Aortopulmonary Window Repair: 15-Year Surgical Experience

Khawar Aizaz, Howaida Al Qethamy, Khalid Al Jubair, Yahya Al Faraidi and

Mohammed R Al Fagih
Asian Cardiovasc Thorac Ann 2001;9:111-114

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The Asian Cardiovascular & Thoracic Annals is the official journal of The Asian Society for
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O RIGINAL CONTRIBUTION
Aizaz AORTOPULMONARY WINDOW REPAIR: 15-YEAR SURGICAL EXPERIENCE

AORTOPULMONARY WINDOW
REPAIR: 15-YEAR SURGICAL
EXPERIENCE
Khawar Aizaz, MBBS, Howaida Al Qethamy, MBBS,
Khalid Al Jubair, MBBS, Yahya Al Faraidi, MBBS,
Mohammed R Al Fagih, MBBS
Department of Cardiac Surgery
Prince Sultan Cardiac Center
Riyadh, Saudi Arabia

ABSTRACT
Aortopulmonary window is an uncommon anomaly. Early surgery is recommended
before permanent pulmonary vascular changes develop. Results were reviewed in
18 patients who underwent aortopulmonary window repair between January 1985
and December 1999. A transaortic approach was employed in 12, a transpulmonary
approach was used in 3, the pulmonary artery flap technique was performed in
2, and an aortopulmonary window was simply ligated in 1 patient. Concomitant
repair of all associated anomalies was carried out, except in 2 patients who had
interrupted aortic arch repaired 6 days before aortopulmonary window repair.
There was no hospital mortality. During a mean follow-up of 43 months (range,
6 to 144 months), there was no late death and all patients were in New York Heart
Association functional class I, except 3 who required reoperation: 2 had pulmonary
artery confluence stenosis 5 to 10 years after aortopulmonary window repair; and
1 required transfer of the right coronary artery from the pulmonary artery to the
aorta 28 months after aortopulmonary window repair. Surgical repair of
aortopulmonary window, even when associated with other cardiac anomalies,
carries a low risk. Early surgical treatment achieved excellent immediate and
long-term results.
(Asian Cardiovasc Thorac Ann 2001;9:111–4)

INTRODUCTION
Aortopulmonary window (APW) is a rare cardiac anomaly
in which there is a localized absence of the conotruncal
septum.1 Anatomically, it consists of a communication
between the ascending aorta and the main pulmonary
artery, with separated aortic and pulmonary valves.
The lesion was first described in an autopsy study by
Elliotson2 in 1830, and its successful closure was initially
reported by Gross3 in 1952. Richardson and colleagues4
described 3 types of APW, depending on the distance
between the defect and the plane of the semilunar valves.
Type I defects are between the ascending aorta and main
pulmonary artery, just above the sinus of Valsalva. Type
II defects are more cephalad between the ascending aorta
and the origin of the right pulmonary artery from the
main pulmonary artery. A type III defect is anomalous
origin of the right pulmonary artery from the aorta.4
PATIENTS AND METHODS
From January 1985 to December 1999, 18 patients with
APW were operated upon at the Prince Sultan Cardiac

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Department of Cardiac Surgery (F-277), Prince Sultan Cardiac Center, P.O. Box 7897, Riyadh 11159, Saudi Arabia.

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AORTOPULMONARY WINDOW REPAIR: 15-YEAR SURGICAL EXPERIENCE Aizaz

Center, Riyadh, Saudi Arabia. There were 10 males (56%)
and 8 females; their ages ranged from 2 weeks to 72
months (mean age, 11.13 months), with 13 (72%) under
1 year of age, of whom 4 were less than 1 month old.
Weights ranged from 2.07 to 14.5 kg (mean, 4.97 kg), and
12 patients (67%) were less than 5 kg (Table 1). Two
patients with associated type A interrupted aortic arch
presented during the neonatal period in a state of
circulatory collapse. Both of these patients required
preoperative intubation and ventilation, and were
supported with inotropics and prostaglandin E1 infusion.
The remaining patients had mild degrees of cardiac failure
and presented with recurrent pulmonary infection and/or
failure to thrive. Electrocardiograms and chest radiographs
were studied in all cases. Standard right and left heart
catheterization with cineangiography was performed in
14 of the 18 patients. Angiographic findings and associated
cardiac anomalies are listed in Table 1.

Table 1. Clinical and Preoperative Cardiac Catheterization Data in 18 Cases of Aortopulmonary Window
Patient Age Weight Aortic/Pulmonary Pulmonary/ Associated
No. Sex (months) (kg) Artery Pressure Systemic Flow Anomaly
1 F 7 4.5 100/75 2.5/1 None
2 F 1 3.8 – – IAA type-A
3 M 1 2.9 75/65 4/1 None
4 M 24 6.6 115/90 2.6/1 None
5 M 72 14.5 110/85 3.5/1 None
6 M 0.5 2.29 – – None
7 F 5 3.4 98/94 3.5/1 None
8 M 8 4.46 75/73 3/1 PDA
9 F 12 5.48 95/94 3/1 TOF
10 M 1 2.07 – – None
11 F 5 4 115/110 3.5/1 IAA type-A
12 M 24 9 80/70 3.8/1 None
13 F 6 4 – – None
14 F 7 3.88 98/80 3/1 PM VSD
15 M 14 5.5 100/78 3.5/1 None
16 M 2 4.7 100/90 3/1 None
17 M 4 5.3 90/85 3/1 PDA
18 F 7 3.2 120/60 1.5/1 None
IAA = interrupted aortic arch, PDA = patent ductus arteriosus, PM VSD = perimembranous ventricular septal defect, TOF = tetralogy of Fallot.

Table 2. Operative Data of 18 Cases of Aortopulmonary Window

Patient Type of Circulatory Follow-Up
No. APW Support Surgical Technique Result (years)
1 II CPB + XC Transaortic Reoperation 12
2 I CPB + XC Transaortic + IAA repair* Good 3
3 II CPB + XC Transpulmonary Good 10
4 II – Direct ligation Reoperation 6
5 II CPB + XC Transaortic Good 12
6 II CPB + XC Transaortic Good 7
7 II CPB + XC Transpulmonary Good 2
8 I CPB + XC Transaortic + PDA ligation Good 0.5
9 I CPB + XC Transpulmonary + TOF repair Good 3
10 II CPB + XC Transpulmonary Good 0.5
11 I CPB + XC Transaortic + IAA repair* Good 1
12 I CPB + XC Transaortic Good 1
13 I CPB + XC Transaortic Reoperation 3
14 I CPB + XC Transaortic + closure of PM VSD Good 0.5
15 I CPB Pulmonary artery flap technique Good 0.5
16 I CPB + XC Transaortic Good 0.5
17 I CPB Pulmonary artery flap technique + PDA ligation Good 2
18 I CPB + XC Transaortic Good 0.5
*IAA repair 6 days before APW repair. APW = aortopulmonary window, CPB = cardiopulmonary bypass, IAA = interrupted aortic arch,
PDA = patent ductus arteriosus, PM VSD = perimembranous ventricular septal defect, TOF = tetralogy of Fallot, XC = crossclamping.

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Aizaz AORTOPULMONARY WINDOW REPAIR: 15-YEAR SURGICAL EXPERIENCE

Table 3. Reoperations in Three Patients

Characteristic Patient No. 1 Patient No. 4 Patient No. 13
Type of APW II II I
Type of operation Transaortic Direct ligation Transaortic
Year of operation 1985 1986 1997
Interval to reoperation 10.6 years 5.2 years 2.3 years
Complication PA confluence stenosis PA confluence stenosis Unrecognized anomalous RCA from PA
Reoperation Enlargement of PA confluence Enlargement of PA confluence Transfer of RCA from PA to aorta
using autologous PP using autologous PP
Comments First patient in the series Bleeding requiring pledgeted Anomalous RCA missed preoperatively
No technical difficulty encountered sutures during APW ligation (no catheterization) and intraoperatively
Transferred to PA by transaortic patch
APW = aortopulmonary window, PA = pulmonary artery, PP = pericardial patch, RCA = right coronary artery.

Surgical repair was carried out through a median
sternotomy. Fifteen patients underwent correction with
the use of cardiopulmonary bypass (CPB), moderate
hypothermia, aortic crossclamping, and a single dose of
antegrade cold blood cardioplegia (25 to 30 mL·kg–1).
Two patients had APW repair using CPB and normo-
thermia without aortic crossclamping, and the APW was
doubly ligated without CPB in 1 patient. The operation
was performed via a transaortic approach with repair of
the defect using a Gore-Tex patch (WL Gore, Flagstaff,
AZ, USA) in 12 patients. A transpulmonary approach
using a Gore-Tex or Dacron patch to close the defect was
undertaken in 3 patients. The pulmonary artery flap
technique (direct closure of the aortic defect with a
pulmonary arterial wall patch and repair of the resultant
defect in the pulmonary artery with an autologous
pericardial patch) was performed in 2 patients. Con-
comitant repair of all associated anomalies was carried
out, except in the 2 patients with interrupted aortic arch;
as both were in a state of circulatory collapse at
presentation, a staged repair was considered safest. Repair
of interrupted aortic arch was undertaken through a
standard left thoracotomy as the first stage, thereby
avoiding the adverse effects of CPB in these sick neonates.
APW repair was then carried out as a second stage 6 days
Table 4. Operative Results and Complications
in 18 Patients
Outcome No. of Patients
Hospital mortality 0 use of CPB without aortic crossclamping. The APW was
Intensive care unit stay (days) 6.75 (range, 1–30)
Hospital stay (days) 13.77 (range, 4–60)
Time to extubation (hours) 78 (range, 12–72)
Reoperation 3 (17%)
Phrenic nerve paresis/paralysis* 2 (11%)
Renal failure requiring peritoneal dialysis† 1 (6%)
Respiratory tract infection requiring reintubation 1 (6%)
Repeat sternotomy for bleeding 1 (6%)
*One patient required plication of the diaphragm. †Renal function
improved to normal before discharge.
later in both cases. The operative data are summarized in
Table 2.
RESULTS
There were no operative or late deaths. Three patients
(17%) required reoperation as shown in Table 3. All 3
made an uneventful recovery after reoperation. Follow-up
ranged from 6 to 144 months (mean, 43 months). The
complications are listed in Table 4. Follow-up data were
obtained in all patients from the records of clinical
examinations and echocardiography findings documented
at the time of the last visit to the outpatient clinic. Fifteen
of the 18 patients (excluding the 3 who required
reoperation) were in New York Heart Association
functional class I, with no residual shunts.
DISCUSSION
APW clinically mimics a large patent ductus arteriosus
or ventricular septal defect with pulmonary hypertension.
However, pulmonary vascular resistance in these patients
tends to increase very rapidly. Prompt diagnosis and
treatment is therefore required.4 The spectrum of APW is
wide so several surgical techniques and modifications
have been described, with or without CPB, through a
transaortic, transpulmonary, or trans-window approach,
and with or without the use of a patch.5–11 In 1992,
Matsuki and colleagues12 described the pulmonary artery
flap technique for closure of APW. Using the same
principle, we recently started using the pulmonary artery
flap technique to repair APW. Our method differs in the
isolated by careful dissection, taking particular care of
the left main coronary artery that can be intimately related
to the posteroinferior surface of the defect. A side-biting
vascular clamp was placed across the defect towards the
pulmonary artery immediately after institution of CPB.
Normothermia was maintained and the heart was kept
beating. The window was divided, taking along with it
the patch of pulmonary arterial wall, which is oversewn,
thereby closing the defect on the aortic side. The side-
biting vascular clamp was removed, the defect in the

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AORTOPULMONARY WINDOW REPAIR: 15-YEAR SURGICAL EXPERIENCE
pulmonary artery was repaired using an autologous
pericardial patch, and the patient was weaned off CPB.
This technique avoids narrowing of the aorta and
pulmonary artery, allows complete separation of the aorta
and pulmonary artery, prevents any residual shunts, and
avoids complications associated with a synthetic patch on
the aorta (aneurysmal dilatation). We used this technique
in 2 patients, and because of the advantages and simplicity,
it will be our procedure of choice in future to repair APW,
especially for a type I defect. Larger defects may require
aortic crossclamping as placing the side-biting vascular
clamp across a larger APW can lead to hemorrhage. In
addition, this technique allows exact tailoring of the
pulmonary artery patch to close the aortic defect, thereby
avoiding a redundant and aneurysmal patch.
APW is an uncommon anomaly often associated with
other cardiac defects, especially type A interrupted aortic
arch (in 26% of cases).13 Despite reports of successful
single-stage repair of APW and interrupted aortic arch,
we believe that a two-stage repair is safer in neonates
who present with severe heart failure and circulatory
collapse.14,15 Surgical repair of APW, even when associated
with other cardiac anomalies, carries a low risk. Early
surgical treatment prevents development of obstructive
pulmonary vascular disease and achieves good immediate
and long-term results.
Presented at the 8th Annual Meeting of The Asian Society
for Cardiovascular Surgery, Fukuoka, Japan, September
6–8, 2000.
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2001, V OL . 9, N O . 2
 Aortopulmonary Window Repair: 15-Year Surgical Experience
Khawar Aizaz, Howaida Al Qethamy, Khalid Al Jubair, Yahya Al Faraidi and
Mohammed R Al Fagih
Asian Cardiovasc Thorac Ann 2001;9:111-114
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Updated Information including high-resolution figures, can be found at:

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