Acta Med Scand 200: 457463, 1976

Prognosis of Patients with Complete Heart Block

or Arrhythmic Syncope
Who Were not Treated with Artificial Pacemakers
A Long-term Follow-up Study of 101 Patients

0. Edhag and A. Swahn

From the Department of Internal Medicine, Karolinska Institutet a1 Serajherlasarettet,

S tockhof m ,Sweden

ABSTRACT. This paper reports the results of a with artificial pacemakers varies from country to
retrospective study carried out with special reference country (12) probably due to differences in the re-
to the survival rate in a series of 101 selected cases sources required. Moreover, opinion is divided as
including patients with complete heart block (CHB) to the therapeutic value or artificial pacing. When
combined or not combined with Adams-Stokes at- this treatment was introduced at the end of the
tacks and patients with arrhythmic syncope with-
1950s, it was used exclusively, at least in Sweden,
out ECG evidence of CHB. All these patients were
treated in our Department during 1958-68, none in cases of CHB combined with arrhythmic syn-
being artificially paced. Twenty-seven patients were copes (14). Subsequently, the indications have been
alive at the end of the follow-up, i.e. 6-15 years widened and at present symptomatic bradycardia
after admission to this Department on account of syn- appears to be generally accepted as an indication
copal episodes or CHB. The survival rate-higher in for artificial pacing irrespective of the underlying
females than males-was lower in the cases of CHB disturbance of rhythm. Treatment by artificial pace-
combined with Adams-Stokes attacks than in the makers is being used increasingly in cases of
cases of asymptomatic CHB. This applied also to the asymptomatic CHH because these patients carry
instances in which a complicating disease such as an adverse prognosis (1 1). Paroxysmal ventricular
ischaemic heart disease (IHD), hypertension, dia-
tachyarrhythmia has likewise been successfully
betes, digitalis intoxication or cardiac enlargement
coexisted. The survival rate in the 68 cases of CHB treated with artificial pacing with a high stimulation
was higher at one year (68%) as well as at 5 years rate, so-called overdriving (21).
(37%) than that reported by other investigators. Pacemaker therapy has been used at the Depart-
When assessing the survival rate in cases treated ment of Thoracic Surgery, Karolinska Hospital,
with artificial pacemakers, it is important to study since 1958 (8). The present paper reports the prog-
the individual case histories with special reference nosis of the patients with CHB combined or not
to a previous or coexisting condition such as IHD, combined with Adams-Stokes attacks or with ar-
hypertension, diabetes or the presence of cardiac en- rhythmic syncope in the absence of ECG evidence
largement. The present results support the view that of CHB, who were not artificially paced. All these
the indications for treatment with artificial pacing
patients were ,admitted to our Department in 1958-
should be wide, albeit that the prognosis in this series
68. During that period, 432 patients were referred
was more favourable than might have been an-
ticipated from observations by others. from this Department to the Department of Tho-
racic Surgery for long-term artificial pacing.
Permanent artificial pacing has radically changed
the prognosis of patients with complete heart block
(CHB) combined or not combined with arrhythmic MATERIAL AND METHODS
syncope (2, 9, 1 I , 13, 21). The incidence of cases From 1958 to 1968, 116 patients with CHB combined with
of CHB combined with arrhythmic syncope treated Adams-Stokes attacks or with arrhythmic syncope with-

Acta Med Scand ZOO

458 0 .Edhag and A . Swahn

- 1 2 3 4 J

year-
6 i 8 9 1 0

Fig. 1. Left: Cumulative survival rate (_+2 S.D.) in the

total material (0-0) compared with an age- and sex-
I
I
I
2
1
3 !
I
I
i

Ye.¶-.
I
6
l
7
l
8
l l
9 1 0

heart block (0-0) compared with 260 patients paced in

Stockholm in 1962-68 (0-0) and with a control group
matched control group (X-X). of the same age and sex (x-x).
Right: Survival rate in the 68 patients with complete

out any ECG evidence of CHB were treated in this Cumulative survival
Department. None of these patients was artificially paced. The cumulative survival was determined in the
Their hospital records were reviewed with special ref-
erence to type of arrhythmia, presence or absence of whole case material as well as in the patients with
Adams-Stokes attacks or VEBs, cause of arrhythmia and CHB. The difference between these two groups
coexistence of a myocardial lesion or any serious extra- with respect to survival is strikingly small (Fig. 1).
cardial disease. The part played by these factors in prog- Survival was also compared with that in a popula-
nosis was then investigated. The local parish offices,
which register births, deaths, marriages and other per-
tion group of the same age and with that in patients
sonal data, were requested to report whether the patient who were treated with long-term artificial pacing in
concerned was alive or dead. In 1974 information about this Department in 1%2-67 (7).
these points was obtained in all cases. The hospital
records of 15 patients, 5 of whom were alive at the end Type of arrhythmia
of the follow-up, were either not available or incomplete CHB was demonstrated electrocardiographically in
and were therefore excluded.
Of the 101 patients presented in this paper, 59 were 68 patients, 33 had syncope combined with one
men and 42 women. Their ages ranged from 18 to 93 or several of the following conditions: AV block,
years (mean of the men 67 (514 S.D.), of the women 64 sinus bradycardia (<40/min), nodal bradycardia,
(+IS S.D.)) at the time of hospitalization. numerous VEBs (>5/min), ventricular tachycardia,
ventricular fibrillation and/or pulselessness on one
or several occasions while treated in this Depart-
RESULTS ment (Table I).
Survival The mean age of the patients with electrocardio-
Twenty-seven patients, I I men (18%) and 16 graphically demonstrated CHB was 67 years (f15
women (38%), were alive and 74 were dead at the S.D.) and of the patients without CHB 66 years
end of the follow-up (p<O.O5). Eight patients had (+I3 S.D.) while they were in hospital. In 34 of
died less than two weeks after CHB had been the 68 patients with CHI3, this conduction defect
recorded for the first time or after the first Adams- was constant and a further 34 patients occasionally
Stokes attack had occured. The follow-up time, had AV-conducted heart rhythm after CHB had
calculated from the first occasion on which the been demonstrated electrocardiographically. Eight
conduction defect was documented electrocardio- (24%) of the patients in the former group and 9
graphically or Adams-Stokes attacks occurred to (25%) in the latter were alive at the end of the fol-
the date when the answers from the parish registers low-up. Of the 35 patients with CHR not demon-
were received, was 6-16 years. strated by ECG, 10 (30%) were alive at the end of
Acta Med Scand 200
Prognosis of unpaced patients 459

Table I. Type of arrhythmia and arrhythmic syncope

Alive Mean age
No. of fS.D.
cases n % (Y.)

CHB and syncope 41 7 17 68f 12

CHB , no syncope 27 10 37 65f 17
2nd degree AV block and syncope 9 4 44 64f13
VF, VT or VEB and syncope 12 5 42 62+11
Sinus arrest and syncope 6 0 80f10
Nodal bradycardia and syncope 2 0
Pulselessness and syncope 4 1 60+ 16
Total 101 27 27 67f 14

the follow-up. The 3 patients with nodal brady- about 5 sec duration. The 3 last-mentioned patients
cardia and 2 of the 4 patients who had had episodes were dead at the end of the follow-up.
of pulselessness while in hospital were dead at the
end of the follow-up. The most favourable prog- Acquired CHB with and without
nosis was found among the patients with second Adams-Stokes attacks
degree AV block or sinus arrest combined with syn-
Forty-six per cent of the patients with CHB uncom-
copal episodes. In 1 1 patients with syncope, the
bined with Adams-Stokes attacks survived, com-
underlying disease was thought to be ventricular
pared with 26% of those in whom such attacks
tachyarrhythmia. Four of the latter patients showed
accompanied CHB (Table I). If the 5 patients with
ECG evidence of ventricular tachycardia (5 or more
presumably congenital CHB, two of whom had
consecutive complexes of at least 0.12 sec, their
Adams-Stokes attacks, are excluded, 40% of the
configurations differing from the other ventricular
patients with CHB uncombined with Adams-Stokes
complexes) and a further patient had ventricular
attacks survived, compared with 24% of those with
fibrillation. Six other patients had numerous VEBs
CHB plus Adams-Stokes attacks. The difference is
(>5/min) or multifocal ectopic beats and syncopal
statistically significant @<0.01).
episodes but were not examined by ECG during the
latter. Six of these 1 I patients were alive but only
one of the 5 patients with electrocardiographically Correlation between prognosis, aetiology
confirmed ventricular tachyarrhythmia was alive and past or coexisting serious
at the end of the follow-up. cardiovascular disease
This series included 4 patients who were pulse- The dividing line between the aetiology of the dis-
less on one or several occasions while in hospital turbance of the conduction system and a previ-
or in whom there was auscultatory evidence of ous or present complicating cardiovascular dis-
cardiac arrest. One of them suddenly lost con- ease is not rigid and it is virtually impossible to
sciousness on three occasions while in hospital. make it so. Coexisting cardiovascular diseases such
On one of these occasions the patient was pulseless as angina pectoris and/or hypertension need not
and no heart sounds were heard. This patient was necessarily be the cause of the conduction defect.
alive at the end of the follow-up. Another patient The cases of second degree AV block and CHB
had more than 20 syncopal episodes before admis- in this series were divided into seven groups ac-
sion. On one occasion he was pulseless for about cording to the presence of a coexisting cardiovas-
10 sec while in hospital. In a further patient who cular disease and the presumptive cause of the
was admitted on account of syncope, there was disturbance of the conducting system (Table 11).
auscultatory evidence of cardiac arrest during at Patients with angina pectoris, hypertension or dia-
least 5 sec. This patient had experienced some 10 betes who developed myocardial infarction, were
syncopal episodes before admission. A fourth pa- included in the myocardial infarction group. None
tient treated in the hospital because of syncopes of the 5 patients with digitalis intoxication pre-
had also repeated episodes of pulselessness of sented in Table I1 had a history of myocardial
Acta Med Scand 200
460 0.Edhag and A. Swahn

Table 11. Relationship between prognosis in cases of 2nd degree AV block or CHB, presumptive cause
of conduction defect and coexisting complicating disease
Alive Mean age
No. of kS.D.
cases n % (Y.)

Congenital CHB 4 4 I00 34f I7

Previous myocardial infarction 13 1 8 7 2 f 10
Angina pectoris, hypertension, diabetes 19 2 11 74k 6
Collagenosis, lues, myocarditis 10 4 40 6 3 f 10
Cause unknown, absence of complicating
disease 24 9 38 a_+
12
Digitalis intoxication, absence of complicating disease 5 0 7 9 f 10
Rheumatic valvular disease 2 1
Total 71 21 67+14

infarction, angina pectoris, hypertension or dia- brosis. One of them had myocarditits and died sud-
betes. A further 2 patients developed digitalis denly in her home at the age of 46 years. Three
intoxication in conjunction with IHD. Both were months before her death she attended this Depart-
dead at the end of the follow-up. Two patients ment to consider the question of pacemaker treat-
developed CHB in conjunction with DC conver- ment. At that time, her heart size was 820 ml/m2
sion. In one of these the conduction defect was BSA. She was considered to be suitable for arti-
intermittent but he died from acute myocardial ficial pacing but was discharged because it was
infarction 17 months later. The prognosis of the thought expedient to postpone treatment until the
patients with a history of myocardial infarction or electronic components of the generator in use at the
digitalis intoxication was very unfavourable (Table time had been improved.
11). Five patients, who developed myocardial in-
farction within two months after CHB had been
recorded electrocardiographically for the first time, Serious extra cardial diseases
died. The combination of a previous myocardial Five patients had cancer or a malignant blood
infarction and angina pectoris, digitalis intoxica- disease but not chronic lymphatic leukaemia; 3 of
tion, hypertension or diabetes mellitus was them showed in addition signs and symptoms
common. Of the 37 patients with second degree suggestive of IHD. All these patients were dead
AV block or CHB associated with one or several at the end of the follow-up. Nine patients had cere-
of these conditions, 34 (92%) were dead at the end brovascular lesions and 3 of them had IHD; all
of the follow-up compared with 36 (61%) patients were dead at the end of the follow-up. One patient
whose past history did not include any of these with CHB had Adams-Stokes attacks on about 20
conditions (p<O.Ol). The 4 patients with congenital occasions shortly before his death in hospital from
CHB were not taken into account when assessing symptoms suggestive of a cerebrovascular lesion.
the prognosis in the latter two groups. However, Necropsy disclosed large subdural haematomas on
the mean age of the patients in the former group both sides.
was about 10 years higher than in the latter.
Of the 4 patients with congenital CHB, 2 had
syncopal episodes which were thought to be in- The relationship between heart size
duced by arrhythmia. Four of the six deceased and survival
patients with collagenosis, lues, myocarditis or val- Information about the heart size was available in 51
vular heart disease, respectively, died suddenly. of the male patients. Eighteen of them were alive
One of these 4 patients had had myocarditis at the end of the follow-up, their relative mean
associated with severe arteriosclerosis of the heart size being 495f 167 ml/m2BSA; in 33 patients
coronary arteries and valves, long-standing peri- who died, the corresponding figure was 600+190
carditis and the myocardium showed severe fi- ml/m2 BSA (p<0.05). Nineteen women were alive

Acta Med Scand 200

Prognosis of unpaced patients 461

at the end of the follow-up, the mean heart size pacing in Sweden at the time the patients in this
being 445+170 ml/m2 BSA; in 18 women who had series were treated in this Department (7).
died, it was 526k 190 ml/m2 BSA. The difference is At present, a malignant blood disease with ar-
not statistically significant. rhythmic syncope is not considered to be a con-
traindication for artificial pacing. Not only human-
itarian but also praclical reasons related to the care
DlSCUSSlON of patients with Adam-Stokes attacks have con-
Many investigations of the prognosis of patients tributed to modifying our view of the indications
with CHB with or without Adams-Stokes attacks for this treatment. It is well known that there is al-
have been carried out ( I , 2, 3, 9, 11, 17, 18, 22, 23, ways a danger of the patient incurring fracture of
24). The survival rate one year after the diagnosis the skull or being otherwise severely hurt in con-
of CHB has been reported to be 5040%. Data on junction with an Adams-Stokes attack. Mental con-
the survival rate in patients who were followed up fusion of a patient with CHB and a slow heart rate
for more than one year, are scarce. The patients does not necessarily contraindicate treatment with
presented in this paper were selected from a large an artificial pacemaker; on the contrary, this symp-
group, in which the other patients were artificially tom should strongly motivate this treatment (7).
paced. They are therefore neither comparable with Cerebrovascular accidents may, however, occur in
those patients nor suitable as controls in subsequent conjunction with Atfams-Stokes attacks. One pa-
series of patients identically treated. tient in this series thus had a subdural haematoma
The patients in this series are all considered to on both sides. This condition should be considered
be potential candidates for artificial pacing on the in a patient who continues to have symptoms sug-
present-day indications for this treatment, possibly gestive of a cerebral lesion after artificial pacing
with the exception of 4 patients with congenital has been instituted.
CHB. However, 2 of the latter had had syncopal The mean age of the patients in this series, 70
episodes and were therefore also potential candi- years, was rather high. The females were somewhat
dates. Nevertheless, it was considered to be of younger than the males but the difference in age
interest to investigate whether the patients in this was not statistically significant. The survival rate
series who were potential candidates for treatment was higher in the women than in the men. There
with artificial pacemakers showed characteristic are two possible explanations of this difference:
features which distinguished them from those who firstly, the difference between men and women in
were not thus treated and were dead at the end of the population in this respect and, secondly, the
the follow-up. All the patients who were alive at the difference between the sexes with respect to
end of the follow-up were requested to report at mortality from IHD. According to the National
this Department to take up artificial pacing for con- Central Bureau of Statistics in Stockholm (1%8)
sideration. the remaining life expectancy in 1962 was 14.0
The hospital records of the patients in this series and 10.2 years for women aged 68 and for men aged
did not invariably afford information about the 71, respectively. Johansson ( 1 1) reported that the
reasons why they were not artificially paced. It survival rate in a series of 204 patients with CHB,
was therefore not possible to study this point in who were not treated with artificial pacing, was
more detail. However, it emerged from the records about 50% at one year. The corresponding percent-
that some patients refused to be artificially paced; age in patients who were artificially paced was
in some cases arrhythmia was associated with a 90-95% at the end of a one-year follow-up (2, 13,
complicating disease, such as cancer or a malignant 19, 20).
blood disease, which were no doubt considered to The patients with CHB in our series did not differ
be contraindications. In some cases the attending from those with arrhythmic syncopes not showing
physician or the patient urged reasons for post- this conduction defect, with respect to survival
poning the treatment and in some cases the attend- (Fig. 1). In view of the small number of patients
ing physician, in agreement with the patient, con- without CHB, it was not considered relevant to
sidered the patient’s advanced age to be a contra- study these patients in greater detail here. A com-
indication. CHB without Adams-Stokes attacks was parison of the prognosis of the CHB patients with
not generally accepted as an indication for artificial and without Adams-Stokes attacks, respectively,

Acla Med Scand 200

462 0.Edhag and A . Swahn

showed that the prognosis was in some cases more diabetes, cardiovascular lesions or heart failure for
favourable in the former group ( I I). This was also excess mortality has been demonstrated recently
observed in the present investigation. (3, 1 I). IHD coexisting with CHB is prognostically
It has been shown that the prognosis of patients an unfavourable factor, also in cases in which the
with CHB varies, depending on the underlying patients are artificially paced (2, 7). It is possible
disease (3). According to Johansson ( I l ) , the prog- that the use of ventricular programmed pulse
nosis of patients with CHB due to “rheumatic heart generators (QRS synchronized or inhibited) will re-
disease”, “miscellaneous conditions” or with an duce the excess mortality in patients with CHB
obscure cause of the conduction defect, is much combined with IHD because this type of pacemaker
more favourable than of patients with CHB com- does not involve the risk of stimulation during the
plicated by a coexisting disease of the coronary vulnerable zone of the cardiac cycle. On the other
arteries or digitalis intoxication. In the series re- hand, it has been demonstrated that only artificial
ported by Johansson, survival was strikingly high stimulation by fast rates, so-called overdriving,
in the cases in which the cause of CHB was deter- prevents tachyarrhythmia (21). The development of
mined. This observation prompted us to compare a pacemaker capable of suppressing tachyarrhyth-
the survival rates of patients with CHB associated mia is in progress. Such pacemakers will make it
with IHD, hypertension or diabetes and of patients possible to treat patients with ventricular tachy-
with CHB not combined with any of these condi- arrhythmia which is refractory to medical treat-
tions. It was found that the prognosis of the patients ment.
with CHB combined with one or several of these It has been reported that some patients with ac-
complicating diseases was signficantly poorer than quired CHB may live many years after the lesion
that of the patients with CHB alone. Admittedly, has been diagnosed (2, p. 75). However, the prog-
these two groups in our series differed with respect nosis of the patients with CHB in this series who
to mean age. However, it is questionable whether were not artificially paced, was comparatively poor
the difference in age was the only factor involved at one year. This is in keeping with the observa-
in the difference in prognosis. Johansson ( I I ) found tions of other investigators.
the survival. to be low at 1 year in patients with In earlier investigations, with shorter follow-up
CHB associated with one or several of the above times than in ours, it was found that in cases of
diseases. In this investigation the prognosis of AV block associated with IHD, diabetes or hyper-
such patients was still found to be poor at the end tension, the prognosis is poor. This finding was
of a prolonged follow-up. confirmed here, and it seems that cardiac enlarge-
The combination of CHB and a disease of the ment, too, is an unfavourable prognostic factor.
coronary arteries does not necessarily imply that Survival at one year in the cases of CHB in our
the latter condition is involved in the causation of series was higher (78%) than that (50%) reported
block (6, 15, 16). It is possible that one of the 5 by Johansson (1 1). The mean age of the patients in
patients in our series who developed myocardial his series was 68 years, i.e. somewhat lower than
idarction within two months of the first occasion in our patients. Johansson reported, however, that
on which there was ECG evidence of CHB, had there was no noteworthy difference between age
previously a painless myocardial infarction which groups in his series, except those over 80 years.
caused the conduction defect. The long-term prog- Although the survival rate in our series was
nosis of patients who develop CHB in conjunction higher at one year than reported by others, it dif-
with acute myocardial infarction is poor. This fered from that in the patients who were treated
applies also to patients with intermittent block with artificial pacemakers (7). This was also true
(10). after a follow-up of 5 years. This observation is
Mortality in the cases of CHB in this series was confirmed by Cosby and Bilitch (2).
higher in the first year of the follow-up compared In patients with CHB combined with arrhythmic
with the subsequent years. This may have been syncope, as well as in patients with AV block com-
due to the fact that the cause of arrhythmia was bined with heart failure as a result of slow heart
a coexisting undetermined disease which may have rate, treatment with artificial pacemakers is indi-
contributed to impair the prognosis. The signifi- cated to relieve the patients from incapacitating
cance of coexisting diseases such as renal failure, symptoms and to prolong their lives.

Acta Med Scand 200

Prognosis of unpaced patients 463

A C K N O W L E DGE ME NTS with and without an artificial pacemaker. Acta Med

Scand (Suppl) 45 I, 1966.
The study was supported by grants from the Swedish
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and the Martha and Gunnar Gordon’s Foundation. in 1972. In: Cardiac pacing. Proceedings of the IVth
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Acta Med Scand 200