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. 2024 Mar 30;26(4):euae049.
doi: 10.1093/europace/euae049.

Management of patients with an electrical storm or clustered ventricular arrhythmias: a clinical consensus statement of the European Heart Rhythm Association of the ESC-endorsed by the Asia-Pacific Heart Rhythm Society, Heart Rhythm Society, and Latin-American Heart Rhythm Society

Radosław Lenarczyk  1 , Katja Zeppenfeld  2 , Jacob Tfelt-Hansen  3   4 , Frank R Heinzel  5 , Thomas Deneke  6   7 , Elena Ene  6 , Christian Meyer  8 , Arthur Wilde  9   10 , Elena Arbelo  11 , Ewa Jędrzejczyk-Patej  12 , Avi Sabbag  13   14 , Markus Stühlinger  15 , Luigi di Biase  16 , Marmar Vaseghi  17 , Ohad Ziv  18   19 , William-Fernando Bautista-Vargas  20 , Saurabh Kumar  21 , Narayanan Namboodiri  22 , Benhur Davi Henz  23 , Jose Montero-Cabezas  2 , Nikolaos Dagres  24 , Petr Peichl  25 , Antonio Frontera  26 , Stylianos Tzeis  27 , Jose Luis Merino  28 , Kyoko Soejima  29 , Christian de Chillou  30 , Roderick Tung  31 , Lars Eckardt  32 , Philippe Maury  33 , Peter Hlivak  34 , Larisa G Tereshchenko  35 , Pipin Kojodjojo  36 , Jacob Atié  37
Affiliations

Management of patients with an electrical storm or clustered ventricular arrhythmias: a clinical consensus statement of the European Heart Rhythm Association of the ESC-endorsed by the Asia-Pacific Heart Rhythm Society, Heart Rhythm Society, and Latin-American Heart Rhythm Society

Radosław Lenarczyk et al. Europace. .

Abstract

Electrical storm (ES) is a state of electrical instability, manifesting as recurrent ventricular arrhythmias (VAs) over a short period of time (three or more episodes of sustained VA within 24 h, separated by at least 5 min, requiring termination by an intervention). The clinical presentation can vary, but ES is usually a cardiac emergency. Electrical storm mainly affects patients with structural or primary electrical heart disease, often with an implantable cardioverter-defibrillator (ICD). Management of ES requires a multi-faceted approach and the involvement of multi-disciplinary teams, but despite advanced treatment and often invasive procedures, it is associated with high morbidity and mortality. With an ageing population, longer survival of heart failure patients, and an increasing number of patients with ICD, the incidence of ES is expected to increase. This European Heart Rhythm Association clinical consensus statement focuses on pathophysiology, clinical presentation, diagnostic evaluation, and acute and long-term management of patients presenting with ES or clustered VA.

Keywords: Arrhythmia; Consensus document; Electrical storm; Sudden cardiac death; Ventricular fibrillation; Ventricular tachycardia.

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Conflict of interest statement

Conflict of interest: Radosław Lenarczyk – nothing to declare, Katja Zeppenfeld – nothing to declare, Jacob Tfelt-Hansen – support received by himself or his institution related to this work - John and Birte Meyer Family Foundation, any other financial support: Johnson and Johnson, Microport, Cytokinetics and Leo Pharma, Frank R. Heinzel – nothing to declare, Thomas Deneke speaker honoraria- Biotronik, Abbott, Biosense Webster, voted member German Cardiology Society leadership team, Elena Ene - travel and proctorship honoraria from Johnson&Johnson, Christian Meyer - Abbott: speaker, Biotronik: consultant, Biosense Webster: consultant, Boston Scientific: consultant, speaker, Arthur Wilde - Associate editor Heart Rhythm, Chair DSMB LEAP trial (unpaid), Member scientific advisory board ARMGO & ThryvTherapeutics (unpaid), Elena Arbelo - Consulting for Bayer and Biosense Webster, Ewa Jędrzejczyk-Patej – nothing to declare, Avi Sabbag – nothing to declare, Markus Stühlinger - speaker honoraria (Biotronik, Medtronic), Luigi di Biase - consultant for Biosense Webster, Stereoataxis and I-Rhythm, has received speaker honoraria/travel from Biosense Webster, St. Jude Medical (now Abbott), Boston Scientific, Medtronic, Biotronik, Atricure, Baylis and Zoll, Marmar Vaseghi – grants NIH R01HL1706262, NIH R01HL148190, honorarium for educational speaking/courses/seminars from Zoll Inc. Medtronic Inc. and Biosense Webster Inc., minor stock in NeuCures Inc, Ohad Ziv – nothing to declare, William-Fernando Bautista-Vargas – nothing to declare, Saurabh Kumar – nothing to declare, Narayanan Namboodiri – nothing to declare, Benhur Davi Henz – nothing to declare, Jose Montero Cabezas - Shockwave Inc- research funding, Penumbra Inc- speaker fees, Nikolaos Dagres – nothing to declare, Peichl Petr – Astra Zeneca, Promed, Abbott, Medtronic, Biotronik, Biosense Webster: speaker fees, consultancy, Frontera Antonio – Abbott, Boston Scientific, Biosense Webster: speaker fees, consultancy, Tzeis Stylianos – Bayer, Pfizer: speaker fees, consultancy, Merino Jose Luis – Sanofi Aventis, Microport, Medtronic, Milestone Pharmaceutical, Biotronik, Zoll Medical: speaker fees, consultancy, Bayer: travel and meeting support, Daiichi Sankyo : Clinical Trial participation, Principal investigator, Milestone : Clinical trial participation, Principal investigator, Abbott : Fellow support, Principal investigator, Medtronic : Fellow support, Principal investigator, Soejima Kyoko – Abbott, Daiichi Sankyo, Medtronic, Johnson and Johnson: speaker fees, consultancy, de Chillou Christian – Abbott, Boston Scientific, Biosense Webster: speaker fees, consultancy, Tung Roderick – Biotronik, Medtronic, Abbott: speaker fees, consultancy, Eckardt Lars - nothing to declare, Maury Philippe - nothing to declare, Hlivak Peter – Pfizer, Boehringer-Ingelheim, Bayer, Novo-Nordisk: speaker fees, consultancy, Tereshchenko Larisa - nothing to declare, Kojodjojo Pipin - nothing to declare Atie Jacob - Johnson & Johnson: speaker fees, consultancy, Boston Scientific: travel and meeting support.

Figures

Graphical abstract
Graphical abstract
Figure 1
Figure 1
Types of ventricular arrhythmias: (A) monomorphic VT, (B) bidirectional VT, (C) polymorphic VT initiated by shortly coupled monomorphic premature ventricular complexes, (D) torsade de pointes in long QT syndrome; macroscopic T-wave alternans prior to VT. VT, ventricular tachycardia.
Figure 2
Figure 2
Manifestations of electrolyte-level disturbances on ECG: (A) hypokalaemia, (B) hyperkalaemia, (C) hypocalcaemia, (D) hypercalcaemia. ECG, electrocardiogram.
Flowchart 1
Flowchart 1
ICD interrogation and reprogramming—inappropriate therapy suspected.*Cardiac oversensing: T-wave oversensing (most common cause of inappropriate shocks in S-ICD); P-wave oversensing; R-wave double counting. #Lead-related oversensing: conductor fracture; insulation breaches; connection problems; lead–lead interaction; air entrapment in the header. @Extracardiac oversensing: myopotentials from skeletal muscle activity (diaphragmatic, pectoral, intercostal rarely); electromagnetic interference (alternating current line breakdown, interference from medical sources). AAD, anti-arrhythmic drug; AF, atrial fibrillation; ATP, anti-tachycardia pacing; CA, catheter ablation; ECG, electrocardiogram; ICD, implantable cardioverter-defibrillator; SVT, supraventricular tachycardia; S-ICD, subcutaneous ICD; TWO, T-wave oversensing.
Flowchart 2
Flowchart 2
ICD interrogation and reprogramming—unnecessary therapy suspected. ICD, implantable cardioverter-defibrillator.
Figure 3
Figure 3
ACLS in an electrical storm., ACLS, advanced cardiovascular life support; CV, cardioversion; DF, defibrillation; POCUS, point of-care ultrasound.
Figure 4
Figure 4
Inappropriate ICD therapy. (A) Fast conducting AF misdetected as VF with ICD shock (arrow), (B) fast conducting AF misdetected as VT with ATP delivered (arrow), (C) noise caused by damaged RV electrode misdetected as VF with ICD shock (arrow). Shock initiates VF. A, atrial signal; AF, atrial fibrillation; Aring, ring pole of atrial electrode; Atip, tip pole of atrial electrode; ATP, anti-tachycardia pacing; CRT, cardiac resynchronization therapy; EGM, intra-cardiac electrogram; F, ventricular fibrillation detected; ICD, implantable cardioverter-defibrillator; LV, signal received by electrode in left ventricle in CRT device; RV, signal received by electrode in the right ventricle; RVring, ring pole of electrode in the right ventricle; RVtip, tip pole of electrode in the right ventricle; STIM, stimulation; VF, ventricular fibrillation; VS or RVs, ventricular sensed event; VT, ventricular tachycardia.
Figure 5
Figure 5
Types of mechanical circulatory support: (A) intra-aortic balloon pump, (B) transvalvular microaxial flow pump (Impella), (C) tandem heart, (D) veno-arterial extracorporeal membrane oxygenator.
Figure 6
Figure 6
Stellate ganglion block. (A) Neck cross-section showing SGB guided by surface landmark technique and palpation (left) and ultrasound (right). (B) Transverse sonographic view of the neck at the level of sixth cervical vertebrae (C6). CaA, carotid artery; IJV, internal jugular vein; LCM, longus colli muscle; SCM, sternocleidomastoid muscle; SG, stellate ganglion; SGB, SG blockade; TH, thyroid.
See this image and copyright information in PMC

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