Outcome After Repair of Tetralogy of Fallot in the
First Year of Life
Christos Alexiou, FRCS, Hyam Mahmoud, MRCPCH, Ahmed Al-Khaddour, MD,
James Gnanapragasam, FRCP, Anthony P. Salmon, FRCP, Barry R. Keeton, FRCP, and
James L. Monro, FRCS
Departments of Cardiac Surgery and Paediatric Cardiology, The General Hospital, Southampton, United Kingdom
Background. The purpose of this study was to evaluate
the early and late outcome after repair of tetralogy of
Fallot in the first year of life.
Methods. Between 1974 and 2000, 89 consecutive in-
fants with a mean age of 6.3 ⴞ 2.6 months (range, 15 days
to 12 months) underwent repair of tetralogy of Fallot
(ventricular septal defect and pulmonary stenosis) by one
surgeon (J.L.M.). Three infants had previous palliative
operations. Sixty-seven procedures were urgent or emer-
gency. A transannular patch was inserted in 69 patients
(77.5%). Follow-up was complete, averaging 13.4 ⴞ 5.6
years (range, 0 to 25.4 years).
Results. There was one operative death (1.1%). Mean
right ventricular to left ventricular pressure ratio postop-
eratively was 0.4 ⴞ 1.1 (in 79 patients, < 0.5). Fourteen
patients underwent reoperations or reinterventions.
There were no reoperations for residual or recurrent
ventricular septal defect. Kaplan-Meier freedom from
reoperation or reintervention for any cause at 20 years
was 85% ⴞ 4.4%, for relief of right ventricular outflow
tract obstruction it was 94% ⴞ 3.1%, and for pulmonary
valve replacement this was 95.4% ⴞ 2.6%. Use of a
T he management of tetralogy of Fallot (TOF) has
evolved over the last few decades, and good results
after one or two-stage repair have been reported [1–15].
The inclusion, however, in some of these reports of
patients having TOF complicated by lesions such as
pulmonary atresia, atrioventricular septal defect, absent
pulmonary valve syndrome, and so forth makes compar-
isons among various treatment protocols difficult. To
achieve a more homogeneous group for analysis we have
excluded such patients from this study.
The optimal age of repair of TOF has been controver-
sial, but because of the reduced operative mortality in the
current era, the realization of the benefits of early repair
on the heart and other organ systems [7, 10, 16, 17], and
the desire to avoid the risks and inconvenience of a
palliative procedure, elective early repair of TOF is now
increasingly being performed [7, 10, 15, 18, 19].
However, questions still remain regarding the long-
Accepted for publication Sept 25, 2000.
Address reprint requests to Dr Monro, Department of Cardiac Surgery,
The General Hospital, Tremona Rd, Southampton SO16 6YD, UK; e-mail:
monro1711@aol.com.
© 2001 by The Society of Thoracic Surgeons
Published by Elsevier Science Inc
transannular patch did not significantly affect the need
for reoperation or reintervention. There was one late
death (leukemia). Kaplan-Meier 20-year survival was
97.8% ⴞ 1.9%. On latest echocardiography, 42 patients
had moderate pulmonary regurgitation, 4 had a right
ventricular outflow tract gradient more than 40 mm Hg,
and 86 had good biventricular function. Twelve-lead
electrocardiography was performed in all and 24-hour
electrocardiography in 61 patients. One patient (1.1%)
exhibited late recurrent ventricular tachycardia requiring
implantation of a defibrillator. The remaining 86 patients
are in New York Heart Association class I with none of
them receiving antiarrhythmic medications.
Conclusions. These data strongly support the concept
of early repair of tetralogy of Fallot. It is associated with
an acceptable operative risk and a low incidence of
significant arrhythmias, and provides long-term survival
similar to that observed in the general population. Late
complications may, however, develop, and long-term
follow-up for their early recognition is essential.
(Ann Thorac Surg 2001;71:494 –500)
© 2001 by The Society of Thoracic Surgeons
term outcome after repair of TOF in infancy. The impact
of the resultant pulmonary regurgitation, for instance, in
those requiring reconstruction of the right ventricular
(RV) outflow tract with a transannular patch (TAP), on
their late hemodynamic condition is uncertain.
Since 1974, it has been our policy to favor primary
repair, rather than palliation, of TOF in symptomatic
infants requiring operation. Exceptions were infants hav-
ing hypoplastic pulmonary arteries (PAs), small-sized left
ventricle (LV), anomalous origin of the left coronary
artery, and multiple ventricular septal defects (VSDs) in
whom a two-stage repair was, mostly, preferred.
Encouraged by the results of this approach we have
from 1988 also performed primary repair in asymptom-
atic infants. The evolution of our practice, regarding the
age at repair, over the study period, resulted in a pro-
gressively higher proportion of patients undergoing de-
finitive repair of TOF during the first year of life (Fig 1).
This article describes the outcome after repair of TOF
in children up to 12 months of age, placing emphasis on
the need for reoperation, the late function of the RV, the
postoperative electrocardiogram (ECG), the incidence of
0003-4975/01/$20.00
PII S0003-4975(00)02444-9
Ann Thorac Surg
2001;71:494 –500
Fig 1. Proportion of patients undergoing repair of tetralogy of Fallot
during the first year of life (gray columns) rose significantly during
the years of the study period, from 26.8% (1975 to 1979) to 81.3%
(1995 to 2000; ␹2 test for trend, p ⬍ 0.0001).
arrhythmias, and the survival and the physical status of
the long-term survivors.
Patients and Methods
Between October 1974 and March 2000, 89 consecutive
infants (51 boys and 38 girls) underwent repair of a
simple TOF (anatomically characterized by a dextro-
posed and overriding aorta, a VSD, and infundibular
pulmonary stenosis) by one surgeon (J.L.M.) in
Southampton. Their mean (⫾ standard deviation) age
was 6.3 ⫾ 2.6 months (range, 15 days to 12 months). Age
distribution is shown in Figure 2. Their mean body
weight was 6.4 ⫾ 1.5 kg (range, 2.1 to 12 kg).
Included in this review were patients operated on by
one surgeon to ensure uniformity in the treatment ap-
proach and operative techniques used over time.
During the same period, 10 infants with TOF under-
went palliative procedures. Nine of them survived and
had definitive repair when they were more than 1 year of
age.
Definitions
The terms emergency and urgent denote the perfor-
mance of nonelective repair within 1 and 7 days, respec-
tively, from the surgical referral by the pediatric
cardiologists.
Operative mortality includes any death occurring
within 30 days after the operation or during the same
hospital admission.
Procedures performed by the cardiologists at recath-
eterization (balloon dilatation, stenting) are defined as
reinterventions.
Clinical and Pathologic Features
Definitive diagnosis was, until around 1980, made by
angiography. Thereafter a combination of echocardiog-
raphy and angiography was used. Seventy-nine patients
exhibited hypoxic spells at various intervals before their
operation, 10 patients had no symptoms, and 66 were on
␤-blockers preoperatively. Mean arterial oxygen satura-
tion was 85.6% ⫾ 10.6% (range, 31% to 100%).
ALEXIOU ET AL 495
REPAIR OF TETRALOGY OF FALLOT IN INFANCY
The operative procedure was elective in 22 (24.7%) and
urgent or emergency in 67 patients. The proportion of
patients undergoing an elective repair before 1988 was
13%, and since then it rose to 43% (p ⫽ 0.002).
Three patients with hypoplastic PAs had a previous
modified Blalock-Taussig shunt before surgical repair in
the first year of life.
Surgical Technique and Operative Data
Cardiopulmonary bypass with deep hypothermia and
circulatory arrest, and surface or core cooling, were used
until the late 1980s. Since then, repair of TOF under
cardiopulmonary bypass alone became standard prac-
tice. Deep hypothermia and circulatory arrest and sur-
face cooling are no longer in use.
Cardiopulmonary bypass with deep hypothermia and
circulatory arrest was used in 70 patients, and hypother-
mic cardiopulmonary bypass alone in 19 patients. Sur-
face cooling was used in 57 patients at a mean tempera-
ture of 18°C ⫾ 2.7°C. Cold crystalloid cardioplegia (St.
Thomas‘s solution) has been used since 1978, and re-
cently, cold blood cardioplegia has been used. The mean
duration of cardiopulmonary bypass was 47.1 ⫾ 16.2
minutes, and the mean duration of circulatory arrest was
49.2 ⫾ 9.1 minutes.
Before 1988 a transventricular approach was invariably
used. A TAP was inserted if the diameter of the narrow-
est point of the RV outflow tract, measured with Hegar
dilators, was less than the minimum acceptable pulmo-
nary valve ring diameter for the patient’s age and weight,
as suggested by Pacifico and coworkers [20]. In the event
of uncertainty, use of a TAP was more likely if a bicuspid
pulmonary valve was present. If a patch was necessary, a
longitudinal incision was made through the pulmonary
valve ring and continued to the bifurcation of the PA. If
an outflow patch was required, a monocusp from an
antibiotic-sterilized aortic homograft was used for pref-
erence before 1983. Since then, homografts have been
more difficult to obtain, and autologous pericardium has
mostly been used.
Since 1988, when from the preoperative investigations
a TAP was thought not to be necessary using criteria as
previously defined (PA to ascending aorta [AA] diameter
Fig 2. Age distribution of patients undergoing repair of tetralogy of
Fallot in the first year of life and number of patients having a
transannular patch (TAP). The differences in the proportion of pa-
tients requiring a transannular patch at various ages were not statis-
tically significant (p ⫽ 0.6).
496 ALEXIOU ET AL
REPAIR OF TETRALOGY OF FALLOT IN INFANCY
ratio ⬎ 0.5 on preoperative angiogram) [21], a transatrial
approach was used. Furthermore, when the insertion of a
TAP was inevitable, the incision in the RV was shorter
and the TAP smaller.
Branch PAs were assessed preoperatively with angiog-
raphy supplemented more recently by echocardiogra-
phy. If they were thought to be of adequate size, a
complete repair was planned, otherwise a Blalock-
Taussig shunt followed by a later repair was preferred.
Hypoplastic PAs remain a contraindication for an early
repair in our unit. At operation, the assessment of the
adequacy of the size of branch PAs was initially based
on the judgment of the operating surgeon. Subsequently,
the McGoon ratio and the Nakata index were used
[22, 23].
Ventricular septal defects were closed with a continu-
ous polypropylene suture, using a polyethylene tereph-
thalate or Gore-Tex (W.L. Gore & Associates, Flagstaff,
AZ) patch, through a transventricular approach in 77 and
a transatrial approach in 12 patients. Resection of infun-
dibular muscle was carried out as required. Interatrial
communications were routinely closed. The RV and LV
pressures were routinely measured before chest closure.
Follow-up
After their discharge from the hospital the patients were
followed up at regular intervals by the pediatric cardiol-
ogists, and echocardiography and 12-lead ECG were
routinely performed.
Data were obtained through a detailed review of the
hospital medical records. Additional information was
sought from the referring physicians, family doctors, and
the patients‘ families as appropriate.
Mean follow-up was 13.4 ⫾ 5.6 years (range, 0 to 25.4
years). Twenty-four patients were followed for up to 10
years and 63 patients for 10.1 to 25.4 years. Follow-up
information was complete within 12 months of the clos-
ing date of this study (March 31, 2000).
Statistics
Continuous data were expressed as mean (⫾ standard
deviation). Proportions were compared with ␹2 or Fish-
er‘s exact test, and means with Student’s t test. Freedom
from time-related events (⫾ standard error from the
mean) was calculated with the Kaplan-Meier method,
and the resulting curves were compared with log rank
test. A p value of less than 0.05 was considered signifi-
cant. Analyses were performed with the SPSS PC version
8 (SPSS Inc, Chicago, IL).
Results
Incidence of Transannular Patching and Early Relief
of Right Ventricular Outflow Tract Obstruction
The mean diameter of the AA was 13.7 ⫾ 2 mm (range, 7
to 20 mm), the mean diameter of the PA was 7.2 ⫾ 1.5 mm
(range, 3 to 15 mm), and the mean PA/AA diameter ratio
was 0.5 ⫾ 1.1. This was less than or equal to 0.5 in 60
patients, equal to 0.6 in 13 patients, 0.7 in 9 patients, 0.8 in
Ann Thorac Surg
2001;71:494 –500
Table 1. Early Postoperative Complications in 13 Patients
(14.6%)
Number
Type of Complication of Patients
Cardiorespiratory failure (prolonged 4
ventilation or inotropic support)
Chest infection 3
Intravascular hemolysis 1
Transient seizures 1
Renal failure 1
Cardiac arrest 1
Coagulase-negative staphylococcal 1
septicemia
Pericardial effusion (open drainage) 1
6 patients, and 1.0 in 1 patient. The pulmonary valve was
bicuspid in 49, tricuspid in 39, and monocuspid in 1
patient.
A TAP was inserted in 69 patients (77.5%), a monocusp
homograft in 33, pericardium in 31, and dura mater in 5.
Until 1988 the incidence of the use of a TAP was 83.3% (45
of 54 patients), and since then it was 68.6% (24 of 35
patients; p ⫽ 0.1).
A PA/AA ratio of less than or equal to 0.5 (p ⬍ 0.00001)
and presence of a bicuspid pulmonary valve (p ⫽ 0.0002)
were significantly associated with the need for a TAP. A
slightly higher proportion of infants up to 6 months of
age required insertion of a TAP, but this may have been
because of chance alone (p ⫽ 0.6; Fig 2).
The mean RV pressure at the end of the operation was
35 ⫾ 7.7 mm Hg (range, 20 to 60 mm Hg), the mean LV
pressure was 86 ⫾ 8.2 mm Hg (range, 66 to 116 mm Hg),
and the mean RV/LV pressure ratio was 0.40 ⫾ 0.08
(range, 0.2 to 0.8). This was less than 0.5 in 79 patients, 0.5
to 0.6 in 8 patients, and 0.7 to 0.8 in 2 patients. The mean
RV/LV pressure ratio among the patients who had a TAP
was 0.39, and it was 0.41 in the 20 patients having a
simple repair (p ⫽ 0.5).
Operative Mortality and Morbidity
One 8-month-old male infant with Down syndrome died
4 days after an uneventful procedure (1.1%). While in the
intensive care unit, he became hypoxic and required high
positive-pressure ventilation. He subsequently had bilat-
eral pneumothoraces and respiratory distress syndrome.
At postmortem examination, there was an unexplained
generalized sloughing off of the tracheobronchial
mucosa.
Thirteen patients (14.6%) had early postoperative com-
plications as shown in Table 1.
Reoperations and Reinterventions
Fourteen patients required 19 further reoperations or
reinterventions at a mean interval of 8.7 ⫾ 7.2 years
(range, 9 months to 21.6 years) with no operative mortal-
ity. Five of them underwent only a reoperation, 5 had a
reoperation and a catheter reintervention, and the re-
maining 4 patients had only a catheter reintervention.
Ann Thorac Surg ALEXIOU ET AL 497
2001;71:494 –500 REPAIR OF TETRALOGY OF FALLOT IN INFANCY

Table 2. Types of Reoperations and Reinterventions in 14

Patients
Number Number
of of
Reoperation Patients Reintervention Patients

PVR 6 Balloon dilatation 4

of hypoplastic
LPA or RPA
Patch enlargement 2 Balloon dilatation 3
of the PAs of RVOT
Relief of RVOTO 1 Balloon dilatation 1
and patch and stenting of
enlargement of RPA
the PA
Insertion of 1 Stenting of RPA 1
pericardial and LPA
cusps into PV
and patch Fig 4. Kaplan-Meier freedom from any reintervention or reoperation
enlargement of for right ventricular outflow tract obstruction at 20 years was 94%
the PAs ⫾ 3.1%.
Total 10 Total 9

LPA ⫽ left pulmonary artery; PA ⫽ pulmonary artery; PV ⫽
pulmonary valve; PVR ⫽ pulmonary valve replacement; RPA ⫽
right pulmonary artery; RVOTO ⫽ right ventricular outflow tract
obstruction.
Types of reoperations (n ⫽ 10) and reinterventions (n ⫽
9) are shown in Table 2.
Twenty-year freedom from reoperation was 91% ⫾
3.3%, and from any reoperation or reintervention this
was 85% ⫾ 4.4% (Fig 3). In the patients in whom a TAP
was inserted, 20-year freedom from reoperation or rein-
tervention was 82.8% ⫾ 5.2%, and it was 86.9% ⫾ 7.6% in
those undergoing a simple repair (p ⫽ 0.8).
One patient required reoperation, and 3 patients re-
quired reinterventions (Table 2) to relieve recurrent RV
outflow tract obstruction (RVOTO) at a mean time of
4.9 ⫾ 5.1 years (range, 0.8 to 15.5 years) postoperatively.
The usual indication was an RV pressure more than two
thirds of systemic pressure measured at cardiac catheter-
ization with the patient anesthetized. Freedom from
reoperation or reintervention for recurrent RVOTO at 20
years was 94% ⫾ 3.1% (Fig 4). For the patients receiving
a TAP, 20-year freedom from reoperation or reinterven-
tion for recurrent RVOTO was 95.4% ⫾ 3.4%, and for the
20 patients undergoing a simple repair it was 89.4% ⫾
6.9% (p ⫽ 0.1).
Six patients, all of whom had a TAP, required replace-
ment of a severely regurgitant pulmonary valve at a
mean time of 14.2 ⫾ 6.7 years (range, 2.4 to 21.6 years)
postoperatively with an antibiotic-sterilized aortic ho-
mograft. The usual indication in an asymptomatic patient
was the presence of severe pulmonary regurgitation with
progressing RV dilatation or ECG changes. In the pres-
ence of symptoms, the threshold for pulmonary valve
replacement (PVR) was substantially lower. The earliest
PVR in the series was performed 2.4 years postopera-
tively in a child who required reoperation for PA stenosis
and also had severe pulmonary regurgitation. The re-
maining 5 patients had a PVR at a mean time of 16.6 ⫾ 5.1
years (range, 8.2 to 21.6 years), 3 of them more than 20
years postoperatively. Overall 20-year freedom from PVR
was 95.4% ⫾ 2.6%. This was 91.8% ⫾ 3.4% in the group
receiving a TAP, and it was 100% in the group of patients
having a simple repair (p ⫽ 0.3; Fig 5). There were no
reoperations for residual or recurrent VSD.

Late Survival and Functional Status

Fig 3. Kaplan-Meier freedom from any reoperation or reintervention
at 20 years was 85% ⫾ 4.4%.
One female patient who had undergone primary simple
repair of TOF at 6 months of age died 2 years later of
leukemia. Twenty-year survival, inclusive of operative
mortality, was 97.8% ⫾ 1.9% (Fig 6).
Recent echocardiographic investigations demonstrated
the presence of an RV outflow tract gradient of more than
40 mm Hg in 4 patients, moderate pulmonary regurgita-
tion in 42, moderately severe RV dilatation in 30, and
good RV and LV function in 86 patients.
At latest 12-lead ECG, 81 patients were in sinus rhythm
with right bundle-branch block pattern, 2 patients had
intermittent first-degree and second-degree heart block
not requiring treatment, 1 patient had occasional ventric-
ular ectopic beats, and 2 patients were in normal sinus
498 ALEXIOU ET AL
REPAIR OF TETRALOGY OF FALLOT IN INFANCY
Fig 5. Kaplan-Meier freedom from pulmonary valve replacement
among patients receiving a transannular patch (TAP ⫽ continuous
line) at 20 years was 91.8% ⫾ 3.4%, but 3 patients had a pulmo-
nary valve replacement more than 20 years postoperatively. None of
the patients who underwent a simple repair (interrupted line) re-
quired a pulmonary valve replacement (p ⫽ 0.3).
rhythm. No patient had a QRS complex equal to or
longer than 180 ms.
A 24-hour Holter ECG was performed in 61 patients.
Fifty-seven of these patients had sinus rhythm with right
bundle-branch block pattern, 2 patients had intermittent
first-degree and second-degree heart block already diag-
nosed by 12-lead ECG, and 1 patient had occasional
ventricular ectopic beats, also diagnosed with 12-lead
ECG. One patient had persistent recurrent tachycardia
requiring antiarrhythmic medications and the implanta-
tion of an automatic defibrillator. This was the only
patient to exhibit significant arrhythmia in this series
(1.1%) and did so 16 years after his original operation.
There has been no case of a patient experiencing
permanent neurologic deficit.
At the latest clinical evaluation all but 1 survivor, the
patient with the defibrillator, were in New York Heart
Association functional class I, leading a normal or nearly
normal lifestyle with none of them receiving antiarrhyth-
mic medications.
Outcome in Neonates
There were 8 neonates (up to 30 days of age) in this series
with a mean age of 24 ⫾ 4.7 days (range, 15 to 30 days)
and a mean body weight of 3.4 ⫾ 0.9 kg (range, 2.1 to
4.2 kg). All had severe cyanosis preoperatively, and 5 had
ductus arteriosus– dependent circulation and were re-
ceiving prostaglandins. None had undergone a previous
operation. At a mean follow-up of 12.9 ⫾ 6.1 years (range,
2 to 22.4 years), all 8 patients are well, with a sinus
rhythm and right bundle-branch block pattern on their
ECG.
Comment
Some previous reports on repair of TOF during infancy
include patients with coexisting conditions, such as pul-
Ann Thorac Surg
2001;71:494 –500
monary atresia, absent pulmonary valve syndrome, atrio-
ventricular septal defects, and so forth. We believe that
this is confusing, making late assessment difficult, and
have therefore described in this series only infants hav-
ing uncomplicated TOF, that is dextroposed and overrid-
ing aorta, VSD, and infundibular pulmonary stenosis.
Early Outcome
The immediate goal of repair of TOF is the restoration of
a normal circulation by closing the VSD and relieving the
RVOTO with the least possible operative mortality. Our
experience in this respect, as evidenced by the low
postoperative RV/LV pressure gradient and the lack of
any reoperation for residual VSD, was rewarding. The
operative mortality of 1.1% in this series, which extends
over the last two and a half decades, was low and
compares favorably with early mortality rates of 0% to
14.3% quoted in other series for one-stage or two-stage
repair in infants, older children, and adults [1–15]. In
their reports on children having repair during an era
similar to ours, Kirklin and colleagues [9, 11] and Ham-
mon and associates [4] found younger age (⬍3 months
and ⬍1 year) and use of a TAP to significantly increase
the likelihood of an early death. None of the patients less
than 3 months of age in our series died early or late, and
the only operative fatality occurred, unexpectedly of
pulmonary complications, in an 8-month-old infant with
Down syndrome who underwent a simple repair.
Transannular Patch and Pulmonary Regurgitation
The reported incidence of insertion of TAP varies widely.
The relatively high overall incidence in this series (77.5%)
reflects the efforts made to adequately relieve the often
severe RVOTO according to the recommendations made
by Pacifico and associates [20]. This has resulted in
obtaining a mean postoperative RV/LV pressure ratio of
0.4, with the highest being 0.8. If we had accepted a
higher postoperative RV/LV pressure ratio, the fre-
quency of transannular patching would have been lower,
and this may have reduced the incidence of regurgitation
and the need for PVR. However, this would have most
Fig 6. Kaplan-Meier survival, inclusive of operative mortality at 20
years, was 97.8% ⫾ 1.9%.
Ann Thorac Surg
2001;71:494 –500
likely been achieved at the expense of an increase in the
requirements for reoperations for residual or recurrent
RVOTO. The decline in the use of TAP over time (83.3%
until 1988 and 68.6% thereafter) was because of the
change in our policy since 1988, favoring elective repair
of TOF in asymptomatic infants with less severe RVOTO.
Whether repair at a younger age increases the need for
use of a TAP remains uncertain. In a report from Toronto
[12], it has been suggested that younger age at repair is
associated with an increased need for transannular
patching. However, in a two-institutional study compar-
ing the results of a protocol of early repair on the
establishment of diagnosis (Boston Children‘s Hospital)
versus palliation in infancy and repair during the second
year of life (University of Alabama), age at repair did not
influence the incidence of use of TAP [11]. Although this
question would be best answered with a prospective
randomized study, our experience would suggest that the
severity of the RVOTO, rather than age at repair, is the
most important determinant of the frequency of use of
TAP (Fig 2).
Transannular patching is often necessary to relieve the
RVOTO, but it induces pulmonary regurgitation, which
may be poorly tolerated, in the all-important immediate
postoperative period. To reduce its early occurrence we
have by preference used monocuspid homografts, ac-
cepting that they will become regurgitant later.
Long-term pulmonary regurgitation, on the other
hand, may lead to RV dilatation and dysfunction, in-
crease the need for reoperations, and provoke malignant
ventricular arrhythmias [24]. Late pulmonary regurgita-
tion invariably developed in the patients who required a
TAP in this series, being severe in 6 patients who re-
quired replacement of a pulmonary valve and moderate
in 42 patients. Its hemodynamic effect, however, was
mostly benign, with 86 of the 87 late survivors having a
good RV and LV function and being in New York Heart
Association functional class I. Similar hemodynamic out-
comes have been observed after repair of TOF during
infancy elsewhere [7, 10].
Reoperations and Reinterventions
The 20-year freedom from reoperation or reintervention
for any cause of 85% (94% for RVOTO and 95.4% for PVR)
would appear to be encouraging.
Use of a TAP was not a significant factor for any further
operations or interventions for PVR in this series. Its
clinical relevance, nevertheless, is apparent: 6 patients
requiring PVR were all among those who received a TAP,
and it is probable that more of these patients will need a
PVR in time.
In a large series from the University of Alabama [9]
comparing the outcome after repair of TOF with or
without use of TAP with an up to 20-year follow-up, TAP
was not a risk factor for reoperation in general, but it
significantly increased the need for a PVR because of
pulmonary regurgitation. The 20-year freedom from PVR
in that series was 88% in the group receiving a TAP and
100% in the group not receiving a TAP [9].
The 91.8% freedom for PVR in the group receiving a
ALEXIOU ET AL 499
REPAIR OF TETRALOGY OF FALLOT IN INFANCY
TAP in our series is good, but it should be noted that 3
patients needed PVR more than 20 years after their
operations (Fig 5).
There was no serious morbidity or mortality at reop-
eration, and the implantation of a homograft in the RV
outflow tract restored good hemodynamics and reversed
the process of the ongoing RV dilatation in all 6 patients,
although 1 patient went on to exhibit recurrent ventric-
ular tachycardia. This stresses the need for a close,
long-term follow-up, so that a PVR can be performed
before irreversible RV damage is established.
Late Arrhythmias and Survival
Previous studies have reported good early and late he-
modynamic outcomes after repair of TOF in older chil-
dren and adults but described a disappointing incidence
of late arrhythmias and a late sudden death rate of up to
6% [7, 25]. The identification of the patients likely to
experience catastrophic events late after repair of TOF
remains a difficult problem, but Gatzoulis and coworkers
[26] found that a QRS complex equal to or more than
180 ms, on the 12-lead ECG, is a good predictor of the
development of ventricular arrhythmias and sudden
death. The 1.1% incidence of significant arrhythmias and
the lack of sudden death in our patients, none of whom
had a QRS complex duration equal to or more than
180 ms on the 12-lead ECG, with a mean follow-up of 14.3
years, are most gratifying features of this study.
Low incidence or no arrhythmias and no sudden
deaths have also been quoted from elsewhere after repair
of TOF during infancy, although with somewhat shorter
follow-up. Walsh and colleagues [7] described an inci-
dence of ventricular arrhythmia of 1% after repair of
TOF in 220 infants (mean follow-up of 60 months),
whereas Touati and associates [10] and Caspi and co-
workers [19] did not encounter ventricular arrhythmias
in their series of 100 and 82 infants, respectively (mean
follow-up, 22 to 24 months). Combined, these results
appear to support the view that ventricular arrhythmias
may be a consequence of endomyocardial fibrosis related
to long-term hypoxemia, and that repair at an early age
may reduce its prevalence and minimize the risk of
sudden death [17, 27].
The long-term survival after repair of TOF with a
variety of protocols at different ages is known to be very
good, although not identical with that of the general
population [7, 9, 11, 14, 28]. The actuarial 20-year survival
of 97.8% (Fig 6) in this series, with the only late death
occurring because of leukemia, is most rewarding and
demonstrates that a normal survival expectancy after
repair of TOF in infancy can be reasonably anticipated.
In conclusion, the results of this study strongly support
the concept of early repair of TOF. In addition to low
operative risk, repair of TOF in infancy is associated with
a low incidence of significant arrhythmias and provides
long-term survival similar to that observed in the general
population. Late complications may, however, develop,
and consistent long-term follow-up for their early recog-
nition and management is essential.
500 ALEXIOU ET AL
REPAIR OF TETRALOGY OF FALLOT IN INFANCY
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