Scimitar syndrome is a rare congenital anomaly consisting in part of right pulmonary
venous return to the inferior vena cava. There is a clear bimodal presentation of
this syndrome with either an infantile manifestation or a pediatric/adult form. The
infantile variant is marked by a higher incidence and severity of associated defects,
heart failure, pulmonary hypertension, and significant mortality. The patient with
the pediatric/adult form is less severely affected and may be asymptomatic on diagnosis.
In this article, we review the historical aspects, presentation, and pathophysiology
of Scimitar syndrome and discuss available treatment strategies. We emphasize the
safe and effective approach developed at Indiana University that obviates both the
need for an intra-atrial baffle or use of cardiopulmonary bypass. The results with
our alternative approach to Scimitar syndrome are summarized and they compare favorably
with other published reports.
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© 2006 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
