1
2
3
4
5
6
still in the sick sinus syndrome. Recently we saw a patient
with sick sinus syndrome manifesting this peculiar phenomenon. Following is a brief account.
REFERENCES
Fischer AM, Kendall B, Van Leuven BD. Hodgkin's disease: a
radiological survey. Clin Radiol 1962; 13:115-19
Weick JK, Kiely JM, Harrison EG Jr, Carr DT, Scanlon PW
Pleural effusion in lymphoma. Cancer 1973; 31:848-53
Case Record of The Massachussetts General Hospital. N Engl J
Med 1981; 305:939-47
Yousem SA, Weiss LM, Colby TV Primary pulmonary Hodgkin's
disease: a clinicopathologic study of 15 cases. Cancer 1986;
57:1217-24
Meis JM, Butler JJ, Osborne BM. Hodgkin's disease involving
the breast and chest wall. Cancer 1986; 57:1859-65
Johnson DW Hoppe RT, Cox RS, Rosemberg SA, Kaplan HS.
Hodgkin's disease limited to intrathoracic sites. Cancer 1983;
52:8-13
CASE REPORT
An 80-year-old woman was admitted to our hospital on August
18, 1987 complaining of exertional dyspnea and orthopnea for two
years. Attacks of syncope and dizziness also occurred without a
definite diagnosis. No history of hypertension and angina could be
elicited. On admission, the patient was semi-recumbent with slight
cyanosis. Physical examination revealed a slightly enlarged heart, a
grade 2 systolic murmur at the apex, and crepitant rales at the right
lung base. Occasional premature beats could be heard. Blood
pressure was 130/80 mm Hg, respirations, 20/min. Chest film
showed left ventricular enlargement and slight pulmonary congestion. Clinical diagnosis was coronary heart disease, left ventricular
failure and possible sick sinus syndrome.
The electrocardiogram on admission (Fig 1, upper strip) revealed
upright, pointed ectopic P' waves, and the P'-P' interval was 0.36
s with a rate of 167/min. Atrial tachycardia was apparent. As seen
in the upper strip of the Figure, P' 1, 3, 5, 7, 9, 11 were conducted
to the ventricles, with progressive prolongation of P'-R intervals
from 0.12, 0.18, 0.21, 0.36, 0.38, and 0.42 s; P'13 was blocked.
Although the decrease in increment did not precisely conform to
the Wenckebach order (0.06, 0.03, 0.15, 0.02, and 0.04) the
diagnosis of atypical Wenckebach conduction could be made; the
longer increments could be explained by concealed retrograde
conduction to the A-V node affecting the antegrade conduction of
the following beat. Every alternate P' wave (P 2, 4, 6, 8, 10, 12) was
nonconducted, manifesting alternate 2:1 block. At the end of the
Wenckebach sequence, there were two blocked P' waves, suggesting
type B 2:1 alternate Wenckebach block; that is to say, Mobitz type
II 2:1 A-V block was distal, and that of Wenckebach conduction
proximal (ladder diagram). The phenomenon of "skipped P" could
be seen before the sixth QRS complex. After P' 22, a long pause
with neither P nor QRS, and lasting 3.48 s appeared, during which
the attending physician noticed a change in consciousness of the
patient. The combination of atrial tachycardia and atrial standstill is
typical of "tachycardia-asystole." Three sinus P waves appeared after
the long pause (second row of the figure); sino-atrial block probably
existed between the first and second sinus P waves. From the fourth
beat on, the cycle 2:1 alternate Wenckebach block resumed. After
treatment with propafenone 150 mg tid, the whole sequence of
ECG abnormalities disappeared.
Atrial Tachycardia, 2:1 Alternate
Wenckebach Periodicity, and Atrial
Standstill*
Wan-chun Chen, M.D., F.C.C.RP; and Zhao-rui Zeng, M.D.
A case of atrial tachycardia, 2:1 alternate Wenckebach
periodicity and atrial standstill is reported in an 80-yearold woman who complained of exertional dyspnea and
occasional syncope for two years. Two blocked P' waves
appeared after each Wenckebach period suggesting type B
alternating Wenckebach phenomenon (Mobitz type II 2:1
A-V block distal, and Wenckebach conduction proximal).
When Wenckebach period appears in alternate beats
while the remaining set of beats exhibit 2:1 A-V block,
the ECG phenomenon is then called 2:1 alternate Wenckebach periodicity This is encountered primarily in atrial
tachyarrhythmias such as paroxysmal atrial tachyeardia,
atrial fibrillation and atrial flutter. It was suggested that two
levels of block in the atrioventricular conducting system
exist, one giving rise to 2:1 block, and the other to Wenckebach periodicityl It is a rather rare ECG event, and rarer
DISCUSSION
The sick sinus syndrome is unique for its protean mani-
*From the Shanghai Sixth People's Hospital, Shanghai, China.
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FIGURE 1. Continuous V1 recording, showing a long pause lasting 3.48 s between two attacks of atrial
tachyeardia with 2:1 alternate Wenckebach conduction.
426
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Atrial Tachycardia (Chen, Zeng)
festations. As originally described, the basic requirement for
diagnosis is inappropriate response to various sinus stimuli.
Thus, there is sinus bradycardia, sino-atrial block and sinus
or atrial standstill on the bradyeardia aspect ofthe syndrome,
while various atrial tachyarrhythmias can occur on the
tachycardia aspect. The bradycardia-tachyeardia syndrome
(BTS) was thus picturesquely coined.2 When asystole follows,
as in the present case, it is then called the bradycardiatachycardia-asystole "syndrome. Periods of bradycardia and
tachycardia were initially thought to follow and alternate in
a random fashion. Recently, an electrophysiologic relationship has been found between the supraventricular tachycardia and bradycardia in some patients with the BTS syndrome. Puech and Slama3 proposed a differentiation between
"syncope due to post-tachycardia atrial standstill" and "supraventricular tachycardia due to atrial bradycardia." In the
former case, atrial standstill occurs only after the atrial
tachyeardia, and long post-tachycardia pauses signify overdrive suppression. Pacemaker may not be necessary, and
treatment should be directed to suppress the atrial tachycardia. In the latter case, the tachycardia more or less
appears to be a direct consequence of sino-atrial block and
vagally induced, as physiologists had long recognized the
role of the vagus in the initiation of atrial tachycardia, or the
tachycardia is actually a junctional reciprocating one, triggered by ajunctional escape beat related to excessive slowing
of the heart. Pacemaker implantation is indicated. In the
present case, a long pause followed the tachyeardia. Evidently it belongs to Puech and Slama's first category2,4
During the Wenckebach sequence of conduction, two or
three dropped P' or F waves can be seen, depending on the
site of blockage.i By using His bundle electrography,67 it
was demonstrated that when the Wenckebach block occurs
proximal to the His bundle, and the 2:1 block in the His
bundle, two blocked P' or F waves would appear after each
Wenckebach cycle. On the other hand, if the site of the
Wenckebach block is situated distal to, or in the His bundle,
and the 2:1 block is proximal to the His bundle, then the
result would be three nonconducted supraventricular impulses. The prevalent view to explain 2:1 alternate Wenckebach periodicity is transverse fissure in the A-V node.67
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5
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7
REFERENCES
Chen WC, Zhen ZR, Wang E. Atrial tachyarrhythmia, alternate
Wenckebach conduction and sinus standstill. Shanghai Med J
1984; 7:723-25
Wan S, Lee GS, Toh C. The sick sinus syndrome. A study of 15
cases. Br Heart J 1972; 34:942-52
Puech PL Slama R. The cardiac arrhythmias (a report of the
Arrhythmia Working Group, French Cardiac Society). Paris:
Roussel Uclal, 1979; 85:119-20
Josephson ME, Wellens HJJ. Tachycardias: Mechanisms, diagnosis, treatment. Philadelphia: Lea and Febiger, 1984; 225
Halpern MS, Nau GJ, Levi RJ, Elizari MN Rosenbaum MB.
Wenckebach periods of alternate beats. Clinical and experimental
observations. Circulation 1973; 48:41-4
Kosowsky BD, Latif P, Radoff AM. Multilevel atrioventricular
block. Circulation 1976; 54:914-21
Amat-y-Leon F, Chuquimia R, Wu D, Denes PE Dhingra RC,
Wyndham C, et al. Alternating Wenckebach periodicity Am J
Cardiol 1975; 36:757-64
Tocainide-Associated Neutropenia:
and Lupus-like Syndrome*
Lawrie D. Oliphant, M. D.;t and
Michele Goddard, M. B., Ch. B. (NZ)t
Neutropenia in association with a lupus-like illness that
developed after the introduction of tocainide therapy is
described. The mechanism of drug-associated neutropenia
and the manifestations of drug-associated lupus are briefly
discussed.
N eutropernia is a rare side effect of tocainide therapy for
which several potential explanations have been offered. The following case report describes tocainide-associated neutropenia in association with a lupus-like illness.
CASE REPORT
A 64-year-old man evaluated for acute-onset presyncope was
found on electrocardiogram to have sustained ventricular tachycardia. He had a history of atherosclerotic heart disease, complicated
by nmyocardial infarction in 1956 and 1973, and was known to have
a left ventricular aneurysm, documented by echocardiogram. Digoxin, furosemide, and potassium supplements were the only
medications. Serum electrolyte levels were rnormal, and serum
digoxin level was therapeutic at 1.4 nM/L (therapeutic range, 0.6
to 2.6 nM/L). There was no history of procainamide or hydralazine
usage. The ventricular tachycardia was initially controlled with
administration of intravenous lidocaine. Oral tocainide (400 mg q
8 h) was then introduced for long-term maintenance therapy; a good
antiarrhythmic response was documented by ambulatory monitoring. No dosage adjustments were made, and drug levels were not
measured. Weekly hemograms were not obtained.
At follow up eight weeks later, the patient related a four-week
history of increasing malaise, fever, and night sweats. Physical
examination disclosed findings compatible with his known atherosclerotic heart disease and left ventricular aneurysm (S4 and
displaced cardiac apex) but was otherwise unremarkable; there was
no evidence ofarthritis, dermatitis or pleuropericai ditis. Laboratory
investigations revealed neutropenia (0.94 x 109/L); normochromic,
normocytic anemia (hemoglobin 109 g/L) with inadequate reticulocyte response (34 x 109/L); and a normal platelet count. A hemogram
eight weeks previously had been normal. The Winthrop sedimentation rate was elevated at 58 mm/h (normal, 0 to 10 mm/h), and on
the peripheral blood smear marked rouleaux formation was demonstrated. Bone marrow aspirate revealed marked depression of the
granulocytic series but preservation of the granulocytic precursors.
Immunologic investigations demonstrated a positive antinuclear
antibody (titer 1:160 homogenous pattern) with anti-DNA binding
of 2 U (normal, less than 25 U) and normal complement titers.
Direct antiglobulin (Coombs' test) was positive for IgG and C3, but
there was no evidence of hemolysis. Urinalysis was unremarkable.
No infectious processes were identified. The cessation of tocainide
therapy resulted in a rapid resolution of the neutropenia, with
normal leukocyte counts being documented ten days later. The
persistence of constitutional symptoms, anemia (hemoglobin 102
g/L), and elevated sedimentation rate four weeks later led to the
introduction of oral corticosteroids (prednisone 30 mg/d). Symptoms
abated, and the anemia resolved in four weeks, allowing the
corticosteroids to be tapered without a recurrence. Follow-up six
*From the Department of Medicine, St. Joseph's Health Centre,
University of Western Ontario, London, Ontario, Canada.
tFellow in Pulmonary and Critical Care Medicine.
tAssociate Professor of Medicine.
Reprint requests: Dr Goddard, St. Joseph's Hospital, London,
Ontario, Canada N6A 4V2
CHEST / 94 / 2 / AUGUST, 1988
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