J Arrhythmia Vol 27 No 4 2011

Case Report

Reel Syndrome:
A Variant Form of Twiddlers Syndrome

Muhammad Munawar MD PhD, Dian L. Munawar MD,

Faris Basalamah MD, Jimmy Pambudi MD
Binawaluya Cardiovascular Center, Jakarta, Indonesia

Reel syndrome is a variant form of twiddlers syndrome. We describe a 53 years old

woman who was referred to our hospital because of symptomatic sinus bradycardia.
Subsequently she underwent dual chamber pacemaker implantation and was sent back to the
previous hospital on the following day. She was referred again because of sudden syncope due
to low heart rate 2 weeks following implantation. Chest X-ray revealed leads had pulled out of
the heart and coiled up around the pacemaker generator; a diagnosis of reel syndrome was
made. The dierence between the two syndromes, risk factors and preventive measures were
discussed.
(J Arrhythmia 2011; 27: 338342)

Key words: Reel syndrome, Pacemaker implantation, Coiling lead, Complication

around the pacemaker generator. The rst report by

Introduction
Twiddlers syndrome is a rare complication of
pacemaker implantation. This syndrome is charac-
terized by coiling of the pacemaker lead due to
rotation of the pacemaker generator on its long axis.
The rst description was reported by Bayliss et al in
1968.1) The syndrome occurs more often among
elderly, obese, dementia and mental disorder pa-
tients.24) Previous reports have noted a tendency of
increased complications in women with twiddlers
syndrome, suggesting lax subcutaneous tissue along
the pendulous breast that may potentially form a
large loose pocket.5,6) Reel syndrome is another
variant of twiddlers syndrome. It occurs due to the
rotation of the pacemaker generator on its transverse
axis with subsequent coiling of the pacemaker leads
Carnero-Varo in 1999 described a 70 year old man in
whom this complication occurred 1 month after
implantation of a single chamber pacemaker to treat
atrial brillation with slow ventricular response.7)
Some literature still use the term twiddlers syn-
drome, although the clinical manifestation conrm
that it is actually reel syndrome.3,810) Most of the
reported cases of reel syndrome describe lead
dislodgement without damage to the lead.3,7,9,11,12)
On the other hand, twiddlers syndrome is frequently
associated with lead dislodgement, either with lead
fracture or insulation leakage.13,14)
In this report, we describe a patient with a dual
chamber pacemaker who presented with sudden onset
of syncope after 2 weeks of implantation and was
subsequently diagnosed as having reel syndrome.

Received 18, March, 2011: accepted 27, April, 2011.

Address for correspondence: Muhammad Munawar MD PhD, Binawaluya Cardiovascular Center, Jl. TB Simatupang 71, Jakarta 13650,
Indonesia. Telephone: +6221-87781606 ext 307 Fax: +6221-8403869 E-mail: muna@cbn.net.id

338
Munawar M Reel syndrome as pacemaker implantation complication

Figure 1
A. ECG showed sinus bradycardia (1st , 2nd and
3rd P wave) and sinus arrest (between 3rd and 4th
P wave) with junctional escape beat (4th QRS
complex). B. One day after implantation showed
tall spike followed by P wave and normal QRS
complex without spike indicating unipolar atrial
lead setting and long AV delay of normal dual
chamber pacemaker function. C. There were 2
types of spikes. Tall spike (black arrow)
indicating unipolar lead from atrium and small
spike (white arrow) indicating bipolar lead
(from right ventricle). Both were not captured
and sensing. The rhythm was marked sinus
bradycardia and junctional escape beat. All
ECGs were recorded at speed of 25 mm/second.

Case report
A 53 year old woman (body height 156 cm, body
weight 60 kg) was referred to our hospital because of
severe bradycardia on 24th February 2010. She has
been treated for ischemic stroke in the previous
hospital for 2 weeks. The presenting symptoms were
hemiparesis of her right extremities, and cranial
nerve paresis including facial, hypoglossal and
cognitive impairment. The resting electrocardiogram
(ECG) demonstrated sinus node disease (Figure 1A).
A dual chamber pacemaker was implanted via the
right subclavian vein. A screw-in ventricular lead
was placed at the high right ventricular septum and
another screw-in atrial lead was placed at the right
atrial appendage. Fixation of both leads was done in
the standard fashion by suturing on lead sleeve with
silk suture. The pacemaker generator was then
connected to both leads and placed into a subcuta-
neous tissue pocket. Both redundant leads were put
under the pacemaker generator. The wound was Figure 2
closed using absorbable suture. The day after Chest X-ray was taken 1 day following pacemaker implantation.
surgery, her ECG, as shown in Figure 1B, conrmed Pacemaker generator and its leads were in proper position. The
that pacemaker function was good. Her chest X-ray path of the leads was clearly through the subclavian venipuncture
(white arrow). Tip of the atrial lead was not clearly seen, but the
also conrmed that the pacemaker generator and its
right ventricular lead was placed at the high right ventricular
leads were in proper position (Figure 2). The patient septum (black arrow).
was sent back to her previous hospital for stroke
management.
On 11th March 2010, the patient was reported to ECG showed both atrial and ventricular spikes
have experienced dizziness and near syncope. Her uncaptured and her own rhythm back to marked

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J Arrhythmia Vol 27 No 4 2011
A B
Figure 3A
Dislodgement of the right atrial and ventricular lead with both tips
was at the superior cava vein. Both leads wrapped around the
pacemaker generator.
sinus bradycardia with junctional escape beat or
rhythm (Figure 1C).
She was referred again to our hospital for further
investigation. We brought the patient to the catheter-
ization room for uoroscopy which revealed that
both leads had been dislodged. Leads were coiling
several times around the generator (Figure 3A) and
the diagnosis of reel syndrome was made. The
patient denied having manipulated the pacemaker,
and we suspected that the generator was uninten-
tionally turned because her left arm was active and
some scratch mark on the skin at the pacemaker
implantation area was clearly seen.
We performed a second operation to reposition the
leads. The leads were found to wrap the pacemaker
generator without any signs of insulation leakage and
conduction damage. The right ventricular lead was
repositioned and placed at the middle right ventric-
ular septum and the right atrial lead was placed at the
right atrial appendage. Both leads were xed with
nonabsorbable suture on its sleeve with surrounding
fascia and muscles. To conrm good xation, the
leads were pulled with adequate strength. Additional
sutures using non-absorbable material were added to
obtain good xation of the pacemaker generator. The
pocket was made within the subpectoral muscle for
adequate xation (Figure 3B). After subsequent fol-
low up, the patient was well, the pacemaker function
was good and no complications have been noticed.
340
Figure 3B
Just after reposition procedure. Both right atrial and ventricular
leads were already placed in proper position. There was no
dierence in X-ray appearance between Figure 2 and Figure 3B
in terms of the pacemaker generator and the leads around it. The
dierence was in its procedure (see text).
Discussion
Twiddlers and reel syndrome both have similar
etiologies. Female gender, large pocket, obesity,
children, older people and dementia can be listed as
contributing factors. Even though the etiologies are
quite similar, the mechanisms may dier. In reel
syndrome, a ratchet mechanism was probably re-
sponsible for this syndrome.15,16) Therefore reel
syndrome is characterized by rotation of the perma-
nent pacemaker on its transverse axis and rolling of
the electrode around the generator. Due to lead or
leads rolling around the pacemaker generator, most
of the time reel syndrome is manifested without any
damage the the lead or leads. A chest X-ray is the
most important and simplest method for diagnostis.
Pacemaker programming is a second possibility. We
also have to know whether there is any lead
insulation leakage or fracture. Repositioning of the
leads into proper position within the heart is a simple
procedure without the need for lead replacement.
Most cases of reel syndrome occur within a month
of implantation, whereas twiddlers syndrome can
occur later, up to one year from implantation
(Table 1). In our case, this complication occurred
within 2 weeks after implantation. Although we
performed lead xation in the standard manner,
unsecure xation may remain unrecognize. Conr-
mation of secure xation of the lead or leads by
Munawar M
Table 1 The dierence between twiddlers and reel syndrome
Twiddlers syndrome
Rotation of the pacemaker
Mechanism
generator on its long axis
Tangling of lead or leads around
X ray
pacemaker generator
Lead damage can occur, either
Consequences on
lead fracture or insulation leakage
lead
Sometimes with depleted battery
Procedure of Replace with a new lead and
treatment maybe pacemaker generator
Occurrence Possibly within a year
pulling each lead with adequate strength may help.
Cooper proposed that leads can spontaneously retract
during normal arm movement, without any con-
scious or unconscious device manipulation by the
patient. Hence, leads must be rmly secured in the
device pocket via their suture sleeves in order to
minimize the risk of retraction, regardless of mech-
anism.17)
Reel syndrome can occur in either pacemaker,
cardiac resynchronization therapy device or in
implantable cardioverter debrillator (ICD) pa-
tients.18,19) It may lead to a series of complications
such as syncopal attack and lethal cardiac arrhyth-
mias, especially in pacemaker-dependent patients, as
well as catastrophic consequences when the ICD is
involved.
Preventive measures such as patient education and
use of a smaller pocket will reduce the risk of
developing the syndrome. Suturing the pacemaker
generator into surrounding tissue, as well as placing
the generator into the pectoral muscle may also
prevent the syndrome. Other preventive measures
include using a compression band around the upper
chest and shoulder, and tightening of the patients
arm for at least ve to seven days.11) The use of a
Dacron patch would stabilize the pulse generator by
promoting tissue in-growth. The necessity for careful
follow-up, especially in the rst few months after
surgery, needs to be underscored. The use of a
Dacron pouch and adequate xation of the device
header should be strongly considered for patients at
risk.20,21)
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Reel syndrome as pacemaker implantation complication
Reel syndrome
Rotation of the pacemaker
generator on its transverse axis
(ratchet mechanism)
Lead or leads rolling around
pacemaker generator
No damage of the leads
Reposition of the lead
Within a month
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