I n c e s s a n t Ven t r i c u l a r

Tachycardia and
F i b r i l l a t i o n : Electrical Storms
Amin Al-Ahmad, MDa, Mohammad Shenasa, MDb,c,*,
Hossein Shenasa, MD, MsCb,c, Mona Soleimanieh, RNb

KEYWORDS
 Electrical storm  Ventricular tachycardia  Ventricular fibrillation

KEY POINTS
 Management of ventricular tachycardia storms is often empiric and typically depends on the iden-
tification of a cause or underlying pathophysiology that needs treatment.
 The use of the electrocardiogram (ECG) and intracardiac ECGs can be useful in deciding on a clin-
ical strategy for treatment of electrical storm.
 Treating the underlying causes and contributing factors is often helpful in addition to the use of
medical therapy and ablation.

INTRODUCTION ablation when done in the setting of electrical storm

also have higher morbidity and mortality than when
Electrical storm is defined as 3 or more episodes performed electively. In an extreme example, ra-
of sustained ventricular tachyarrhythmias/ventric- diofrequency ablation (RFA) in the setting of VT/
ular fibrillation (VT/VF) or appropriate implantable- VF storm in patients with ventricular assist devices
cardioverter defibrillators (ICD) shocks that occur has been reported as high as 80% over a 6-month
within a period of 24 hours.1,2 Most commonly, VT follow-up period.2,5,6 Neural modulation (left stel-
storm is seen in individuals with structural heart dis- late ganglion blockade) has recently been reported
ease and ICDs. Before ICDs, most patients with as an effective method in rare cases of VT storm.7
electrical storm did not survive. VT storm can also Similarly, renal denervation has been used in the
be seen in patients with a structurally normal heart, management of ventricular arrhythmia storm in pa-
such as those with ion channel mutation.3 The over- tients with cardiomyopathy.6
all mortality related to electrical storm is very high.1 In this communication, VT/VF storm is used
Many times electrical storm occurs in the setting of interchangeably with electrical storm.
end-stage cardiomyopathy or in the setting of se-
vere medical and metabolic comorbid conditions.4
In addition, patients who survive the storm often are SPECIFIC CONTRIBUTING FACTORS IN
treated with medications that have a high level of ELECTRICAL STORM
side effects and adverse effects, such as Amiodar-
one. Innovative procedures such as VT or VF Box 1 shows the common causes of VT/VF storm.
cardiacEP.theclinics.com

The authors have nothing to disclose.

a
Texas Cardiac Arrhythmia Institute, 3000 N. IH 35 Suite 720, Austin, TX 78705, USA; b Heart & Rhythm
Medical Group, 105 North Bascom Avenue, San Jose, CA 95128, USA; c Department of Cardiovascular Services,
O’Connor Hospital, San Jose, CA, USA
* Corresponding author. Heart & Rhythm Medical Group, 105 North Bascom Avenue, Suite 204, San Jose,
CA 95128.
E-mail address: [email protected]

Card Electrophysiol Clin 6 (2014) 613–621

http://dx.doi.org/10.1016/j.ccep.2014.05.010
1877-9182/14/$ – see front matter Ó 2014 Elsevier Inc. All rights reserved.
614 Al-Ahmad et al

Box 1 example of drug-induced polymorphic VT and tor-

Causes of VT/VF electrical storm sades de pointes due to administration of Ibutilide
for conversion of AF. Amiodarone can commonly
1. Electrolyte and metabolic imbalance cause prolongation of the QT interval; however, it
2. Myocardial ischemia and infarction rarely causes proarrhythmia. Unfortunately, when
Amiodarone does cause proarrhythmia, it often
3. Drug-induced proarrhythmia
manifests as electrical storm. Also, given the pro-
4. VT/VF Storm in Patients with ICDs longed drug half-life with Amiodarone, patients
5. VT storm in inherited channelopathies affected with electrical storm can have multiple
6. Electrical storm in patients with congestive episodes even after the drug is discontinued.
heart failure Commonly, electrical storm is related to pause-
dependent early premature ventricular contrac-
7. Unknown causes
tions (PVCs). Treatments that increase the heart
rate and prevent pause-dependent arrhythmia
such as pacing or use of isoproterenol can be
helpful.
Electrolyte Imbalance
VT Storm in Patients with ICDs
Electrolyte imbalance can often contribute to elec-
trical storm.4 In patients with hyperkalemia, the Electrical storm in patients with ICDs can occur in
electrolyte imbalance can lead directly to ventricu- about 10% to 20% of this population.4 The inci-
lar arrhythmias, although not always as a “storm.” dence is higher in patients who receive ICDs as a
Patients with electrical storm can often have hypo- secondary than primary prevention. Recipients of
kalemia. Both hyperkalemia and hypokalemia can cardiac resynchronization therapy may present
manifest as electrocardiogram (ECG) changes with electrical storm, especially in the early phase
before onset of ventricular arrhythmias. Correction after implantation.11–13 Recent advances in the
of the electrolyte imbalance is an important step in management of patients with ICDs have led to rec-
the early management of these patients. ommendations for increased use of antitachycar-
dia pacing as well as increasing the time to
Myocardial Ischemia and Infarction detect and to prevent unnecessary shocks.14 In
addition to the pain related to the ICD shocks, pa-
Acute ischemia can sometimes lead to electrical tients often have psychological manifestations of
storm, usually VF. Chronic myocardial ischemia posttraumatic stress syndrome.
can also set the stage for reentrant arrhythmias.
Patients with electrical storm should be evaluated
VT Storm in Inherited Channelopathies
for ischemia, and when possible, it should be
reversed. In addition, treatment with b-blockers Patients with inherited syndromes may present
can decrease both ischemia and ventricular ar- with electrical storm. Conditions such as the
rhythmias. Patients with atrial fibrillation (AF) who Brugada syndrome can have electrical storm
inherently have irregular heart rate often with with no apparent precipitating factor.15–18
short-long-short sequence in the presence of Fig. 3 shows an example of recurrent VT in a pa-
ischemia and infarction may trigger recurrent fast tient with Brugada syndrome who also received
VT/VF, especially when antiarrhythmic medica- an ICD.
tions are on board. Fig. 1 shows an example of
incessant VT in a patient after a myocardial infarc- Electrical Storm in Patients with Congestive
tion. Frequent premature ventricular complexes Heart Failure
precede VT, and a 3-beat VT triggers sustained
monomorphic VT. Urgent coronary angiography Patients with congestive heart failure (CHF) are
and revascularization of a subtotal left anterior de- the most common patients presenting with elec-
scending coronary artery abolished ventricular trical storm. Most cases do not have an identifi-
arrhythmias. able ischemic cause or clear electrolyte
abnormalities. Worsening CHF can be a precipi-
tating factor, but is not always present: many
Drug-induced Proarrhythmia
cases have not had any clinical decompensation.
Proarrhythmia is most commonly related to pro- Electrical storm is a poor prognostic indicator in
longation of the QT interval. Common culprits are patients with CHF. Figs. 4 and 5 shows an
antibiotics,8 antiarrhythmic medications,9 and example of recurrent VT/VT in patients with end-
antipsychotic medications.10 Fig. 2 shows an stage heart failure (HF).
 Incessant Ventricular Tachycardia and Fibrillation 615

Fig. 1. Incessant VT in a patient with anterior wall myocardial infarction detected during Holter monitoring.

ECG MARKERS OF VT STORM nonsustained VT are also sometimes seen before

an electrical storm event.
There are no specific ECG characteristics or pre- ECG manifestations of ischemia such as ST
dictors of VT storms. However, in some cases, depression are commonly seen with prolongation
ECG findings can be useful and may help lead to of the QT interval and lead to dispersion of refrac-
potential therapy. Frequent monomorphic PVCs toriness of the QT interval, which in turn can lead to
can be seen initiating polymorphic VT. Polymor- the development of recurrent ventricular arrhyth-
phic VT can occur in normal hearts with PVCs of mias. In severe cases, the T wave alternates in a
usually benign morphology such as those origi- beat-by-beat fashion resulting in visible or
nating from the right and left ventricular outflow microvoltT-wave alternans, which is a worrisome
tract region. In addition, PVCs initiating VF in sign and often precedes VF by only minutes.
patients with ischemic heart disease can be mono- Prolongation of the QT interval also is associ-
morphic and originate in a region near the ated with an increase in episodes of ventricular
Purkinje system19 or in the peri-infract area in pa- arrhythmias. Often, this can be seen as the result
tients with prior infarction. Frequent episodes of
616 Al-Ahmad et al

Fig. 2. (A) Top tracing is baseline in Atrial Fibrillation (AF). Middle tracing is after Ibutilide infusion and shows AF
and torsades de pointes (TdP). Lower tracing shows conversion from AF to sinus rhythm (SR) (B) Ibutilide infused
TdP in different patient than panel A.
 Incessant Ventricular Tachycardia and Fibrillation 617

Fig. 3. (A) Twelve-lead ECG of a patient with Brugada syndrome. Note ST-elevation of V1 (arrow). (B) Twelve-lead
ECG of a patient with Brugada syndrome and ventricular bigeminy.

of administration of medications that prolong Patients with arrhythmogenic right ventricular

the QT interval. Patients with a congenital long dysplasia (ARVD) will commonly have repolariza-
QT syndrome and a very long QT interval also tion abnormalities as well as abnormalities of the
have an increased risk of recurrent ventricular terminal portion of the QRS. Identification of the
arrhythmias. ECG manifestations of ARVD or the Brugada syn-
In patients with the Brugada syndrome, the drome in a patient with electrical storm can help
ECG manifestations of an atypical right bundle correctly identify the underlying condition and
branch block can often be more obvious in certain can be useful in the initiation of therapy for electri-
clinical states such as fever and may be associ- cal storm in these patients.
ated with a higher risk of developing clinical Another abnormality associated with electrical
arrhythmias. storm is the catcholaminergic polymorphic
618 Al-Ahmad et al

Fig. 3. (continued). (C) Rapid VT (same patient as in A and B) with a heart rate of 248 bpm. (D) Rapid nonsus-
tained VT (same patient as in A–C).
 Incessant Ventricular Tachycardia and Fibrillation 619

Fig. 4. VT storm in patient with CHF.

ventricular tachycardia (CPVT) syndrome. factors that led to these individual episodes, all
Although the baseline 12-lead ECG does not while stabilizing the patients often with the use of
reveal any abnormalities, the ECG of the VT often intravenous anti-arrhythmic medications. Often
shows bidirectional VT (see article by Methachit- the use of more than one medication is needed
tiphan and colleagues). Identification of bidirec- to stabilize the patient. Occasionally, if the patient
tional VT should lead to consideration of CPVT is not improved with the use of medications, seda-
or medication toxicity such as digoxin. tion with the use of general anesthetics may be
Other than the ECG, because most patients with indicated.
electrical storm have an ICD, the use of intracar- Other potential contributing factors, such as elec-
diac ECGs can also be useful. For example, exam- trolyte abnormalities and ischemia, should be
ination of the ECG preceding the shock can often reversed. In patients with acute HF, optimization of
reveal frequent premature ventricular beats. In HF status is important. Medications that may have
addition, the morphology of these beats (at least caused proarrhythmia should be discontinued.
in the near-field and far-field ECG) can occasion- RFA should be considered once the patient is sta-
ally be useful to identify these as monomorphic bilized. In some cases, RFA is needed because of
versus polymorphic beats; a repetitive beat that difficulty in stabilizing the patient with medical ther-
triggers ventricular arrhythmias can be a target apy. RFA can target the initiating premature beat, if
for ablation. present, or can be useful to target the arrhythmia
directly or via substrate modification in cases with
INFARCTION ELECTRICAL STORM hemodynamically intolerable episodes. Last, the
MANAGEMENT use of deep sedation and mechanical support,
such as ventricular assist devices, can be useful in
Treatment of electrical storm consists of first un- the management of these patients. Heart transplant
derstanding the potential cause and contributing at specialized centers may also be considered.
620 Al-Ahmad et al

Fig. 5. From a patient with congestive heart failure with ejection fraction of 25% awaiting ICD implantation.
Panels (A-D) shows sinus rhythm with ventricular bigeminy. Note the episodes of torsades de pointes was initiated
consistently with a short long short sequence.

SUMMARY 6. Remo BF, Preminger M, Bradfield J, et al. Safety and

efficacy of renal denervation as a novel treatment of
Electrical storm is a challenging condition. The use ventricular tachycardia storm in patients with cardio-
of the ECG and intracardiac ECGs can be useful in myopathy. Heart Rhythm 2014;11:541–6.
deciding on a clinical strategy for treatment. Treat- 7. Bourke T, Vaseghi M, Michowitz Y, et al. Neuraxial
ing the underlying causes and contributing factors modulation for refractory ventricular arrhythmias:
is often useful in addition to the use of medical value of thoracic epidural anesthesia and surgical
therapy and ablation. left cardiac sympathetic denervation. Circulation
2010;121:2255–62.
REFERENCES 8. Huang BH, Wu CH, Hsia CP, et al. Azithromycin-
induced torsade de pointes. Pacing Clin Electrophy-
1. Verma A, Kilicaslan F, Marrouche NF, et al. Preva- siol 2007;31:1579–82.
lence, predictors and mortality significance of the 9. Eckardt L, Breithardt G. Drug-induced ventricular
causative arrhythmia in patients with electrical storm. tachycardia. In: Zipes DP, Jalife J, editors. Cardiac
J Cardiovasc Electrophysiol 2004;15:1265–70. electrophysiology, From Cell to Bedside. 6th edition.
2. Nayyar S, Ganesan AN, Brooks AG, et al. Venturing Philadelphia: Elsevier Saunders; 2014. p. 1001–8.
into ventricular arrhythmias storm: a systemic review 10. Beach SR, Celano CM, Noseworthy PA, et al. OTc
and meta-analysis. Eur Heart J 2013;34:560–9. prolongation, torsades de pointes and psychotropic
3. Haissaguerre M, Extramiana F, Hocini M, et al. Map- medications. Psychosomatics 2013;54:1–13.
ping and ablation of ventricular fibrillation storms in 11. Brigadeau F, Kouakam C, Klug D, et al. Clinical pre-
patients with ischemic cardiomyopathy. dictors and prognostic significance of electrical
4. Eifling M, Razavi M, Massumi A. The evaluation and storm in patients with implantable cardioverter defi-
management of electrical storm. Tex Heart Inst J brillators. Eur Heart J 2006;27:700–7.
2011;38(2):111–21. 12. Arya A, Haghjoo M, Dehghani MR, et al. Prevalence
5. Carbucicchio C, Santamaria M, Trevisi N, et al. Cath- and predictors of electrical storm in patients with
eter ablation for the treatment of electrical storm in implantable cardioverter-defibrillator. Am J Cardiol
patients with implantable cardioverter-defibrillators: 2006;97:389–92.
short-and long-term outcomes in a Prospective 13. Turitto G, El-Sherif N. Cardiac resynchronization ther-
Single-Center Study. Circulation 2008;117:462–9. apy: a review of proarrhythmic and antiarrhythmic
 Incessant Ventricular Tachycardia and Fibrillation 621

medications. Pacing Clin Electrophysiol 2007;30: 17. Nademanee K, Veerakul G, Chandanamattha P,

115–22. et al. Prevention of ventricular fibrillation episodes
14. Moss AJ, Schuger C, Beck CA, et al. Reduction in in Brugada syndrome by catheter ablation over the
inappropriate therapy and mortality through ICD anterior right ventricular outflow tract epicardium.
programming. N Engl J Med 2012;367:2275–83. Circulation 2011;123:1270–9.
15. Nademanee K, Taylor R, Bailey WE, et al. Treating 18. Dinckal MH, Davutoglu V, Akdemir I, et al. Incessant
electrical storm: sympathetic blockade versus monomorphic ventricular tachycardia during febrile
advanced cardiac life support-guided therapy. Cir- illness in a patient with Brugada syndrome: fatal
culation 2000;102:742–7. electrical storm. Europace 2003;5:257–61.
16. Veerakul G, Nademanee K. Treament of electrical 19. Haissaguerre M, Shah DC, Jais P, et al. Role of Pur-
storms in Brugada syndrome. Journal of Arrhythmia kinje conducting system in triggering of idiopathic
2013;29:117–24. ventricular fibrillation. Lancet 2002;359:677–8.