AbstractA 16-year-old girl with a known history of asymptomatic Wolff-Parkinson-White syndrome exhibited signs of left ventricular (LV) septal akinesia and LV dysfunction during routine follow-up. A 12-lead surface ECG showed pre-excitation, a predominantly negative delta wave in V1 and left axis deviation, which was consistent with the presence of a right free-wall accessory pathway. Radiofrequency ablation of the anterolateral right atrium around the local shortest atrium-to-ventricle interval created the accessory pathway block. An echocardiogram taken one month after the procedure revealed that LV septal wall motion had normalized and that LV ejection fraction had improved from 50% before the ablation to 64% after the ablation. Most previous reports of asymptomatic patients of WPW with LV septal dyskinesia and dysfunction have described right septal or posteroseptal accessory pathways. This patient reported here represents a rare case with right free-wall accessory pathway and LV dysfunction without tachycardia

Barth, P.G.

Liebaers, I.

Lissens, W.

Meirleir, L. de

Ruitenbeek, W.

Seneca, S.

Vreken, P.

Wanders, R.J.

Wijburg, F.A.

Radboud Repository

Cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene.

Pyruvate dehydrogenase (PDH) complex deficiency, a common cause of congenital lactic acidosis, is mostly due to mutations in the X-linked gene coding for the E1alpha subunit of the complex. We have studied two unrelated girls presenting a static encephalopathy with spastic quadriplegia, microcephaly and seizures and in one girl, hypocalcaemia, a new finding in PDH complex deficiency. PDH deficiency was diagnosed in adolescence and both girls had low PDH complex activity in muscle but normal amounts of all subunits on Western blotting, and a normal lactate/pyruvate ratio in blood and CSF. Mutation analysis of the E1alpha gene at the cDNA or DNA level revealed an arginine to histidine substitution at amino acid position 288 (R288H) in the girl with hypocalcaemia and a 12 bp insertion, predicting a four amino acid duplication at the c-terminal end of the protein in the second girl. They both carried a normal and a mutated E1alpha gene and X-inactivation studies showed skewed patterns. CONCLUSION: Mutation identification in pyruvate dehydrogenase complex deficiency remains important especially for the determination of the recurrence risk and for reliable genetic counselling in couples with an affected chil

Barth, P. G.

Wijburg, F. A.

Wanders, R. J.

de Meirleir, L.

NARCIS 

Cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene

Mine, Takanao

Kodani, Takeshi

Hamaoka, Mamoru

Masuyama, Tohru

Elsevier - Publisher Connector 

An Asymptomatic Case of Wolff-Parkinson-White Syndrome with Right-sided Free-wall Accessory Pathway and Left Ventricular Dysfunction 

Takanao Mine

Takeshi Kodani

Mamoru Hamaoka

Tohru Masuyama

Crossref

Journal of Arrhythmia

An Asymptomatic Case of Wolff-Parkinson-White Syndrome with Right-sided Free-wall Accessory Pathway and Left Ventricular Dysfunction

A 16-year-old girl with a known history of asymptomatic Wolff-Parkinson-White syndrome exhibited signs of left ventricular (LV) septal akinesia and LV dysfunction during routine follow-up. A 12-lead surface ECG showed pre-excitation, a predominantly negative delta wave in V1 and left axis deviation, which was consistent with the presence of a right free-wall accessory pathway. Radiofrequency ablation of the anterolateral right atrium around the local shortest atrium-to-ventricle interval created the accessory pathway block. An echocardiogram taken one month after the procedure revealed that LV septal wall motion had normalized and that LV ejection fraction had improved from 50% before the ablation to 64% after the ablation. Most previous reports of asymptomatic patients of WPW with LV septal dyskinesia and dysfunction have described right septal or posteroseptal accessory pathways. This patient reported here represents a rare case with right free-wall accessory pathway and LV dysfunction without tachycardia

Takanao Mine, MD

Takeshi Kodani, MD

Mamoru Hamaoka, MD

Tohru Masuyama, MD

Directory of Open Access Journals

An Asymptomatic Case of Wolﬀ-Parkinson-White
Syndrome with Right-sided Free-wall Accessory Pathway
and Left Ventricular Dysfunction
Takanao Mine MD, Takeshi Kodani MD, Mamoru Hamaoka MD, Tohru Masuyama MD
Department of Internal Medicine, Cardiovascular Division, Hyogo College of Medicine,
Nishinomiya, Japan
A 16-year-old girl with a known history of asymptomatic Wolﬀ-Parkinson-White
syndrome exhibited signs of left ventricular (LV) septal akinesia and LV dysfunction during
routine follow-up. A 12-lead surface ECG showed pre-excitation, a predominantly negative
delta wave in V1 and left axis deviation, which was consistent with the presence of a right
free-wall accessory pathway. Radiofrequency ablation of the anterolateral right atrium around
the local shortest atrium-to-ventricle interval created the accessory pathway block. An
echocardiogram taken one month after the procedure revealed that LV septal wall motion had
normalized and that LV ejection fraction had improved from 50% before the ablation to 64%
after the ablation. Most previous reports of asymptomatic patients of WPW with LV septal
dyskinesia and dysfunction have described right septal or posteroseptal accessory pathways.
This patient reported here represents a rare case with right free-wall accessory pathway and
LV dysfunction without tachycardia.
(J Arrhythmia 2011; 27: 145–149)
Key words:
Pre-excitation, Septal akinesia, Left ventricular dysfunction, Dyssynchrony, Radiofrequency
ablation
Introduction
Supraventricular tachycardia (SVT) is a common
arrhythmia in patients with Wolﬀ-Parkinson-White
(WPW) syndrome.1) Left ventricular (LV) dysfunc-
tion may arise in those patients with symptomatic
WPW syndrome. It has been recognized that frequent
or incessant SVT may induce dilated cardiomyop-
athy, i.e., tachycardia-induced cardiomyopathy.2,3)
Echocardiographic abnormalities have been reported
in WPW syndrome since the 1970s. A signiﬁcant
observation from these reports is the relation between
the location of the accessory pathway and the LV
wall motion.4–6) Recently, an accessory pathway has
been reported to contribute to LV septal dyskinesia
and LV dysfunction in the absence of documented
SVT.7–11) Many patients exhibit right septal or
posteropesptal accessory pathways. Herein, we report
a case of WPW syndrome with right-sided free-wall
accessory pathway and LV dysfunction.
Address for correspondence: Takanao Mine MD, Department of Internal Medicine, Cardiovascular Division, Hyogo College of Medicine,
1-1 Mukogawa-cho, Nishinomiya 663-8501, Japan. Tel: 81-798-45-6553 Fax: 81-798-45-6551 E-mail: mine@hyo-med.ac.jp
Received 7, December, 2010: accepted 30, March, 2011.
145
Mine T An Asymptomatic Case of Wolﬀ-Parkinson-White Syndrome
Case Report
Case Report
A-16-year-old female was diagnosed with WPW
syndrome ten years prior (when 6-years-old), but she
had been asymptomatic. At the age 13, she underwent
a medical examination at our hospital, which showed
pre-excitation in a 12-lead surface ECG (Figure 1A).
A predominantly negative delta wave in V1 and
left axis deviation indicated the presence of a right
free-wall accessory pathway. Transthoracic echocar-
diography at that time showed a dilated coronary
sinus, normal LV function and dimension, LV
ejection fraction (LVEF) of 58%, and LV end-
systolic diameter (LVDd) of 44mm. A persistent
left superior vena cava was suspected. She exhibited
no residual symptoms, so any treatment for WPW
syndrome was not indicated. Since that time, she
underwent routine medical examination at our hos-
pital on an annual basis. At the age of 16, trans-
thoracic echocardiography showed left ventricular
septal akinesia, left ventricular dysfunction, LVEF
of 50%, and LVDd of 51mm. Physical examination
and chest X-rays were otherwise normal. The patient
and her parents expressed a preference for catheter
ablation to improve cardiac function.
After obtaining informed consent from the patient
and parents, right atriography, electrophysiological
study and catheter ablation were performed. Biplane
right-atriography was performed in the 30-degree
right anterior oblique and the 60-degree left anterior
oblique views (Figure 2A and B). Catheters were
placed at the right atrium, the right ventricle, the
bundle of His and the tricuspid annulus along the
anterolateral right atrium after insertion into the
coronary sinus via the right femoral vein. During
sinus rhythm, the earliest ventricular activity was
recorded from the lateral right atrial electrode
(Halo 17-18) (Figure 2C). The AH interval was
80ms and the HV interval was 30ms. During
right ventricular apex pacing, the earliest retrograde
atrial activity was recorded from the lateral right
atrial electrode (TA 17-18) (Figure 2D). Thus, the
accessory pathway was mapped to the right-sided
free-wall. An 8mm-tip catheter was used for map-
ping and ablation. Radiofrequency (RF) application
with a target temperature of 55 C and a power of 30
watts around the shortest delta wave-to-ventricle
interval showed a persistent accessory pathway
block after 4.5 seconds of RF (Figure 3).
An echocardiogram taken one month after abla-
tion revealed that the left ventricular septal wall
motion had normalized, while the LVEF and LVDd
improved to 64% and 48mm, respectively. Figure 4
shows the parametric imaging obtained from 3-D
A B
Figure 1
A: Surface 12-lead electrocardiogram (ECG) with a heart rate of 77 bpm, predominantly negative delta wave in V1 and left axis deviation
before the catheter ablation. B: ECG with a heart rate of 81 bpm and ﬁrst degree atrioventricular block after the catheter ablation.
J Arrhythmia Vol 27 No 2 2011
146
echocardiography before and after the ablation. The
ﬁgure shows a bullseye map using the volume
change curve analysis, with the blue area indicating
early excitement. As illustrated, the blue area in the
anteroseptal site disappeared after the ablation. On
follow-up testing, the delta wave has not reappeared,
and electrocardiography shows only ﬁrst degree
atrioventricular block (Figure 1B).
Discussion
Our group has occasionally encountered cases
of heart dysfunction in patients with WPW syn-
drome. Most of these patients have symptoms
consistent with supraventricular tachycardia (SVT),
including possible tachycardia-induced cardiomyop-
athy. However, there have been some reports of
decreased cardiac function in patients with WPW in
the absence of SVT, and these patients (most
of whom were infants or children) tend to exhibit
segmental dyskinesia of the myocardium and/or
global LV dysfunction.7–11) Pre-existing areas may
lead to dyssynchrony in LV motion and result in
global LV dysfunction.
Several papers have reported that RF ablation
resulted in mechanical resynchronization, reverse
remodeling, and improved LV function. In those
reports, most patients exhibited right septal or
posteroseptal accessory pathways.7–11) These patients
may have wider pre-existing areas than patients with
left or right-sided free-wall accessory pathways.
Further, Iwasaku et al. reported a case of a patient
A
B
C
D
RV
RV
TA
TA
His
His
HRA
HRA
V1
V6
HRA 1-2
HBE 1-2
HBE 3-4
HBE 5-6
TA 1-2
TA 3-4
TA 5-6
TA 7-8
TA 9-10
TA 11-12
TA 13-14
TA 15-16
TA 17-18
TA 19-20
RVA 1-2
V1
V6
HRA 1-2
HBE 1-2
HBE 3-4
HBE 5-6
TA 1-2
TA 3-4
TA 5-6
TA 7-8
TA 9-10
TA 11-12
TA 13-14
TA 15-16
TA 17-18
TA 19-20
RVA 1-2
Figure 2
A: Right-atriography in the right anterior oblique views. Multi-polar catheters were positioned at the high right atrium (HRA), the right
ventricle (RV), the Bundle of His (His) and the tricuspid annulus (TA). B: Right-atriography in the left anterior oblique views. C: During
sinus rhythm, the earliest ventricular activity was recorded from the lateral right atrial electrode (TA 17-18). D: During right ventricular
apex pacing, the earliest retrograde atrial activity was recorded from the lateral right atrial electrode (TA 17-18).
I, II, V1, V6: surface electrocardiographic leads I, II, V1, V6, HRA: intracardiac electrocardiogram of HRA; HBE: intracardiac
electrocardiogram of the bundle of His; TA: intracardiac electrocardiogram of the tricuspid annulus; RVA: intracardiac electrocardiogram
of the right ventricular apex
Mine T An Asymptomatic Case of Wolﬀ-Parkinson-White Syndrome
147
with WPW syndrome with a right-sided free-wall
accessory pathway and dilated cardiomyopathy
(DCM), in which LV function improved following
RF ablation.12) In patients with decreased LV cardiac
function, such as those with DCM, LV dyssynchrony
can further compromise cardiac function. By con-
trast, the present case is relatively unqiue in that
right-sided free-wall accessory pathway and LV
dysfunction was present in the absence of structural
heart disease.
The present case described a patient with WPW
syndrome with right-sided free-wall accessory path-
way that exhibited LV myocardial dyskinesia and
dysfunction. On routine follow-up, she denied any
symptoms that could be attributed to paroxysmal
SVT. After treatment, LV septal wall motion
normalized and LV function improved. These results
suggest that pre-excitation resulted in dyssynchrony
and led to LV dysfunction in this patient. After RF
ablation, an electrocardiogram showed ﬁrst degree
atrioventricular block. In this case, the pre-excited
activation might have propagated faster than the
normal conduction system, and the pre-excited
myocardium might have been larger relative to that
in more typical cases with a right-side free-wall
accessory pathway.
LV septal akinesia or LV dysfunction may be
incidentally detected during routine follow-up in a
patient with WPW syndrome. However, because
such patients do not universally undergo routine
A
B
C
D
ABL
ABL
V1
V6
HRA 1-2
HRA 3-4
HBE 1-2
HBE 3-4
HBE 5-6
TA 1-2
TA 3-4
TA 5-6
TA 7-8
TA 9-10
TA 11-12
TA 13-14
TA 15-16
TA 17-18
TA 19-20
ABL 1-2
ABL 3-4
ABL Uni
RVA 1-2
RVA 3-4
V1
V6
HRA 1-2
HRA 3-4
HBE 1-2
HBE 3-4
HBE 5-6
TA 1-2
TA 3-4
TA 5-6
TA 7-8
TA 9-10
TA 11-12
TA 13-14
TA 15-16
TA 17-18
TA 19-20
ABL 1-2
ABL 3-4
ABL Uni
RVA 1-2
RVA 3-4
Figure 3
A: Successful ablation site in the right anterior oblique views. The ablation catheter (ABL) was positioned at the anterolateral site of the
tricuspid annulus. B: Successful ablation site in the left anterior oblique views. C: Intracardiac electrocardiogram of ablation in the
successful ablation site. The local atrium-to-ventricle interval is 50ms. D: Radiofrequency (RF) energy delivery with a target temperature
of 55 C and a power of 30 watts resulted in the accessory pathway block after 4.5 seconds of RF.
I, II, V1, V6: surface electrocardiographic leads I, II, V1, V6, HRA: intracardiac electrocardiogram of HRA; HBE: intracardiac
electrocardiogram of the bundle of His, TA: intracardiac electrocardiogram of the tricuspid annulus; ABL: intracardiac electrocardiogram
of the tip of the ablation catheter; ABL Uni: unipolar electrocardiogram of the ablation catheter; RVA: intracardiac electrocardiogram of
the right ventricular apex
J Arrhythmia Vol 27 No 2 2011
148
follow-up, this pathology may go undetected in an
otherwise asymptomatic patient. Because it is diﬃ-
cult to predict whether or not LV dysfunction will
occur, we advocate routine follow-up and testing for
patients with WPW syndrome, even when asympto-
matic. If LV dysfunction is revealed on routine
testing, RF ablation should be considered.
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Figure 4 Upperpanel:
Parametric imaging ob-
tained from 3-D echo-
cardiography before the
ablation. Lower panel:
Parametric imaging ob-
tained from 3-D echo-
cardiography after the
ablation. These ﬁgures
show a bullseye map
using volume change
curve analysis and the
blue area indicating ear-
ly excitation. The blue
area in the anterior and
anteroseptal site is evi-
dent before the ablation
and disappears after the
ablation.
Mine T An Asymptomatic Case of Wolﬀ-Parkinson-White Syndrome
149


An Asymptomatic Case of Wolff-Parkinson-White Syndrome with Right-sided Free-wall Accessory Pathway and Left Ventricular Dysfunction

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