The Annals of Thoracic Surgery, Vol 19, 180-190, Copyright © 1975 by The Society of Thoracic Surgeons
Correction of absence of the aortopulmonary septum by creation of concentric great vessels
PO Daily, NJ Sissman, MJ Lipton and NE Shumway
An 18-year-old patient is reported who had simultaneous occurrence of
aortopulmonary septal absence, complete interruption of the aortic arch,
and patent ductus arteriosus. This is the only known patient who has had
total anatomical correction of this combination of defects. Correction was
accomplished by staged procedures; the first stage consisted of
obliteration of the patent ductus and establishment of aortic continuity;
this was followed one year later by the second stage, which included a
unique method for repair of absence of the aortopulmonary septum. The
surgical desirability and embryological implications of criteria for
differentiating truncus arteriosus from aortopulmonary septal defect are
presented. Follow-up physiological evaluations and the patient's death from
the effects of increasing pulmonary vascular disease (despite early
symptomatic improvement) approximately two years after completion of the
repair underscore the importance of early diagnosis and operative
intervention.
