Ventricular Fibrillation
A Possible Mechanism of Sudden Death in Patients
with Wolff-Parkinson-White Syndrome
By LEONARD S. DREIFUS, M.D., ROBERT HAIAT, M.D., YOSMO WATANABE, M.D.,
JAIME ARRIAGA, M.D.,
AND
NORMAN REITMAN, M.D.
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SUMMARY
A case of Wolff-Parkinson-White syndrome associated with ventricular fibrillation
is presented. The control of recurrent ventricular fibrillation with large doses of digitalis and other antiarrhythmic drugs, including propranolol, lidocaine, procainamide,
and quinidine, is discussed. As far as we can determine, this is the first human case in
which the precise onset of ventricular fibrillation was documented. Possible mechanisms are presented that may produce ventricular fibrillation in patients with WolffParkinson-White syndrome associated with atrial flutter or fibrillation.
Additional Indexing Words:
Pre-excitation
Ventricular vulnerability
Wolff-Parkinson-White tachycardia
Electronic demand pacemaker
Atrial fibrillation
T HE PURPOSE of this paper is to report
a case of ventricular fibrillation in a
patient with Wolff-Parkinson-White (W-PW) syndrome. Such an association is rare
and has been documented in only six
previous observations. It may account for
some instances of unexplained sudden deaths
in the W-P-W syndrome.
control her recurrent tachycardia. Although the
rhythm was moderately well-controlled for three
or four weeks, she had to be readmitted to St.
Michael's Hospital in November, 1969, because of
the recurrence of persistent tachycardia.
The patient was subsequently transferred to
the Hahnemann Hospital for further evaluation.
On admission, the physical examination revealed
an obese, slightly lethargic white female in no
acute distress. Her blood pressure was 100/60
mm Hg, and her pulse was irregularly irregular at
180 beats/min. Neck vein distention was present
at 35°. The previously implanted pacemaker was
palpated in the left anterior chest wall. All
peripheral pulses were palpable. The right calf
was tender. Heart tones were distant but no
murmur, rub or gallop was present. Decreased
breath sounds were noted at the right base. The
examination of the other systems was not
contributory.
The electrocardiogram taken on admission (fig.
2) revealed atrial fibrillation with an average
ventricular response of 190 beats/min. The QRS
complexes were wider and more bizarre. Evidence of actual delta waves could not be seen in
this tracing although the QRS configuration was
similar to figure 1, and type A pre-excitation was
suggested. Confirmation of atrial fibrillation was
made by a right atrial electrogram (fig. 3).
The chest roentgenogram revealed cardiomegaly and a right pleural effusion which was
Case Report
A 63-year-old female was admitted to the
Hahnemann Hospital on December 1, 1969. She
had a history of supraventricular paroxysmal
tachycardia with narrow QRS complexes for
many years. Numerous electrocardiograms
showed type A W-P-W configuration (fig. 1).
The patient had been doing well until September,
1969, when she noted recurrent episodes of
lightheadedness, without palpitations. She was
then admitted to St. Michael's Hospital in
Newark, New Jersey, for implantation of a
transvenous demand pacemaker in an attempt to
From the Division of Cardiology, Hahnemann
Medical College, Philadelphia, Pennsylvania, and
Middlesex General Hospital, New Brunswick, New
Jersey.
Received August 26, 1970; revision accepted for
publication December 16, 1970.
520
Circulation, Volume XLIII, April 1971
VENTRICULAR FIBRILLATION IN W-P-W SYNDROME
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Figure 1
A sinus rhythm is present. Prominent delta waves are seen in this tracing, showing
W-P-W type A.
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taken
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of
2
admission, showing atrial fibrillation with
190 beats/mmn).
thought to be associated with a pulmonary
infarction. The results of all laboratory studies
were normal, including electrolytes and serum
enzymes. An acute myocardial process was not
identified.
Hospital Course
Intravenous propranolol at a rate of 2 mg/hour
was started, and the patient was given 0.25 mg of
digoxin intravenously in an attempt to slow the
ventricular rate. Two hours later, a second dose of
0.25 mg of digoxin was administered, and,
because of the contiiued arrhythmia, a third dose
Circulation, Volume XLIII, Api 1971
*
an
irregular ventricular
response
of 0.25 mg of digoxin was given two hours later
(midnight). At 2 a.m. (December 2, 1969), the
patient suddenly lost consciousness. The heart
sounds were no longer perceptible. On the
electrocardiogram (fig. 4), ventricular fibrillation
was identified and was reverted by a direct
current precordial shock. Because a rapid ventricular response continued even after the ventricular
fibrillation had been reverted, 0.25 mg of digoxin
was given again on two occasions. A second
episode of ventricular fibrillation occurred at 6
a.m. that morning, and was again reverted by
direct current precordial shock. A lidocaine drip
DREIFUS ET AL.
522
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Figure 3
The right atrial electrogram (RAE) shows atrial fibrillation. Arrows indicate pacemakerinduced beats.
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Figure 4
Direct current defibrillation terminated the ventricular fibrillation. (A.) Onset of ventricular
fibrillation. (B.) Ventricular fibrillation. (C.) Following direct current shock, electronic pacemaker controls the heart. (D.) 15 minutes later, supraventricular tachycardia.
Circulation, Volume XLIII, April 1971
VENTRICULAR FIBRILLATION IN W-P-W SYNDROME
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Figure 5
The ventricles are under
to the atria.
the
control of
a
demand
started but quickly discontinued due to the
drowsiness and confusion.
Intravenous quinidine was given at a rate of 100
mg every 2 hours, as well as intravenous
procainamide which was given at an average rate
of 2 mg/min. Digoxin (0.25 mg intramuscular)
was continued daily. These drugs were alternately
titrated during the first week in order to achieve
an appropriate regimen which would control the
patient's heart rate without engendering hypotension. A therapeutic dose of 131I (18 mCi) was
administered on December 9, 1969.
On this regimen, no further evidence of
tachycardia was noted, and sinus rhythm was
predominantly present. Slowing of the ventricular
rate below 58 beats/min allowed the demand
pacemaker to escape and control the ventricular
rate (fig. 5).
The patient stayed in the hospital for 27 days,
and the remainder of her course was uneventful.
She was maintained on intravenous heparin
because of the possible presence of a pulmonary
infarction. During the second week, the following
antiarrhythmic drugs were administered orally:
propranolol (20 mg), quinidine (200 mg),
procainamide (250 mg, four times daily), and
digoxin (0.25 mg daily). The patient was
discharged (December 27, 1969) on this regimen, and was free from both paroxysmal
tachycardia and heart failure until August,
1970.
type
pacemaker with retrograde conduction
Discussion
was
onset of extreme
Circulation, Volume XLIII, April 1971
Cardiac arrhythmias, especially supraventricular tachyeardias, are well known to occur
in patients with W-P-W syndrome.'-5
Reports of ventricular tachycardia in patients with W-P-W syndrome have been
reviewed by Langendorf et al.,2 Giraud et al.,3
and Newman et al.4 According to Langendorf
et al., no unequivocal case of ventricular
tachycardia could be demonstrated, as independent atrial activity could not be identified.
Furthermore, in their review they said: "It is
likely that these so-called ventricular tachycardias were in fact, supra-ventricular tachycardias with widened and slurred ventricular
complexes."2
Although the prognosis of W-P-W syndrome is usually said to be benign in the
absence of underlying cardiopathy, several
sudden and unexpected deaths in these
patients have been previously reported. Okel6
reviewed 22 patients and reported an additional case of sudden death associated with
W-P-W syndrome. Ten other previously reported cases were identified during the
preparation of this manuscript.2 714 The
DREIFUS ET AL.
524
Table 1
Ventricular Fibrillktion in Wolff-Parkinson-White Syndrome: Review of Seven Cases
Authors
Case no.
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Age
Sex
Fox et al.18
1
1952
70
Ahlinder et al.19
2
1963
Touche et al.20
Okel6
3
Wojtasik et al.13
Kaplan et al.2'
Dreifus et al.*
5
6
7
4
Year
Symptoms
VF
Atrial fibrillation
F
Occasional dizziness & palpitations
Yes
Transient episode 3
months later
42
M
None
Yes
Concomitant
1966
1968
21
33
M
M
Paroxysmal tachycardia 1 year ago
Transient atrial fibrillation
Yes
Yes
None
Concomitant
May, 1969
Aug., 1969
18
27
63
F
F
F
6 years ago
None
Palpitations for 5 years
Recent palpitations
Yes
Yes
Yes
None
None
Concomitant
1970
Abbreviations: VF = ventricular fibrillation; DCD = direct current defibrillation.
*Case reported by the authors in this paper.
mechanism of sudden death has not been
demonstrated with accuracy. Ventricular fibrillation as a possible mechanism of sudden
death in patients with W-P-W syndrome has
been observed in only six other instances.
(table 1).
The two observations of ventricular fibrillation in W-P-W syndrome reported by
Schwartz and Jezer (1934) 15 and Gould
and Mundal (1951)16, and mentioned by
Prinzmetal et al.,17 are questionable because
neither the presence of W-P-W complexes nor
ventricular fibrillation was clearly identified.
Fox et al.18 reported in 1952 the first
documented case of W-P-W syndrome associated with ventricular fibrillation. In this
observation, ventricular fibrillation occurred 4
hours after the intravenous administration of
500 mg of procainamide.
Five other cases have been reported subsequently.6 13. iP 21 In one of these patients, an
autopsy showed myocarditis, interatrial lipomatous hypertrophy, and a prominent right
moderator band.20 All of the other patients
were successfully resuscitated, usually by one
or two direct current precordial shocks. It is
noteworthy that all patients presented had a
previous history of palpitations or proved
paroxysmal tachyeardias, and in three cases,
including this present report, atrial fibrillation
was present at the time ventricular fibrillation
occurred.
In the case presented here, one might argue
that ventricular fibrillation was due to the
presence of the previously inserted pacemaker, and represented a complication of the
electronic pacing rather than a hazard of W-PW syndrome. This is unlikely, however, since
the pacemaker was of the demand, Rinhibited type (Medtronics 5841), and electrical stimulation occurred only after 1 sec of
asystole.
The pathogenesis of ventricular fibrillation
associated with W-P-W syndrome is unclear.
Previous observations concerning the mechanism of premature atrial and ventricular
stimulation, with the production and the
interruption of recurrent tachycardia as well
as the effective results of surgical ligation of
the atrioventricular (A-V) junction or the
accessory pathway, may offer several clues to
the onset of ventricular fibrillation.
Durrer and Roos22 indicated that, depending upon the timing of premature atrial systole
and the state of refractoriness of the His and
Kent bundles, excitation of the ventricles
could occur either predominantly through one
of these two conduction pathways or through
both. On the other hand, they said that if a
premature ventricular stimulus was given at a
Circulation, Volume XLIII, April 1971
VENTRICULAR FIBRILLATION IN W-P-W SYNDROME52
Emergency
treatment
None
1 DCD;
External
massage
2 DCD
1 DCD
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DCD
1 DCD
2 DCD
Additional remarks
Follow-up
Immediate evolution
Spontaneous reversion
to sinus rhythm
Alive
Alive
Doing well 3 months later
Alive
Died
Doing well a few weeks later
Alive
Alive
Alive
Sudden death 6 months later
Doing well
Doing well 8 months later
Ventricular fibrillation occurred after injection
of procainamide (Pronestyl) (500 mg)
Autopsy report
sharply defined delay interval of about 320
retrograde P wave was followed by
a supraventricular tachycardia with normal
msee, the
excitation of the ventricles.
Durrer and Roos22 also suggested that the
pre-excitation syndrome could cause a tachycardia resembling a ventricular mechanism by
direction.
enter
the
the
would
normnal
Basically, impulse
A-V transmission system in a retrograde
fashion to activate the atria and then reenter
the ventricles through the bypass. Hence,
depending on whether the circus movement
moved antegrade through the normal A-V
conduction system or through the bypass, the
a circus movement in a reverse
....
525
....
....
....
....
....
Normal selective coronary arteriography
Myocardial infarction 1965; demand
pacemaker inserted 1969
resulting tachycardia could resemble either a
supraventricular or ventricular mechanism.
It was further noted that the ligation of the
A-V node23 or the accessory pathway24 could
permanently prevent recurrent tachycardia,
and that both pathways must be intact for
initiation and perpetuation of the W-P-W
tachycardia.2 On the other hand, in the
presence of atrial flutter or fibrillation, competition for the normal A-V transmission system
and the bypass fibers can occur. If conduction
is predominantly through the normal A-V
conduction system, the QRS complexes may
remain narrow. But if conduction through the
bypass fibers occurs in rapid sequence, the
....
....
..
....
..
...
..
.
....
....
..
..
A L J~
....
...
Figure 6
Atrial fibrillation is present. The ventricular response is rapid and irregular. The second
ventricular complex following the long pause occurs on the apex of the preceding T wave.
Several multiform beats are then followed by ventricular fibrillation. Note that ventricular
fibrillation did not occur with rapidly occurring beats prior to pause (star). The pause may
have altered the refractory period of the ventricles.
Circulation, Volume XLIII, APril 1971
.
.........
.
.
526
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ventricular rate may become excessive with
wide bizarre QRS complexes.
Several of the cases of W-P-W syndrome
with "ventricular tachycardia" are, therefore,
probably due to either atrial flutter or atrial
fibrillation, with the impulse being predominantly conducted through the bypass fibers
rather than through the normal A-V transmission system.
It does not seem unreasonable to postulate
that in the case reported here, in which the
precise moment of the onset of ventricular
fibrillation was recorded in the presence of
atrial fibrillation, impulses passed through the
accessory fibers at a rapid frequency and
reached the ventricles during the vulnerable
period of ventricular excitability (fig. 6). According to Boineau and Moore,25 who made
similar experimental observations, ventricular
fibrillation occurred repeatedly in the dog as a
direct result of the rapid ventricular rate
permitted by the accessory pathway subsequent to induced atrial fibrillation. The
mechanism engendering ventricular fibrillation may be similar in this case. This situation
is physiologically not possible without accessory pathways because the refractory period of
the A-V junctional tissues produces 2:1 or
higher conduction ratios, thereby preventing
excitation of the ventricles in the vulnerable
period.26
Conduction block in the bypass fibers has
also been described.27 The fact that fatal
ventricular fibrillation does not ensue more
often in the presence of atrial fibrillation with
pre-excitation is probably explained on this
basis.
Although ventricular pacing by the already
implanted demand pacemaker was fortuitous
in the case presented here, suppression of all
A-V conduction and electronic pacing appears
to offer a method of therapy in patients with
the W-P-W syndrome and persistent atrial
fibrillation (fig. 5). Subsequent to this case,
one other similar case has been successfully
managed in a similar way.
Digitalis may have partly contributed to the
onset of ventricular fibrillation in the case
currently reported. However, it was continued
DREIFUS ET AL.
after successful defibrillation and control of
conduction in both the normal and the
accessory A-V pathways by appropriate drug
administration. Furthermore, it has been
clearly shown that ventricular fibrillation can
be produced in the absence of digitalis.25 Also,
factors favoring a longer vulnerable period of
excitability, such as myocardial ischemia, may
have contributed to the onset of ventricular
fibrillation. However, it should be emphasized
that any of these factors in the absence of WP-W are capable of precipitating ventricular
fibrillation. Nevertheless, it is quite clear that
the rapid succession of supraventricular impulses with subsequent QRS complexes falling
on the T wave of the previous beat may have
led to the onset of ventricular fibrillation in
the case we have presented here (fig. 6).
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Ventricular Fibrillation: A Possible Mechanism of Sudden Death in Patients
with Wolff-Parkinson-White Syndrome
LEONARD S. DREIFUS, ROBERT HAIAT, YOSHIO WATANABE, JAIME
ARRIAGA and NORMAN REITMAN
Circulation. 1971;43:520-527
doi: 10.1161/01.CIR.43.4.520
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