European Heart Journal (2003) 24, 787–788
Editorial
Right ventricular tachycardia—arrhythmogenic
right ventricular cardiomyopathy or idiopathic?
Hans Kottkamp *, Gerhard Hindricks
Department of Electrophysiology, University of Leipzig—Heart Center Cardiology, Struempellstrasse 39,
D-04289 Leipzig, Germany
See doi:10.1016/S1095-668X(02)00654-1, for the
article to which this editorial refers.
The majority of right ventricular tachycardias ex-
hibit a left bundle branch block pattern with an
inferior axis. The classical differential diagnosis is
either idiopathic right ventricular outflow tract
tachycardia or arrhythmogenic right ventricular
dysplasia/cardiomyopathy. Recently, the entity of
right ventricular outflow tract tachycardia has been
extended to outflow tract tachycardias with poten-
tial origins in the right ventricular outflow tract,
the left ventricular outflow tract or the aortic sinus
of valsalva.1,2 Especially the R/S transition in the
precordial leads of the 12-lead surface ECG has
been useful to differentiate between a left or right-
sided origin, with the classical left bundle branch
block pattern indicating to the right side and a
premature transition at V1 to V3 indicating more
towards the left side or the aortic sinus. In some
cases, the definite origin may only be defined peri-
interventionally. In general, right ventricular out-
flow tract tachycardia occurs in the absence of
structural heart disease and has a favorable prog-
nosis.3 In some studies, however, structural abnor-
malities have been described using magnetic
resonance imaging.4 Clinical manifestations include
nonsustained or sustained ventricular tachycardias
that are related to physical exercise in a significant
portion of the cases.
Arrhythmogenic right ventricular cardiomy-
opathy is characterized pathologically by fibro-
fatty replacement of the right ventricular
myocardium.5,6 Although segmental right ventricu-
* Corresponding author. Tel.: +49-341-865-1413; fax:
+49-341-865-1460
E-mail address: kotth@medizin.uni-leipzig.de (H. Kottkamp).
0195-668X/03/$ - see front matter © 2003 The European Society of Cardiology. Published by Elsevier Science Ltd. All rights reserved.
doi:10.1016/S0195-668X(02)00847-3
lar disease is most commonly seen, evolution to a
more diffuse right ventricular involvement and also
left ventricular abnormalities have been described.
Clinical manifestations include depolarization/
repolarization abnormalities in the right precordial
ECG leads, structural abnormalities of the right
(and rarely left) ventricle, ventricular tachycardias
and presentation with sudden cardiac death, and
a family history of the disease. Criteria for diag-
nosis of arrhythmogenic right ventricular dysplasia
include global and/or regional dysfunction, histo-
logic tissue characteristics, repolarization and
depolarization/conduction abnormalities, arrhyth-
mias and a family history.5,7 The fulfillment of the
diagnosis of right ventricular dysplasia has been
proposed by the presence of two so-called major
criteria, one major plus two minor criteria, or four
minor criteria. Theoretically, the definite diagnosis
of right ventricular dysplasia is based on the histo-
logical demonstration of transmural fibro-fatty re-
placement of right ventricular myocardium at
either necroscopy or surgery. Obviously, the clini-
cal value with respect to findings at necroscopy or
surgery as diagnostic criteria is very limited and, in
addition, assessment of the diagnosis based on right
ventricular endomyocardial biopsy specimens is
also inherently difficult for several reasons.5–7 In
clinical praxis, the histological demonstration of
transmural fibro-fatty replacement therefore does
not play a major role.
The study by O'Donnell et al.8 in this issue of
the European Heart Journal addresses clinical and
electrophysiological differences between patients
with arrhythmogenic right ventricular dysplasia and
right ventricular outflow tract tachycardia. The
study presents important data because the clinical
problem is analyzed comprehensively with clini-
788
cal data, electrocardiographic data, echo data,
magnetic resonance imaging data, invasive electro-
physiologic data as well as results of catheter abla-
tion including a relatively long follow-up period.
Needless to say that the proposed major and minor
criteria were applied for differentiation between
the two clinical entities by the authors. Interest-
ingly, a mean number of only 1.2 major criteria was
found in the patient group with arrhythmogenic
right ventricular cardiomyopathy. The higher mean
number of 3.0 minor criteria was therefore neces-
sary for the diagnosis emphasizing the puzzle-like
steps in reaching the diagnosis of arrhythmogenic
right ventricular cardiomyopathy. The value of
magnetic resonance imaging is critically high-
lighted in the present study with 54% of the patients
with idiopathic right ventricular tachycardia show-
ing some structural abnormalities. In contrast, no
structural abnormalities were found using echocar-
diography or right ventricular cine angiography in
the idiopathic patient group.
Among the key information of the present study
is the reported data from the invasive electrophysi-
ological studies.8 A first criterion for differentiation
was the mode of induction, with 82% of the cardio-
myopathy patients being inducible with critically
timed extrastimuli versus only 3% in the idiopathic
group indicating the reentrant mechanism of the
ventricular tachycardias in the majority of patients
with arrhythmogenic right ventricular cardio-
myopathy. Secondly, the electrophysiologic study
revealed a range of one to six morphologies of
inducible ventricular tachycardias (mean 1.8), with
71% of the patients having more than one mor-
phology. In contrast, the patients with idiopathic
ventricular tachycardia all had only one mor-
phology. The presence of more than one mor-
phology in the cardiomyopathy group is best
explained by the broader arrhythmogenic substrate
allowing the perpetuation of more than one reen-
trant circuit or at least the presence of more than
one exit site. The third invasive criterion of frag-
mented potentials also relates to the substrate
created by the fibro-fatty myocardial replacement
in arrhythmogenic right ventricular cardiomyopa-
thy with the creation of zones of slow and/or zigzag
conduction. The direct electrogram recordings dur-
ing the electrophysiologic study revealed frag-
mented potentials in 82% of the cardiomyopathy
Editorial
patients and, therefore, were much more sensitive
than the surface ECG correlate, i.e. the epsilon
potentials, that were found in only 30% of the
cardiomyopathy patients. In addition, the specifi-
city of fragmented potentials remained high since
fragmented potentials could only be detected in a
single patient (3%) in the idiopathic group.
Overall, the study by O'Donnell comprehensively
describes the differences between patients with
idiopathic right ventricular outflow tract tachy-
cardia and arrhythmogenic right ventricular cardio-
myopathy from a clinical point of view. This also
includes the possibility of re-grouping a patient
with suspected idiopathic ventricular tachycardia
towards the cardiomyopathy group after inducing
additional ventricular tachycardia morphologies
and the recording of fragmented potentials during
the invasive electrophysiologic study. In the indi-
vidual patient, such a re-grouping is not academi-
cal, given the striking differences in therapy, i.e.
catheter ablation versus implantable defibrillator,
and also in prognosis.
References
1. Callans D, Menz V, Schwartzman D et al. Repetitive mono-
morphic tachycardia from the left ventricular outflow tract:
electrocardiographic patterns consistent with a left ventricu-
lar site of origin. J Am Coll Cardiol 1997;29:1023–7.
2. Kanagaratnam L, Tomassoni G, Schweikert R et al. Ventricu-
lar tachycardias arising from the aortic sinus of valsalva: an
under-recognized variant of left outflow tract ventricular
tachycardia. J Am Coll Cardiol 2001;37:1408–14.
3. Wilber DJ, Baerman J, Olshansky B et al. Adenosine-sensitive
ventricular tachycardia. Clincial characteristics and response
to catheter ablation. Circulation 1993;87:126–34.
4. Carlson MD, White RD, Trohman RG et al. Right ventricular
outflow tract tachycardia: detection of previously unrecog-
nized anatomic abnormalities using cine magnetic resonance
imaging. J Am Coll Cardiol 1994;24:720–7.
5. McKenna WJ, Thiene G, Nava A et al. Diagnosis of arrhyth-
mogenic right ventricular dysplasia/cardiomyopathy. Br
Heart J 1994;71:215–8.
6. Gemayel C, Pelliccia A, Thompson PD. Arrhythmogenic right
ventricular cardiomyopathy. J Am Coll Cardiol 2001;
38:1773–81.
7. Corrada D, Fontaine G, Marcus FI et al. Arrhythmogenic right
ventricular dysplasia/cardiomyopathy: need for an inter-
national registry. Circulation 2000;101:E101–6.
8. O'Donnell, D, Cox, D, Bourke, J. et al. Clinical and electro-
physiological differences between patients with arrhyth-
mogenic right ventricular dysplasia and right ventricular
outflow tract tachycardia. Eur Heart J 2003;24:801–810.