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Ann Thorac Surg 1987;44:35-39
© 1987 The Society of Thoracic Surgeons

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Articles

Palliation of Cardiac Malformations Associated with Right Isomerism (Asplenia Syndrome) in Infancy

From the Dipartimento Medico-Chirurgico di Cardiologia Pediatrica and Servizio di Anatomia Patologica, Ospedale Pediatrico Bambino Gesú, Roma, Italy

Accepted for publication November 26, 1986.

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 < .02). Guidelines for the palliative management of right isomerism are suggested.

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