Exercise prescription using an insertable cardiac

monitor in a patient with catecholaminergic

polymorphic ventricular tachycardia
Mizuki Momoi, MD,* Yoshinori Katsumata, MD, PhD,*† Masaharu Kataoka, MD, PhD,*‡
Hiroyuki Yamagishi, MD, PhD,x Keiichi Fukuda, MD, PhD,*
Seiji Takatsuki, MD, PhD, FHRS*
From the *Department of Cardiology, Keio University School of Medicine, Tokyo, Japan, †Institute for
Integrated Sports Medicine, Keio University School of Medicine, Tokyo, Japan, ‡Second Department of
Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan, and
x
Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.

Introduction
Catecholaminergic polymorphic ventricular tachycardia KEY TEACHING POINTS
(CPVT) is a lethal arrhythmic disease that can cause sudden
 Catecholaminergic polymorphic ventricular
cardiac death in young patients, with variants in the ryano-
dine receptor 2 (RyR2) as the primary causative gene.1
tachycardia (CPVT) is often misdiagnosed as
Hence, an accurate diagnosis is essential for appropriate man- epilepsy and early diagnosis is vital for its precise
agement. However, its diagnosis is usually delayed because treatment.
of misdiagnosis as epilepsy.2–4  Recognizing the frequency of daily arrhythmia and
With respect to treating the disease, exercise restriction is supervision of compliance regarding exercise
vital in preventing the occurrence of lethal arrhythmia, and restriction is of paramount importance in the
some medications, such as beta blockers and flecainide, are
management of CPVT.
considered effective.5 Conversely, it is still unclear whether
an implantable cardiac defibrillator (ICD) improves the prog-  An insertable cardiac monitor is highly useful as a
nosis of CPVT.6,7 In addition, some patients may refuse to diagnostic and a follow-up tool in patients with
undergo ICD implantation.7 Herein, we present a case of CPVT and it helps to make their daily lives easier.
CPVT in a 19-year-old woman who was implanted with an
insertable cardiac monitor (ICM), which was effective in  Genetic testing including the trio analysis is one of
diagnosis and management, including exercise restriction. the effective ways to make an early and accurate
diagnosis of CPVT, especially in cases where clinical
diagnosis is difficult.
Case report
A 19-year-old woman had been suffering from syncope with
exertion since the age of 5 years. Based on the abnormal find-
ings of her electroencephalogram ([EEG] spike and wave syncope-related events has worsened. She had no other
complex generalized), she was diagnosed with epilepsy. significant past medical history. On physical examination,
Although she had been treated with 3 types of anticonvul- no abnormal findings were observed.
sants, the syncope recurred; moreover, the frequency of A 12-lead electrocardiogram (ECG) showed sinus brady-
cardia without a coved-type ST-segment elevation in the ante-
KEYWORDS Exercise restriction; Genetic disorders; Insertable cardiac
rior precordial leads, J wave, and QT prolongation. Peripheral
monitor; Lifestyle; Secondary prevention; Ventricular tachycardia blood tests, echocardiography, cardiac computed tomography,
(Heart Rhythm Case Reports 2022;8:17–21) and cardiac magnetic resonance imaging were all unremark-
Funding: This research did not receive any specific grant from funding able. An exercise test reproducibly induced polymorphic
agencies in the public, commercial, or not-for-profit sectors. Conflict of inter- premature ventricular contractions (PVCs) at 7 metabolic
est: The authors declare that there is no conflict of interest. Address reprint equivalents (METs) and nonsustained bidirectional and poly-
requests and correspondence: Dr Yoshinori Katsumata, Department of
morphic ventricular tachycardia at 10 METs. A drug tolerance
Cardiology, Keio University School of Medicine, 35 Shinanomachi
Shinjuku-ku Tokyo, Japan 160-8582. E-mail address: goodcentury21@ test also provoked a nonsustained bidirectional ventricular
keio.jp. tachycardia after epinephrine injection. The arrhythmia

2214-0271/© 2021 Heart Rhythm Society. Published by Elsevier Inc. This is an open access article https://doi.org/10.1016/j.hrcr.2021.10.004
under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
18 Heart Rhythm Case Reports, Vol 8, No 1, January 2022

Figure 1 The electrocardiogram from an insertable cardiac monitor during syncope. Electrocardiography with an insertable cardiac monitor revealed
bidirectional sustained ventricular tachycardia following ventricular fibrillation coupled with a syncopal attack while climbing stairs. The ventricular fibrillation
resolved spontaneously.

disappeared after the initiation of continuous intravenous fle- Following discharge, her rhythm was continuously moni-
cainide injections. Despite these clinical findings, it was diffi- tored using ICM. At first, it was difficult for her to recognize
cult to differentiate whether the syncope was due to epilepsy precisely how much intensive exercise daily caused the
attacks or lethal arrhythmia, as EEG abnormalities were pre- arrhythmia. Furthermore, nonsustained ventricular tachy-
sent. Therefore, we inserted an ICM that confirmed ventricular cardia and PVCs were often recorded during light exercises,
fibrillation during syncope (Figure 1). In the ICM record, an such as riding a bicycle up a hill or running lightly on level
incremental pattern of PVCs was observed prior to ventricular ground (Figure 3). Despite her having palpitations, there
tachycardia, consistent with her palpitations. Ventricular were no episodes of syncope. At the outpatient visit, we
tachycardia and ventricular fibrillation appeared while climb- shared the record with the patient, and the exercise restriction
ing the stairs. This lethal arrhythmia spontaneously converted level was modified according to the log data of arrhythmia.
to sinus rhythm without electrical defibrillation. Although she The safe threshold of her daily activity could be easily
had no family history of sudden cardiac death, we performed comprehended by confirming the ECG findings, which
trio whole-exome sequencing of her family. Genetic analysis made her compliant with the exercise restriction. Half a
revealed a de novo heterozygous c.12388A.C missense month after discharge, the arrhythmic events decreased in
variant (S4130R) in RyR2. The minor allele frequency was frequency, and her symptoms stabilized. Anticonvulsant
,0.01 in all public databases. agents were gradually reduced; however, special attention
First, we treated her with optimal pharmacological therapy was given to the possible recurrence of epilepsy. The patient
using 5 mg of bisoprolol fumarate and 200 mg of flecainide, has been doing well for approximately 2 years, with no prob-
which was found to be highly effective during the drug lems.
tolerance test. An ICD was not implanted because its efficacy
was reportedly limited and the patient refused it.7 Although Discussion
potentially lethal arrhythmic events were frequently induced Since patients with pathogenic variants in the RyR2 often have
by low-intensity exercise even after drug administration, sym- the same abnormal EEG findings as patients with epilepsy,8 it
pathetic denervation was unacceptable owing to its aversion would be extremely difficult to distinguish CPVT from epi-
to specific complications. Therefore, catheter ablation of repro- lepsy in early phases, especially during childhood; however,
ducible bidirectional PVCs localized in the left ventricular infer- a previous study reported that video EEG monitoring was use-
oseptal and left ventricular outflow tract areas was performed ful for distinguishing epilepsy from CPVT.9 It enables contin-
(Figure 2). An exercise test at 10 METs showed no ventricular uous and long-acting monitoring of electrical activities in the
tachycardia after successful catheter ablation or optimal brain. In addition, continuous ECG monitoring using ICM
pharmacological therapy. The postdischarge exercise restric- may be an alternative method because ICM may directly iden-
tion was set at 10 METs according to the exercise test. tify the existence of a lethal arrhythmia during syncope, with
Momoi et al Insertable Cardiac Monitor and Exercise Restriction 19

Figure 2 3D mapping, pace mapping, and activation mapping. Catheter ablation was performed as a therapy for catecholaminergic polymorphic ventricular
tachycardia. The red tags indicate ablation points. A: 3D mapping, pace mapping, and activation mapping of the green tag of premature ventricular contractions
(PVC) arising from the left ventricular inferoseptum. B: 3D mapping, pace mapping, and activation mapping of the green PVC tag arising from the left ventricular
summit area.

high sensitivity and specificity. ICM may monitor the ECG exercise caused arrhythmia. Simultaneously, physicians easily
continuously without interruption; therefore, it can reflect recognized both symptomatic and asymptomatic events.
arrhythmias that are closely related to daily life. These benefits were thought to have led to effective
According to current guidelines,1 lifestyle modifications management and stability of her condition. Thus, accurate
are also recommended in patients with CPVT because exercise recognition of arrhythmia thresholds and complete removal
causes lethal arrhythmias in accordance with catecholamine of triggers are important for the prevention of fatal
release. Therefore, recognizing the frequency of arrhythmias arrhythmias.11
in daily life and compliance with exercise restriction is of para- ICD implantation is recommended when CPVT patients
mount importance. Cheung and colleagues10 argued that the experience cardiac arrest or recurrent syncope despite
restriction of moderate activity could also be needed to prevent optimal therapy. However, a previous study reported poor ef-
lethal arrhythmias, in addition to avoiding extreme exercise. ficacy of ICD shocks for triggered arrhythmias.6 Moreover,
However, some patients cannot obey the exercise restriction, an observational study revealed that 42% of CPVT patients
especially if they are young. In our case, the patient was moni- who experienced cardiac arrest were younger and did not
tored continuously via ICM after the diagnosis of CPVT. receive ICD implantation.7 In our case, the patient, who
Although she had frequently experienced palpitations while was a young woman, refused to receive ICD implantation.
exercising, such as running or cycling, she recorded the Further investigation into the indications for ICD in patients
episodes with ICM and recognized how much intensive with CPVT is required.
20 Heart Rhythm Case Reports, Vol 8, No 1, January 2022

Figure 3 The electrocardiogram from an insertable cardiac monitor during follow-up. The insertable cardiac monitor showed premature ventricular contraction
when the patient ran in her daily life after the initiation of beta blockers and flecainide.

With respect to PVC ablation, it is not recommended as Acknowledgments

the standard therapy according to the current guideline.1 We would like to thank Editage (www.editage.com) for
However, a previous study using a mouse model reported English language editing.
that the Purkinje network was critically associated with ar-
rhythmogenic triggers in CPVT and may be a viable thera-
peutic target.12 A single case report showed the References
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Momoi et al Insertable Cardiac Monitor and Exercise Restriction 21

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