Brief Report Acta Cardiol Sin 2004;20:31-6

Surgical Repair of Aortopulmonary

Window in Infants
Chieh-Jen Wu, Bor-Yen Lin, Tung-Ho Wu, Anna Lo, Jun-Yen Pan, Pei-Luen Kang,
Kai-Sheng Hsieh1 and Shu-Min Kuo

Background: Aortopulmonary window is a rare congenital heart defect resulting from a communication between
the main pulmonary artery and the ascending aorta. Surgical closure is indicated in all patients with
aortopulmonary window and should be performed at the time of diagnosis to prevent irreversible pulmonary
vascular disease.
Methods: From 1990 to 2002, 4 infants with aortopulmonary window underwent surgical correction. The defects
ranged from 0.4 to 0.6 cm in size and they were all proximal type. Associated cardiac lesions were present in 2
cases, including 1 case with coarctation of the aorta and patent ductus arteriosus, and the other with atrial septal
defect. These procedures were performed under the cardiopulmonary bypass and the associated cardiac lesions
were corrected simultaneously.
Results: There was neither early nor late death. During the period of follow-up (10 months to 12 years), no
residual defect was noted and neither distortion nor stenosis of great artery was found.
Conclusions: Early correction of aortopulmonary window at the time of diagnosis is advised. Repair of
aortopulmonary window can be performed in neonates and infants with excellent results.

Key Words: Aortopulmonary window · Aortopulmonary septal defect · Congenital heart disease

INTRODUCTION APW usually have symptoms of congestive heart failure

Aortopulmonary window (APW), also known as
aortopulmonary septal defect, is a communication
between the ascending aorta and the main pulmonary
artery in the presence of two normally formed semilunar
valves, separate aortic valve and pulmonary valve. APW
is a rare cardiac lesion representing approximately
0.1~0.2% of all congenital heart disease.1-3 Elliotson first
described this cardiac anomaly in 1830.4 Patients with
Received: April 15, 2003 Accepted: September 24, 2003
Division of Cardiovascular Surgery, Department of Surgery; and
1 Surgical closure is indicated in all patients with APW and
Department of Pediatrics, Kaohsiung Veterans General Hospital,
Kaohsiung, Taiwan.
Address correspondence and reprint requests to: Dr. Shu-Min Kuo,
Division of Cardiovascular Surgery, Department of Surgery,
Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan. No. 386,
Ta-Chung 1st Road, Kaohsiung 804, Taiwan. Tel: 886-7-342-3232
ext. 3039; Fax: 886-7-342-0956; E-mail:mryen@isca.vghks.gov.tw
and pulmonary hypertension. Irreversible pulmonary
vascular disease, which is highly lethal, can occur as early
as the first year of life.5 If untreated surgically, 40% of
affected patients will die of intractable heart failure during
the first year of life, and a large number of survivors will
succumb to sequels of congestive heart failure or
pulmonary vascular disease in childhood.6 Anatomically,
Mori and associates classified APW into 3 types. 7
Proximal defects, the most common, are located just
above the sinus of Valsalva; distal defects are located in
the upper portion of the ascending aorta; total defects
involve the majority of the ascending aorta. Different
classifications of the APW have been described.1,8
should be performed at the time of diagnosis. In 1948,
Gross first performed surgical ligation of an APW and
closure of a patent ductus arteriosus (PDA) through left
thoracotomy.9 In 1953, Scott and Sabiston described a
closed method for division and suture of an APW

31 Acta Cardiol Sin 2004;20:31-6

 Chieh-Jen Wu et al.

between clamps. 10 In 1957, Cooley reported the first ventricle. Cardiac catheterization established the definitive
successful repair of an APW utilizing cardiopulmonary diagnosis and moderate pulmonary artery hypertension
bypass.11 The introduction of cardiopulmonary bypass with a peak pulmonary artery systolic to aortic systolic
allowed safer and more reliable different techniques to be pressure ratio greater than 0.5.
applied, including the first transaortic closure of an APW Patient 3, an 18-day-old boy, 2.2 kg, was referred to
reported by Wright et al. in 1968,12 and the first use of a our hospital after birth immediately due to intrauterine
patch to close an APW described by Deverall et al. in growth retardation with low birth body weight of 2250
1969.13 We report our experience of 4 cases with APW gm and imperforated anus. Surgical intervention of
and describe the diagnostic methods, surgical techniques, colostomy was carried out on the second day. Chest
and the results in these patients. roentgenography showed cardiomegaly with cardiothoracic
ratio of 60% and pulmonary hypervascularity. Two-
dimensional echocardiography revealed an APW (Figure
MATERIAL AND METHODS 1), PDA and severe aortic coarctation.
Patient 4, a 29-day-old girl, 3.0 kg, was admitted to
Between October 1990 and May 2002, we performed our hospital with progressive tachypnea and heart
2200 cases of congenital heart surgery in our institution. murmur. Chest roentgenography showed cardiomegaly
Four patients presented with aortopulmonary window and with cardiothoracic ratio of 60% and pulmonary
underwent repair. It accounts for approximately 0.18% of hypervascularity. Two-dimensional echocardiography
all cardiac defects receiving surgical repair. There were 2 revealed an APW and atrial septal defect (ASD). Cardiac
male and 2 female infants ranging in age between 18 days catheterization (Figure 2) confirmed the diagnosis, with
and 6 months (Table 1).
The first patient, a 6-month-old girl, 5.4 kg, had a
history of recurrent upper respiratory infections, and
obvious chest retraction at rest had been noted for 1
month before referred to our hospital. Cardiomegaly was
noted by chest roentgenography, and the cardiothoracic
ratio was near 60%. Two-dimensional echocardiography
revealed a large aortopulmonary window, enlarged left
atrium and left ventricle with moderate degree of mitral
regurgitation. The findings of cardiac catheterization were
APW with left to right shunting and severe pulmonary
hypertension, while the pulmonary artery pressure to
systemic pressure ratio was greater than 70%.
Patient 2, a 49-day-old boy, 3.5 kg, he suffered from
progressive dyspnea and poor feeding since 2 weeks
before admission to our hospital. Chest roentgenography
showed cardiomegaly with cardiothoracic ratio more than
Figure 1. Preoperative echocardiogram of case 3, high parasternal
50%. Two-dimensional echocardiography revealed an short-axis view showing the aortopulmonary window (arrow) between
APW and mild dilatation of the left atrium and left the ascending aorta (Ao) and main pulmonary artery (PA).

Table 1. Clinical data

Case Sex Age Weight (Kg) Major symptoms Associated anomalies
1 F 6 months 5.4 Recurrent pulmonary infection None
2 M 49 days 3.5 Failure to thrive None
3 M 18 days 2.2 Congestive heart failure PDA, Coarctation
4 F 29 days 3.0 Congestive heart failure ASD

Acta Cardiol Sin 2004;20:31-6 32

 Surgical Repair of APW in Infants
Table 2. Investigative data, surgical intervention and outcomes
Pulmonary/Systemic Pulmonary/Systemic
Case pressure ratio Flow ratio Location of defect
1 0.7 (65/90) 3 Proximal
2 0.5 (50/100) 2.5 Proximal
3 None Proximal
4 0.7 (55/80) 3 Proximal
Figure 2. Preoperative aortogram of case 4, showing rapid filling of
the main pulmonary artery through the aortopulmonary window
(arrow) during ascending aortic angiography.
pulmonary artery hypertension at the ratio 0.7 of systemic
pressure.
Surgical technique
In cases 1, 2, and 4, through a median sternotomy,
cardiopulmonary bypass was instituted with bicaval and
aortic cannulation. The right and left pulmonary arteries
were temporarily snared on the commencement of bypass.
Under moderate hypothermia, the heart was arrested with
cold crystalloid cardioplegic solution injecting into the
aortic root. In cases 1 and 2, a vertical incision was made
over the main pulmonary artery. The defects were seen to
be about 6 mm and 5 mm in diameter, respectively, and
they were proximal type (Type I). The pulmonary artery
branches were assessed carefully. Teflon patches were
used to close the windows. The aortic cross-clamping
times were 28 and 26 minutes, respectively. The
33
Technique (approach) Type of repair Result
Transpulmonary Teflon patch Good
Transpulmonary Teflon patch Good
Transwindow Pericardial patch Good
Transwindow Teflon patch Good
pulmonary bypass times were 49 and 54 minutes,
respectively. In case 4, a vertical incision was made over
the anterior wall of the defect lesion. One 4-mm proximal
defect (Type I) was inspected. The patch was sewn to the
circumference of the defect, and the anterior edge of the
patch was then sandwiched between the incision edges of
the anterior aspect of the window by continuous 6-0
polypropylene running suture. The ASD was repaired
simultaneously. The aortic cross-clamping time was 30
minutes, and the cardiopulmonary bypass time was 64
minutes.
In case 3, who suffered from APW, PDA and
coarctation, cardiopulmonary bypass was established with
the ordinary bicaval cannulation and arterial cannula
connecting to innominate artery via a 4-mm Gore-Tex
vascular graft and an additional descending aorta
cannulation. The PDA was divided. The ductus arteriosus
tissue and the aortic coarctation lesion were resected. The
aorta was end-to-end anastomosized directly. Cardiac
arrest was then achieved as per the procedures described
above. A 4-mm proximal defect (Type I) was identified
through an incision over the anterior wall of the defect
lesion. It was closed with a glutaraldehyde-treated
pericardium patch. The aortic cross-clamping time was 27
minutes, and the cardiopulmonary bypass time was 126
minutes.
RESULTS
All 4 patients had good recovery from operation
(Table 2). They survived well, without any cardiac events.
They are followed up regularly by echocardiography.
These studies have documented normal left ventricular
function, no residual aortopulmonary defect, no evidence
of narrowing or stenosis for great arteries, and no
evidence of semilunar valve distortion. The first case,
who presented with moderate mitral regurgitation (MR)
pre-operation, his heart function improved post-operation
Acta Cardiol Sin 2004;20:31-6
 Chieh-Jen Wu et al.
with trivial to mild MR. The third patient, who required
repair of aortic coarctation, showed no evidence of
stenosis. They are all active and asymptomatic and in
New York Heart Association class I at follow-up ranging
from 10 months to 12 years (mean 5.5 years).
DISCUSSION
Aortopulmonary window, a rare congenital heart
anomaly, is caused by abnormal development of the
embryologic truncoconal septum (aorticopulmonary
septum), which normally separates the primitive arterial
trunk into the ascending aorta and the pulmonary artery.
This lesion represents by underdevelopment of the
truncoconal septum, failed fusion or incompletely
formation of the conotruncal ridges, resulting in the
aortopulmonary window. 14,15 Jacobs and associates 8
defined aortopulmonary window as a communication
between the main pulmonary artery and the ascending
aorta in the presence of 2 separate semilunar valves. The
presence of 2 separate semilunar valves distinguishes
aortopulmonary window from truncus arteriosus.
Commonly, the defect is large enough to function as a
nonrestrictive left-to-right shunt, resulting in congestive
heart failure, pulmonary hypertension and early
development of pulmonary vascular disease.3,5,6
In the past, APW was more frequently reported as an
isolated lesion. In our experience, 50% of cases with
APW had associated cardiac anomalies, 1 with ASD and
the other patient with PDA and coarctation of the aorta. In
recent reported series, 1 half to 2-third of patients with
APW were associated with major cardiac defects. 16-18
These cardiac defects include ventricular septal defect,
ASD, PDA, interrupted aortic arch, coarctation of the
aorta, tetralogy of Fallot, transposition of the great
arteries, and coronary anomalies.2,3,6,19
Many different classifications of APW have been
advocated. Earliest, Mori and associates7 classified APW
into 3 types, proximal, distal, and total. Proximal defects
are located just above the sinus of Valsalva, a few
millimeters above the semilunar valves, which is the most
common type. Distal defects are located in the uppermost
portion of the ascending aorta. Total defects consist of the
majority of the ascending aorta. Richardson and associates
proposed an alternative classification,1 which included both
the APW lesions and anomalous right pulmonary artery
Acta Cardiol Sin 2004;20:31-6
origin from the ascending aorta. The Richardson system
describes 3 types of anomalies of APW: Type I, typical
aortopulmonary septal defect or window, is situated
between the ascending aorta and the main pulmonary
artery just above the sinus of Valsalva; it is due to
incomplete septation of the aortopulmonary trunk. Type II,
distal aortopulmonary septal defect, is located more
distally, in which the ascending aorta communicated with
the origin of the right pulmonary artery and the right
pulmonary artery is from the main pulmonary artery. This
anomaly is due to abnormal migration of the sixth aortic
arch. Type III, consists of anomalous origin of the right
pulmonary artery from the ascending aorta. This type is the
result of unequal septation of the aortopulmonary trunks or
truncus arteriosus. Ho and associates modified Mori’s
anatomic classification, which is more useful to
cardiologists considering transcatheter device for APW
closure.8 They kept the terminology but added some
additional description. Proximal defect are distinguished
with little inferior rim separating the APW from the
semilunar valves but with good superior rim. Distal defects
are noted with a well-formed inferior rim but little superior
rim. Total defects, also called confluent defects, are noted
with little superior and inferior rims. Intermediate defects
are recognized with adequate both superior and inferior
rims. This defect is the most suitable group for
transcatheter device closure. Our 4 cases all were proximal
defects.
The initial diagnostic approach to APW was with
cardiac catheterization.18 Current diagnostic techniques
emphasize non-invasive assessment, and 2-dimensional
echocardiography is very useful and facilitated in
diagnosis of an APW.3,6,16,19 In transthoracic studies, the
communication between the aorta and pulmonary artery
can be best visualized from a variety of views, including
subcostal coronal view, parasternal short and long-axis
views and high parasternal short-axis view. It allows
adequate demonstrations of the exact location and
morphological features of the defect and associated cardiac
anomalies. However, cardiac catheterization is necessary
sometimes to provide information about pulmonary artery
pressure and pulmonary vascular resistance.
Surgical closure is indicated in all patients with
aortopulmonary window, and it should be undertaken as
early as possible after diagnosis. Since the first report
about successful repair of an APW by Gross in 1952, 9
many authors have reported their results with a variety of
34
 Surgical Repair of APW in Infants
different techniques. In 1957, Cooley reported the first
successful repair of an APW utilizing cardiopulmonary
bypass. 11 In 1966, Johanson and coworkers described the
first transwindow approach 20 and Putnam and Gross
proposed the transpulmonary approach.21 In 1968, Wright
and associates performed the first transaortic direct
closure of an APW.12 The first use of a patch to close an
APW was described by Deverall et al. in 1969. 13 We
employed transpulmonary approach for APW in our first
two cases. Neither coronary artery injury nor pulmonary
stenosis or distortion was found. During the follow-up
period (12 years and 8 years, respectively), no
reintervention was needed. Currently, most centers
recommend repairing defects through transaorta or
transwindow with a patch placement.3,16-18 It gives better
exposure of the defect, the edge is easily identified, and
the orifices of the coronary arteries and aortic valve are
noted to carefully avoid injury.
In conclusion, most patients with APW develop
severe clinical features in early infancy. Neonatal repair
or early surgical closure is indicated as soon as the
diagnosis is established due to easily developed
congestive heart failure, and to prevent the development
of pulmonary hypertension. Associated cardiovascular
anomalies should be repaired simultaneously. Repair of
APW can achieve good results with low mortality and
morbidity, even in early infancy, and late postoperative
results are excellent.
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