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British Journal of Healthcare and Medical Research - Vol. 11, No. 3
Publication Date: June 25, 2024
DOI:10.14738/bjhmr.113.16959.
Cillis, N. I., Bodziock, G. M., Ferris, T. S., Patel, N., Pothuraju, T. V., & Bhave, P. D. (2024). Is the Advanced Cardiac Life Support
Algorithm Always Correct? A Case of Catecholaminergic Polymorphic Ventricular Tachycardia. British Journal of Healthcare and
Medical Research, Vol - 11(3). 73-78.
Services for Science and Education – United Kingdom
Is the Advanced Cardiac Life Support Algorithm Always Correct?
A Case of Catecholaminergic Polymorphic Ventricular
Tachycardia
Nicole I. Cillis
Department of Internal Medicine,
Atrium Health Wake Forest Baptist Medical Center,
One Medical Center Blvd, Winston Salem, NC 27157
George M. Bodziock
Cardiovascular Medicine,
Atrium Health Wake Forest Baptist Medical Center,
One Medical Center Blvd, Winston Salem, NC 27157
Taylor S. Ferris
Department of Internal Medicine,
Atrium Health Wake Forest Baptist Medical Center,
One Medical Center Blvd, Winston Salem, NC 27157
Nikhil Patel
Cardiovascular Medicine,
Atrium Health Wake Forest Baptist Medical Center,
One Medical Center Blvd, Winston Salem, NC 27157
Tejit V. Pothuraju
Department of Internal Medicine,
Atrium Health Wake Forest Baptist Medical Center,
One Medical Center Blvd, Winston Salem, NC 27157
Prashant D. Bhave
Cardiovascular Medicine,
Atrium Health Wake Forest Baptist Medical Center,
One Medical Center Blvd, Winston Salem, NC 27157
ABSTRACT
A 19-year-old male with catecholaminergic polymorphic ventricular tachycardia
(CPVT) presented with outside-of-hospital cardiac arrest in electrical storm.
Ventricular arrhythmias persisted while following the Advanced Cardiac Life
Support (ACLS) algorithm using epinephrine boluses. Upon deviation from the ACLS
algorithm and use of beta blockers, ventricular arrhythmias ceased and patient was
successfully stabilized. Treatment of CPVT electrical storm is challenging due to
limited guidelines. Antiarrhythmic agents such as beta blockers and flecainide are
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British Journal of Healthcare and Medical Research (BJHMR) Vol 11, Issue 03, June-2024
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among the primary treatment options. This case report highlights a unique example
of how deviation from traditional ACLS may be necessary to best treat CPVT storm.
Keywords: Catecholaminergic polymorphic ventricular tachycardia, ventricular
arrhythmia, electrical storm, ACLS
INTRODUCTION
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited
arrhythmogenic disorder linked to mutations in the cardiac ryanodine receptor (RYR2) and
cardiac calsequestrin 2 (CASQ2) [1,2]. CPVT is characterized by adrenergic-induced
bidirectional and polymorphic ventricular tachycardia (VT) [1,2]. Due to mutations in these
receptors, the sarcoplasmic reticulum inappropriately leaks calcium leading to a large amount
of calcium in the cytosol which can predispose patients with CPVT to ventricular arrhythmias
due to delayed afterdepolarizations [1,3].
CPVT usually presents as syncope or sudden cardiac death in children with structurally normal
hearts at an average age of 8 years old [1,2]. In patients with CPVT, adrenergic stimulation, such
as emotional stress or exercise, results in intracellular calcium mishandling and ventricular
arrhythmias [1]. The mortality rate of untreated CPVT is reportedly as high as 31% by the age
of 30 years old [1].
The resting electrocardiogram (ECG) findings include a normal QTc, which is an important
distinction, as it differentiates polymorphic VTs arising from congenital long QT syndrome from
CPVT [1]. However, as heart rate increases, these patients will develop monomorphic
premature ventricular beats then transition into bidirectional VT, polymorphic VT, and
ultimately ventricular fibrillation (VF) [1,2]. The reverse sequence in arrhythmias occur as the
heart rate downtrends [1].
Ambulatory management of CPVT is focused on lifelong beta blockers (BBs), notably nadolol.
BBs are usually effective for the prevention of recurrent arrhythmias, although there have been
reports of recurrence of cardiac events/ incomplete prevention [4,5]. An implantable
cardioverter defibrillator (ICD) should be considered in these patients who continue to have
arrhythmias despite BB usage [6]. However, ICDs come with a potential to induce electrical
storms due to inappropriate shocks. An alternative antiarrhythmic agent, flecainide, has shown
promise, in conjunction with conventional drug therapy, to reduce exercise-induced ventricular
arrhythmias in patients with CPVT [6,7]. Specifically, flecainide directly blocks the RYR2
channels, thus preventing calcium release preventing ventricular arrhythmias [6,7]. A potential
treatment to consider in refractory cases to BBs is left cardiac sympathetic denervation, in
which the left stellate ganglion (T1) and thoracic ganglia (T2-T4) are resected, interrupting
norepinephrine release, thus providing an anti-fibrillatory effect [2]. However, this technique
has side effects and requires an experienced surgeon [1,2]. Recommended lifestyle medications
include limiting competitive sports, strenuous exercise, and stressful situations [2].
Treatment of patients with CPVT storm is challenging because of limited guidelines. Consistent
with treatment for prevention, BBs are first line during acute electrical storm. Intravenous BBs
can be combined with IV procainamide as well. Additionally, deep sedation and mechanical
circulatory support (MCS) should be considered when electrical storm is refractory to IV
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Cillis, N. I., Bodziock, G. M., Ferris, T. S., Patel, N., Pothuraju, T. V., & Bhave, P. D. (2024). Is the Advanced Cardiac Life Support Algorithm Always
Correct? A Case of Catecholaminergic Polymorphic Ventricular Tachycardia. British Journal of Healthcare and Medical Research, Vol - 11(3). 73-78.
URL: http://dx.doi.org/10.14738/bjhmr.113.16959.
antiarrhythmics [3]. According to the current 2020 Adult Basic Life Support (ACLS) algorithm,
the first drug of choice is epinephrine for a patient in VF/pulseless VT [8]. However, in patients
with CPVT, epinephrine can rather be arrhythmogenic. Here, we present a case of VT storm
successfully treated by deviating from the ACLS algorithm.
CLINICAL CASE
A 19-year-old male with medical history notable for single chamber ICD implant for CPVT with
remote cardiac arrest in childhood presented with out-of-hospital cardiac arrest. In the field,
the patient was intubated and defibrillated for VF. Upon arrival to the hospital, he developed
multiple episodes of bidirectional VT degenerating to VF requiring defibrillation [Fig.1].
He was admitted to the cardiovascular intensive care unit with VT storm, requiring extensive
CPR with chest compressions and numerous external shocks, ultimately requiring MCS for
support. He was initiated on intravenous amiodarone, lidocaine, and procainamide. During the
ACLS algorithm with chest compressions and intermittent epinephrine boluses, it became
apparent that the arrhythmia burden worsened after epinephrine. In addition, with resuming
chest compressions immediately after defibrillation, it was clear that a narrow complex rhythm
degenerated back into VT and VF during continued chest compressions prior to the next
scheduled pulse check [Fig. 2].
Fig. 1: 12-lead electrocardiograms from admission. (A) Presenting ECG with sinus rhythm. (B)
Repeat ECG with sinus rhythm and T wave inversion in inferior and lateral leads. (C) Repeat
ECG with frequent short-coupled premature ventricular contractions in a pattern of bigeminy.
(D) Final ECG with beat-to-beat alternation of frontal QRS axis consistent with bidirectional
ventricular tachycardia.
The code team elected to cease epinephrine boluses and perform an early pulse check after the
next defibrillation. With this, sinus rhythm with a pulse was achieved after the next
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defibrillation, and the patient transiently maintained normal sinus rhythm, but again
degenerated into VF. The decision was made to pursue VA ECMO. With MCS, the patient’s
medical regimen was consolidated to procainamide and BB as hemodynamics tolerated. He
remained electrically quiescent for the remainder of the hospitalization.
Ultimately, the patient was decannulated and extubated. His home nadolol and flecainide were
resumed. The patient later revealed he had stopped taking his home medications, leading to his
arrest. At outpatient follow-up, he was doing well and surgical sympathectomy was discussed
as a future management option.
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Cillis, N. I., Bodziock, G. M., Ferris, T. S., Patel, N., Pothuraju, T. V., & Bhave, P. D. (2024). Is the Advanced Cardiac Life Support Algorithm Always
Correct? A Case of Catecholaminergic Polymorphic Ventricular Tachycardia. British Journal of Healthcare and Medical Research, Vol - 11(3). 73-78.
URL: http://dx.doi.org/10.14738/bjhmr.113.16959.
Fig. 2: (A) Telemetry tracing with two vectors showing ventricular bigeminy and then
bidirectional VT which further degenerates into VF, along with the corresponding arterial
waveform in red. (B) Additional tracing shows attempted defibrillation followed by chest
compressions. There appears to be a narrow complex rhythm after defibrillation that
corresponds to the arterial waveform (narrow complexes highlighted with red circles and
corresponding arterial wave form highlighted with red arrows), but this degenerates back into
VF during chest compression. (C) Final tracing shows return of a narrow complex rhythm after
subsequent defibrillation. This was recognized and early pulse check was performed, which
revealed a perfusing sinus rhythm.
DISCUSSION
This case highlights an example when deviation from traditional ACLS protocol is necessary.
Electrical storm, due to CPVT, is characterized by adrenergic-induced ventricular arrhythmias.
This patient was initially treated following the ACLS algorithm with persistence of ventricular
arrhythmias. Chest compressions and defibrillation resulted in increased adrenergic tone
paradoxically creating a cycle of worsening electrical storm. As such, the team deviated from
the algorithm by withholding additional epinephrine boluses and administered IV BB and
procainamide, with return of sinus rhythm. Additionally, an early pulse check immediately after
defibrillation appeared to help prevent the return to VT. Algorithms provide a guideline for
medical practice, however, this case provides a unique example of how deviation from
traditional ACLS may be necessary to best treat CPVT storm.
CONCLUSIONS
CPVT is a rare inherited disorder that can cause cardiac arrest from ventricular arrhythmias in
the setting of elevated catecholamines. During resuscitative efforts of VT storm, avoidance of
epinephrine and use of IV antiarrhythmics are best steps of management in ACLS.
References
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