49
Aortopulmonary Window
JOHN F. KEANE AND DONALD FYLER
DEFINITION
An aortopulmonary window (aortopulmonary fenestra-
tion, aortopulmonary septal defect) is a hole between
the ascending aorta and the main pulmonary artery. There
must be two distinct and separate semilunar valves before
this diagnosis can be made.
PREVALENCE
Aortopulmonary window is a rare anomaly; 34 patients
have been seen in the past 14 years at Children’s Hospital
Boston (Exhibit 49-1).
EMBRYOLOGY
There is failure of aortopulmonary septation that,
although in many ways reminiscent of truncus arteriosus
communis, probably develops by a different mechanism.1
In terms of associated lesions, among series with 10 or more
patients reported in the past decade, an average of 57%
had other defects, the most common being ventricular
septal defect (13%), interrupted aortic arch (12%), and
tetralogy of Fallot (8%).2–8
ANATOMY
An aortopulmonary window consists of an opening
between the ascending aorta and the main pulmonary artery
midway between the similar valves and the pulmonary artery
bifurcation, distally at the bifurcation or encompassing both
these areas.1,9
PHYSIOLOGY
The physiologic effects are those of a left-to right shunt,
such as with ventricular septal defect or persistent patent
ductus arteriosus. As pulmonary resistance drops in the
days and weeks after birth, there is increasing left-to-right
shunting, and ultimately, if the window is big, congestive
heart failure develops in the first weeks of life. Because
these defects are most often large, there is usually
pulmonary hypertension at systemic levels. Without repair,
pulmonary vascular disease develops later, possibly as early
as the end of the first year of life.
CLINICAL MANIFESTATIONS
The clinical features in those without additional lesions
are indistinguishable from those seen in a patient with a
persistently patent ductus arteriosus. With a smaller open-
ing, the murmurs are those of a classic patent ductus arte-
riosus (see Chapter 34). With larger defects, the murmurs
are not necessarily continuous, and evidence of congestive
heart failure or pulmonary hypertension may dominate the
auscultatory findings. In those with an additional defect,
the clinical features may be quite different; for example,
with an interrupted aortic arch when the patent ductus
becomes restrictive or closes, femoral pulses may be
impalpable and the baby can become critically ill within
hours.
783
784 Congenital Heart Disease

Exhibit 49–1
Children’s Hospital Boston Experience
Aortopulmonary Window
1973–1987 1988–2002
N (%) N (%)

All Patients 19 34
All other lesions 10 (50) 22 (65)
IAA 1 (5) 8 (26)
TOF 4 (21) 5 (15)
VSD 8 (42) 4 (12)
Surgical mortality 2 (11) 2 (6)*

*Of our 31 surgical patients (median age, 15 days), 2 died,

one a 1.9-kg baby in 1993 and the other a 7-year-old with
severe pulmonary vascular obstructive disease (1998).
IAA, interrupted aortic arch; TOF, tetralogy of Fallot;
VSD, ventricular septal defect.
FIGURE 49–1 Parasternal short-axis echocardiogram of an
aortopulmonary window. The window (double-headed arrow)
between the aorta (AO) and pulmonary artery (PA) is seen, as
well as the relationship to the right (R) and left (L) pulmonary
artery origins.

artery branches, whereas a persistent ductus arteriosus is

Electrocardiography
more distal and usually has some length.
The electrocardiographic patterns are the same as those Surgically important information that is readily derived
seen in patients with patent ductus arteriosus. from the echocardiogram includes the distance between
the proximal border of the defect and the semilunar valves
and coronary arteries, as well as the distance between the
Chest X-Ray
distal border and the pulmonary arteries. Although the
The chest x-ray shows evidence of left-to-right shunting
and pulmonary hypertension indistinguishable from that
seen in patients with patent ductus arteriosus.

Echocardiography
The hyperdynamic function and the enlarged chambers
provide useful clues to the presence and location of a
left-to-right shunt. The defect itself can be easily visuali-
zed and the shunting documented by Doppler examina-
tion (Fig 49-1). The diagnosis is made by scanning the
aortopulmonary septum in multiple views (including sub-
xiphoid, parasternal, and suprasternal notch). Imaging the
defect in multiple views and detecting the flow through it
by color-flow Doppler mapping (Fig. 49-2) allows confir-
mation of the true defect rather than false dropout. At the
level of the defect, the joined vessels are elliptical in cross-
section, whereas in contrast, false dropout is characterized
by persistence of the normal circular contour of each vessel.
The distinguishing feature between aortopulmonary
window and truncus arteriosus is the presence of two sepa- FIGURE 49–2 Color-flow Doppler mapping of the aortopul-
rate semilunar valves in the former. The aortopulmonary monary window in Fig. 49-1, showing flow toward the transducer
window is proximal to, or at the level of, the pulmonary (red signal) from the aorta into the pulmonary artery.
 Aortopulmonary Window
Exhibit 49–2
incidence of additional defects is quite high, these are all
now readily identifiable by careful echocardiography.
Cardiac Catheterization
From an anatomic or hemodynamic standpoint, this is
no longer necessary. The only indication at this time is if
device closure is planned; there have been a few reports of
such management with success in small defects without
other lesions.10,11
MANAGEMENT
Catheter-delivered device closure of a small uncompli-
cated lesion is an option in a few. For most, surgical
closure, including repair of any other lesions, should be
undertaken with minimal delay after diagnosis in infants.
A brief period of intensive anticongestive therapy is prob-
ably useful preoperatively, with prostaglandin E1 treatment
a necessity in those with interrupted aortic arch and ductal
restriction. In a recent report from our hospital covering a
785
26-year period, among 38 patients operated on at median
age of 5 weeks and weight of 3.9 kg, actuarial survival
was 88% at 10 years, and patch closure through the aorta
or defect rather than the pulmonary artery achieved the
best outcome.3 Surgical results are nowadays excellent
(Exhibit 49-2), with only rare occurrences of right
pulmonary artery stenosis or residual leaks, the latter in those
either ligated or repaired through the pulmonary artery.
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