47
Truncus Arteriosus
JOHN F. KEANE AND DONALD C. FYLER
DEFINITION
Truncus arteriosus is characterized by a single arterial
vessel that originates from the heart, overrides the ventric-
ular septum, and supplies the systemic, coronary, and
pulmonary circulations, all from the proximal ascending
vessel. When the pulmonary circulation is supplied from
the descending aorta by collateral vessels or a ductus arte-
riosus, the anatomy is considered to be that of tetralogy of
Fallot with pulmonary atresia. There must be no remnant
of a separate main pulmonary artery connected to the heart
and no evidence of a separate pulmonary valve if one is to
be confident of the presence of true truncus arteriosus.
PREVALENCE
Truncus arteriosus is a rare lesion. Reported incidence
rates range from 0.006 to 0.043/1000 live births.1,2 Overall,
among all patients seen by us from 1988 to 2002, this was
the major lesion in 0.3% (Exhibit 47-1). The common asso-
ciation with DiGeorge syndrome and deletion of chromo-
some 22q11 is well recognized and discussed in detail,
together with recurrence risk data in Chapter 5.
EMBRYOLOGY
In the normal embryo, septation of the single truncus
arteriosus into aorta and main pulmonary artery together
with aortic and pulmonary valve formation occurs by the end
of the fifth week. Shortly thereafter, conal septum forma-
tion is completed. It is thought that disturbances during
these weeks, supported by experimental data3–5 result in
conotruncal abnormalities including truncus arteriosus.
ANATOMY
A single arterial trunk, larger in diameter than the normal
aorta at a comparable age, arises from the heart. The trunk
is positioned above the ventricular septum, being domi-
nantly over either ventricle (the right in 42%, the left in
16%, and equally shared in 42%). The truncal valve is rarely
normal, often having thickened and deformed leaflets (72%)6
that are variably stenotic or, more often, incompetent (50%).7
Valve anatomy is variable, with 42% tricuspid, 30% bicuspid,
24% quadricuspid, and 1% unicuspid in one study,6 and 69%,
9%, 21%, and 1%, respectively, in a literature review.7
A malalignment ventricular septal defect, which is rarely
small, is present in almost all patients. This ventricular
defect type, the overriding great artery and frequent right-
sided aortic arch are reminiscent of tetralogy of Fallot,
another conotruncal abnormality. The pulmonary arteries
usually arise from the truncus within a short distance of the
valve, as a single short vessel that divides into right and left
pulmonary arteries (Fig. 47-1)8,9 or as two separate vessels,
usually without stenosis, from the posterior wall of the
truncus. Occasionally, a pulmonary artery is absent.10–12 An
interrupted aortic arch is a common associated lesion in
11% to 14% of patients (Exhibit 47-1),6 as are extracardiac
anomalies (21% to 48%).8,13 Coronary artery anomalies are
frequent—these include a prominent right conal vessel, high
or low origins of the vessels, greater than normal frequency
of posterior descending origin from the left coronary
artery, and a single coronary artery.14–16
767
768 Congenital Heart Disease

Exhibit 47–1
Children’s Hospital Boston Experience: Truncus Arteriosus Complicating Factors
Complicating Diagnoses 1973–1987 1988–2002
93 patients Deaths % 171 patients Deaths %
Interrupted aortic arch 10 70 24 25
Truncal stenosis 22 23 69 20
Truncal regurgitation 52 44 138 14
Mitral valve disease 13 31 83 12
There were 171 children with the diagnosis of truncus arteriosus: 62% were younger than 2 months when first seen: 22%
were older than 6 months.
Some patients had more than one complicating diagnosis: survival with interrupted aortic arch has improved considerably.
Life-table analysis of all 171 children with truncus arteriosus first seen between 1988 and 2002 compared with 49 seen 1983
to 1987, without respect to type of treatment. Late survival has improved.

Life-table analysis of 131 children undergoing reparative surgery at Children’s Hospital Boston (median age 19 days) 1988
to 2002 compared to 36 between 1983 and 1987. Survival is improving: The 14 early postoperative deaths included three
in the interrupted aortic arch group and all three with severe truncal regurgitation and/or stenosis.
 Truncus Arteriosus
FIGURE 47–1 There are similarities between the Collett and
Edwards and the Van Praagh classifications of truncus. Type 1 is
the same as A1. Types II and III are grouped as a single type A2,
because they are not significantly distinct embryologically or
therapeutically. Type A3 denotes unilateral pulmonary artery
atresia with collateral supply to the affected lung. Type A4 is
truncus associated with an interrupted aortic arch (13% of all cases
of truncus arteriosus).
From Hernanz-Schulman M, Fellows KE. Persistent truncus arte-
riosus: pathologic, diagnostic and therapeutic considerations.
Semin Roentgenol 20:121–129, 1985, with permission.
PHYSIOLOGY
The truncus receives the output of both ventricles and,
within a short distance, the blood destined for pulmonary
circulation is diverted to the lungs (Fig. 47-2). In the absence
of pulmonary stenosis, the pulmonary arterial pressure is
equal to that in the truncus. The amount of pulmonary blood
flow is determined by the presence of pulmonary stenosis
or by the level of pulmonary arteriolar resistance. In the
absence of pulmonary stenosis, the expected reduction in
pulmonary resistance in the first days of life is not well toler-
ated; survival depends on persistence of some pulmonary
arteriolar resistance to prevent flooding of the lungs.
Pulmonary blood flows are greater than normal, the exact
amount determining the systemic arterial saturation. When
a main pulmonary artery arises immediately beyond the
truncal valve, the pulmonary circulation may largely derive
from the right ventricle, the pulmonary arterial oxygen satu-
ration being less than that observed in the aorta. Truncal
regurgitation of some degree is present in most patients,
and when severe, the added burden of the regurgitant flow
may be more than can be tolerated. The amount of blood
passing through the truncal valve to supply the systemic
blood flow and the pulmonary blood flow, as well as the
regurgitant blood flow, is greatly excessive and often leads to
early congestive heart failure. Because of the large pulmonary
769
FIGURE 47–2 Diagram of the hemodynamics in a patient with
truncus arteriosus. Note the equilibration of systemic and pulmonary
pressures; the complete mixing of systemic and pulmonary blood
as evidenced by similar systemic and pulmonary oxygen concen-
trations; and the large left-to-right shunt as evidenced by
high pulmonary oxygen saturation. Ao, aorta; LA, left atrium;
LPA, left pulmonary artery; LV, left ventricle; RA, right atrium;
RPA, right pulmonary artery; RV, right ventricle.
From Nadas’ Pediatric Cardiology, ed Fyler DC, Hanley &
Belfus, Philadelphia, 1992.
blood flow, development of pulmonary vascular obstructive
disease is common among survivors who have not undergone
surgery: it can occur as early as 6 months of age.
CLINICAL MANIFESTATIONS
Most of these babies are found to have a cardiac problem
within the first weeks of life, either because of a murmur
or the appearance of tachypnea and/or costosternal retrac-
tions. The infants are sometimes visibly cyanotic, although
tachypnea and even costal retractions are more common
presenting symptoms and this evidence of congestive heart
failure is usually readily apparent. The peripheral pulses are
bounding. There is generally an easily palpable and often
visible right ventricular impulse. There is usually a systolic
murmur at the left sternal border, sometimes associated
with the diastolic murmur of aortic regurgitation. There is
frequently a prominent ejection click audible at the apex
or the left sternal border, and the second heart sound is
usually single although this may be difficult to detect in a
770 Congenital Heart Disease
small sick infant. An apical diastolic flow rumble due to
relative mitral stenosis and a huge pulmonary blood flow
is common. Once pulmonary vascular obstructive disease
has developed, the clinical features reflect the elevated
pulmonary vascular resistance (see Chapter 10).
Electrocardiography
The electrocardiogram may show left or right ventricular
hypertrophy, with left or combined ventricular hypertrophy
being more common.
Chest Radiography
Chest radiograph shows that the heart is large; pulmonary
vasculature is increased. There may be a right aortic arch.
Echocardiography
From a subxiphoid long-axis view, the echocardiogram
shows the overriding truncal root and its leftward posterior
originating pulmonary artery is visualized atop a large
conoventricular septal defect together with truncal valve
thickness and mobility (Fig. 47-3). The atrial and ventricular
septa, atrioventricular valves and truncal valve commissural
details may be evaluated using a short-axis subxiphoid view.
Truncal valve stenosis may be quantitated using Doppler
from apical, suprasternal or right sternal border views, and
regurgitation from parasternal or apical transducer locations.
Coronary artery origins and branching details, including
FIGURE 47–3 Subxyphoid echocardiographic image showing
the right and left ventricles connected by a large ventricular
septal defect immediately below a thickened truncal valve. The
pulmonary arteries (arrow) arise directly from the single arterial
root, which continues to the ascending aorta (Ao).
ostial to pulmonary artery origin measurements, are available
from parasternal long and short-axis views.
The proximal pulmonary artery anatomy, including distal
branches, that is the truncus type (Fig. 47-1) is displayed
using parasternal short-axis and suprasternal notch views.
The ascending and transverse aortic arch, brachiocephalic
arterial and ductal origin anatomy can be outlined on a
suprasternal notch short-axis view.
Cardiac Catheterization
Given the precise anatomic details, together with truncal
valve function information provided by echocardiography
in these patients who are neonates when first seen (from
which low pulmonary resistance may be inferred), there
is little need for catheterization. The only preoperative
indications for such a study would include (a) uncertainty
about anatomic information (Fig. 47-4) or (b) suspicion of
pulmonary vascular obstructive disease, which does occur
even in infants with this lesion. The purpose of the study
in the latter is to evaluate pulmonary resistance response
to vasodilators such as oxygen and nitric oxide.
MANAGEMENT
Since (a) most of these patients will develop heart failure
in the neonatal period, (b) pulmonary vascular obstructive
disease is not uncommon by age 1 year,17 and (c) surgical
techniques have improved greatly, repair, including any asso-
ciated lesions, is usually undertaken within days of diagnosis
(Exhibit 47-1). In earlier years, palliative pulmonary artery
banding was an initial approach but resulted frequently in
FIGURE 47–4 A, Cineangiogram in the left anterior oblique
view showing a truncus arteriosus 1. The descending aorta is
supplied by a ductus arteriosus (arrow), through which the catheter
entered the common truncus. B, the small transverse arch (arrow)
ends in an interrupted aortic arch.
From Nadas’ Pediatric Cardiology, ed Fyler DC, Hanley &
Belfus, Philadelphia, 1992.
 Truncus Arteriosus
severe obstruction/hypoplasia/atresia of vessels and even
vascular obstructive disease in one or both lungs.18
Thus, where facilities (surgery, intensive care, cardiology)
are available, after a brief period (days at most) of intensive
medical treatment if necessary, surgical repair is undertaken.
In the uncomplicated patient this includes ventricular septal
defect closure and placement of a valved homograft from
the right ventricle to the detached pulmonary arteries.
Aortic arch interruption or coarctation is readily repaired
(direct end-end anastomosis or patch plasty) at the same
time. Early mortality rates for repair in recent years have
ranged from 0% to 12.5%.19–22
The most difficult associated lesions to deal with are
stenosis or regurgitation of the truncal valve. In general, mild
degrees of either are left alone as these will likely remain
unchanged or even improve postoperatively. Prosthetic
valve placement at this age is often not possible, but a plasty
procedure can be beneficial in some.23
These patients require life-long follow-up. All will need
replacement of the right ventricular-pulmonary homograft
because of increasing obstruction at least once. Replacement
can be postponed for a period of some 2 years by stent
placement at catheterization.24 Pulmonary artery stenoses
centrally are encountered, as is aortic obstruction follow-
ing coarctation or interruption repair, all of which can be
significantly improved by balloon dilation with or without
stent placement at catheterization. Bacterial endocarditis
prophylaxis is, of course, mandatory in all.
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