological disorders, particularly severe
anxiety, and depression.29
Prompt identification of VES is critical as it warrants rapid man-
agement. The approach depends on whether or not the patient
has an ICD implanted. In those patients without an ICD, the clini-
cian must scrutinize the twelve-lead electrocardiogram of the pre-
senting rhythm. The majority of VES present with monomorphic
VT, which is a re-entrant arrhythmia generated by non-
homogenous myocardial scarring. Polymorphic VT and VF, on the
other hand, are seen in acute ischaemia, electrolytes disarrange-
ment, prolonged QT intervals, and genetic channelopathies. Often
times, differentiating monomorphic VT vs. supraventricular tachy-
cardia (SVT) with aberrancy can be challenging.30 Several algo-
rithms have been published to aid in the differential of VT from
SVT.31–33 In cases of ambiguity, especially in the setting of struc-
tural heart disease, a wide-complex tachycardia should be treated
as VA unless proved otherwise.34,35 If the situation permits, an as-
tute clinician should make a mental checklist to rule-out the fol-
lowing conditions in all wide complex tachycardias:
A. SVT with a pre-existing bundle branch block: Thus, comparison to a
prior electrocardiogram is paramount.
B. SVT with rate-related aberrant conduction: Right bundle branch aber-
rancy more common but can present with left bundle branch block
as well.36
C. Antidromic atrioventricular reciprocating tachycardia: By extension, any
SVT that conducts antegrade over an accessory pathway can pro-
duce a wide QRS complex. Pre-excitation with a delta wave can be
recognized by careful review of QRS morphology.
D. Electrolyte disorders such as hyperkalaemia.
E. Drugs: The list predominantly includes sodium channel blockers
such as the Vaughan Williams Class I antiarrhythmic drugs
(Quinidine, Procainamide, Flecainide, among others). If time permits,
a thorough history should be obtained to exclude the use of recrea-
tional drugs such as cocaine and amphetamines that can have similar
QRS complex widening effects.
F. Paced rhythms: Certain specific causes such as pacemaker-mediated
tachycardia, and normal upper-rate behaviour must be considered
in patients with ICDs.
In patients with ICDs who experience device therapies, the first
step must be to verify if the therapies were indeed appropriate.
G.N. Kowlgi and Y.-M. Cha
Shocks for reasons other than VA are termed inappropriate shocks
and can occur in as high as 40% of ICD recipients despite novel dis-
criminating device algorithms.37–39 Some of the established reasons
for inappropriate therapies include SVT with rates in the VT/VF zone,
oversensing in the ventricular lead: such as T-wave oversensing and
lead malfunction with noise detection associated with lead fracture,
or loss of lead insulation.37,40
Management
Initial assessment
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It is imperative to determine haemodynamic instability if present on
initial evaluation. In cases of haemodynamic decompensation, resour-
ces and personnel should be immediately diverted to execute ad-
vanced cardiac life support with effective cardiopulmonary
resuscitation.5,41 Patients without a pulse and rhythm consistent with
VA, should be emergently defibrillated.42
Simultaneously, a systems check must be run to identify any
reversible causes of VES. Electrolyte disorders must be rapidly cor-
rected with particular attention to hypokalaemia and hypomagnese-
mia.43–45 The cardiac catheterization lab must be alerted if acute
ischaemia is suspected as an aetiology. Decompensated heart failure,
hypoxia, and lack of adherence to antiarrhythmic drugs must be iden-
tified swiftly. Certain unique scenarios might call for specific manage-
ment, which will be addressed in a separate section to follow.
Patients with pre-existing systemic comorbidities or acute end-organ
damage must be triaged to critical care units, preferably a cardiac in-
tensive care unit.15,28
Device evaluation and reprogramming
Verification of the appropriateness of ICD therapies is the first step.
In cases where inappropriate therapies are detected, turning off ICD
shock therapy should precede any other intervention.46 In situations
when access to device programmers is limited, a magnet placed on
the ICD can serve to deactivate tachycardia therapies. Inappropriate
shocks generate a hyperadrenergic state, which in turn can precipi-
tate VES by itself.47 Even when treatments are appropriate, if the pa-
tient is haemodynamically stable, consideration must be given to
temporarily deactivate shock therapies. For re-entrant monomorphic
VT, programming longer detection times, and enhancing ATP thera-
pies should be attempted.1,48–50Anti-tachycardia pacing has been
proven to be safe and effective when compared to shocks, and most
importantly, cause much less patient discomfort.51,52
Sometimes, device pacing algorithms can prove to be proarrhyth-
mic. Examples include algorithms to minimize ventricular pacing,
where device-generated pauses and short-long-short sequences can
lead to VA.53,54 Furthermore, LV pacing as seen in cardiac resynchro-
nization therapy (CRT) can result in triggered VA with premature
ventricular complexes (PVCs), or re-entrant VA by promoting het-
erogeneous conduction.55 If it is established that CRT was recently
programmed and was a potential source of proarrhythmia, then dis-
abling LV pacing can potentially mitigate the storm episode.
Medical therapies
Antiarrhythmic medications are the backbone of VES management
(Table 1). Rapid administration of antiarrhythmics is required as part
Table 1 Summary of drugs used for ventricular electrical storm describing the mechanism of action, dosing strategies, pharmokinetics, monitoring parameters,
and key adverse effects

Vaughan Drug Channels affected Dose Half-life Metabolism Specific use Monitoring Adverse effects
Ventricular storm

Williams
class
...................................................................................................................................................................................................................................................................................................
IA Quinidine INa, IKr, Ito, M, alpha Quinidine sulfate: 200–600 6–8 h Predominant hepatic VT/VF (Brugada syndrome, Prolongs QTc, QRS; use Diarrhoea, gastritis, VA, TdP,
mg PO q6–12 h; quinidine short QT syndrome) with caution in HF, AVB, blood dyscrasias
gluconate 324–648 mg PO G6PD deficiency dizziness, headache
q8 h; IV loading dose 800 increases DFT (Cinchonism)
mg/50 mL, maintenance 50
mg/min
Procainamide INa, IKr IV: bolus 10 mg/kg over 20 2–5 h Hepatic and renal ACS; pre-excited AF mimick- Prolongs QTc, QRS. TdP, AVB, HF exacerbation,
min, maintenance 2–3 g/24 (NAPA) ing VT/VF NAPA levels; increases Lupus-like syndrome
h; oral: 500–1250 mg q6 h DFT
1B Lidocaine INa IV: bolus 1–1.5 mg/kg, can re- 7–30 min Hepatic Ischaemic VT/VF Check Lidocaine levels; Delirium, psychosis, seizures,
peat up to total of 3 mg/kg, QTc can shorten tinnitus, bradycardia, AVB,
maintenance: 1–4 mg/min sinus arrest
Mexiletine INa 150–300 mg PO q8–12 h 10–14 h Hepatic metabolism VT/VF; helpful in LQTS3 QTc can shorten Tremors, ataxia, HF, AVB
via CYP2D6. Renal
excretion
II Propranolol Non-selective IV: 1–3 mg q5 min to a maxi- 3–6 h Extensive first-pass ef- VT/PVC; LQTS Slows SA node; increase Bradycardia, hypotension,
b-blocker mum of 5 mg; PO: 10–40 (immediate fect; hepatic metab- AV node refractoriness AVB, bronchospasm,
mg q6 h immediate release; release) olism via CYP2D6 nightmares, dizziness
60–160 mg q12 h extended
release
Nadolol Non-selective 40–320 mg daily 20–24 h Not metabolized. VT/PVC; LQTS; CPVT Slows SA node; increase Bradycardia, hypotension,
b-blocker Excreted AV node refractoriness AVB, bronchospasm, dizzi-
unchanged in the ness, cold extremities
urine
Metoprolol b1-receptor IV: 5 mg q5 min up to 3 doses; 3–4 h Hepatic metabolism VT, PVC Slows SA node; increase Bradycardia, hypotension,
PO: metoprolol tartarate AV node refractoriness AVB, fatigue, depression,
25–100 mg q12 h diarrhoea
Esmolol b1-receptor IV: bolus: 0.5 mg/kg, mainte- 9 min Metabolized by RBC VT Slows SA node; increase Bradycardia, hypotension,
nance: 0.05 mg/kg/min esterases AV node refractoriness AVB, dizziness, nausea
III Sotalol IKr; b1,2 IV: 7 5 mg q12 h; PO: 80–160 12 h Renal VT, VF, PVC Prolongs QTc (monitor TdP, bradycardia, hypoten-
mg q12 h on initiation), slows SA sion, AVB, fatigue,
node; increase AV depression, diarrhoea
Continued
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 1772

Table 1 Continued

Vaughan Drug Channels affected Dose Half-life Metabolism Specific use Monitoring Adverse effects
Williams
class
...................................................................................................................................................................................................................................................................................................
node refractoriness;
decreases DFT
Amiodarone INa, ICa, IKr, IK1, IKs, Ito, IV: bolus 300 mg for VF/pulse- 4–14 weeks Hepatic VT, VF, PVC Prolongs QTc, QRS, Hypotension, bradycardia,
Beta receptors, less VT arrest; 150 mg for slows SA node; in- AVB, TdP, corneal micro-
Alpha receptor, stable VT; maintenance: 1 crease AV node refrac- deposits, thyroid abnor-
nuclear T3 receptor mg/min  6 h, then 0.5 mg/ toriness; increases DFT malities, nausea,
min  18 h; PO: 400 mg  constipation, photosensi-
q 8–12 h for 7–14 days, tivity, skin discolouration,
then 200–400 mg daily peripheral neuropathy,
tremor, hepatitis, cirrho-
sis, pulmonary fibrosis, or
pneumonitis
IV Verapamil ICa IV: 2.5–5 mg q 15–30 min; 3–7 h Fascicular VT, RVOT VT Slows SA node; increase Hypotension, AVB, brady-
PO: sustained release 240– AV node refractoriness cardia, exacerbation of
480 mg/day HFrEF, oedema, headache,
rash, gingival hyperplasia,
constipation

Alpha, alpha-adrenergic receptor; AV, atrioventricular; AVB, atrioventricular block; Beta, beta-adrenergic receptor; CPVT, catecholaminergic polymorphic ventricular tachycardia; DFT, defibrillation threshold; h, hours; HF, heart failure;
HFrEF, HF with reduced ejection fraction; Ica, L-type calcium channel current; IK1, inward rectifier potassium channel; IKr, rapid delayed rectifier potassium current; IKs, slow delayed rectifier potassium current; INa, fast inward sodium current;
Ito, transient outward potassium current; IV, intravenous; LQTS, long QT syndrome; min, minutes; NAPA, n-acetyl procainamide; PO, per oral; PVC, premature ventricular complex; QTc, corrected QT interval; RVOT, right ventricular out-
flow tract; T3, triiodothyronine; TdP, torsades de pointes; VF, ventricular fibrillation; VT, ventricular tachycardia.
G.N. Kowlgi and Y.-M. Cha

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Ventricular storm 1773

of initial resuscitative measures.5,41 Studies have demonstrated that

antiarrhythmic drugs can assist in significantly reducing VES recur-
rence, albeit without impacting mortality.56

i. Beta-blockers: The surge in adrenaline is a robust mechanistic consid-

eration in VES episodes. Hence, the application of b-blockers to blunt
the sympathetic nervous system makes intuitive sense. Due to a rapid
onset of action and short half-life, esmolol can be used as an intrave-
nous infusion in the acute setting.57 In patients with VA after a recent
myocardial infarction, b-blockers can decrease VA
recurrence.58,59Non-selective b-blockers such as propranolol are
preferred over metoprolol or bisoprolol.34,60,61 The superiority of
propranolol over metoprolol could be secondary to a higher central

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nervous system concentration owing to its lipophilic nature. A recent
article described the use of amiodarone with propranolol vs. meto-
prolol in acute VES management.62 In this study, Chatzidou et al.62
reported earlier termination of VA in the propranolol arm along with
less time in intensive care and fewer recurrences of VA. Caution
must be exercised when using b-blockers in patients with decompen-
sated heart failure, as that may precipitate cardiogenic shock.63
ii. Amiodarone: Amiodarone is placed under class III in the Vaughan
Williams classification, which consists of potassium-channel blockers.
However, amiodarone is genuinely a mixed bag such that it can block
sodium channels, and can also function as a b-blocker and a calcium
channel blocker.64 Amiodarone has a slow onset of action, and the
half-life ranges around 6–8 weeks. Thus, a loading dose is recom-
mended at the outset (1–1.5 g/day in divided doses). Notably, in the
acute setting, amiodarone exerts its action predominantly by the b-
blocker component (h), and the rest of the channels take longer to
block (days to weeks).65 Owing to its multi-channel blocking proper-
ties, amiodarone is one of the most effective initial drugs adminis-
tered in VES.5,66 For out-of-hospital cardiac arrests due to VA,
Amiodarone has shown a higher survival to hospital admission as
compared to Lidocaine.67,68 However, more recent trials by
Kudenchuk et al.69 have failed to reproduce this effect. Santangeli et
al.56 in a recent meta-analysis have suggested that treatment with
amiodarone may result in increased mortality in patients with ICDs.
The long-term use of amiodarone is fraught with side effects, thus
impacting its use. The adverse effects include corneal deposits in the
majority of users, photosensitivity in 25–75% patients, elevated liver
enzymes in up to 30% patients, hepatitis/cirrhosis <3%, pulmonary fi-
brosis in less than a fifth of the patients, thyroid gland derangements,
skin pigmentation, among others.66
iii. Sotalol: Sotalol formulations consist of the L- and D-enantiomers.
The D-isomer is a potassium channel blocker only, and L-isomer has
an additional non-selective b-blocker effect. D-sotalol has been
shown to increase mortality when studied in randomized controlled
trials in patients with systolic heart failure, and prior acute myocardial
infarctions.70 Sotalol has been demonstrated to have superiority over
lidocaine in the acute termination of VA calling for its mention in the
guidelines.5,71 Based on the optical pharmacological therapy in
cardioverter-defibrillator patients (OPTIC) trial, sotalol was reported
to reduce ICD shocks.72 However, amiodarone and b-blockers were
shown to be more effective in preventing ICD shocks as compared
to sotalol alone.72
iv. Lidocaine and Mexiletine: Lidocaine is a Class IB antiarrhythmic agent
that exerts its action by sodium channel blockade.73 Lidocaine prefer-
entially works in ischaemic myocardium, promoting its administration
in VA occurring during/after acute myocardial infarctions.10,74 The ef-
ficacy of Lidocaine in terminating VA in non-ischaemic VT has been
reported to be 8–30%; thus, it is less commonly used as solo therapy
in these scenarios.34 Another drug with a similar pharmacologic
Figure 2 Illustration describing thoracic epidural anaesthesia. A
Tuohy needle is used to gain access to the epidural space. An epidu-
ral catheter is advanced beyond the Touhy needle and secured in
place. Lack of blood or cerebrospinal fluid aspiration is used to ex-
clude intravascular or intrathecal catheter placement. At the initia-
tion of thoracic epidural anaesthesia, a 1-mL injection of bupivacaine
is administered via the epidural catheter, followed by an infusion at
2 mL/h of bupivacaine. The dose can be titrated according to the ar-
rhythmic response.
profile is Mexiletine. Mexiletine is given orally and is often used in
conjunction with class III AAD to enhance VA control.
v. Procainamide and Quinidine: Procainamide is a Class IA agent which is a
potent sodium channel blocker.73 Procainamide is a two-in-one drug
as its metabolite, N-acetyl procainamide, works as a potassium chan-
nel blocker. Another property that makes procainamide unique is its
cardiac ganglion blocking function, which can be useful in curbing re-
sistant arrhythmias.75 Procainamide has been demonstrated to be su-
perior to amiodarone and lidocaine in separate studies.76,77
Gastrointestinal discomfort can be quite common, but caution must
be exercised to detect lupus-like syndrome which may occur with
chronic therapy.10,56 Quinidine is another Class IA agent which can
be used for VES, with particular efficacy in patients with Brugada syn-
drome, early repolarization syndrome, and short QT syndrome.5
Sedation
When VES remains intractable despite aggressive anti-arrhythmic
therapies, deep sedation, along with mechanical ventilation, must be
considered. The goal is to alleviate the sympathetic overdrive by
achieving Richmond Agitation-Sedation Scale values below 2.78
Preference is given to opioid analgesics and benzodiazepines over
propofol owing to lesser negative inotropic effects.79,80 Sedation, in
addition to directly suppressing arrhythmogenesis, also serves by
buying time for the critical care team to identify and target reversible
causes, if any. Our group has previously demonstrated in a study of
65 patients with VA and severe cardiomyopathy that VA and skin
sympathetic nerve activity can be suppressed effectively by general
anaesthesia.81
1774
Figure 3 Illustration describing the anatomy and landmarks for
stellate ganglion block. (A) The classic approach is shown. The cri-
coid cartilage is palpated, and the vascular bundle is displaced later-
ally, and the needle tip is inserted perpendicular to the skin. (B) The
cross-section at the level of the C6 with the classical approach on
the left of the figure, and the ultrasound-guided approach on the
right is shown. Note that in the ultrasound-guided approach, the
needle course is lateral and inferior to the vascular bundle. (C)
Image of stellate ganglion blockade guided by ultrasonography. The
red arrow shows the path of the needle. CA, carotid artery; IJ, inter-
nal jugular vein; LC, longus colli muscle; SCM, sternocleidomastoid;
SG, area of stellate ganglion; TH, thyroid; VB, vertebral.
Catheter ablation
The predominant presenting arrhythmia in VES is monomorphic VT,
which is potentiated by heterogeneous myocardial scar.19 Catheter
ablation (CA) works by homogenizing the myocardial scar, thus po-
tentially limiting re-entrant circuits.10 CA finds a role in patients who
have refractory VT despite medications or have adverse effects from
the drugs (Supplementary material online, Table S2).5 A meta-analysis
by Nayyar et al.82 meticulously analysed 39 publications and reported
that after more than a year of follow-up, there was 83% survival, and
60% freedom from VA recurrence. CA has since been established to
be superior to medical therapy based on multiple trials in terms of
VA recurrence and ICD shocks.10,56,83,84 Randomized trials are yet to
show a mortality benefit of CA in VES, that some retrospective stud-
ies have described.10,11 However, in patients with polymorphic VT or
focally triggered VF storm after a myocardial infarct, CA of the trig-
gers has shown to decrease VA recurrences and improve mortality.85
With regard to the timing of CA, Frankel et al.86 have demonstrated
that early referral for CA for VT results in better 1-year VT-free
survival.
While CA has established a strong foothold in the management of
VA in ICM, the same cannot be said about NICM due to the relative
G.N. Kowlgi and Y.-M. Cha
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Figure 4 Illustration describing thoracoscopic cardiac sympa-
thetic denervation. Two to three small incisions are made over the
left chest along the mid-axillary line. A camera is used to provide a
magnified vision of the surgical field. (A) The visualization of thoracic
ganglia after tissue dissection is shown. (B and C) The pleura being
incised with an electrocautery hook dissector to access the sympa-
thetic chain are shown. (D) Reflects the removal of T-4 thoracic
ganglia to complete the cardiac sympathetic denervation. Note that
the stellage ganglion has not been excised to avoid iatrogenic
Horner’s syndrome.
paucity of data.87 A prospective trial of 227 patients based in
Germany, demonstrated short-term success in NICM; however,
long-term outcomes were worse compared to ICM.88 More re-
cently, 267 patients with NICM were compared with 196 patients
with ICM by Muser et al.,87 and they reported that CA of VES in both
groups had similar VA recurrence and mortality outcomes. The elec-
trophysiologist must have a lower threshold of mapping and ablating
in the epicardium for NICM, given the nature of scar distribution in
certain disease conditions.83,87 Shirai et al.89 have described that non-
scar Purkinje fibre VTs can be seen in about 5% of patients with struc-
tural heart disease undergoing VT ablation, and thus a predominant
substrate-based ablation may not be sufficient in such cases.
In patients where the traditional endocardial and epicardial CA
have failed to prevent VA recurrences, novel alternative technologies
have emerged.90 When VT circuits are deemed to be deep intramu-
ral, strategies such as half-normal saline irrigation91 and bipolar abla-
tion, have been described.92 Retrograde coronary venous ethanol
infusion has been suggested for refractory VT, particularly those aris-
ing from the LV summit.93 Needle ablation has surfaced as another
promising option for refractory intramural VT where a 27-gauge nee-
dle can be inserted into the tissue for deep energy delivery.94
Autonomic modulation
Curtailing the adrenergic surge can be a critical weapon when
VES is refractory to aggressive antiarrhythmic drugs and sedation
Ventricular storm 1775

Ventricular electrical storm ≥3 episodes of VT, VF

STEP 1: Acute Care/initial assessment

Hemodynamically unstable Hemodynamically stable

ACLS STEP 2: Rhythm analysis

Cardioversion/defibrillation

VT/VF Supraventricular tachycardia

STEP 3: Identify reversible causes Manage per guidelines

• Ischemia
• Electrolytes
• Acidosis

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• Drugs
• Heart failure
• Others

STEP 4: Medical therapies

• Amiodarone
• b-blockers
• Procainamide
• Lidocaine

STEP 5: Deep sedation/intubation

STEP 6: Alternative effective management

Catheter ablation: Hemodynamic support

High rate pacing for Autonomic Device
MMVT, PVC or Balloon pump
brady-mediated modulation: programming
ischemia triggered ECMO
PMVT, Long-QT SGB, TEA ATP
PMVT, VF, Brugada LVAD

Figure 5 Flowchart depicting the proposed algorithm for the management of ventricular electrical storm. ACLS, advanced cardiac life support;
ATP, anti-tachycardia pacing; Balloon pump, intra-aortic balloon pump; ECMO, extracorporeal membrane oxygenation; ICD, implantable cardi-
overter-defibrillator; LCSD, left cardiac sympathetic denervation; Long-QT, long-QT syndrome; LVAF, left ventricular assist device; MMVT, mono-
morphic VT; PMVT, polymorphic VT; SGB, stellate ganglion blockade; TEA, thoracic epidural anaesthesia; VA, ventricular arrhythmia; VES,
ventricular electrical storm; VF, ventricular fibrillation; VT, ventricular tachycardia.

strategies.25,95 Autonomic modulation has been described to treat
VES in prior studies.96–99 Multiple techniques of autonomic modu-
lation exist with the most popular ones being thoracic epidural
anaesthesia (TEA), percutaneous stellate ganglion block (SGB),
thoracoscopic, or open cardiac sympathetic denervation (CSD).15
Autonomic modulation has been used for LQTS and CPVT asso-
ciated VA for many years.17,100 In the last few years, the applica-
tion of neuraxial modulation has been described in patients with
structural heart disease.98
Injection of a local anaesthetic agent such as bupivacaine into the
thoracic epidural space constitutes TEA. In most circumstances, TEA
serves as a temporizing measure, to control VES, until more definitive
treatment strategies are executed.99 Figure 2 depicts the TEA proce-
dure. An epidural catheter is advanced into the epidural space be-
yond a Touhy needle and secured in place. Lack of blood or
cerebrospinal fluid aspiration is used to exclude intravascular or intra-
thecal catheter placement. At the initiation of TEA, a 1-mL injection
of bupivacaine is administered via the epidural catheter, followed by
an infusion at 2 mL/h of bupivacaine. The dose can be titrated accord-
ing to the arrhythmic response. Thoracic epidural anaesthesia, while
temporary, has shown promising efficacy in acute suppression of
VES.99,101
Stellate ganglion block is similar but involves the injection of local
anaesthetic into the left or bilateral stellate ganglia.98 Stellate ganglion
block can be quickly performed at the bedside using ultrasound guid-
ance in our centre. In the largest series of 30 patients with VES under-
going urgent SGB, At 24 h, 60% of patients were free of VA. in those
with frequent ICD therapies, VA episodes were significantly reduced
by 92% from 26 ± 41 to 2 ± 4 in the 72 h after SGB.95 Figure 3A–C
illustrates ultrasound-guided SGB. The stellate ganglion is located be-
R
hind the carotid artery. A 22-gauge, 2-inch spinal needle (PAJUNKV)
is used and advanced in-plane in a posterior-to-anterior direction to
the anterior surface of the longus coli muscle to avoid all vascular
structures. A 7 mL of bupivacaine is injected after a negative
aspiration.95
Left CSD involves thoracoscopic or open surgical removal of the
lower half of the left stellate ganglia and the T2-4 thoracic ganglia
(Figure 4).97,102 Cardiac sympathetic denervation has been shown to
reduce ICD shocks significantly in patients with resistant VES, with bi-
lateral CSD having a slight edge over left CSD.96 Cardiac sympathetic
denervation might be more effective in rapid VT, partially attributing
to elevated sympathetic output, while it is less effective in patients
with scar driven slow VT.103 Cardiac sympathetic denervation is use-
ful in some patients with idiopathic VF or polymorphic VT when
1776 G.N. Kowlgi and Y.-M. Cha

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Figure 6 Procedural details from an extensive ablation performed in a patient with non-ischaemic cardiomyopathy due to cardiac sarcoidosis pre-
senting with electrical storm. Patient had four prior failed ablations for ventricular tachycardia including a septal needle ablation and had breakthrough
episodes despite lidocaine, amiodarone, quinidine, and mexiletine and a stellate ganglion block. (A) The clinical VT which had left bundle branch type
morphology with a late transition, and a right inferior axis. (B) A 98% pace-map match from posteroseptal right ventricular outflow tract. (C) A right
anterior oblique of the left ventricle with red dots representing ablation lesions. (D) A postero-anterior projection of the right ventricle with red dots
representing ablation lesions. This patient did not have any recurrent ventricular tachycardia but succumbed to respiratory failure.

other measures have failed.104 In a prospectively enrolled 17 patients
with LQTS and high risk of sudden death who underwent left cardiac
sympathetic denervation, the mean left arm skin nerve activity de-
creased significantly, possibly reflecting reduced sympathetic
tone. These patients had no ventricular arrhythmic event at 1-year
follow-up.105
Haemodynamic support during
ventricular electrical storm
Institution of mechanical haemodynamic support should be consid-
ered early in the management of haemodynamically unstable arrhyth-
mias when conventional therapy fails (Figure 5). Extracorporeal
Ventricular storm
membrane oxygenation (ECMO) is a form of heart-lung bypass that
can be used to support VES patients for days or weeks until the vul-
nerable myocardial substrate settles down. ECMO or other mechani-
cal supporting systems are especially useful in weaning catecholamine
infusions after cardiac surgery or intervention and help terminate
catecholamine-driven electrical storm while restoring systemic circu-
lation.106 In a meta-analysis including 2465 adult and 82 paediatric
patients, a substantial mortality benefit was observed among high-risk
patients, as identified with PAINESD risk score or suffering from elec-
trical storm and treated with prophylactic mechanical circulatory
support.107
Performing CA in patients with VES and underlying cardiomyopa-
thy comes with the expected risks of haemodynamic decompensa-
tion.87 Baratto et al.106 studied 64 patients with haemodynamically
unstable VTs undergoing CA and demonstrated that prophylactic
use of ECMO, allowed safe procedural completion in 92% of the
patients, with non-inducibility of VT in 69%, and almost 90% survival
at a median follow-up of 21 months. Mathuria et al.108 studied a co-
hort of 93 patients with structural heart disease undergoing CA for
VT and reported improved survival with prophylactic use of percuta-
neous LV assist device, as opposed to rescue use.
Special scenarios
There are certain unique situations where specific treatment strate-
gies have to be followed. Herein, we describe some salient points
about a few special conditions. This is not meant to be an in-depth re-
view of these entities.
i. Idiopathic VT/VF: In structurally normal hearts, the triggering PVCs
are most commonly mapped to the outflow tracts, Purkinje system,
or the papillary muscles (left ventricle or right ventricle).109 The
mechanism of outflow tract VT is most frequently delayed after-
depolarization, and can respond to iv adenosine or verapamil.
Fascicular VT or Belhassen VT are classically known to be verapa-
mil-sensitive.110Long-term ablation outcomes are excellent.111
ii. Brugada syndrome: The medical management of Brugada syndrome
includes IV isoproterenol. Owing to its ability to block the transient
outward potassium current (Ito current), quinidine can prove effica-
cious in the long-term treatment of Brugada syndrome. Cilostazole
has been shown to be useful in managing refractory VF in Brugada
syndrome with early repolarization syndrome.112 Epicardial
right ventricular outflow tract substrate modification has been
proved to be very effective for VA associated with Brugada
syndrome.113
iii. Long QT syndrome: Specific lifestyle measures and trigger-avoidance
depends on the subtype of LQTS. b-blockers and flecainide form
the mainstay of medical management, and if there are VA recur-
rences, then left, or bilateral CSD can be considered.100,114
Intentional atrial pacing have been shown as an adjunctive strategy
to stabilize the QT interval and reduce VA episodes.115
iv. Short QT syndrome: Quinidine is the only antiarrhythmic drug of
proven efficacy in short QT syndrome. Atrial fibrillation is a frequent
accompaniment, and rhythm-control with propafenone has been
studied to be beneficial.116
v. Early repolarization syndrome: Certain patients with early repolariza-
tion syndrome can develop VF. Quinidine and isoproterenol have
been shown to be beneficial in this population.117
vi. Catecholaminergic polymorphic VT: Similar to LQTS, b-blockers, and
flecainide are the first-line treatment options, with CSD as the op-
tion for refractory cases.
1777
vii. Arrhythmogenic right ventricular cardiomyopathy (ARVC): For patients
with ARVC, avoidance of exercise is critical in preventing VES. For
refractory VES, CA has been proven to be efficacious. However,
patients tend to suffer from chronic heart failure symptoms, that
may progress despite arrhythmia mitigation.118
viii. Cardiac sarcoidosis: VT ablation in patients with cardiac sarcoidosis
can be challenging. Several VT morphologies may be identified, and
mapping of both ventricles is often needed (Figure 6). A third of
patients may be epicardial ablation.119
Long-term management
After the initial VES episode is controlled, prompt consideration
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must be given to the requirement of ICD implantation if the patient is
without one. If no reversible causes are identified, then an ICD im-
plantation for secondary prevention is encouraged by the current
guidelines.5In-hospital and post-discharge cardiac rehabilitation is rec-
ommended and has been shown to not increase the risk of recurrent
VES.120
Conclusion
Ventricular electrical storm is a cardiac emergency that requires swift
intervention. It is associated with poor short and long-term out-
comes. An astute clinician must be aware of rapid troubleshooting on
initial assessment, be familiar with the multitude pharmacotherapeu-
tic options, and refer for CA when warranted. A multipronged ap-
proach is frequently needed for device interrogation, advanced
cardiac life support, cardiac intensive care for monitoring and drug
administration, electrophysiological assistance for CA, and surgical
backup for CSD if indicated. While we have come a long way over
the last few decades, further research would be paramount to im-
prove the outcomes of patients with VES.
Supplementary material
Supplementary material is available at Europace online.
Conflict of interest: none declared.
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