Patients with pulmonary atresia and intact ventricular septum (PA/IVS) have right
ventricle-to-coronary artery fistulous communications in 30% to 60% of cases,
1
,
2
,
3
,
4
and a subset of these patients develop coronary artery obstructive lesions.
5
In such cases, a portion of left ventricular myocardium may have a right ventricle–dependent
coronary circulation (RVDCC) via the coronary artery fistulas.
2
Surgical repair by establishment of right ventricle-to-pulmonary artery continuity
can be fatal when the resulting reduction in right ventricular pressure significantly
compromises myocardial perfusion.
2
,
6
,
7
Management options for infants with RVDCC include aortopulmonary shunts, staged surgery
for single ventricle palliation, aorta-to-right ventricle shunts, and cardiac transplantation,
although outcome data in this rare condition are limited.
3
,
8
,
9
,
10
,
11
,
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Since 1986, we have pursued staged surgical palliation directed toward a Fontan procedure
for all patients with PA/IVS and RVDCC. We now report intermediate-term follow-up
data for this cohort.To read this article in full you will need to make a payment
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References
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Article info
Publication history
Published online: August 16, 2004
Accepted:
June 16,
2000
Received in revised form:
June 16,
2000
Received:
April 3,
2000
Footnotes
☆Dr. Powell was supported in part by Grant 2T32HL07527-123A from the National Institutes of Health–National, Heart, Lung, and Blood Institute, Bethesda, Maryland.
Identification
Copyright
© 2000 Excerpta Medica Inc. Published by Elsevier Inc. All rights reserved.
