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Richard A. Jonas
Outcomes for surgical repair of tetralogy of Fallot with pulmonary stenosis are often
considered to be a useful benchmark for the assessment of congenital surgical programs.
This procedure, including preoperative assessment and postoperative management, falls in
the middle of the spectrum of difficulty of all the congenital cardiac procedures. Within this
anomaly itself, there is a wide spectrum of severity and difficulty. However, it is important
to distinguish tetralogy with pulmonary stenosis or “simple tetralogy” from the much more
complex anomaly tetralogy of Fallot with pulmonary atresia. Tetralogy of Fallot with pul-
monary stenosis is virtually always operable. It is also a progressive condition that is
therefore best repaired in early infancy following echocardiographic diagnosis alone.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 12:39-47 © 2009 Published by
Elsevier Inc.
Figure 1 This figure from the study by Shimazaki et al illustrates that the right (A) and left (B) mediastinal pulmonary
arteries are usually close to normal size in tetralogy of Fallot with pulmonary stenosis. As one moves more proximally
the pulmonary trunk (PT), pulmonary valve annulus and RV infundibulum become increasingly small relative to
normal (ie, z score is less). (Reprinted with permission.1)
the underdevelopment of the main pulmonary artery and alignment VSD as well as to allow patch enlargement of the
pulmonary annulus, were well defined in a comprehensive full length of the underdeveloped infundibulum.
radiographic study by Shimizaki et al (Fig. 1).1
One of the important variables in the infundibular ob-
struction of tetralogy is the length of the conal septum. In Coronary Artery Distribution
some forms of tetralogy the conal septum is completely ab- Moving through the spectrum of tetralogy from normality,
sent and there is a small fibrous band only below the pulmo- the aorta moves forward relative to the pulmonary artery and
nary valve. This allows excellent exposure of the VSD the distance that the left main coronary artery must traverse
through a very short ventricular incision. On the other hand, in order to pass posteriorly behind the pulmonary artery
if there is a long conal septum, then generally a longer infun- before bifurcating into the anterior descending and circum-
dibular incision is required both to expose the anterior mal- flex coronary artery increases. Not surprisingly, therefore, a
Early primary repair of tetralogy of Fallot 41
Figure 3 Van Praagh has emphasized that the two principal features of tetralogy of Fallot, namely a VSD and infundib-
ular obstruction, result from underdevelopment of the subpulmonary conus. The infundibular septum no longer sits
between the two divisions of the septal band because it is anteriorly displaced. There is an anterior malalignment VSD
and muscular subpulmonary stenosis. (a) Normal anatomy. (b) Tetralogy of Fallot.
In addition to the multiple developmental advantages for 90% at birth to less than 75% to 80%. This should be an
the child, there are additional important economic and psy- indication to move ahead relatively urgently with surgery.
chosocial advantages for the young family with a child born
with tetralogy of Fallot. It is not difficult to empathize with Cyanotic spells. Cyanotic spells are usually observed in a
young inexperienced parents who are told by their physician child who is initially agitated. This is followed by a period of
that their child has a potentially fatal cardiac condition that cyanosis with an arterial oxygen saturation of less than 20%
can result in sudden cyanotic spells and death if the child is to 30%. In a classic cyanotic spell, the child subsequently
allowed to become agitated. One can only imagine the fear becomes gray, pale, and comatose, presumably related to a
that a single cry at night from this child will cause. One fall in cardiac output secondary to hypoxia. Cyanotic spells
option adopted by many centers is to defer reparative surgery can result in brain injury and death and should be an indica-
beyond 6 to 12 months and to undertake a preliminary shunt tion for immediate hospitalization and surgical repair. In the
if the child becomes symptomatic within that timeframe.11,12 past, both parents and hospital staff were taught maneuvers
The family must then endure a second hospitalization with its to reverse cyanotic spells; although today it should be rare
attendant stresses and costs. It seems infinitely preferable that a child progresses to the point of having recurrent cya-
from the perspective of the family, as well as the cost to notic spells. The child’s agitation should be treated with mor-
society, to undertake single-stage elective repair early in in- phine. Systemic vascular resistance can be increased by plac-
fancy before the development of symptoms. ing the child in a knee-to-chest position. (This is equivalent
to the “squatting” position often adopted by the older, un-
treated child.) Oxygen should also be given. If necessary, the
Specific Indications for Surgery child should be anesthetized, intubated, paralyzed, and ven-
tilated. Systemic resistance can be further increased pharmaco-
Symptoms logically with alpha-adrenergic agents such as phenylepheph-
Prostaglandin-dependent neonate. Occasionally a child will rine (Neo-Synephrine; Bayer Healthcare, Pittsburgh, PA) and
be born with a sufficiently severe degree of fixed right ven- the catecholamine drive exacerbating the spell can be dimin-
tricular outflow tract obstruction that the child is prostag- ished by administration of a beta-blocking agent such as pro-
landin-dependent from birth. In this setting, it is particularly pranolol.
important to exclude the presence of important aortopulmo-
nary collateral vessels as the child is likely to be close to
Elective Repair
having tetralogy of Fallot with pulmonary atresia. Mediasti-
nal continuity of the true pulmonary arteries should also be At present, it is our practice to schedule elective repair fol-
carefully confirmed. lowing diagnosis, which is usually made within a few days of
birth. This allows the parents and caregivers the time to plan
Worsening cyanosis. Right ventricular outflow tract ob- necessary logistics and for the financial aspects of the proce-
struction almost always progressively worsens in the first dure to be put in place, usually over a 4- to 6-week period.
weeks and months of life, usually due to increasing muscular Over this time frame, ductal tissue that may extend into the
outflow tract obstruction, but on occasion because of wors- origin of the left pulmonary artery may declare itself as a
ening valvar stenosis. This may result in the resting oxygen possible stenosis at the origin of the left pulmonary artery.
saturation gradually decreasing from a level of greater than Although there was concern that repair in early infancy re-
Early primary repair of tetralogy of Fallot 43
Figure 4 (a) A ventriculotomy approach is preferred for repair of tetralogy of Fallot when there is moderate or severe
infundibular obstruction. The incision should be extended across the annulus (transannular incision) when the z score
of the pulmonary annulus is smaller than two to three standard deviations below normal. (b) Autologous pericardium
lightly tanned with glutaraldehyde is ideal for use as a right ventricular outflow tract patch. The patch should usually
be pear shaped as indicated. (c) Placement of a transannular pericardial right ventricular outflow tract patch. (d) On
occasion there may be an adequate pulmonary annulus but moderate or severe hypoplasia of the supravalvar main
pulmonary artery. (e) Two separate patches are placed, one in the main pulmonary artery for management of supra-
valvar pulmonary stenosis and one in the infundibulum of the right ventricle for infundibular obstruction.
44 R.A. Jonas
Figure 5 (a) Division of the parietal (free wall) and septal extensions of the conal septum will allow the conal septum to
move posteriorly when the VSD patch is anchored to it. (b) Placement of multiple interrupted pledgetted 5/0 sutures
for closure of the anterior malalignment VSD. In this patient there is considerable muscular separation between the
tricuspid and aortic valves so that sutures can be placed into the muscle ridge in this area. Sutures would usually not
be separated from each other as depicted here. The inset (c) demonstrates that sutures are placed on the right
ventricular aspect of the septum. The muscle bar separates the edge of the VSD from the conduction bundle.
sulted in a higher probability of transannular patch place- has facilitated the performance of repair of tetralogy using
ment this appears in fact not be borne out by experience with bicaval cannulation and continuous cardiopulmonary bypass
elective repair.13 in babies weighing as little as 2 to 2.5 kg. Moderate hypo-
thermia (eg, 25 to 28°C) can be used, although many groups
today employ a mild degree of hypothermia or even normo-
Contraindications to thermia. For the very premature and small child (eg, less than
Early Primary Repair 1.5 to 2 kg), we continue to use a single venous cannula and
There are essentially no contradictions to early primary re- usually a period of deep hypothermic circulatory arrest for
pair, including anomalous anterior descending coronary ar- VSD closure.
tery, multiple VSDs, and discontinuous pulmonary arteries.
In the newborn period, the child with an anomalous coro- Ventricular or Transatrial Approach
nary artery can usually be managed with a transatrial ap- Although either approach is reasonable, the ventricular ap-
proach and placement of outflow patches above or below or proach offers several advantages. The hypoplastic infundib-
both relative to the coronary artery to avoid conduit place- ulum can be enlarged without unduly aggressive resection of
ment. Multiple muscular VSDs can frequently be closed by muscle, which can lead to extensive endocardial scar forma-
direct suture, often using adjacent muscle bundles, particu- tion. The VSD is closed without undue traction on the tricus-
larly the moderator band, which can be folded into the mus- pid annulus, which can cause injury to the tricuspid valve
cular defect using multiple interrupted doubly pledgetted and conduction bundle. However, a number of important
sutures.14 technical considerations must be observed if the ventricular
approach is to be used correctly:
Technique of Repair
1. Decision regarding transannular patch: If the pulmonary
Cardiopulmonary Bypass Setup annulus and the pulmonary artery are more than two to
The development of small, thin-walled, right angle venous three standard deviations below normal, a transannular
cannulas down to 10 Fr caliber (eg, Terumo, Tokyo, Japan) patch is indicated (Fig. 4).
Early primary repair of tetralogy of Fallot 45
Figure 6 (a) It is not uncommon in the patient with tetralogy of Fallot to have virtual fibrous continuity between the
aortic and tricuspid valves. There may be a fibrous rim in this area that may represent a remnant of the membranous
septum. (b) The sutures may be safely placed in the fibrous rim or, alternatively, must be placed some distance below
the VSD edge to avoid injury to the bundle of His. (c) Placement of interrupted pledgetted sutures using the remnant
of the membranous septum. (d) Completed VSD closure using a patch of double velour knitted Dacron.
2. Location of the ventriculotomy: No right ventricular cor- 8. Management of the foramen ovale: It is very important in
onary arteries should be divided, other than the conal the young infant that the foramen ovale should be left
coronary branch of the anterior right ventricular branch of patent in order to allow right to left decompression so
the right coronary artery. as to maintain cardiac output in the postoperative pe-
3. Length of the ventriculotomy should be limited. riod. The mild degree of cyanosis that results is well
4. Division of infundibular muscle bundles should not in- tolerated in early infancy.
clude division of the moderator band (Fig. 5).
5. VSD closure: Tricuspid chordae must be carefully pre-
served (Figs. 5, 6).
Results of Surgery
6. Dimensions of the outflow patch: An autologous pericar- In an important study described by Kirklin et al,13 the results
dial patch treated with 0.6% glutaraldehyde for 20 to of an approach of early primary repair were compared with a
30 minutes should be used. The patch should enlarge two-stage approach. One hundred consecutive patients from
the infundibulum and pulmonary annulus to a normal the University of Alabama were compared with 100 consec-
size for the child’s surface area. utive patients from Children’s Hospital Boston undergoing
7. Avoidance of coronary artery injury caused by suturing: It correction of tetralogy of Fallot between 1984 and 1989. The
is important that suture lines not cause undue tension authors concluded that there was a possible disadvantage of
on the epicardium, which may compress coronary ar- the two-stage approach using preliminary shunting and later
teries. repair. Other excellent results have been described by Karl et
46 R.A. Jonas
al,11 who described a review of 366 patients with a median pulmonary valve replacement in the native right ventricular
age of 15.3 months. There were two hospital deaths for a outflow tract. Currently, such valves are limited to placement
hospital mortality of 0.5%. Hennein et al15 described no hos- in regurgitant conduits.
pital deaths among 30 neonates with symptomatic tetralogy
of Fallot.
The Role of the Monocusp Valve
Long-Term Results Although some centers have expressed enthusiasm for place-
ment of a monocusp valve at the time of initial tetralogy
After Early Primary Repair
repair follow-up, studies have suggested that the function of
A recent review of 49 long-term survivors of early repair of such “valves” is transient at best. For example, Gundry et al20
tetralogy of Fallot with a median follow-up of 23.5 years described 19 patients who had monocusp valves placed and
described an actuarial survival of 85% at 20 years.16 Freedom
were assessed up to 24 months postoperatively. Although 16
from reintervention was 93% at 5 years and 79% at 20 years.
of 19 patients had component monocusp valves immediately
No significant differences were found between patients with
postoperatively, by 24 months only one of seven patients had
and without a transannular patch. Right ventricular outflow
a competent valve. Similar findings were described by Bigras
tract obstruction was the most common indication for late
et al.21
reoperation; 41 of 45 long-term survivors for whom recent
follow-up was available were in NYHA Class 1.
12. Fraser CD, McKenzie ED, Cooley DA: Tetralogy of Fallot: Surgical man- 17. Gladman G, McCrindle BW, Williams WG, et al: The modified Blalock-
agement individualized to the patient. Ann Thorac Surg 71:1556-1561, Taussig shunt: Clinical impact and morbidity in Fallot’s tetralogy in the
2001 current era. J Thorac Cardiovasc Surg 115:25-30, 1997
13. Kirklin JK, Blackstone EH, Jonas RA, et al: Morphologic and surgical 18. Hirsch JC, Mosca RS, Bove EL: Complete repair of tetralogy of Fallot in
determinants of outcome events after repair of tetralogy of Fallot with the neonate: Results in the modern era. Ann Surg 232:508-514, 2000
pulmonary stenosis: A two institution study. J Thorac Cardiovasc Surg 19. Geva T: Indications and timing of pulmonary valve replacement after
103:706-723, 1992 tetralogy of Fallot repair. Semin Thorac Cardiovasc Surg Pediatr Card
14. Jonas RA: Comprehensive Surgical Management of Congenital Heart Surg Ann 9:11-22, 2006
Disease. London; Arnold; 2004, p 251 20. Gundry SR, Razzouk AJ, Boskind JF, et al: Fate of the pericardial mo-
15. Hennein HA, Mosca RS, Urcelay G, et al: Intermediate results after nocusp pulmonary valve for right ventricular outflow tract reconstruc-
complete repair of tetralogy of Fallot in neonates. J Thorac Cardiovasc tion. J Thorac Cardiovasc Surg 107:908-912, 1994
Surg 109:332-344, 1995 21. Bigras JL, Boutin C, McCrindle BW, et al: Short-term effect of mono-
16. Bacha EA, Scheule AM, Zurakowski D, et al: Long-term results after cuspid valves of pulmonary insufficiency and clinical outcome after
early primary repair of tetralogy of Fallot. J Thorac Cardiovasc Surg surgical repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 112:33-
122:154-161, 2001 37, 1996