Correction of absence of the aortopulmonary septum by creation of concentric great vessels.

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.