Repair of Truncus Arteriosus
With Interrupted Aortic Arch
Ralph S. Mosca, MD
T runcus arteriosus (TA) is a conotruncal anomaly in
which a single great vessel arises from the heart and gives
rise to the aorta, coronary arteries, and pulmonary arteries
(PA). It is often seen in concert with a large subarterial ven-
tricular septal defect (VSD).1 The more common associated
anomalies include a patent foramen ovale or atrial septal
defect (90%), coronary ostial or branching variations (30-
50%), right aortic arch (25%), interrupted aortic arch (IAA)
(10-15%), aberrant subclavian artery (5-10%), and persistent
left superior vena cava (5-10%).2
Two major schemes have been commonly used to classify
TA, those of Collett and Edwards3 and those of Van Praagh
and Van Praagh.4 Accepting perforce the presence of a VSD
and IAA, we use the former to describe the PA anatomy (Fig.
1). The system of Celoria and Patten5 is most often used to
describe the IAA anatomy (Fig. 2). The majority of cases of
IAA in association with TA are either type B (85-90%) or type
A (5-9%).6 The truncal valve often has the appearance of a
fused aortic and pulmonary valve and may have 1 to 5 or
more leaflets.7 Significant stenosis is present in about 5% of
cases and moderate or greater regurgitation is present in ap-
proximately 25%.8
Preoperative Care
The pathophysiology of TA is characterized by a total admix-
ture of the pulmonary and systemic circulations (atrial, ven-
tricular, and truncal levels) in the presence of elevated pul-
monary blood flow (PBF). Newborns are typically not in
distress and may appear pink, although the systemic oxygen
saturations are often 85 to 90%. As pulmonary vascular re-
sistance (PVR) decreases, PBF (which occurs in both systole
and diastole) increases, leading to pulmonary overcirculation
and signs of congestive heart failure. The pressure and vol-
ume overload on the lungs is known to lead to early devel-
opment of pulmonary vascular obstructive disease in nonop-
erated patients.9 The systemic and coronary circulations may
suffer from relative hypoperfusion and ischemia may occur.
This can be further exacerbated by significant degrees of
truncal valve insufficiency.
NYU Langone Medical Center, New York, New York.
Address reprint requests to Ralph S. Mosca, MD, NYU Langone Medical
Center, 530 First Avenue, Suite 9V, New York, NY 10016. E-mail:
Ralph.mosca@nyumc.org
1522-2942/$-see front matter © 2010 Elsevier Inc. All rights reserved.
doi:10.1053/j.optechstcvs.2010.07.002
Medical management is limited and focused on stabiliza-
tion of the circulation. Even in the presence of IAA, prosta-
glandin infusions are rarely necessary. Once the diagnosis is
made, surgical repair should be undertaken in the first week
of life. The onset of tachypnea often heralds the fall in PVR
and need for repair.
Operative Technique
The important preoperative anatomic features, aortic arch
anatomy and the specific type of IAA, degree of regurgitation
and/or stenosis of the truncal valve, coronary artery anatomy,
location and number of VSDs, functional status of the tricus-
pid valve, and the size of the atrial septal defect, are well
delineated by echocardiography. The goal of the surgical re-
pair is closure of septal defects, separation of the pulmonary
and systemic circulations, and re-establishing aortic arch
continuity.
At induction and before cardiopulmonary bypass (CPB),
anesthetic care should be aimed at avoiding further pulmonary
overcirculation and possible myocardial ischemia. Thus, ma-
neuvers that tend to increase PBF such as alveolar hyperoxia or
hypocarbia and agents that increase heart rate or myocardial
oxygen demand should be avoided.
Surgical approach is through a median sternotomy. Given the
prevalence of DiGeorge syndrome in patients with TA, the pres-
ence or absence of a thymus should be noted. Any thymic tissue
that obstructs the view can be resected. A portion of pericar-
dium can be harvested if planned for use in the right ventric-
ular (RV) outflow tract reconstruction. A pericardial well is
established. A right atrial purse-string is placed to help retract
the right atrial appendage and through which systemic hep-
arinization is performed. A survey of the anatomy is made to
confirm the diagnosis and locate the pulmonary and coro-
nary arteries. The right PA (RPA) is encircled with a large silk
snare. This will be tightened as CPB begins or earlier if myo-
cardial ischemia becomes an issue. The CPB circuit is pre-
pared for dual arterial and either single atrial or bicaval ve-
nous cannulation. Although it is possible to adequately
perfuse and cool the lower body with a single ascending
arterial cannula, often a second arterial cannula is placed in
the proximal ductus arteriosus to perfuse the lower body.
Typically CPB is established with arterial cannulation in the
distal TA and right atrial venous cannulation. The RPA is
223
224 R.S. Mosca

Figure 1 Collett and Edwards classification scheme for truncus arteriosus.

immediately snared; the left PA (LPA) is then more easily
dissected, encircled, and gently snared. At this point the sec-
ond arterial cannula can be placed to optimally perfuse the
lower body (Fig. 3A). The CPB perfusate temperature is then
gradually lowered to cool the patient for at least 20 minutes to
a nasopharyngeal and rectal temperature of 18 to 20°C. So-
dium nitroprusside (1-5 ␮g/kg/min) and Isoforane
(0.5-2.0%) are utilized during cooling and titrated according
to the patient’s blood pressure. The alpha stat blood gas man-
agement technique is employed throughout the bypass pe-
riod. The head is packed in ice to optimize cooling. On oc-
casion, a left-sided vent placed through the right superior
pulmonary vein may help alleviate left heart distension that
may occur as a result of truncal valve insufficiency.
The TA, brachiocephalic vessels, PA, ductus arteriosus,
and descending thoracic aorta are widely mobilized to con-
firm the anatomy and allow for the greatest visualization and
manipulation during the repair. Exposure to the brachioce-
phalic vessels can be improved with either a superior skin
stitch or looping and gentle retraction of the inominate vein.
Care is taken to avoid injury to the left recurrent laryngeal
nerve and phrenic nerve. If present, an aberrant right subcla-
vian artery may need to be divided to mobilize the descend-
ing thoracic aorta. Once again the site of origin of both the left

Figure 2 Classification of interrupted aortic arch as described by Celoria and Patton. Type A, at aortic isthmus; type B,
between left common carotid artery and left subclavian artery; type C, between inominate artery and left common
carotid artery. Asc. ao. ⫽ ascending aorta; Desc. ao. ⫽ descending aorta; IA ⫽ innominate artery; LCC ⫽ left common
carotid artery; LSCA ⫽ left subclavian artery.
Repair of TA with IAA 225

Figure 3 (A) Appearance of truncus arteriosus type 2.

Cannulation of the ascending aorta and the proximal
ductus arteriosus are shown. Pulmonary artery snares
are tightened on commencing CPB and cooling is begun.
(B) The truncus, brachiocephalic vessels, PDA, and de-
scending aorta are widely mobilized. This can be facili-
tated by removal of the arterial cannula in the PDA after
sufficient cooling. (C) The arterial cannula is removed;
the head vessels snares are tightened and antegrade car-
dioplegia is given. The proximal tie on the PDA and the
LPA snare are retained and can be used to improve ex-
posure to the aortic arch and descending aorta. (C-E) A
transverse incision is made anteriorly over the pulmo-
nary arteries and extended both superiorly and inferi-
orly, removing the pulmonary artery branches from the
truncus. Care must be taken to avoid injury to the cor-
onary ostia and the aortic valve commissures. Ao. ⫽
aorta; LSCA ⫽ left subclavian artery; RA ⫽ right artery;
RV ⫽ right ventricle; SVC ⫽ superior vena cava.
226 R.S. Mosca
Repair of TA with IAA 227

and the right PA from the truncal root is confirmed. Snares
are placed around the brachiocephalic vessels for use after
arrest.
Before myocardial and/or hypothermic circulatory arrest,
the conduct of the operation should be determined. In gen-
eral it can be thought of as separate yet sequential steps: (1)
inspection and removal of the PA from the truncal root; (2)
repair of the aortic arch interruption; (3) VSD closure; (4)
distal RV-PA conduit connection; (5) proximal RV-PA con-
duit connection. If a homograft is to be used for the RV-PA
connection, it should be chosen and thawed at the beginning
of the operative procedure.
After 20 minutes of cooling, the systemic flow is reduced to
one fourth of full flow. In addition, if further mobilization of
the descending aorta is needed, the arterial cannula in the
patent ductus arteriosus (PDA) can be removed, improving
visualization. The PDA is doubly tied and divided. The prox-
imal tie can be used for retraction during the arch repair.
Preparations are made for giving cardioplegia and initiating a
period of circulatory arrest (Fig. 3B). The arterial pump head
is stopped and the patient is exsanguinated into the pump
oxygenator (Fig. 3C). The head vessels are snared and
through a side port on the arterial cannula, 15 mL/kg of
cardioplegia (500 mL Normasol-R, 25 mEq potassium chlo-
ride, and 25 mEq sodium bicarbonate) is delivered in a 1:1
ratio with blood at approximately 4°C. The heart is inspected
to ensure that the left ventricle does not distend in the pres-
ence of an often incompetent truncal valve. Topical ice slush-
saline is used for further protection. Once completed, the
arterial cannula is removed. The snare on the RPA is re-
moved. The loop on the LPA can be removed or retracted
inferiorly if it helps with exposure.
The truncal root is transected just above (2 mm) the origin
of the PA (Fig. 3C-E). This helps visualize the PAs as well as
the truncal valve commissures and leaflets and coronary ar-
tery orifices. The left coronary artery is often intimately asso-
ciated with the inferior aspect of the pulmonary artery con-
fluence and must not be injured. The PA are thus removed
with a portion of truncal wall and then mobilized from
right to left hilum. This will facilitate the distal RV-PA
homograft connection and allow the PA conduit and PA
bifurcation to sit to the left of the ascending aorta (Fig.
4A).
Next the IAA, typically type B, is repaired. Division of the
truncal root and wide mobilization of the head vessels and
descending aorta facilitate this step. On occasion, the left
subclavian artery originates quite distally and can tether the
descending aorta down in the mediastinum. If mobilization
of this vessel does not prove adequate, it can be ligated and
divided. In type B aortic arch interruption, the left common
carotid artery heads straight up into the neck and there is no
“transverse aortic arch.” Two techniques can be employed to
fix the arch. If the gap between the proximal and distal ends
is not extreme and can be brought together with little or no
tension, incisions can be made in the arch and left common
carotid artery with counterincisions in the descending aorta
and up the LSCA and a primary end-to-end anastomosis
performed. Great care must be taken to ensure that these
lumens will be adequate, under little or no tension, and that
this does not compromise the aortopulmonary window, risk-
ing impingement on the RPA or left mainstem bronchus.
More often the back wall can be repaired primarily and a
patch fashioned to augment the undersurface of the aorta,
reducing any tension and allowing it to be tailored in width
and length to the proximal aortic root (Fig. 4B). The proximal
ascending aorta is then sewn end to end to the newly recon-
structed arch (Fig. 4C). The cross-clamp can be reapplied
and CPB resumed at this point.
The VSD is then approached through a longitudinal ven-
triculotomy. Fine silk stay sutures are used to bring the RV
toward the surgeon and delineate the lateral dimensions of
the incision (Fig. 4D). Care must be taken to avoid injury to
the left anterior descending coronary artery either by the
incision, by retraction on the edges of the ventriculotomy, or
with the sutures repairing the defect later. In addition, the
ventriculotomy incision should be started at a point inferior
and leftward of where the aortic valve will be located. Once
inside the RV, any abnormal parietal attachments are divided
or resected and the VSD margins are identified (Fig. 5A). The
VSD is most often outlet and muscular, making the conduc-
tion system somewhat remote. However, it can extend into
the membranous septum. The VSD is then closed using a
polytetrafluoroethylene (PTFE) patch. The patch is oversized
slightly and sewn to the RV side of the septum. Along the
aortic valve, stitches are placed close to the annulus to avoid
residual ventricular level shunting through trabeculations.
Along the superior edge the patch is sewn to the edge of the
ventriculotomy (Fig. 5B). This helps to enlarge the left ven-
tricular outflow tract and avoid injury to the “aortic” valve.
Normally, a patent foramen ovale is left to allow for periop-
erative shunting at the atrial level.
The RV-PA conduit is now constructed. The cross-clamp
can be removed; the myocardium can be inspected and sys-
temic rewarming can begin. A number of different conduits
can be utilized to re-establish RV-PA continuity including
aortic or pulmonary homografts, bovine jugular vein, or syn-
thetic valved or nonvalved conduits. In general, an aortic

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Figure 4 (A) Anatomy is shown after division of the truncal root and removal of the pulmonary artery branches. The
dotted lines indicate arteriotomies for repair of the ascending aorta and arch. (B) Exposure to the descending aorta can
be facilitated by the use of a “Spoon Potts” type clamp. The back wall is repaired after excising all residual ductal tissue.
A portion of pulmonary allograft tissue is thawed and trimmed to augment the aorta. (C) The reconstructed ascending
aorta is reanastomosed to the proximal aortic root. (D) The VSD is exposed via a right ventriculotomy. It is directed to
the left of the aorta. The truncal valve extends somewhat inferiorly and care must be taken to avoid injuring it at the
superior end of the incision. Silk stay sutures improve the exposure. MPA ⫽ main pulmonary artery; PA ⫽ pulmonary
artery; PDA ⫽ patent ductus arteriosus; RV ⫽ right ventricle.
228 R.S. Mosca

Figure 5 (A) View of the outlet VSD via the right ventriculotomy. Here the VSD is shown as outlet muscular in nature;
thus, the conduction system is remote from the defect. (B) Closure of the VSD with a PTFE patch. Superomedially the
patch is sutured close to the aortic valve annulus to prevent residual VSDs through trabeculations. Superiorly the patch
is sewn to the edge of the right ventriculotomy. (C) Distal homograft to PA bifurcation anastomosis. Segments of
running polypropylene are used to prevent a “purse-string” distortion. (D) The posterior aspect of the proximal
homograft to RV anastomosis. Approximately one third of the circumference of the homograft is sutured to the superior
aspect of the RV incision. (E) The repair is completed with a hood of glutaraldehyde-treated pericardium or PTFE. The
dimensions for the patch are shown in (D). VSD ⫽ ventricular septal defect.
Repair of TA with IAA 229

Figure 5 (Continued)
230
homograft (10-12 mm) works well in small neonates. The
distal portion of the conduit is trimmed and beveled to ac-
commodate the pulmonary artery confluence and orientated
in such a way to sit well on the right ventriculotomy. The
proximal muscular cuff is freshened up and shortened as
necessary. The distal anastomosis is performed with seg-
ments of running suture with care to avoid purse-stringing
the PAs (Fig. 5C). (Of note, the distal conduit anastomosis
can also be performed after the IAA repair and before recon-
struction of the ascending aorta if visualization appears to be
difficult.) The conduit is then filled with saline and the pos-
terior wall is oriented to the ventricular incision. The back
wall is sewn in place for approximately one third to one half
of its circumference (Fig. 5D). The repair is finished with a
hood of glutaraldehyde-treated pericardium or PTFE patch
(Fig. 5E).
As the patient is rewarmed, preparations are made for sep-
aration from CPB. A pair of right atrial pressure monitoring
and medication infusion lines are brought through the lower
portion of the incision and cut to length. Temporary pacing
wires can be placed if there are any rhythm issues or to allow
for raising the heart rate. Some inotropic support can be
helpful; often intravenous milrinone and low-dose epineph-
rine are used. The endotracheal tube is suctioned; the patient
is ventilated and gradually weaned from CPB. Transesopha-
geal or epicardial echocardiography is used to inspect the
repair. Particular attention is paid to the VSD closure, left and
RV outflow tracts, and function of the truncal and tricuspid
valves. Any significant blood pressure gradient from the left
R.S. Mosca
ventricle to the descending aorta should be investigated. Di-
rect “needle” evaluation of the RV pressure can help detect
any residual RV outflow problems, or in their absence ele-
vated PVR. Systemic acidosis, hypercarbia, and hypoxemia
must be avoided to help keep PVR low. In the patient with
tenuous hemodynamics, sedation with fentanyl and tempo-
rary paralysis may help minimize rapid changes in PVR. In-
haled nitric oxide can be used to further relax the pulmonary
arterial bed.
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