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The Annals of Thoracic Surgery, Vol 44, 35-39, Copyright © 1987 by The Society of Thoracic Surgeons

ARTICLES

Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy

R Di Donato, D di Carlo, C Squitieri, E Rossi, A Ammirati, B Marino and C Marcelletti

Right isomerism is characterized by the combination of an obstructed pulmonary outflow tract and a total anomalous pulmonary venous connection (TAPVC), which is obstructed in nearly half of the patients. Fourteen patients less than 2 years of age with right isomerism have been seen in our unit. Thirteen of them underwent palliation consisting of a systemic-pulmonary shunt (10 patients) or a combination of shunt and TAPVC repair (3). There were 7 hospital deaths (54%). The presence of obstructed pulmonary venous drainage was the major risk factor in the surgical treatment of these complex cardiac anomalies (p less than .02). Guidelines for the palliative management of right isomerism are suggested.

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