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Aortopulmonary Window
Introduction
“window,” there is usually no length to the communicating channel. The origin of the right pulmonary artery
from the aorta is sometimes considered to be an
example of the most extreme type of aortopulmonary
window. Either the right or (less commonly) the left
coronary artery may arise on the pulmonary side of the
window. About half of the cases of aortopulmonary
window occur as isolated lesions, while the other half
has associated major or minor cardiac malformations.
These include interrupted aortic arch, tetralogy of
Fallot, persistently patent arterial duct, and ventricular
septal defect.
Surgical repair of aortopulmonary window is done
virtually always as an open heart procedure with cardiopulmonary bypass or, in small infants, with low flow
or profound hypothermia and total circulatory arrest. A
patch of Dacron or other material is used to close the
defect, taking care to leave the orifices of the coronary
arteries in continuity with the aorta. Associated malformations are generally repaired at the same time.
Communications between the ascending aorta and
pulmonary artery constitute a spectrum of malformations which is collectively designated “aortopulmonary
window,” “aortic septal defect,” or “aorticopulmonary
window.” The communication is distal to the aortic and
pulmonary valvar leaflets, but may be found in any
position where the great vessels are contiguous, from
the sinus of Valsalva to the origin of the brachiocephalic
vessels.
Aortopulmonary windows have been subclassified
into Type I (proximal defects between the left lateral
wall of the ascending aorta and the pulmonary trunk),
Type II (defects involving the right pulmonary artery
and its origin – also called distal defects), and Type III
(a large defect combining the other two types). It is
probably more useful to view them as a continuum of
pathology. The proximal communications tend to be
very large and round or oval-shaped, while smaller,
more rare and distal lesions may join the posterior
aorta to the anterior right pulmonary artery, similar to
a Waterston anastomosis. As suggested by the name
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36
A Practical Atlas of Congenital Heart Disease
Innominate
Artery
Window
LPA
PA
LAA
Ao
RAA
3.1 Aortopulmonary window. The external appearances of a large
proximal defect are seen from the front.
Innominate
Artery
Window
RPA
LPA
LCA
AoV
RCA
3.2 The aorta has been opened in the case above to show the large aortopulmonary window just above the orifice of the left coronary artery.
The orifice of the right coronary artery lies close to the window, although
this is not immediately apparent in the photograph. The origins of the
right and left branch pulmonary arteries are seen through the window.
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Aortopulmonary Window
Patch
A
B
LPA
RPA
1
2
LCA
3
AoV
3.3 Repair of the aortopulmonary window may be done through an incision in the aorta (1), through
the window itself (2) with a sandwich technique, or through an incision in the pulmonary artery (3).
Cannulation must be sufficiently distal on the lateral side of the aorta (A) or transverse aortic arch (B) to
permit safe application of a clamp above the window; the branch pulmonary arteries are temporarily
occluded to maintain a perfusion pressure and prevent flooding of the lungs on bypass.
3.5 The patch is lowered down when the suture line has cleared the coronary arterial
orifices. It may be carried into the pulmonary artery when a coronary artery lies on that
side of the window. Alternatively, a flap of vessel wall may be used to tunnel the aorta
to the coronary artery. As the suture line approaches the origin of the right pulmonary
artery (star), it is deviated into the aorta to avoid any narrowing of that vessel.
3.4 Exposure through an aortic incision allows visualization of the
coronary arterial orifices, both of which must be identified with certainty.
A patch of either prosthetic material or composite prosthetic material/
pericardium is then inserted with a continuous monofilament suture.
Sutures near the coronary orifice are placed accurately, with the patch at
a distance for good visualization.
3.6 The aortotomy is closed, working from proximal to distal end, so that the aortic
leaflets and coronary arterial orifices may be visualized at the bottom of the suture line.
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A Practical Atlas of Congenital Heart Disease
Suggested Reading
Kutsche LM, Van Mierop LHS. Anatomy and pathologenesis of aorticopulmonary septal defect. Am J Cardiol 1987;59:443.
Burroughs JT, Schumutzer KJ, Linder F, Neuhans G.Anomalous origin
of the right coronary artery with aortico-pulmonary window and
ventricular septal defect. J Cardiovasc Surg 1968;3:142.
Mori K, Ando M, Takao A, Ishikawa S, Imai Y. Distal type of aortopulmonary window; report of four cases. Br Heart J 1978;40:681.
Casillas JA, Delcon JP, Villagra F, Checa SL, Sanchez PA, Gomez R,
Fortuny R, MaBrito J. Aortopulmonary window with anomalous
origin of the right coronary artery from the pulmonary trunk.
Texas Heart Inst J 1986;13:323.
Penkoske PA, Castaneda AR, Fyler DC, Van Praagh R. Origin of pulmonary artery branch from ascending aorta; primary surgical
repair in infancy. J Thorac Cardiovasc Surg 1983;85:537.
Castaneda AR, Kirklin JW. Tetralogy of Fallot with aorticopulmonary
window. Report of two surgical cases. J Thorac Cardiovasc Surg
1977;74:467.
Ravikumar E, Whight CM, Hawker RE, Celermajer JM, Nunn G,
Cartmill TB. The surgical management of aortopulmonary
window using the anterior sandwich patch closure technique.
J Cardiovasc Surg 1988;29:629.
Doty DB, Richardson JV, Falkovsky GE, Gordonova MI, Burakovsky
VI. Aortopulmonary septal defect; hemodynamics, angiography
and operation. Ann Thorac Surg 1981;32:244.
Richardson JV, Doty DB, Rossi WP, Ehrenhaft JL. The spectrum of
anomalies of aortopulmonary septation. J Thorac Cardiovasc Surg
1979;78:21.
Gula G, Chew C, Radley-Smith R, Yacoub M. Anomalous origin of the
right pulmonary artery from the ascending aorta associated with
aortopulmonary window. Thorax 1978;33:265.
Van Praagh R, Van Praagh S. The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases. Am J Cardiol
1965;16:406.
Ho SY, Gerlis LM, Anderson C, Devine WA, Smith A. The morphology
of aortopulmonary windows in regard to their classification and
morphogenesis. Cardiol Young 1994;4:146.
Kirkpatrick SE, Girod DR, King H. Aortic origin of the right pulmonary artery; surgical repair without graft. Circulation 1967;36:
777.
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