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Transcript
Sick Sinus Syndrome after Surgery for Congenital
Heart Disease
By RONALD D. GREENWOOD, M. D., AMNON ROSENTHAL, M.D.,
LAURENCE J. SLOSS, M.D., MICHAEL LACORTE, M.D., AND
ALEXANDER S. NADAS, M.D.
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SUMMARY
The course and prognosis of 16 infants and children with sick sinus syndrome associated with cardiac surgery is reviewed. The dysrhythmia was observed most often after extensive atrial reconstructive surgery in
patients with transposition of the great arteries and with atrial septal defect. In 12 (75%) of the patients, sick
sinus syndrome was detected in the immediate postoperative period. Tachyarrhythmias and bradyarrhythmias were present in 12 and isolated bradyarrhythmias in four. Temporary pacing was used in two
and permanent pacing was required in five. Death in two non-paced patients was attributable to
arrhythmias. Postmortem examination in one patient revealed a suture in the sinoatrial node. Careful attention to the anatomy of the sinoatrial node, its artery and the internodal tracts during surgery may prevent
the development of sick sinus syndrome. In patients with dysrhythmia, a careful search should be undertaken to document the abnormal rhythm with the use of Holter monitoring. The insertion of a pacemaker is
indicated in patients with tachyarrhythmias requiring cardioversion or antiarrhythmic drug -therapy and
those without adequate lower escape mechanisms.
THE TERM SICK SINUS SYNDROME (SSS)
from January 1960 through June 1974 for patients with SSS.
Sixteen patients developed SSS following cardiac surgery
and form the basis of this report; four children with SSS, not
related to cardiac surgery, were excluded. During this
period, approximately 40 patients underwent a Mustard
operation, 450 underwent tetralogy repair and 500 underwent closure of an atrial septal defect. To determine the
true incidence of the dysrhythmia, the records of 102 randomly selected patients undergoing tetralogy of Fallot
repair (1962-1972) and 35 consecutive patients representing
all patients undergoing the Mustard operation from
September 1972 through June 1974 were reviewed.
The patients reported were on continuous electrocardiographic monitors for the first few days postoperatively
and for longer periods if dysrhythmias were present. Standard 12 lead electrocardiograms were obtained in the first
postoperative day, prior to discharge, and within one year
after surgery. Frequent rhythm strips were taken
throughout the hospitalization and on follow-up visits. A
Holter monitor was employed in six of 16 patients during
the recovery period and after discharge in 11 of 16. Each
patient was monitored during sleep and waking periods.
was first used by Lown' to describe failure of the
sinus node to assume control following cardioversion
of chronic atrial fibrillation. In recent years, the term
SSS has been used to describe a clinical entity which
includes various disturbances in sinus or atrial
rhythm. It is often used interchangeably with the term
tachycardia-bradycardia syndrome.2`5 The SSS, a
potentially fatal arrhythmia, has been attributed in
adults3' 4 to pathologic changes in the sinus node,
atria, and atrioventricular junction due to coronary
artery disease, cardiomyopathy, hypertensive heart
disease or to unknown etiology. The syndrome has
been recognized with increasing frequency following
surgical repair in children with congenital heart disease. This study describes our experience with SSS
following cardiac surgery at The Children's Hospital
Medical Center.
Methods
Definition
The files in the Medical Records Department and the
Departments of Cardiology and Cardiovascular Surgery at
The Children's Hospital Medical Center were searched
SSS was defined as the occurrence of sinus bradyeardia (a
sustained rate of less than 50 per minute for patients five
years of age or older and less than 55 per minute for younger
children), with or without arrest or exit block, associated
paroxysmal atrial fibrillation, flutter, tachycardia or the
presence of symptoms. Junctional rhythm (defined as less
than the slowest normal sinus rate for that age) without
documented sinus bradyeardia, sinus arrest or exit block,
was not considered to represent SSS unless the arrhythmia
persisted or recurred frequently after discharge from the
hospital. Patients with accelerated junctional rhythm or
tachyarrhythmias without bradyarrhythmias, or with
chemical or toxic etiology for the dysrhythmia (e.g.,
hypokalemia, digitalis toxicity) were not included. Also ex-
From the Department of Cardiology, The Children's Hospital
Medical Center and the Department of Pediatrics, Harvard Medical
School, Boston, Massachusetts.
Supported in part by Training Grant HL05855, Program Project
HL10436 from the National Institutes of Health, and a grant-in-aid
from the American Heart Association.
Address for reprints: Ronald D. Greenwood, M.D. The
Children's Hospital Medical Center, 300 Longwood Avenue,
Boston, Massachusetts 02115.
Received January 17, 1975; revision accepted for publication
March 12, 1975.
208
Circulation, Volume 52, August 1975
POSTOPERATIVE SSS
209
Table 1
Postoperative Rhythm in 35 Consecutive Mustard
Operations
Normal sinus rhythm
Minor rhythm abnormalities
Abnormal P wave
Coronary sinus rhythm
No. of
patients
Percent
8
6
23%
17%
6
9
7
8
17%
26%
(4
(2)
Junctional rhythm
Accelerated junctional rhythm
Supraventricular tachyeardia
Conduction abnormalities
10 Atrioventricular block
Right bundle branch block
Trifascicular block
Complete heart block
Transient atrioventricular dissociation
Sick sinus syndrome
(1)
'3)
(1)
(1)
(2)
a
20%
23%C
14%
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eluded were patients with bradyeardia associated with ventilatory problems, transient dysrhythmias following cardioversion, patients whose only abnormality was sinus
bradyeardia or junctional rhythm for short periods and
patients with junctional rhythm associated with transient or
permanent complete heart block. By adherence to these
criteria, a number of patients with less severe probable SSS
were omitted.
Results
Occurrence
SSS occurred in five of 35 (14%) patients with Dtransposition of the great arteries undergoing consecutive Mustard operations (table 1) and in none of
102 randomly selected patients following repair of
tetralogy of Fallot (table 2).
SSS developed after surgery in 16 patients ranging
in age, at the time of diagnosis, from 11 months to 22
Table 2
Postoperative Rhythm in 87 Survivors of 102 Patients
Undergoing Intracardiac Repair for Tetralogy of Fallot
Postoperative rhythm
Normal sinus rhythm
Conduction disturbance
Right bundle branch block
Right bundle branch block and left
anterior hemiblock
Left anterior hemiblock
Complete heart block
10 Atrioventricular block
Ventricular dysrhythmias
Premature ventricular contractions
Ventricular tachycardia
Atrial dysrhythmias
Coronary sinus rhythm
Paroxysmal atrial tachycardia
Atrial flutter
Junctional tachycardia
Junctional escape rhythm
Sinuts bradycardia
Sick sinus syndrome
Circulation, Volume 52, August 1975
No. of patients
Percent
6
70
8
80
9
1
3
10
1
3
11
8
3
9
3
1
1
2
2
1
3
0
1
1
2
2
1
3
0
years (table 3). It followed the Mustard operation in
five patients (31%), atrial septal defect closure in six
(38%), tetralogy of Fallot repair in four (25%), and
aortic valve replacement following ventricular septal
defect closure in one (6%). The atrial septal defect was
of the sinus venosus type and associated with partial
anomalous pulmonary venous return in two patients
(cases 8, 15), of the secundum variety in three patients
(cases 3, 13, 16) and a common atrium in one (case 6).
Three of the five patients undergoing Mustard operation had a previous surgical atrial septectomy.
Surgical Technique
Surgery was performed in all patients utilizing
cardiopulmonary bypass with additional hypothermia
employed in two (cases 2, 7). Superior vena caval cannulation was performed in all and an atriotomy was
performed in 13/16. All operations resulted in
hemodynamically satisfactory repair except in patient
4 who has a persistent ventricular septal defect.
Clinical Description
In 12 of 16 patients (75%), including all those with
transposition, the arrhythmia occurred immediately
after the operative procedure and in the remaining
four (25%) two to 48 months after surgery. Delayed
onset of arrhythmia was noted in three of four patients
with tetralogy and one with atrial septal defect.
Tachy-bradyarrhythmias were present in 12
patients and bradyarrhythmias alone in four. Among
the former, the tachyarrhythmias noted were atrial
flutter (7), atrial fibrillation (2) and supraventricular
tachycardias (8). The bradyarrhythmias in this group
included sinus arrest (4), sinus bradyeardia (10),
asystole (1), nodal escape rhythm (12), sinoatrial block
(2) and predominant ventricular escapes in one. The
patients with isolated bradyarrhythmias exhibited
sinus bradyeardia (three patients), sinus arrest (two
patients) and nodal escape (three patients). Associated
conduction abnormalities observed included right
bundle branch block in three (patients 1, 3, 5),
trifascicular block in two (patients 4, 13) and
trifascicular block with transient complete heart block
in one (patient 10). Dynamic electrocardiogram
recorded via Holter 12-24 hour monitors was extremely helpful in defining the type and severity of
the arrhythmia (table 4). Periods of sinus arrest made
after initial diagnosis (e.g., patients 2, 12) were helpful in determining treatment.
Therapy
Pharmacologrc treatment employed was quinidine
in seven patients (cases 1, 3, 4, 5, 9, 14, 16), isuprel in
two patients (cases 13, 15) and digitalis in 13 patients
(cases 1-9 and 11, 12, 14, 16), seven of whom received
the latter drug primarily for congestive heart failure.
GREENWOOD ET AL.
210
Table 3
Clinical Presentation and Course of Patients Developing Sick Sinus Syndrome after Surgery
Patient
1
2
3
4
5
6
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7
8
9
10
11
12
13
14
15
16
Cardiac diagnosis
Surgery (age yr)
Postop time of
diagnosis of SSS
Major rhythms at
time of diagnosis
Total repair (7); residual Immediately FL, SVT, SB, SA,
CA, JR
ventricular septal
defect closure (20)
Immediately JR, SB
Atrial septal defect
D-transposition of
the great arteries
creation (5d); Mustard
(11/12)
FL, FI, SB, JR
13o mos
Pulmonary stenosis; Total repair (6)
atrial septal defect,
secundum
FL, AT, SB, JR
48 mos
Tetralogy of Fallot Total repair (18)
2 mos
FL, SA, SB, JR
Tetralogy of Fallot Total repair (6)
Immediately SVT, JR, SB, SA
Atrial septal defect, Common atrium repair
(20)
common atrium,
endocardial cushion
defect
D-transposition of
Immediately SB, JR, SA
Mustard (1)
the great arteries
Atrial septal defect, Total repair (1 4/12)
Immediately SAB, AT, JR
sinus veniosus
Atrial septal defect
Immediately FI, FL, JR, SB
D-transposition of
creation (3 wk);
the great arteries
Mustard (4 4/12)
SB
9 mos
Tetralogy of Fallot Total repair (7 8/12)
Immediately SVT, JR
D-transposition of Atrial septal defect
creation (4 d);
the great arteries
MViustard (2)
Immediately SB, JR, Vent Ex,
D-transposition of Mustard palliative
SVT
the great arteries
(10 5/12)
Atrial septal defect, Total repair (7 8/12)
Immediately SB, SA, JR, SVT
secundum;
ventricular
septal defect
Ventricular septal
Ventricular septal
Immediately SAB, SB, JR, FL,
defect-total repair (8);
PAT
defect; aortic
regurgitation
aortic valve
replacement (14)
Atrial septal defect, Total repair (5)
Immediately SA, JR
sinus venosus
Immediately FL, SB, JR
Atrial septal defect, Total repair (5%12)
secundum
Tetralogy of Fallot
Pacemaker
Outcome
Alive (A) Time after
or
diagnosis
dead (D) of SS (yr)
Permanent
A
1 5/12
Temporary
A
10/12
0
A
2 7/12
Permarnent,
0
0
A
1)
D
2 2/12
2 7/12
6 davs
0
A
3/12
0
A
3/12
Permanent
A
1 4/12
Permanent
0
A
A
8 3/12
1 4/12
Permanent
A
2,/12
Temporary
A
10/12
0
A
5 7/12
0
A
1 1/12
0
A
11 9/12
Abbreviations: SSS = sick sinus syndrome; Postop = postoperative period; FT = fibrillation (atrial); FL = flutter (atrial);
SVT = supraventricular tachycardia; SB - sinus bradyeardia. SA = sinus arrest; CA = cardiac arrest; JR = junctional rhythm;
AT = atrial tachycardia; SAB = sinus block; Vent Ex = ventricular escape beats.
Cardioversion was employed in eight patients (cases 1,
3, 4, 6, 8, 9, 14, 16) to terminate tachyarrhythmias.
Permanent epicardial pacemakers were placed in five
patients, for cardiac arrest in one (patient 1), inadequate escape rhythm with extremely slow ventricular
rate in two (patients 9, 12, one of whom - patient 9
- exhibited syncopal episodes with a rate of 18/min),
and trifascicular block in two (patients 4, 10, one of
whom - patient 4 - exhibited syncope). A temporary pacemaker was employed in two additional
patients (cases 2, 13) because of an inadequate rate
and was maintained in the latter patient for one
month. Patient 6 developed syncope and hypotension
and died before a pacemaker could be placed.
Follow-up
Fourteen of the 16 patients are alive and have been
followed from two months to 12 years after the onset
of SSS. The predominant rhythm in these patients,
when last seen, was normal sinus rhythm in two
(patients 8, 14), sinus bradyeardia in one (patient 13),
sinus rhythm with sinus arrest and junctional escape
in one (patient 2), junctional with wandering
pacemaker in one (patient 15), junctional in three
(patients 7, 11, 16), atrial flutter with block in one
(patient 3) and paced in five (patients 1, 4, 9, 10, 12).
Two of 16 patients (cases 5, 6) died with
arrhythmias six days and 2 7/12 years after
documented onset of SSS. Postmortem studies were
Circulation, Volume 52, August 1975
POSTOPERATIVE SSS
211
Table 4
Holter Monitoring of Patients with Sick Sinus Syndrome
Patient
1
2
3
7
8
11
12
13
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14
15
16
Time
postoperatively
1 mo
1 wk
10/12
3
9/12
10 dy
3 mo
5 dy
12 dy
2 h' mo
13 mo
17 dv
20 dy
22 dy
23 dy
14 dy
28 dy
10 mo
Hours of
monitoring
12
12
12
24
12
yr
yr
12
12
12
12
12
24
24
24
24
12
12
12
5 yr
2 dy
8 mo
11 9,/12 vr
12
12
24
12
Findings
JR, SA, atrial bigeminy
JR, NSR
SA, JR, WAP, NSR
FL, varying block
NSR, JR
JR
2:1 SA exit block, SA
FL, varying block
NSR
JR, SVT
JR
JR, 4.5 Sec. SA
SA, multiple, 2.4 sec.
Multiple SA
SA, then paced
NSR
SB, nonconducted atrial
bigeminy
SA 1.2 see.. vent. esc.
SA
WAP, JR
WAP
Abbreviations: JR = junctional rhythm; SA - sinus
arrest; NSR = normal sinus rhythm; WAP = watidering
atrial pacemaker; FL = flutter (atrial); SVT = supraventricular tachycardia; SB
sinus bradyeardia; vent. esc. =
ventricular escape beats.
=
performed in both. In case 5, the tetralogy of Fallot
was well repaired but a surgical suture had been
placed in the area of the sinoatrial node. In case 6, the
repair of the common at$$ium was accomplished by
construction of a dacron baffle diverting blood from
the right superior vena cava, hepatic veins and left
superior vena cava to the right side of the heart and
from the widely separated pulmonary veins to the left
side of the heart. The baffle was large and produced
some obstruction of the mitral valve.
Discussion
Incidence
SSS is characterized by the occurrence of marked
sinus bradyeardia or arrest or sinoatrial exit block with
or without an escape rhythm (junction or ventricular)
or atrial tachyarrhythmias (fibrillation, flutter,
tachycardia). Symptoms and complications associated
with dysrhythmias vary greatly.
Although not termed SSS, identical dysrhythmias
have been previously described following cardiac surgery. Sinus bradyeardia, sinoatrial block or arrest, escape rhythms or tachyarrhythmias have been noted
following the Mustard operation,6 -13 Blalock-Hanlon
procedure,7' 14 Senning operation,7 atrial septal defect
repair" and less commonly in ventricular septal
defect repair,'7' 21, 22 tetralogy of Fallot repair'5' 21, 23
and other cardiac operations.'5' 24
Circulation, Volume 52, August 1975
Atrial surgery, the Mustard operation in particular,
highest incidence of
dysrhythmias. In one series, only three of 60 patients
surviving Mustard operation have remained free of
dysrhythmias.f A significant number of these exhibited passive dysrhythmias including sinoatrial
is associated with the
block, wandering pacemaker, junctional rhythm
or
atrioventricular dissociation. Tachyarrhythmias
(supraventricular tachycardia and atrial flutter) were
also present. It is not clear whether these conditions
coexisted in some patients with bradyarrhythmias.
One patient with junctional rhythm and recurrent
atrial flutter died after surgery. Among our patients
undergoing the Mustard procedure only eight of 35
(22%) were free of any dysrhythmia and five (14%) exhibited classical SSS (table 1). Among a randomly
selected group of survivors after repair for tetralogy at
our institution, none exhibited SSS (table 2). Atrial
septal defects of the sinus venosus variety represent a
small proportion of all patients with atrial septal
defects and the interatrial communication is usually
small. However, the location of the defect allows a
high incidence of sinus dysfunction.25
Pathology and Mechanism
Pathologic changes of the sinus node, atria and
atrioventricular junction have been noted in adults
with S5.5 Injury to these structures at surgery may
produce SSS and an inadequate escape rhythm. In
patients developing SSS after cardiac surgery, the
dysrhythmia may be related to injury to or around the
sinus node, the sinoatrial nodal artery or the internodal atrial tracts. Pathologic studies of patients with
postoperative dysrhythmias have shown abnormalities
in the sinoatrial node and sinoatrial nodal artery.6 7 In
addition, clinical and pathologic studies have shown
that damage to the internodal pathways during a
Mustard, Senning or Blalock-Hanlon operation is
associated with rhythm disturbances, usually junctional rhythm.7 The mechanisms of the bradycardia
may be a disorder of impulse generation in the sinus
node or disordered conduction from the sinus node.3' 4
In addition, in patients with inadequate escape
rhythm in response to the sinus bradyeardia or arrest
episodes, we may presume that there are abnormalities in automaticity of the atrioventricular junction. The paradox of rapid ventricular response to the
tachyarrhythmias yet inadequate escape rhythm is explained by the observation that atrioventricular conduction and automaticity are related to different portions of the atrioventricular junctional tissues.5
Prevention
Modifications of the Mustard procedure by using
the right atrial appendage rather than superior vena
cava for cannulation, making the atriotomy anterior to
212
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the sulcus terminalis and fixation of the intra-atrial
baffle away from the superior vena cava have been
suggested as preventive measures in avoiding
dysrhythmias.6 Modification in repair of sinus venosus
atrial septal defect might include swinging of the
superior margin of the patch posteriorly into the left
atrium instead of anchoring it to the cristt terminalis.
In all operations involving cardiopulmonary bypass,
anchoring sutures to cannulae in the superior vena
cava or placement of sump suckers may produce injury to the sinoatrial node or its artery and result in
SSS. Confining the atriotomy, if possible, to the
trabeculated portion of the right atrium and
avoidance of the crista terminalis and other conduction tissues may result in fewer dysrhythmias.
Awareness of the variation in origin of the sinoatrial
nodal artery26 should lead to its avoidance at surgery
and may also reduce the incidence of damage to the
node. We have not noted this syndrome in any
patients operated under inflow occlusion.
Diagnosis
The Holter monitor provides an excellent tool for
evaluating patients suspected of having SSS. Episodes
of sinus arrest, severe bradycardia and short bursts of
tachyarrhythmias in asymptomatic patients were
diagnosed in many of our patients only by use of the
Holter monitor. The decision to place a permanent
pacemaker in a child is a difficult one and documentation of serious arrhythmias is mandatory in making
the appropriate decision. Failure to recognize these
dysrhythmias may lead to syncope and death. In contrast to adult patients, infants may not manifest symptoms. The absence of symptoms in infancy should not
preclude further investigation or treatment. Patients
with definite SSS may have normal sinus rhythm during the course of their disease. It is likely that a spectrum of abnormalities exists from patients with severe
unremitting sinus disease to those with intermittent
and mild disease which is not easily recognizable or
diagnosed.
Treatment
Pharmacologic therapy alone has been unsuccessful
in treatment of SSS.3' However, it is often required in
the control of tachyarrhythmia. Electrical pacing has
been frequently employed because it not only
successfully protects from complications associated
with bradyarrhythmias but may also be effective in
suppressing tachyarrhythmias27-29 and preventing
adverse effects on the conduction system when the addition of antiarrhythmic agents is required.3-5 30 the
optimum therapy in the postoperative patient is
dependent upon specific dysrhythmias present. During the immediate postoperative period, evaluation
GREENWOOD ET AL.
should include adequate monitoring with Holter
monitor and temporary pacing via epicardial wires left
at the time of surgery. No treatment is necessary in
asymptomatic patients with sinus bradyarrhythmias
and documented adequate sustained junctional escape mechanism (case 7). If symptoms occur, the escape rate is inadequate and dysrhythmia persists for
more than a few weeks, a permanent demand
pacemaker is indicated. If tachyarrhythmias are present, a demand pacemaker and antiarrhythmic drug
treatment are indicated. Death probably occurred in
two of our patients from the use of pharmacologic
treatment without pacing. Antiarrhythmic drug
therapy alone is unsatisfactory since the drugs utilized
(quinidine, propranolol, digoxin) can further suppress
sinus or lower escape mechanisms. Cardioversion is
best attempted with a temporary transvenous demand
pacemaker in place due to the possibility of both conversion asystole and inadequate sinus response.
Any patient with SSS who is not paced should be
carefully followed for an indefinite period of time. We
feel that any well documented episode of sinus failure
without an adequate escape rhythm should be treated
with a permanent pacemaker. It is worth emphasizing
that the subgroup of patients with SSS in whom the
escape mechanism is inadequate is at particular risk
from bradyarrhythmias producing syncope or death.
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Sick sinus syndrome after surgery for congenital heart disease.
R D Greenwood, A Rosenthal, L J Sloss, M LaCorte and A S Nadas
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Circulation. 1975;52:208-213
doi: 10.1161/01.CIR.52.2.208
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