Surgical Management of Aortopulmonary Window

Isidoro Di Bella and Dennis J. Gladstone

Ann Thorac Surg 1998;65:768-770

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Surgical Management of Aortopulmonary Window
Isidoro Di Bella, MD, and Dennis J. Gladstone, FRCS
Department of Cardiac Surgery, Royal Group of Hospitals, Belfast, Northern Ireland
Background. Aortopulmonary window is a rare anom-
aly, and a variety of surgical techniques have been
described for its closure.
Methods. We treated 6 infants with aortopulmonary
window between 1993 and 1995. Three had associated
type A interrupted aortic arch, and another had a muscu-
lar ventricular septal defect. The diagnosis was made by
echocardiography, confirmed by cardiac catheterization
in 4 infants. In 1 very sick neonate with interrupted arch,
diagnosis of the window was considerably delayed. In 4
patients, we closed the window by using a flap of
pulmonary artery, which was reconstructed without us-
ing a patch. In 2 neonates with interrupted arch we
A ortopulmonary window (APW) is a rare congenital
cardiac anomaly first described by Elliotson in 1830
in an autopsy study [1]. We report our experience of 6
patients with APW who presented for surgical treatment.
In this series all repairs were accomplished using autol-
ogous tissue.
Material and Methods
Between February 1993 and September 1995, 6 patients
presented with APW and subsequently underwent repair
in the Cardiac Surgical Unit of the Royal Victoria Hospi-
tal, Belfast (Table 1).
The three patients with associated type A interruption
of the aortic arch (IAA) all presented during the neonatal
period; 2 were in a state of circulatory collapse with
severe cardiac failure precipitated by ductal closure, but
the other had only mild symptoms (patient 2). The
remaining patients had no major associated anomaly and
presented at more than 1 month of age. Their cardiac
failure was less severe and manifested more as failure to
thrive, complicated by bronchiolitic infection in 1 patient
(patient 3).
Initial diagnoses were made by two-dimensional echo-
cardiography and were subsequently confirmed by car-
diac catheterization in 4 patients.
In the first patient of the series, there was considerable
delay in diagnosing the APW. This patient presented in a
state of circulatory collapse and the initial diagnosis was
of severe aortic coarctation. At emergency operation,
type A IAA was diagnosed and treated by direct anasto-
mosis between descending aorta and arch. Postopera-
Accepted for publication Sep 22, 1997.
Address reprint requests to Mr Gladstone, Royal Victoria Hospital,
Grosvenor Rd, Belfast BT12 6BA, Northern Ireland.
© 1998 by The Society of Thoracic Surgeons
Published by Elsevier Science Inc
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anastomosed the mobilized descending aorta directly to
the aortic defect of the aortopulmonary window, closing
the pulmonary artery with a pericardial patch.
Results. There were no hospital deaths, and all patients
are in New York Heart Association functional class I at a
mean follow-up of 30 months. Echocardiography shows
no significant distortion of the great vessels.
Conclusions. The techniques described achieve excel-
lent results using only autologous tissues with the po-
tential for normal growth.
(Ann Thorac Surg 1998;65:768 –70)
© 1998 by The Society of Thoracic Surgeons
tively, he was in renal failure for several days, and his
subsequent slow recovery and persistent cardiac failure
were initially attributed to his poor preoperative status,
as no additional intracardiac anomaly was seen on echo-
cardiography. The clinical picture was further compli-
cated by the development of severe infection with respi-
ratory syncytial virus, requiring prolonged ventilatory
support. The diagnosis of APW was eventually made at
3.5 months of age, and shortly thereafter he underwent a
further successful operation and made a good recovery.
In patient 2, whose cardiac failure was well controlled
on medical therapy, operation was delayed because of
low birth weight. Two of the remaining 4 patients (pa-
tients 3 and 5) required preoperative ventilation, but all
of the operations were performed semielectively once the
patients had been stabilized on medical therapy.
The surgical approach adopted in 4 patients was to
close the APW using a simple pulmonary flap technique.
After institution of cardiopulmonary bypass with moder-
ate hypothermia, either the APW itself or the main
pulmonary artery (PA) distal to it was occluded, followed
by ascending aortic cross-clamping and administration of
cold cardioplegia solution.
A semicircular flap of arterial wall was created by
incising the main PA anteriorly a few millimeters lateral
to the APW, and then extending this incision to diamet-
rically opposite points at the superior and inferior mar-
gins of the defect (Fig 1). Care was taken to ensure that
there was no anomalous coronary orifice that might be
compromised by the repair. The flap was then sutured to
the posterior margin of the APW using continuous
polypropylene (Fig 2).
Air was vented from the ascending aorta and the
cross-clamp removed to test for leaks on the suture line.
During rewarming, the cut edge of the PA was closed to
the adventitia of the aorta using the same suture (Fig 3).
0003-4975/98/$19.00
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Ann Thorac Surg DI BELLA AND GLADSTONE 769
1998;65:768 –70 OPERATION FOR AP WINDOW

Table 1. Presenting Features and Operative Data of the Patients

Patient Associated Age at Weight
No. Sex Anomalies Operation (kg) Procedure

1 M Type A IAA 8 days 2.8 Aortoplasty

111 days 4.6 Pulmonary flap repair
2 F Type A IAA and large PDA 85 days 3.4 Descending aorta to aortic window anastomosis
3 M Small VSD 92 days 4.6 Pulmonary flap repair
4 F ... 114 days 4.6 Pulmonary flap repair
5 M Type A IAA 17 days 3.5 Descending aorta to aortic window anastomosis
6 F ... 58 days 3.2 Pulmonary flap repair

IAA 5 interrupted aortic arch; PDA 5 patent ductus arteriosus; VSD 5 ventricular septal defect.

In the remaining 2 patients with IAA, a one-stage Comment

repair was performed. The PA was detached from the
aorta, and the mobilized descending aorta was directly
anastomosed to the aortic window. The PA was repaired
using a patch of autologous pericardium. (In patient 2,
the initial plan was to connect the APW directly to the
patent ductus arteriosus, using a baffle within the PA, but
this approach was abandoned when operative manipu-
lations caused ductal constriction.)
Results
All patients made a good recovery from operation. All are
well from the cardiac viewpoint, and taking no cardiac
medication at a mean follow-up of 30 months. Each has
been studied by echocardiography at follow-up on at
least one occasion, but none has yet been subjected to
further invasive investigation. These studies show no
evidence of PA narrowing and normal flow patterns.
Patients who required repair of interrupted aortic arch
show no evidence of anastomotic narrowing on two-
dimensional imaging. Doppler measurements in the de-
scending aorta show maximum flow velocities that give
small calculated gradients (mean 13 mm Hg). In our
experience, patients with such findings do not show
significant gradients on invasive measurement.
Aortopulmonary window was first described by Elliotson
[1] and is a result of incomplete septation between the
ascending aorta and the PA. Richardson and colleagues
[2] described three types of APW, depending on the
distance between the defect and the plane of the semi-
lunar valves. All of our patients had type I APW accord-
ing to their classification.
In our series, 4 of 6 patients had associated cardiac
anomalies, in three patients type A IAA. This association
has previously been reported in 26% of cases, in a review
of the literature, by Kutsche and van Mierop [3].
Aortopulmonary window may be readily diagnosed by
echocardiography. However, as our first patient demon-
strated, the findings can be easily misinterpreted on
multiple examinations if the operator is not alert to the
diagnosis. The finding of type A IAA in isolation should
be treated with special suspicion. Any doubt about the
diagnosis may be resolved by cardiac catheterization.
Since Gross [4] first successfully ligated an APW in
1948, several surgical techniques and modifications have
been described, with or without extracorporeal circula-
tion, through a transaortic or transpulmonary approach,
and with or without the use of a patch [5–11]. Matsuki
and associates in 1992 [12] and Messner and colleagues in
1994 [13] have described similar pulmonary flap tech-
niques for closure of APW, although they both used
pericardium to repair the pulmonary artery.

Fig 2. The pulmonary flap is sutured to the posterior margin of the

Fig 1. Line of incision on the main pulmonary artery. (Ao 5 as- aortopulmonary window. (Ao 5 ascending aorta; PA 5 main pul-
cending aorta; PA 5 main pulmonary artery.) monary artery.)

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770 DI BELLA AND GLADSTONE
OPERATION FOR AP WINDOW
Fig 3. The cut edge of the pulmonary artery is sutured to the adven-
titia of the aortic wall. (Ao 5 ascending aorta; PA 5 main pulmo-
nary artery.)
If the pulmonary flap is based across the diameter of
the defect, as we describe, the length of the flap does not
need to be as great as if it is more anteriorly and narrowly
based. We have found that our repair gives a normal
cylindric shape to the aorta using only a small amount of
PA wall. All of our patients showed marked dilatation of
the main PA and direct closure was achieved without
causing significant distortion. We believe that patching
should only be necessary in special situations where a
larger pulmonary flap is required to close a more com-
plex defect, or to direct blood to an anomalous coronary
origin.
In the 2 patients in whom APW and IAA were diag-
nosed together at presentation, we anastomosed the
descending aorta directly to the aortic defect of the APW.
This option, which was also reported by Burke and
Rosenfeld in 1994 [14], in a patient with anomalous origin
of the right PA, seems to achieve good results without
significant compression of the right pulmonary artery or
left main bronchus, despite the more proximal connec-
tion to the ascending aorta.
In conclusion, APW is an uncommon anomaly that can
be easily overlooked if not sought specifically. It is often
associated with other cardiac anomalies, especially type
A interruption of the aortic arch, which is almost never
found in isolation.
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Ann Thorac Surg
1998;65:768 –70
The techniques we describe are simple and effective
means of repairing AP window with and without
interrupted aortic arch. The absence of foreign mate-
rials should give good growth potential with minimal
distortion of the vessels and excellent functional re-
sults.
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 Surgical Management of Aortopulmonary Window
Isidoro Di Bella and Dennis J. Gladstone
Ann Thorac Surg 1998;65:768-770

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