What is the Optimal Age for Repair of Tetralogy of Fallot?

Glen S. Van Arsdell, MD; Gyaandeo S. Maharaj, MD; Julie Tom, RN, MPh; Vivek K. Rao, MD;
John G. Coles, MD; Robert M. Freedom, MD; William G. Williams, MD; Brian W. McCrindle, MD

Background—Controversy regarding the timing for the repair of tetralogy of Fallot centers around initial palliation versus
primary repair for the symptomatic neonate/young infant and the optimal age for repair of the asymptomatic child. We
changed our approach from one of initial palliation in the infant to one of primary repair around the age of 6 months,
or earlier if clinically indicated. We examined the effects of this change in protocol and age on outcomes.
Methods and Results—The records of 227 consecutive children who had repair of isolated tetralogy of Fallot from January
1993 to June 1998 were reviewed. The median age of repair by year fell from 17 to 8 months (P⬍0.01). The presence
of a palliative shunt at the time of repair decreased from 38% to 0% (P⬍0.01). Mortality (6 deaths, 2.6%) improved with
time (P⫽0.02), with no mortality since the change in protocol (late 1995/early 1996). Multivariate analysis for
physiological outcomes of time to lactate clearance, ventilation hours, and length of stay, but not death, demonstrated
that an age ⬍3 months was independently associated with prolongation of times (P⬍0.03). Each of the deaths occurred
with primary repair at an age ⬎12 months. The best survival and physiological outcomes were achieved with primary
repair in children aged 3 to 11 months.
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Conclusions—On the basis of mortality and physiological outcomes, the optimal age for elective repair of tetralogy of
Fallot is 3 to 11 months of age. (Circulation. 2000;102[suppl III]:III-123-III-129.)
Key Words: tetralogy of Fallot 䡲 survival 䡲 physiology

O pinions regarding the optimal time for the repair of tetral-

ogy of Fallot vary.1–5 Debate continues regarding the use of
initial palliation versus primary repair in symptomatic neonates.
A debate also exists about the timing of repair for the asymp-
tomatic infant.
For many years, the protocol at the Hospital for Sick Children
in Toronto was to repair children with tetralogy of Fallot at an
age of ⬇18 months.1 Infants presenting with cyanosis or spells
were initially palliated with a Blalock-Taussig shunt. In the latter
part of 1995 and early 1996, an institutional shift in protocol was
made to perform the primary repair of tetralogy of Fallot at ⬇6
months of age. Infants who were symptomatic before that age
were repaired primarily.
Data were collected and reviewed for children who were
operated on both before and after the change in protocol. We
evaluated the changes in patient demographics, operative
strategies, and outcomes on the basis of the year of repair. A
separate analysis based on age at repair was performed to
define the optimal age for repair.
disorders were not excluded. We are not aware of any exclusion of
children with tetralogy of Fallot from operative therapy. Repair of
tetralogy of Fallot, in its isolated form was identified in 227 consecutive
children. These children are the subject of this report. Hospital records,
clinic records, and computer database echocardiographic reports were
reviewed. The data reviewed is listed in Table 1.
Statistical Methods
Data are described as frequencies, medians with ranges, and
means⫾SD, as appropriate. Where data are missing, the number of
non-missing values is given. Trends over time (by year) were sought
with Mantel-Haenszel ␹2, Fisher’s exact test, and Pearson and
Spearman correlation coefficients. Patients were divided into 3
groups by age of repair. Clinical experience suggested that outcomes
for either morbidity or mortality were less favorable in the very
young and in children ⬎1 year old. For morbidity and mortality, age
at repair was initially tested as a continuous variable, with significant
associations noted. However, this analysis seemed to oversimplify
the relationships with age. Therefore, we divided the variable age
into 3 logical categories. Thus, we could define more complex
associations, particularly relationships with greater morbidity or
mortality at one or both extremes of age. The 3 groups were
compared using ␹2, ANOVA, and Kruskal Wallis ANOVA.

Methods Results
The Hospital for Sick Children in Toronto, Division of Cardiovascular
Surgery computer database was searched for all cases of tetralogy of Cohort Description
Fallot between January 1993 and June 1998. Those patients with
pulmonary atresia, absent pulmonary valve syndrome, or an atrioven- Preoperative Characteristics
tricular septal defect were excluded (those with an atrioventricular septal There were 130 male children (57%). The median age of
defect were recently reported).6 Children with other metabolic or genetic repair was 14 months (range, 8 days to 9.6 years), the mean

From the Divisions of Cardiac Surgery and Cardiology (R.M.F., B.W.M.), The Hospital for Sick Children, Toronto, and the Departments of Surgery
and Pediatrics (R.M.F., B.W.M.), the University of Toronto, Toronto, Canada.
Correspondence to Dr Glen Van Arsdell, Suite 1525, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8. E-mail glen.vanarsdell@sickkids.on.ca
© 2000 American Heart Association, Inc.
Circulation is available at http://www.circulationaha.org

III-123
 III-124 Circulation November 7, 2000

TABLE 1. Data Reviewed TABLE 1. Continued

Demographics and preoperative data Postoperative parameters
Age Presence of junctional ectopic tachycardia
Weight Central venous pressure
Body surface area Serum arterial lactates
Sex Ventilation time
Associated lesions Intensive Care Unit days
Medications Ascites/peritoneal drain
Previous operations Mortality
Ventilator time Hospital days
Intensive Care Unit days Discharge echocardiography
Hospital days Right ventricular outflow tract gradient
Preoperative catheterization Tricuspid insufficiency grade
Left pulmonary artery size Pulmonary insufficiency grade
Right pulmonary artery size Right ventricular function grade
Main pulmonary artery size Left ventricular function grade
Pulmonary annulus size Ventricular septal defect leaks
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Aortic annulus size Effusion

Descending aortic size Date of chest tube removal
Nakata index Follow-up interventions
McGoon index
Echocardiographic data weight was 9.4⫾4.5 kg (n⫽226), and the mean body surface
Right ventricular outflow tract gradient area was 0.44⫾0.12 m2 (n⫽226). The mean preoperative
Saturation hemoglobin level was 14.0⫾2.1 mg/dL (n⫽208), and the
Hemoglobin mean preoperative arterial saturation was 85⫾11% (n⫽204).
Tetralogy spells Previous procedures were performed in 28% of the children
Operative characteristics (62 of 222); they included 49 systemic to pulmonary artery
Surgeon shunts, 13 balloon dilations of the pulmonary outflow tract or
Cardiopulmonary bypass time pulmonary arteries, 1 repair of an aortopulmonary window,
Cross-clamp time
and 1 right ventricular outflow patch with pulmonary arterio-
plasty (some children had ⬎1 procedure). An anterior de-
Repair type (transventricular or transatrial)
scending coronary artery crossing the right ventricular out-
Use of transannular or outflow patch
flow tract or an accessory anterior descending coronary artery
Pulmonary arterioplasty
was identified in 15 patients (7%).
Use of a monocusp and type of monocusp
Use of post-bypass modified ultrafiltration Operative Characteristics
Use of atrial fenestration
Mean total cardiopulmonary bypass time for repair was
126⫾44 minutes (n⫽225), and the mean cross-clamp time
Post-repair hemodynamics
was 50⫾17 minutes (n⫽225). A transventricular closure of
Right ventricular systolic pressure
the ventricular septal defect (transventricular repair) was
Right atrial systolic pressure
performed in 108 patients, and a transatrial closure of the
Right atrial saturation ventricular septal defect (transatrial repair) was performed in
Pulmonary artery saturation 112 patients (n⫽220). In those having a transatrial repair, 70
Central venous/right atrial pressure (63%) had a small infundibular patch placed as well. A
Reoperation transannular patch was used in 131 children (59%, n⫽221),
Use of intraoperative transesophageal echocardiography of which 91 had placement of a monocusp valve (n⫽220).
Transesophageal echocardiographic estimated right ventricular The type of monocusp that was predominantly used (nearly
outflow gradient 70%) is described by Gundry et al.7
Pulmonary valve competence Branch pulmonary arterioplasty was performed in relation
Tricuspid valve competence to a previous shunt to the pulmonary artery in 16 children
Ventricular septal defect patch leak (33% of 49 total shunts). In those children having an
Right ventricular function abnormal anterior descending coronary system (n⫽15), the
Left ventricular function
repair was performed transatrially in all but 2. Despite the
aberrant or accessory anterior descending coronary artery, a
transannular patch was placed in 53% of these children (8 of
 Van Arsdell et al
Figure 1. Median age trends by year of repair. TOF indicates
tetralogy of Fallot.
the 15). No conduits were used in association with coronary
anomalies (there was 1 death in this subset).
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The mean right ventricular systolic pressure after repair
was 37⫾10 mm Hg (n⫽203), the mean pulmonary artery
systolic pressure was 26⫾10 mm Hg (n⫽177), and the mean
systolic systemic pressure was 76⫾11 mm Hg (n⫽197).
Central venous pressures measured in the operating room
after repair were 10⫾3 mm Hg (n⫽142). Modified ultrafil-
tration after repair was performed in 90% of the children (185
of 205).
Postoperative Characteristics
The median length of stay in the intensive care unit was 4
days (range, 1 to 103 days; n⫽211). The median time to
extubation was 26 hours (range, 2 hours to 101 days; n⫽213).
Time to normalized serum lactate ranged from 0 hours to 9
days (median, 6 hours; n⫽148) after arrival in the intensive
care unit.
Reoperation for residual lesions was performed in 6 chil-
dren (3%); 2 had residual ventricular septal defects, and the
others had right pulmonary arterioplasty, superior vena cava
stenosis, atrial septal defect closure, and conversion from a
valve sparing procedure to a transannular patch. Junctional
ectopic tachycardia was noted in 7% (14 of 214). Death
before hospital discharge occurred in 6 children (2.6%). Each
of these deaths occurred in a child receiving a transannular
patch, and each occurred before the change in protocol in the
beginning of 1996. None of the deaths occurred in a child
with a previous palliative shunt.
Characteristics and Results by Year of Repair
The median age of repair fell from 17 months in 1993 to 8
months in 1998 (Figure 1). The number of children having a
previous systemic to pulmonary artery shunt fell from 38%
(n⫽32) to 0% (n⫽24) for 1993 and 1998, respectively (Table
2). Preoperative use of ␤-blockers increased from 18% in
1993 to 42% in 1998 (Table 2). Hospital mortality also
significantly decreased between 1993 and 1998 (Figure 2).
Operative technique changed from primarily a transven-
tricular approach to transatrial repair (Table 2). Other trends
by year (detailed in Table 2) included a diminished use of a
Optimal Age for Repair of Tetralogy of Fallot III-125
monocusp transannular patch with later year of repair and
increased use of post-bypass modified ultrafiltration. No
change occurred in the use of a transannular patch over time.
Cardiopulmonary bypass and cross-clamp times increased.
A comparison of median cumulative hospital stays for
infants ⬍3 months of age who had a systemic to pulmonary
artery shunt followed by later repair and for infants ⬍3
months of age who had primary repair showed a trend toward
less total hospital time in the primary repair group. This did
not reach significance (shunt and subsequent repair: median,
32 days; range, 21 to 63 days; primary repair: median, 21
days; range, 6 to 112 days; P⫽0.06).
Physiological Outcomes by Year of Repair
The median time to a normal lactate level fell from 19 hours
to 3 hours between 1993 to 1998 (Table 2). Additionally, the
median time to extubation decreased from 43 to 16 hours. By
year of repair, no change occurred in right ventricular outflow
gradient, as measured by echocardiography at the time of
discharge. No change occurred in the mean postoperative
right ventricular pressure by intraoperative direct catheter or
echocardiographic measurement. The mean right ventricular
to systolic pressure ratio stayed constant over the 5 years.
Details of the above findings are shown in Table 2.
Results by Age at Repair
To determine the distribution of morbidity and mortality in
the entire database, age at repair was divided into the
following 3 groups: ⬍3 months, 3 to 11 months, and ⬎12
months. These data were analyzed for outcomes.
Time to serum lactate normalization was least in children
⬍3 months of age and greatest for those ⬎12 months of age.
Details of age-related data are shown in Table 3. Those
children ⬍3 months of age at repair had substantially more
ascites and a greater use of a peritoneal drain. The median
time in the intensive care unit and median time to extubation
were longest for those children ⬍3 months of age and
equivalent for those aged 3 to 11 months and those aged ⬎12
months (Figure 3). Hospital deaths were significantly higher
in those children ⬎12 months of age compared with those ⬍3
months and those aged 3 to 11 months (P⫽0.02; Figure 4).
Transannular Patches
Children receiving a transannular patch had a longer median
time to extubation (patch: median, 29 hours; range, 2 hours to
101 days; n⫽123; no patch: median, 23 hours; range, 3 hours
to 71 days; n⫽85; P⫽0.04) and a longer time to discharge
from the intensive care unit (patch: median, 4 days; range, 1
to 103 days; n⫽121; no patch: median, 3 days; range, 1 to 72
days; n⫽85; P⬍0.01) when compared with those who did not
have a transannular patch. A tendency existed toward having
a poorer grade of right ventricular function, as measured by
echocardiography at hospital discharge, if a transannular
patch had been placed (patch: good function, 87; mild
decrease, 15; moderate decrease, 3; poor function, 1; n⫽106;
no patch: good function, 58; mild decrease, 3; moderate
decrease, 1; poor function, 0; n⫽62; P⫽0.06). Transannular
patches were associated with more pulmonary insufficiency,
as determined by echocardiography at discharge (patch: trace
 III-126 Circulation November 7, 2000

TABLE 2. Other Trends by Year of Repair

1993 1994 1995 1996 1997 1998

Value n Value n Value n Value n Value n Value n P

Preoperative data
␤-Blocker 18% 33 33% 46 29% 35 40% 48 49% 41 42% 24 ⬍0.01*
BT shunt present at 37% 32 24% 46 38% 34 18% 45 10% 41 0% 24 ⬍0.01*
complete repair
Operative data
Transatrial repair 34% 29 33% 45 41% 34 43% 47 78% 41 88% 24 ⬍0.01*
Transannular patch 58% 31 56% 45 73% 34 71% 48 53% 40 35% 23 0.1*
Creation of monocusp 27% 30 46% 46 67% 33 61% 46 27% 41 4% 24 0.05*
Modified ultrafiltration 65% 23 91% 44 97% 30 87% 47 98% 40 100% 21 ⬍0.01*
post-bypass
Mean cardiopulmonary 128⫾49 33 107⫾27 45 111⫾24 34 137⫾58 48 146⫾46 41 126⫾33 24 ⬍0.01
bypass time, min
Mean cross-clamp time, 51⫾23 33 42⫾10 45 42⫾14 34 49⫾16 48 61⫾15 41 57⫾10 24 ⬍0.01
min
Mean post-repair RV 37⫾7 30 37⫾11 41 37⫾11 31 36⫾9 40 36⫾10 40 39⫾10 21 0.76
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pressure, mm Hg
Mean RV/systemic 0.56⫾0.40 28 0.50⫾.12 38 0.47⫾0.12 30 0.48⫾0.12 37 0.47⫾0.11 39 0.50⫾0.12 19 0.22
systolic ratio
Postoperative data
Junctional ectopic 4% 28 7% 142 9% 34 8% 48 8% 40 0% 22 0.80*
tachycardia
Median time to 43 (11–961) 28 35 (7–616) 40 52 (10–2430) 52 21 (2–306) 48 20 (2–2254) 40 16 (7–215) 23 ⬍0.01
extubation, h
Median time to normalize 19 (5–207) 5 8 (0–130) 25 11 (0–64) 26 6 (0–48) 37 3 (0–54) 35 3 (0–9) 20 ⬍0.01
lactate, h
Median echocardiographic 10 (0–40) 22 17 (0–102) 27 20 (10–61) 19 17 (5–46) 31 17 (8–55) 19 20 (0–89) 11 0.19
RVOT gradient at discharge
Data are mean⫾SD, median (range), or percent. n indicates No. of patients. BT indicates Blalock-Taussig; RV, right ventricular; and RVOT, right ventricular outflow
tract.
*Mantel Haenszel ␹2; other P values are Spearman and Pearson correlation coefficients.

insufficiency, 2; mild, 17; moderate, 15; severe, 43; n⫽77; no
patch: trace insufficiency, 2; mild, 15; moderate, 15; severe,
15; n⫽47; P⫽0.01); however, a monocusp valve lessened the
grade of insufficiency (median grade insufficiency with a
monocusp valve was mild; range, none to severe; n⫽50;
P⫽0.01).
Transatrial Versus Transpulmonary Repair
More transatrial repairs were performed in recent years.
When compared with transventricular repairs, transatrial
repairs had longer cross-clamp times (transatrial: median, 56
minutes; range, 27 to 157 minutes; n⫽111; transventricular:
median, 41 minutes; range, 22 to 91 minutes; n⫽108;
P⬍0.01), a lower percentage of transannular patching (43%
versus 57% in transventricular repair; both n⫽108; P⫽0.01),
a shorter time to extubation (transatrial: median, 21 hours;
range, 2 hours to 94 days; n⫽106; transventricular: median,
33 hours; range, 3 hours to 101 days; n⫽96; P⬍0.01), and
fewer days in intensive care (transatrial: median, 3 days;
range, 1 to 103 days; n⫽105; transventricular: median, 4
days; range, 2 to 72 days; n⫽95; P⬍0.01). Right ventricular
function at hospital discharge was not different between the 2
groups (transatrial: good function, 69; mild decrease, 5;
moderate decrease, 2; poor function, 1; n⫽77; transventric-
ular: good function, 71; mild decrease, 13; moderate de-
crease, 2; poor function, 0; n⫽86; P⫽0.06).

Independent Factors Associated With Outcomes

Figure 2. Hospital deaths by year of repair. The shaded area The independent factors associated with the outcomes of time
indicates a policy change to primary repair. to normal serum lactate level, time to extubation, and time to
 Van Arsdell et al Optimal Age for Repair of Tetralogy of Fallot III-127

TABLE 3. Trends and Outcomes by Age of Repair

Age 3–11
Age ⬍3 Months Months Age ⱖ12 Months

Value n Value n Value n P

Preoperative data
␤-Blocker use 35% 17 43% 75 31% 135 0.02
Room air saturation, O2 76⫾12% 14 86⫾9% 67 85⫾11% 123 ⬍0.01
Hemoglobin, mg/dL 13.4⫾2.7 17 13.6⫾1.7 65 14.3⫾2.2 126 0.04
Operative data
Transatrial repair 59% 17 62% 73 44% 130 ⬍0.03
Atrial fenestration 59% 17 13% 75 4% 135 ⬍0.01
Mean systolic RV pressure, mm Hg 33⫾7 14 35⫾8 67 38⫾10 122 0.09
Postoperative data
Junctional ectopic tachycardia 6% 17 7% 69 6% 128 1.0
Time to normalize serum lactate, h 3 (0–130) 15 4 (0–25) 56 7 (0–207) 77 ⬍0.01
Clinically significant ascites or 47% 17 10% 71 6% 127 ⬍0.01
peritoneal drainage
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Data are mean⫾SD, median (range), or %. RV indicates right ventricular.

hospital discharge were determined with general linear re- Discussion

gression modeling. There were an insufficient number of
deaths to include this as an outcome. Variables tested in the
models were preoperative use of ␤-blockers, preoperative
oxygen saturation, preoperative hemoglobin, presence of a
palliative shunt, date of operation, age at repair (⬍3 months,
3 to 11 months, and ⬎12 months), operative cross-clamp and
cardiopulmonary bypass times, transatrial versus transven-
tricular repair, use of a transannular patch, and the presence of
postoperative junctional ectopic tachycardia. Age ⬍3 months
and earlier year of operation were independent factors asso-
ciated with prolonged time to lactate clearance. Independent
factors associated with longer intubation were age ⬍3
months, higher preoperative hemoglobin, the occurrence of
junctional ectopic tachycardia, and a shorter cross-clamp
time. Independent factors associated with increased total
hospital days were age ⬍3 months, lower preoperative
oxygen saturation, junctional ectopic tachycardia, and the
presence of a palliative shunt at the time of definitive repair.
The details of this analysis are shown in Table 4.
One of the problems with analyzing data on the repair of
tetralogy of Fallot is that many studies span large periods of
time.1,2,4 Therefore, advances in multiple areas of treatment
are reflected in the outcomes. The substantial changes in
treatment protocol at this institution regarding both the timing
of repair and a move to primary repair provide an opportunity
to study the effects on outcomes over broad age ranges in a
relatively short time frame (5.5 years). This is a unique
opportunity to analyze age-related outcomes with a minimal
era effect.
Year of Repair
In this cohort, median age of repair decreased with later year
of repair and there were fewer previously present systemic to
pulmonary artery shunts. The use of a transatrial rather than
a transventricular repair increased over time. The shift to
more transatrial repairs was a natural evolution and not a
planned change. Univariate analysis demonstrated an im-
provement in physiological outcome measurements with the
use of transatrial repair. When year of repair was controlled in
the multivariate model, transatrial repair was not an indepen-

Figure 3. Median time to cessation of mechanical ventilation

and discharge from the intensive care unit (ICU) by age. Figure 4. Hospital deaths by age at repair.
 III-128 Circulation November 7, 2000

TABLE 4. Independent Factors Associated With Outcome early. These infants still had prolonged intubation times when
Parameter Estimate P
compared with those aged 3 to 11 months and ⬎12 months.
We interpret this as an indicator of greater physiological
Time to lactate normalization
stress. In the multivariate model, age ⬍3 months was an
Age independent risk factor associated with a prolonged time to
⬍3 months 13.6 0.03 normalization of serum lactate, time to extubation, and length
3–11 months ⫺2.7 0.51 of hospital stay. Nevertheless, outcomes in this group were
⬎12 months 0 䡠䡠䡠 good (no mortality; n⫽17). The caveat to this finding is that
Later date of repair (per day) ⫺0.02 ⬍0.01 if one makes surgical decisions on the basis of probabilities,
R2⫽0.17 P⬍0.01 Root mean square error⫽20.9 Intercept⫽244.0
one must accept that with greater numbers, a mortality
difference is possible because of the duration of illness.
Time to extubation, h
Mitigation of this possibility might occur with the develop-
Age
ment of greater institutional expertise with increasing cumu-
⬍3 months 442.0 ⬍0.01 lative experience.
3–11 months ⫺2.3 0.96 The lower age limit for better tolerance of repair may be
⬎12 months 0 䡠䡠䡠 ⬍3 months. However, because the number of patients
Higher preoperative hemoglobin (mg/dL) 3.0 ⬍0.01 younger than 3 months of age is small, a meaningful
Junctional ectopic tachycardia assessment of age at repair of ⬍28 days, 28 to 60 days, and
No ⫺63.0 0.04
61 to 90 days is not possible. It could be that the natural
physiological change in tolerance is 2 months or some other
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Yes ⫺0 䡠䡠䡠 time. However, the data are clear that very young infants
Shorter operative cross-clamp time (min) ⫺2.7 0.03
tolerate the procedure less well than older infants.
R2⫽0.24 P⬍0.01 Root mean square error⫽261.2 Intercept⫽⫺77.0 Although we have changed to primary repair of symptom-
Hospital days atic neonates rather than initial palliation followed by repair
Age before 1 year of age, the data from this cohort is insufficient
⬍3 months 15.9 ⬍0.01 to document if this is the best course of action. Combined
3–11 months ⫺0.61 0.75 hospital and intensive care unit days (a reflector of cumula-
⬎12 months 0
tive cost) for systemic to pulmonary artery shunt followed by
䡠䡠䡠
later repair was nearly significantly greater (P⫽0.06) than
Junctional ectopic tachycardia
primary repair alone in a child ⬍3 months. With a few more
No ⫺9.6 ⬍0.01
patients in each arm, this number would likely have achieved
Yes 0 䡠䡠䡠 significance. Ungerleider et al8 previously outlined this con-
Presence of palliative shunt cept in a small cohort.
No ⫺5.1 0.02 We abandoned the routine use of a Blalock-Taussig shunt
Yes 0 䡠䡠䡠 in the symptomatic infant with tetralogy of Fallot because of
Preoperative oxygen saturation (%) ⫺0.2 0.05 the previously documented morbidity and interval mortality
R2⫽0.20 P⬍0.01 Root mean square error⫽11.9 Intercept⫽40.5
reported by this institution9 and others.10,11 We did not
identify anatomic criteria, such as an anomalous anterior
descending coronary artery crossing the right ventricular
dent factor for improved physiological outcome. Interest- outflow tract or small pulmonary arteries, in the present
ingly, in the multivariate model, a shorter cross-clamp time cohort that would have precluded primary repair. It is
was a risk factor for longer intubation time. Shorter cross- possible that specific contraindications to primary repair
clamp times were associated with earlier year of repair and exist, such as the comorbidities of cerebral hemorrhage or
transventricular repairs. The increased preoperative use of infection.
␤-blockers in the later years of the study was not a univariate Not all reports have shown important problems with
or independent risk factor for measured outcomes. In general, neonatal shunts in a biventricular setting.4 Outstanding out-
␤-blockers were used to stabilize a child for elective repair. comes have also been achieved by performing a shunt for
Because we had few deaths, a multivariate analysis could not symptoms in early infancy and following this with complete
be done for mortality. A significant improvement in survival repair within the next year or so.4 A multi-institutional
with later year of repair and the improvement in physiological observational study based on institutional practice (such as
parameter measurements, such as serum arterial lactate and those done by the Congenital Heart Surgeons Society) would
duration of intubation, over time suggest a better physiolog- be helpful to answer this question. Experience at this institu-
ical tolerance of the procedure with the new protocol. tion with balloon dilation of the right ventricular outflow tract
for symptomatic tetralogy of Fallot in early infancy has not
Age of Repair been gratifying because of a failure to reliably relieve
When screening the data for tolerance to the procedure, it is cyanosis. We no longer use this approach except under
clear that age is important. Most infants who had repairs unusual circumstances.
performed at ⬍3 months of age were operated on during the As a group, infants aged 3 to 11 months had the most rapid
later years, when there was a concerted effort to extubate recovery from operative therapy, as measured by reflectors of
 Van Arsdell et al
physiological tolerance, when compared with those at other
ages. No deaths occurred in this age group. We think this
indicates the greatest physiological tolerance to repair and,
therefore, the optimal age for repair.
The group of children ⬎12 months at the time of repair had
the least favorable outcomes (6 deaths in 135 treated; 4.4%
mortality; P⫽0.02). Despite the fact that each of these deaths
occurred in a child who did not have a previous shunt, the
multivariate model showed previous palliation to be a risk for
longer hospitalization. We speculate that the volume-loading
provided by the systemic to pulmonary artery shunt protects
against the unfavorable right ventricular diastolic stiffness
found in some older children but may unfavorably affect
systolic function.
Transannular Patch
Patients receiving a transannular patch tolerated the repair
less well. Each of the deaths occurred in a child who received
a transannular patch. In the multivariate model; however, a
transannular patch was not a risk factor for prolonged time to
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lactate clearance, extubation, or hospital stay. Preservation of
the pulmonary annulus in tetralogy of Fallot has been
reported as ⬎80% by Yasui et al12 when a concerted effort
toward preservation was made. Improved outcomes without a
transannular patch by univariate analysis and the fact that
surgeon variance seems to exist in the use of a transannular
patch suggest that efforts to preserve the pulmonary annulus
and valve are favorable in the short-term.
Transatrial Transpulmonary Repair Versus
Transventricular Repair
The favorable effects on intensive care and ventilation time
seen in the transatrial repair patients are probably accounted
for by the fact that most of the transatrial repairs occurred in
infants aged 3 to 11 months and were performed in more
recent years. A suggestion of a better grade of right ventric-
ular function on the predischarge echocardiogram existed in
the transatrial repair group (P⫽0.06). Stellin et al13 and Miura
and colleagues14 had similar findings of ventricular function
with transatrial repair.
Conclusions
Substantial changes in the treatment protocol for tetralogy of
Fallot were made. These changes are characterized by a shift
Optimal Age for Repair of Tetralogy of Fallot III-129
to primary repair at a younger age and an increasing percent-
age of transatrial repairs. Outcomes improved with the
change in strategy. By multivariate analysis, infants ⬍3
months of age had statistically significant evidence of greater
physiological stress but not mortality. The unfavorable out-
comes seen in this study occurred in children aged ⬎12
months at primary repair. On the basis of reflectors of
physiological tolerance and mortality, the optimal age for
elective repair of tetralogy of Fallot is 3 to 11 months.
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 What is the Optimal Age for Repair of Tetralogy of Fallot?
Glen S. Van Arsdell, Gyaandeo S. Maharaj, Julie Tom, Vivek K. Rao, John G. Coles, Robert M.
Freedom, William G. Williams and Brian W. McCrindle

Circulation. 2000;102:Iii-123-Iii-129
doi: 10.1161/01.CIR.102.suppl_3.III-123
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