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, C. Marcelletti

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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.

Original languageEnglish
Pages (from-to)35-39
Number of pages5
JournalAnnals of Thoracic Surgery
Issue number1
Publication statusPublished - 1987

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery


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