Clinical Review & Education

Challenges in Clinical Electrocardiography

A Case of Ventricular Arrhythmia

When the Right Isnt Right
Shing Ching, MBBS; Chiu Sun Yue, MBBS, MRCP

A woman in her 60s presented to the emergency department
with palpitation and dyspnea. She had multiple syncopal episodes
months prior. Her blood pressure was 154/78 mm Hg, her pulse
was 184 beats per minute, and
her arterial oxygen saturation
Video at
was 98% breathing ambient air.
jamainternalmedicine.com
Her electrocardiogram (ECG) is
shown (Figure 1A). The arrhythmia did not respond to adenosine.
Following amiodarone infusion, a second ECG test was performed
(Figure 1B).
Questions: What is the cause of the arrhythmia? Does this
patient need an implantable cardioverter-defibrillator (ICD)?
Interpretation and Clinical Course
The first ECG (Figure 1A) shows a regular wide-complex tachycar-
dia. Bumps that are variably related to QRS complexes, best seen
in lead V1, are P waves marching through the tachycardia, indicat-
ing atrioventricular dissociation (Figure 1A, red arrowheads). The QRS
of a different morphology at the right side of the strip, occurring at
an expected interval from the previous beat, and preceded by a P
wave (Figure 1A), is a fusion beat. This ECG is diagnostic of ventricu-
lar tachycardia (VT). The tachycardia has a left-bundle branch block
(LBBB) pattern, an inferior axis and predominant R in lead 1, prob-
ably originating from right ventricular outflow tract (RVOT).
The patient was pharmacologically cardioverted to sinus rhythm
(Figure 1B). This ECG is a clue to the etiology of the arrhythmia. First,
following each RSR complex in lead V1 are low-amplitude depolariza-
tions that are epsilon waves, a feature of fragmented RV conduction
(Figure 2). A corollary of the same phenomenon is terminal activa-
tion delay, which is taken from the nadir of the S wave to the end of
all depolarization before the T wave. It is prolonged at 90 millisec-
onds (normal being < 55 milliseconds). Second, T waves are in-
verted in leads V1 to V5. Thus, this patient meets 2 major and 1 minor
criteria of arrythmogenic right ventricular cardiomyopathy (ARVC)1:
(1) epsilon wave in right precordial leads; (2) inverted T waves be-
yond V3; and (3) sustained VT of an LBBB morphology and inferior axis.
The patients coronary angiogram showed only minor irregu-
larities. Transthoracic echocardiographic findings revealed dilated

Figure 1. Patient Electrocardiograms

A Initial electrocardiogram

V1
aVR
V4

V2

aVL
I

V5

V3
II aVF
V6

B Electrocardiogram following pharmacologic cardioversion

V1 V4 A, Initial electrocardiogram showing

aVR
I regular wide-complex tachycardia
with left bundle-branch block
morphology and inferior axis. P-waves
march through the QRS indicative of
V2 V5 atrioventricular dissociation (red
II
aVL arrowhead). A fusion beat (star) is
noted at the right side of the strip
preceded by a P-wave (black
arrowhead); B, Electrocardiogram
III V3 V6 following pharmacologic
aVF cardioversion. QRS in V1 appears
fragmented. Note T wave inversion in
leads V1 to V5.

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 Clinical Review & Education Challenges in Clinical Electrocardiography

Figure 2. Enlarged View of Lead V1
TAD
Enlarged view of lead V1. Note that this is not right bundle-branch block. Black
arrowhead points to the epsilon wave. Terminal activation delay (TAD), taken
from nadir of the S wave to the end of all depolarization before the T wave, is
prolonged at 90 milliseconds (normal being < 55 milliseconds).
RV, areas of dyskinesia and thinning over RV free wall, dilated RV out-
flow tract, depressed left ventricular (LV) ejection fraction, and LV
posterolateral wall thinning (Figure 3, Video1, 2, and 3), thereby
meeting 1 more major criteria of ARVC1: regional RV akinesia and
RVOT dilation. Cardiac magnetic resonance (CMR) imaging was not
performed because of a metallic hip implant.
The patient was diagnosed as having ARVC after meeting 3
major and 1 minor criteria, and received an ICD for secondary pre-
vention. She continued to experience VT terminated by antitachy-
cardia pacing and shocks. The addition of amiodarone to her treat-
ment led to significant improvement. Genetic testing did not show
pathogenic mutations. No other family members have symptoms.
Discussion
Arrhythmogenic right ventricular cardiomyopathy is an inheritable car-
diomyopathy characterized by progressive fibrofatty replacement of

Figure 3. Transthoracic Echocardiogram

A Parasternal short axis B Parasternal long axis

C Subcostal four-chamber D Tricuspid valve systolic gradient

A, Parasternal short axis view at the aortic valve level showing a dilated RV outflow tract; B, dilated proximal RV outflow tract shown in parasternal long axis view;
C, subcostal four-chamber view demonstrating RV free wall akinesia and thinning (white arrowhead); D, normal peak tricuspid regurgitation velocity indicating
absence of significant pulmonary hypertension.

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 Challenges in Clinical Electrocardiography Clinical Review & Education

right, or both ventricular walls, caused by mutations of desmosomal
proteins that are identified in about half of cases. Arrhythmogenic right
ventricular cardiomyopathy preferentially affects the subtricuspid tri-
angle, bordered by the RV free wall, apex, and outflow tract,2 result-
ing in RV free wall dyskinesia and RV outflow tract dilation. It may also
involve the posterior and lateral walls of the LV while sparing the sep-
tum. These features can be subtle on echocardiographic results, be-
coming more apparent in advanced disease. Cardiac magnetic reso-
nance imaging enables quantification of RV function, and
characterization of RV fat and fibrosis, but is not to be used without
caveats. For example, the presence of adipose tissue in the RV wall is
not always pathological; normal RV wall motion can appear dyssyn-
chronous and difficult to distinguish from early disease.2 Indeed, CMR
findings are more often overread, and echocardiographic findings are
more often underread for ARVC.3
The scarred RV conducts more slowly and has abnormal repo-
larization, giving rise to epsilon waves and right precordial T-wave in-
version. Terminal activation delay, a signature of slow RV conduc-
tion, identifies ARVC with good sensitivity,1 especially because epsilon
waves are present only in 1% to 9% of newly diagnosed patients. In
advanced disease, epsilon waves are more prevalent, associated with
poorer RV function and higher risk for ventricular arrhythmias.4
Electrical instability is a hallmark of ARVC that may predate RV
dysfunction. Most commonly VT is monophormic, has an LBBB mor-
phology, a superior, inferior, or undetermined axis, and may mimic id-
iopathic RVOT VT. The differentiation is important because ARVC is
potentially fatal and is not cured by radiofrequency ablation. T-wave
inversion in leads V1 to V3 in sinus rhythm is more common in ARVC
and more rare in idiopathic RVOT VT; it is specific but not sensitive.5
Therefore, further diagnostic tests may be necessary for individuals
with red flags, including family history of sudden death and recur-
rent VT.
ARVC is diagnosed by a constellation of clinical, electrocardio-
graphic, and imaging findings as outlined in the revised Task Force
Criteria.1 They are grouped under the headings of RV dysfunction,
tissue characterization of RV wall on endomyocardial biopsy, repo-
larization abnormalities, depolarization abnormalities, arrhyth-
mias, and family history, each describing a set of major and minor
criteria. Depending on the criteria fulfilled, the diagnosis is deemed
definite, borderline, or possible. Patients suspected of having ARVC
may have less pronounced RV dysfunction that may not meet
imaging criteria. It is important to consider other tests that may con-
tribute to diagnosis, including signal-averaged ECG, Holter moni-
toring, and genetic testing.
ARVC is associated with ventricular arrhythmia and sudden
death. Aborted sudden cardiac death, sustained VT, and severe ven-
tricular dysfunction define a high-risk population in whom ICD im-
plantation is beneficial.6 Since arrhythmias may be adrenergically
mediated, all patients are advised against strenuous exercise and re-
ceive a blocker. Nevertheless, some may continue to experience
VT, requiring treatment with anti-arrhythmic drugs.
Take-Home Points
ARVC causes syncope, ventricular arrhythmias, and sudden car-
diac death.
Epsilon waves are present in only a minority of patients. Terminal
activation delay is a more sensitive ECG marker of RV fragmented
conduction.
ARVC is diagnosed by demonstrating a constellation of clinical, elec-
trocardiographic, imaging, and tissue findings outlined in the re-
vised Task Force Criteria.
Overreliance on imaging may result in overdiagnosis or underdi-
agnosis. Other clinical aspects have to be considered, including ECG,
family history, and morphology of arrhythmia.
Strenuous exercise is contraindicated. All patients should receive
a -blocker, and ICD for high risk individuals. Family screening
should be offered to confirmed cases.

ARTICLE INFORMATION
Author Affiliations: Division of Cardiology,
Department of Medicine and Geriatrics, United
Christian Hospital, Hong Kong, China.
Corresponding Author: Shing Ching, MBBS,
Department of Medicine and Geriatrics, United
Christian Hospital, Hip Wo Street, Kwun Tong, Hong
Kong, China (aj12029@gmail.com).
Section Editors: Zachary D. Goldberger, MD, MS;
Nora Goldschlager, MD; Elsayed Z. Soliman, MD,
MSc, MS.
Published Online: May 31, 2016.
doi:10.1001/jamainternmed.2016.1982.
Conflict of Interest Disclosures: None reported.
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