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Rohinton K. Balsara
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Ann Thorac Surg 1984;38:146-150
© 1984 The Society of Thoracic Surgeons

Articles

Congenital Cardiac Anomalies Associated with the DiGeorge Syndrome: A Neonatal Experience

Louis M. Marmon, M.D., Rohinton K. Balsara, M.D., Robert Chen, M.D., Jeffrey M. Dunn, M.D.*

Section of Pediatric Cardiothoracic Surgery, St. Christopher's Hospital for Children, and Temple University School of Medicine, Philadelphia, PA

Accepted for publication February 10, 1984.

* Address reprint requests to Dr. Dunn, Section of Pediatric Cardiothoracic Surgery, St. Christopher's Hospital for Children, 5th and Lehigh Ave, Philadelphia, PA 19133

The DiGeorge syndrome is a rare congenital abnormality of absent or hypoplastic thymus and parathyroid glands. Thirty neonates who had cardiac lesions and the DiGeorge syndrome are reviewed. The early mortality for 10 neonates undergoing palliative procedures was 80%. Seventy-five percent of the deaths were secondary to sepsis.

Twenty neonates did not undergo palliative procedures. In this group, early mortality was 60% and late mortality was 65%. Sixty percent of the deaths in this group were associated with sepsis, with cardiac failure responsible for the remaining deaths. Survival in both groups has improved with appropriate treatment of the immunological and metabolic consequences of the DiGeorge syndrome.




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