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The Journal of Thoracic and Cardiovascular Surgery, Vol 104, 189-194, Copyright © 1992 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
VA Starnes, ML Griffin, PT Pitlick, D Bernstein, D Baum, K Ivens and NE Shumway
Over the past 3 years, 35 newborn infants have been referred for surgical
management of hypoplastic left heart syndrome. Surgical palliation
(first-stage Norwood) or cardiac transplantation was offered. Twenty-four
families (68%) chose palliation and 11 families (32%) chose cardiac
transplantation. Of the 11 infants listed for cardiac transplantation, five
underwent transplantation. Because of a lack of donors after an average
wait of 25 days (19 to 31), the remaining six infants underwent palliation,
with no perioperative deaths. Of the 30 infants undergoing palliation,
including crossovers, 20 (67%) survived the first operative stage. Among
the last 19 infants undergoing palliation in 1990, the early survival was
84%. Risk factors determined for poor outcome were year of operation (p
less than 0.001) and circulatory arrest time greater than 50 minutes (p
less than 0.001). Among the 13 infants undergoing palliation with a
circulatory arrest time of less than 50 minutes, there were 12 survivors
(92%); among 12 having a circulatory arrest time of more than 50 minutes,
there were four survivors (33%). At intermediate follow-up, six infants
have undergone second-stage procedures (Glenn), with five survivors. There
were eight late deaths, four caused by respiratory infections and four
caused by cardiac problems, including a thrombosed shunt in one infant.
Three of five infants are alive and doing well after cardiac
transplantation. Size of aorta, tricuspid regurgitation, and ventricular
wall thickness did not prove to be risk factors. Given the existing data,
we believe these infants should be managed selectively on the basis of
donor availability and family wishes.
ARTICLES
Current approach to hypoplastic left heart syndrome. Palliation, transplantation, or both?
Department of Cardiothoracic Surgery, Stanford University Medical Center, Calif.
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