Original Article TOF Repair in Young Infants and Toddlers Acta Cardiol Sin 2012;28:137-144

Congenital Heart Disease

Long-Term Outcomes of Patients with Tetralogy

of Fallot Repaired in Young Infants and Toddlers
Shuenn-Nan Chiu,1 Jou-Kou Wang,1 Ming-Tai Lin,1 En-Ting Wu,1 Chun-An Chen,1 Shu-Chien Huang,2
Chung-I Chang,2 Yih-Sharng Chen,2 Ing-Sh Chiu2 and Mei-Hwan Wu1

Background: Total repair of tetralogy of Fallot (TOF) during young infancy had been recently advocated, but recent
outcomes leave this question undecided.
Methods: Between 1992 and 2002, 259 consecutive TOF patients received total repair in our hospital. To avoid
confounding by previous shunt operations, we excluded those patients receiving staged shunt operation in the
following analysis. Therefore, a total of 217 TOF patients who received one stage total repair before age 3 in our
hospital were enrolled (early group: 38 patients 0-6 months old; late group: 179 patients 6 months-3 years old). We
reviewed the medical records and confirmed the patients’ survival status from the National Health Database.
Results: Baseline characteristics showed fewer emergent operations (1.7% vs. 13.2%, p = 0.005) and fewer
transannular patches required (70.9% vs. 86.8%, p = 0.029) in the late group. However, the early group had longer
intubation periods and intensive care stays. After the 1994 patient-years follow-up, the 10-year actuarial survival
and reintervention-free survival rate was 97.4% and 89.4%, respectively in the early group, and 95.5% and 93.5%
respectively in late group, which showed no statistically significant difference. The major risk factor for
reintervention was small pulmonary artery size. Severe pulmonary regurgitation correlated with transannular patch,
which tended to be higher in the early group.
Conclusion: Although patients with TOF repaired within first 6 months of life had prolonged postoperative
recovery and an increased chance of transannular patch usage, outcomes were comparable to those patients with
TOF repaired later. Therefore, symptomatic infants can receive total repair of TOF early to avoid the need for use of
a palliative shunt. However, when total repair is undertaken on an elective basis, the procedure may be delayed until
the patient is older than 6 months of age.

Key Words: Infant · Long-term outcome · Surgery · Tetralogy of Fallot

INTRODUCTION perioperative support, outcomes have substantially im-

proved in the last 20 years. However, residual hemo-
It has been more than five decades since the first dynamic abnormalities were still commonplace in many
total repair of tetralogy of Fallot (TOF) by Lillehei et al. patients, with further reintervention possibly required.
in 1955. 1 With advances in surgical techniques and Primary total repair during early infancy had been ad-
vocated for the last ten years.2 The advantages of early
repair include shorter duration of hypoxia, shorter dura-
Received: April 12, 2011 Accepted: January 19, 2012 tion of right ventricular hypertension and a potentially
1
Department of Pediatrics; 2Department of Surgery, National Taiwan
University Hospital and Medical College, National Taiwan University, lower incidence of late arrhythmias. However, there are
Taipei, Taiwan. some concerns regarding early primary repair, including
Address correspondence and reprint requests to: Dr. Mei-Hwan Wu, elevated need for use of transannular patch, increased
National Taiwan University Children’s Hospital, No. 8, Chung-Shan
South Road, Taipei 100, Taiwan. Tel: 886-2-2312-3456 ext. 71524; severity of pulmonary regurgitation (PR), and higher re-
Fax: 886-2-2311-8435; E-mail: [email protected] intervention and mortality rate.3-8 Though several reports

137 Acta Cardiol Sin 2012;28:137-144

 Shuenn-Nan Chiu et al.

had shown favorable early and mid-term results of pri- by elective, symptomatic and emergent operation. Symp-
mary repair during young infancy,9-16 the data regarding tomatic patients in our study were defined as those who
long-term outcomes are still limited.14,16-18 In our hos- have frequent blue spell attacks requiring beta blocker
pital, total correction of TOF during early infancy therapy, or those who were O2 or Prostaglandin E1 de-
(within six months of birth) began in the early 1990s. pendent. Emergent operation was defined as an opera-
With more than fifteen years experience, we have here tion performed either immediately during a hypoxic
investigated the benefits and risks of early total repair by spell attack, or immediately after a hypoxic spell was
comparing those patients with TOF repaired during the medically controlled. Among these 217 patients, 198 had
first six months of life with those repaired between six preoperative cardiac catheterization data for pulmonary
months and three years of age. size evaluation, 7 other patients had preoperative com-
puted tomography results, and the remaining 12 patients
had only preoperative echocardiography reports. The
PATIENTS AND METHODS McGoon index was defined as the sum of the diameters
of the left and right pulmonary artery divided by the dia-
From January 1992 to December 2002, 259 consecu- meter of the descending aorta at the diaphragm level.21
tive TOF patients who received total repair by the age of We also obtained clinical and echocardiographic fol-
3 years in this institution were studied. Patients with pul- low-up data. We mailed questionnaires to or had tele-
monary atresia or absent pulmonary valve were ex- phone interviews with those patients who were lost to
cluded. The data collection was in accordance with re- follow-up for more than 2 years. In addition, we linked
gulations and approved by the institutional review board to the National Health database for death records of
at National Taiwan University Hospital. Among these those patients to check and confirm the survival status
259 patients, 42 of them received a shunt operation first. for all patients.
They had smaller initial pulmonary artery size, smaller The echocardiography machines used during the
initial operative age, and more commonly required emer- study period include the Acuson Sequoia C256 (Acuson,
gent operation. Because staged shunt operation is an im- Mountain View, CA, USA) and the Hewlett Packard
portant risk factor of reintervention and late mortality, Sonos iE33 (Philips Medical Systems, Andover, MA,
(Figure 1), and given that the procedure was gradually
discarded as part of the current surgical strategy,19,20 we
also excluded those patients who received a previous
shunt operation in the following analysis to avoid con-
founding. Therefore, a total of 217 patients who received
one stage total TOF correction without previous shunt
operation were enrolled in our study. They were further
divided into two groups, an early group and a late group.
The early group (n = 38) was defined as those who re-
ceived total cardiac repair from 0 to six months of age,
and the late group (n = 179) was defined as those who
were repaired between the age of six months and three
years. Preoperative echocardiography, cardiac cathe-
terization, and operation data were reviewed. Right ven-
tricular outflow tract (RVOT) reconstruction methods
included RVOT transannular patch repair, RVOT patch
without crossing pulmonary annulus, and transatrial-
Figure 1. Reintervention-free survival curve between those who did
transpulmonary approach without RVOT patch (infun-
and did not receive previous shunt operation. Solid line: those with pre-
dibulectomy only). For those patients who had surgery vious shunt operation. The p value comparing these two groups by
from 0 to 6 months of age, we further divided the group log-rank analysis is 0.006.

Acta Cardiol Sin 2012;28:137-144 138

 TOF Repair in Young Infants and Toddlers

USA). We reviewed the videotape records of the echo- defect was defined as pulmonary versus systemic shunt
cardiography evaluation of each patient, and rechecked (Qp/Qs) greater than 1.5 upon cardiac catheterization, or
the left ventricular function, PR and pulmonary stenosis further necessary intervention. RVOT aneurysm was de-
severity to assure consistency. The PR was graded as fined as aneurysmal formation occurring at RVOT.
severe degree if regurgitated flow was from branch pul-
monary artery, moderate degree if regurgitated flow was Statistics
from distal half main pulmonary artery, and mild degree The statistics used in our study included use of the
if regurgitated flow was from proximal half of pulmo- chi-square test and Fisher’s exact test for comparisons of
nary artery.22 For left ventricular function assessment, clinical characteristics between groups. The Wilcoxon
we defined left ventricular ejection fraction (measured rank sum test was applied for comparisons of the dura-
by M mode method) ‡ 55% as normal.23 However, be- tion of ventilator use, intensive care unit stay and length
cause paradoxical septal motion is common in repaired of admission. Independent Student’s t-test was used for
TOF patients, we also used Simpson’s biplane method. other numeric data comparisons. Log-rank test was used
To evaluate echocardiographic accuracy, we also com- for comparisons in Kaplan-Meier event-free survival
pared the results from magnetic resonance imaging curves, and logistic regression and Cox regression were
(MRI) with echocardiography data in 36 of our patients. applied for regression analysis. Statistical significance
The RVOT and pulmonary stenosis were graded as se- was defined as a p value less than 0.05.
vere degree if the pressure gradient across RVOT and the
main pulmonary artery was greater than 60 mmHg, or
further intervention was necessary. Severe peripheral RESULTS
pulmonary stenosis was defined as pressure gradient
across one branch pulmonary artery that was greater The basic clinical characteristics of these TOF pa-
than 50 mmHg, with marked preferential pulmonary tients who received one stage total TOF repair are shown
flow to one lung, or estimated right ventricular pressure in Table 1. Basic data was also compared between 38
greater than 60 mmHg. Large residual ventricular septal early repair patients and 179 late repair patients. A ge-

Table 1. Clinical characteristics of the 217 patients (38 in early group and 179 in late group) with tetralogy of Fallot operated on
within the age of 3 years. p value less than 0.05 was typed as bold and Italic
Subgroup analysis
All (n = 217) p value
Early group (n = 38) Late group (n = 179)
Operation age (months) 14.4 ± 8.1 4.6 ± 1.2 16.5 ± 7.3 < 0.001 <
Gender (M/F) 148/69 28/10 120/59 0.424
Emergent operation 8 (3.7%) 05 (13.2%) 03 (1.7%) 0.005
Syndrome combination 16 (7.4%)0 1 (2.6%) 15 (8.4%) 0.316
PA size (McGoon index) 0.308
< 1.5 6 (2.9%) 1 (2.7%) 5 (2.9%)
1.5-2 58 (27.6%) 14 (37.8%) 44 (25.4%)
>2 146 (69.5%)0 22 (59.5%) 124 (71.7%)0
Presence of MAPCA 4 (1.8%) 0 4 (2.2%) 1.000
Surgical method 0.029
Transannular patch 160 (73.7%)0 33 (86.8%) 127 (70.9%)0
RVOT patch only 34 (15.7%) 1 (2.6%) 33 (18.4%)
Valve sparing surgery 23 (10.6%) 04 (10.5%) 19 (10.6%)
Total follow-up (years) 9.2 ± 3.3 7.7 ± 2.3 9.5 ± 3.4 < 0.001 <
Echocardiography follow-up (years)* 8.0 ± 3.6 7.0 ± 3.1 8.3 ± 3.7 0.092
* include only those with data available more than 2 years after operation. MAPCA, major aortopulmonary collateral arteries; PA,
pulmonary artery; RVOT, right ventricular outflow tract.

139 Acta Cardiol Sin 2012;28:137-144

 Shuenn-Nan Chiu et al.

netic syndrome was found in 7.4% of TOF patients, in- slightly elevated chance of delayed sternum closure or
cluding 1.9% with documented CATCH 22 syndrome, reoperation in the early repair group. There were 8
0.9% with Down syndrome, 1.5% with VATER associa- (3.7%) early deaths, which were all from the late group:
tion, and the other 3.2% having other chromosomal 4 died of cardiopulmonary collapse within 5 days after
anomaly or a combination with gastrointestinal or brain operation, 2 died of neurological complications, and
malformation. Patients in the early group were more another 2 died of postoperative infection. Multivariate
likely to receive emergent total repair (13.2% vs. 1.7%, logistic regression (on those factors including sex, pul-
p = 0.005) and require transannular patch (86.8% vs. monary artery size, major aortopulmonary collateral
70.9%, p = 0.029) for RVOT reconstruction. The total arteries existence, age at operation, syndromic combina-
follow-up period was longer in the late group than in the tion, operation era, emergent operation, and transannular
early group. This may be related to the fact that rela- patch usage) showed operations conducted prior to 1997
tively fewer patients received early repair in our in- as a significant risk factor for early mortality [odds ratio
stitution in the early 1990s. (OR) 0.55 for every increasing year, 95% confidence in-
terval 0.38-0.80, p = 0.002]. The perioperative mortality
Survival status rate before 1997 and after 1998 were 8/101 (7.9%) and
The immediate outcomes of those patients receiving 0/116 respectively (p = 0.002, Table 3).
one stage repair in the early group and late group are There were only one late death (after discharge or
shown in Table 2. We noted longer duration of in- more than 1 month after operation) in the whole study
tubation, prolonged stay in the intensive care unit, and a cohort. He died of cardiac tamponade due to post-

Table 2. Surgical results and follow-up data after total repair of the 217 Patients with tetralogy of Fallot. p value less than 0.05 was
typed as bold and Italic
All (n = 217) Early group (n = 38) Late group (n = 179) p value
Short term outcome
Total bypass time (mean ± SD) 108.9 ± 28.8 117.2 ± 28.5 107.0 ± 28.7 0.055
Aorta cross clamp (mean ± SD) 083.9 ± 24.6 087.3 ± 24.3 083.1 ± 24.7 0.380
Ventilator day (median) 2.0 3.5 1.0 < 0.001 <
Intensive care unit day (median) 3.5 5.5 3.0 < 0.001 <
Delay sternum closure or early reoperation 09 (4.1%) 04 (10.5%) 5 (2.8%) 0.052
Chylothorax 25 (9.7%) 3 (7.9%) 17 (9.5%)0 1.000
Transient atrioventricular block* 12 (5.5%) 05 (13.2%) 7 (3.9%) 0.039
Severe infection 07 (3.2%) 2 (5.3%) 5 (2.8%) 0.354
Resuscitation early postoperatively 10 (4.6%) 2 (5.3%) 8 (4.5%) 0.688
Early mortality 08 (3.7%) 0 8 (4.5%) 0.356
Hemodynamics at follow-up
Moderate or severe PR† 100 (69.9%) 22 (73.3%) 78 (69.0%) 0.823
Severe PR† 027 (18.9%) 08 (26.7%) 19 (16.8%) 0.292
Left ventricular ejection fraction (%) 69.4 ± 6.7 67.5 ± 7.0 69.9 ± 6.5 0.081
Significant residual VSD† 01 (0.7%) 0 1 (0.9%) 1.000
RVOT aneurysm† 13 (9.4%) 03 (10.0%) 10 (9.3%)0 1.000
More than trivial AR† 08 (5.6%) 0 8 (7.1%) 0.204
Severe PS or peripheral PS† 016 (11.2%) 05 (16.7%) 11 (9.7%)0 0.329
Reintervention 18 (8.3%) 04 (10.5%) 14 (7.8%)0 0.528
Late mortality 01 (0.5%) 1 (2.5%) 0 0.182
* all resolved within two weeks after operation although one received permanent pacemaker one week after operation; † include only
175 patients with echocardiography data available more than 2 years after operation.
AR, aortic regurgitation; PR, pulmonary regurgitation; PS, pulmonary stenosis; RVOT, right ventricular outflow tract; VSD, ventricular
septal defect.

Acta Cardiol Sin 2012;28:137-144 140

 TOF Repair in Young Infants and Toddlers

Table 3. Perioperative mortality rate according to operation year

Operation year 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002
Mortality number 0 3 2 1 2 0 0 0 0 0 0
Operation volume 8 19 17 13 22 22 33 27 18 20 18
Mortality rate 0 15.8% 11.8% 7.7% 9.1% 0 0 0 0 0 0

pericardiotomy syndrome 2 months postoperatively after DISCUSSION

successfully repaired at 5 month of age. The 10-year
actuarial survival rate by Kaplan-Meier analysis was From this study cohort, we had two major findings:
97.4% in the early group, and 95.5% in the late group (1) early total repair of TOF in patients from 0 to six
(Figure 2A, p = 0.60). Regression analysis showed no months of age was associated with slower postoperative
specific factors for late mortality. recovery and a higher risk of transannular patch use, but
that late outcomes, including reintervention rate, were
Late outcomes: hemodynamics and comparable to those repaired between six months and
reintervention three years of age; and (2) small pulmonary arterial size
There were no significant differences in hemo- was the most important risk factor for reintervention.
dynamic parameters between the early and late groups Therefore, we advocate direct total repair for symptom-
(Table 2). Through echocardiographic follow-up, we atic young infants with TOF because of the favorable
found the percentage of severe PR and moderate PR was long-term outcome. However, because of the higher risk
18.9% and 69.9% respectively, after 8.0 ± 3.6 years of of transannular patch usage, total repair surgery on an
follow-up. Severe PR was common in early repair pa- elective basis in early infant stage may be delayed until
tients, but not significantly different from the late group late infancy.
(26.7 vs. 16.8%, p = 0.292). In our study, there were 36 In the early 1990s, Sousa et al. reported that the
patients receiving concomitant MRI examination for PR mortality rate of primary TOF repair in patients from 0
study. We found a good correlation between the PR de- to 6 months of age was comparable to the sum mortality
gree by echocardiography and the PR index by MRI rate from both staged initial palliation and later repair.2
study, with a correlation coefficient of 0.581 (p < 0.001, Since then, several studies had described favorable early
Figure 3). None of our patients had impaired left ven- results of primary repair of TOF in young infants.9-16 In
tricular function during follow-up, both in the early and the present study, although the postoperative course,
late group (Table 2). None of those patients who under- including sternum closure, ventilator support and inten-
went MRI imaging had impaired left ventricular func- sive care unit stay, was relatively prolonged in the early
tion. Using multivariate regression analysis, we found group as compared to the late group, the total cardio-
that use of the transannular patch at repair was the major pulmonary bypass time and aortic cross clamp time were
risk factor for severe PR (OR 10.1, 95% confidence in- similar. This implies that the surgical technique may not
terval 1.31-77.5, p = 0.026). The usage of a transannular be the problem, but that young infant postoperative care
patch was significantly more common in those who un- was more complicated.6,12,13 Nonetheless, postoperative
derwent early repair than in the late repair group (86.8% morbidity, such as by chylothorax, severe infection, or
vs. 70.9%, p = 0.029). severe cardiopulmonary compromise requiring resuscita-
The 10-year reintervention-free survival rate was tion, was similar in both groups. Therefore, prolonged
89.4% and 93.5% in early and late groups, respectively hospital stay may infer only a delayed recovery of car-
(p = 0.314, Figure 2B). Reintervention included trans- diopulmonary function after operation rather than higher
catheter intervention in 12 patients, primarily for pulmo- complication rate in the early group. With improved op-
nary or peripheral pulmonary stenosis. Multivariate Cox erative technique and postoperative care, early infant re-
regression analysis on those factors noted above showed pair is no longer considered as a risk of early mortality.
small pulmonary artery size as the independent risk fac- In the present study, we have identified that the ma-
tor for reintervention (OR 2.25, p = 0.027). jor risk factor for reintervention was small pulmonary

141 Acta Cardiol Sin 2012;28:137-144

 Shuenn-Nan Chiu et al.

Figure 3. Pulmonary regurgitation index by magnetic resonance

imaging compared with pulmonary regurgitation grading by echo-
cardiography. Using Pearson correlation method, we can find good a
correlation between these two methods with correlation coefficient
0.581 and p value less than 0.01. The upper and lower margin of the box
indicates the upper and lower quartile, and the line within the box
indicates the median. Abbreviations: MRI, magnetic resonance imag-
ing; PR, pulmonary regurgitation.

tion, the risk of late reintervention in the early group was

not higher than that in the late group. Reintervention in
this study mainly involved dilatation and stenting of the
pulmonary or peripheral pulmonary stenosis. The 10-
year reintervention free survival rate after total repair
was 92.7%, which was close to results obtained in
B previous reports (ranged from 76.6 to 94.2%). 3,10,11
Figure 2. (A) Kaplan-Meier survival curve and (B) reintervention-free
survival curve between early and late repair group. There were no sta- Therefore, primary total repair in symptomatic young
tistically significant differences when comparing these two events be- infants for appropriate candidates, instead of shunt pal-
tween early and late groups. Sold line: early repair group, dash line: liation, may be preferred to avoid shunt-related mor-
late repair group.
bidity and mortality.
As just mentioned, one of the advantages of early
arterial size. In addition, previous shunt operation was total repair is a shorter duration of hypoxia, which may
also found to increase late reintervention rate, which is cause reduced ventricular dysfunction. 3,6 However, in
consistent with findings in previous reports.19,20 Given our study, we did not find any patients presenting left
the possibility of acute shunt occlusion and late reinter- ventricle dysfunction by echocardiography in either
vention, the use of staged shunt operation has gradually group. In one large scale multicenter study, approxi-
been discarded, and a one stage operation is now the pre- mately 21% of adult TOF patients had more than a mild
ferred procedure.24 For those receiving one stage opera- degree of left ventricle dysfunction measured by M

Acta Cardiol Sin 2012;28:137-144 142

 TOF Repair in Young Infants and Toddlers

mode method, which is significantly higher than in our sessment by echocardiography, there was still no stan-
study.23 In that earlier study, the significant risk factors dard measurement. The jet width, length, and localiza-
for left ventricle dysfunction included operation age tion of regurgitant flow have all been used.32 In recent
(mostly older than 10 years of age), duration of previous studies, they found the branch pulmonary artery diastolic
shunt operation, right ventricle dysfunction, prolonged flow reversal had a good correlation with PR severity by
QRS duration and arrhythmia status. Such discrepancy MRI, which is also shown in our study.31,33 Finally, we
between their study and our study could be explained by had not yet introduced transcatheter pulmonary valve
different measurement methods and a different study implantation; therefore, reintervention arising from pul-
population. In their study, they only used the M mode monary regurgitation was underscored as some eligible
method, which can be biased by paradoxical septal mo- patients for pulmonary valve implantation refused addi-
tion in repaired TOF patients. Besides, we did not enroll tional surgery.
staged shunt operation patients, and most patients in the
earlier study underwent surgery at an older age (even
older than 10 years old) which were all regarded as CONCLUSION
high-risk groups. As none of our patients had left ven-
tricle dysfunction, we may speculate that total TOF re- This study demonstrated that TOF patients repaired
pair before 3 years may carry a low risk of left ventricle from 0 to 6 months of age, though accompanied by a
dysfunction.18 more complicated postoperative course and higher chance
In repaired TOF subjects, moderate to severe degree of transannular patch usage, had long-term outcomes
pulmonary regurgitation is common and may be present comparable to those repaired between 6 months and 3
in more than half of the patients. 4,25 However, the in- years of age. Therefore, symptomatic young infants
fluence of early repair on the PR status in these patients could receive total TOF repair early, to avoid the com-
is still unclear.10,12,13,20 Two mid-term follow-up results plications of shunt operation. But for those to be re-
showed a high percentage of moderate to severe PR paired on an elective basis, total repair should be de-
(more than moderate PR 53.4% and severe PR 22%) in layed until the infant is over six months of age, to avoid
these early repair patients.9,26 However, long-term fol- transannular patch usage and potentially severe PR.
low-up data is limited.14 As the PR severity is often re-
lated to transannular patch usage, and the transannular
patch is more often used in the early group patients, it ACKNOWLEDGMENTS
may explain why PR is a common late problem in early
repaired patients. PR in repaired TOF patients may lead Grant: National Science Council in Taiwan (grant:
to right ventricular dilatation and subsequent ventricular NSC 97-2314-B-002-070).
arrhythmia from electromechanical interaction. 7,8,25-28
Therefore, we suggest that total repair of TOF, on an
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