European Journal of Echocardiography 2006 7(5):379-382; doi:10.1016/j.euje.2005.10.010
Copyright © 2005, The European Society of Cardiology
Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery: Two cases highlighting the importance of complete pre-operative echocardiographic evaluation of the coronary arteries in all conotruncal anomalies
Steven C. Greenwaya,
Timothy J. Bradleya,*,
Christopher A. Caldaroneb,
Norman H. Silvermanc,
Frank L. Hanleyd and
Jeffrey F. Smallhorna
aDivision of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8
bDivision of Cardiovascular Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
cDivision of Cardiology, Department of Pediatrics, Stanford University School of Medicine, Stanford, California, USA
dDivision of Cardiovascular Surgery, Department of Surgery, Stanford University School of Medicine, Stanford, California, USA
Received 29 June 2005; received in revised form 18 October 2005; accepted after revision 31 October 2005.
* Corresponding author. Tel.: +1 416 813 7610; fax: +1 416 813 7547. timothy.bradley{at}sickkids.ca
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Abstract
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This report describes two infants with an aortopulmonary window
in association with anomalous origin of the right coronary artery
from the pulmonary artery. In both cases the diagnosis was made
pre-operatively by transthoracic echocardiography, with the
initial clue being extensive collateral flow within the myocardium.
In each case there was surgical confirmation of the echocardiographic
findings. These two cases demonstrate that coronary artery evaluation
should be an integral part of every new echocardiographic evaluation,
particularly in the setting of conotruncal anomalies.
Keywords: Aortopulmonary window; Anomalous origin of the coronary artery from the pulmonary artery; Echocardiography
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Introduction
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Aortopulmonary window with anomalous origin of a coronary artery
from the pulmonary artery, is a rare but important lesion.
1,2 If left undiagnosed, the potential associated myocardial ischemia
due to coronary steal, may be further compounded by the additional
aortic run-off lesion. Thus far, the majority of cases have
been diagnosed at the time of angiocardiography or surgical
repair of an associated defect.
2–5 We report two infants
with an aortopulmonary window, in association with anomalous
origin of the right coronary artery from the pulmonary artery
(ARCAPA), in whom the diagnosis was made pre-operatively by
transthoracic echocardiography.
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Case 1
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The first case was referred at 6weeks of age to the Division
of Cardiology at the Hospital for Sick Children for the assessment
of a cardiac murmur. The infant was asymptomatic, with clinical
findings suggestive of a patent ductus arteriosus and electrocardiographic
evidence of left ventricular hypertrophy in the absence of myocardial
ischemia. This case had only echocardiographic evaluation. Of
note, there was no evidence of either significant mitral or
tricuspid valve regurgitation. There was evidence of collateral
coronary artery flow within the myocardium. In the precordial
short axis view at the level of the aorta and pulmonary artery,
a dilated left coronary system was noted, with antegrade color
Doppler flow (
Fig. 1A). In the same view the right coronary
artery could be identified arising from the posterior and medial
sinus of the pulmonary valve and color Doppler flow demonstrated
retrograde flow into the main pulmonary artery (
Fig. 1B). With
angulation in the same transducer location, high velocity continuous
color flow could be seen arising from a small (3mm) aortopulmonary
window that was located in a more antero-superior location (
Fig. 1C).
There was no evidence of pulmonary hypertension, with normal
septal curvature and a peak instaneous gradient across the aortopulmonary
window of 61mmHg while the systemic systolic blood pressure
was 96mmHg. At surgery the right coronary artery was directly
reimplanted into the facing aortic sinus, with closure of the
aortopulmonary window and the post-operative course was uneventful.
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Figure 1 Transthoracic echocardiography (2D and color Doppler): (A) high precordial short axis view demonstrating the dilated left coronary system, with antegrade flow; (B) same view showing the origin of the right coronary artery from the pulmonary artery, with retrograde flow into the pulmonary artery indicated by the arrows; and (C) with angulation above the aortic valve sinuses to demonstrate the small aortopulmonary window indicated by arrows. (AO, aorta; CIRC, circumflex coronary artery; LAD, left anterior descending coronary; PA, pulmonary artery; RCA, right coronary artery).
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Case 2
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The second case was referred at 3months to Stanford University
Medical Center with the diagnosis of a patent ductus arteriosus
for coil occlusion. This infant was also asymptomatic with electrocardiographic
findings of left ventricular hypertrophy. Again by echocardiography,
dilatation of the left sided chambers was noted with good biventricular
function and no atrioventricular valve regurgitation. Collateral
coronary artery flow within the myocardium and a small (2–3mm)
aortopulmonary window was seen. In this case cardiac catheterization
with angiocardiography was performed prior to surgical intervention
and demonstrated mildly increased pulmonary pressures and a
dilated left coronary system with retrograde flow in the right
coronary artery into the main pulmonary artery. The surgical
surface anatomy in the second case is shown (
Fig. 2). Surgical
reimplantation of the right coronary artery and closure of the
aortopulmonary window was similarly performed and the post-operative
course was uneventful.
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Figure 2 Surgical anatomy. The aortopulmonary window is indicated by the arrow and the anomalous right coronary artery arising from the pulmonary artery runs down beside the two asterisks. Note the dilated left coronary system marked by the smaller arrows. (AO, aorta; PA, pulmonary artery).
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Discussion
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These two cases demonstrate the importance of performing a complete
echocardiographic evaluation in all new patients, including
an assessment of the coronary arteries. This is especially important
now that echocardiography is often likely to be the only imaging
obtained prior to many neonatal and infant repairs. The initial
echocardiographic clue to the diagnosis in both of these cases
was the extensive collateral flow within the myocardium, which
if seen should suggest a coronary artery run-off lesion, be
it an anomalous coronary artery or a significant coronary artery
fistula. The echocardiographic findings are consistent with
reports of isolated ARCAPA, that is a dilated left coronary
system with retrograde flow from the coronary artery into the
pulmonary artery.
6
Anomalous origin of the either the left or right coronary artery may occur in isolation or in association with other forms of congenital heart disease, in particular conotruncal abnormalities.2–5 If coronary abnormalities are not identified prior to surgical correction of an associated lesion, then there may be dire consequences for the patient.2 Both of our cases had acquired coronary collaterals which preserved, but these may have not have been adequate in the post-operative period, if the associated ARCAPA had not been identified.
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References
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- Grunenfelder J., Zund G., Vogt P.R., Turina M.I. Aortopulmonary window with anomalous origin of the right coronary artery. Ann Thorac Surg (1999) 67:233–235.[Abstract/Free Full Text]
- McMahon C.J., DiBardino D.J., Undar A., Fraser C.D. Jr. Anomalous origin of left coronary artery from the right pulmonary artery in association with type III aortopulmonary window and interrupted aortic arch. Ann Thorac Surg (2002) 74:919–921.[Abstract/Free Full Text]
- Izumoto H., Ishihara K., Fujii Y., Oyama K., Kawazoe K. AP window and anomalous origin of right coronary artery from the window. Ann Thorac Surg (1999) 68:557–559.[Abstract/Free Full Text]
- Morell V.O., Feccia M., Cullen S., Elliott M.J. Anomalous coronary artery with tetralogy of Fallot and aortopulmonary window. Ann Thorac Surg (1998) 66:1403–1405.[Abstract/Free Full Text]
- Albertal J., Lynch F.G., Vaccarino G., Vrancic M., Pichinini F., Albertal M. Anomalous origin of right coronary artery. Circulation (2001) 103(13):E73–E75.[Web of Science][Medline]
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Abstract
Introduction