AORTIC-ARCH COMPLEX ANOMALIES - 20-YEAR EXPERIENCE WITH SYMPTOMS, DIAGNOSIS, ASSOCIATED CARDIAC DEFECTS, AND SURGICAL REPAIR

To better understand the clinical presentation and surgical management of children with aortic arch complex anomalies (''vascular rings''), a retrospective study was performed on patients who presented to Child ren's National Medical Center between the years 1969 and 1989. A total of 59 patients were identified ranging in age at admission from birth to 14 years, of whom 35 (59%) were male and 24 (41%) female. Patients were classified into four major subtypes based on their surgical anat omy, with 29 (49%) patients having right aortic arch and left ductus/l igamentum arteriosus, 21 (36%) double aortic arch, 6 (10%) anomalous l eft pulmonary artery, and 3 (5%) arch artery anomaly (no ring); 19 pat ients (32%) had associated cardiac defects. The mean (+/-SD) age at on set of symptoms was 4.6 +/- 14.0 months, and the age at surgical repai r was 18 +/- 34 months. There were no intraoperative mortalities, but two late deaths occurred, Three (5%) patients had a surgical complicat ion, In contrast to previous studies, the incidence of nonring lesions was lower and associated cardiac defects higher. Forty-nine percent o f patients had symptoms present at birth, and patients with associated cardiac disease did not present earlier than those without. In patien ts with right aortic arch and left ductus/ligamentum arteriosus, few h ad an anomalous left subclavian artery. Finally, equal dominance of th e arches was most frequent in patients with double aortic arch, Aortic arch complex anomalies present symptomatically in a variety of ways, and noninvasive methods are used to identify the specific lesion and a ssociated cardiac defects. Surgical repair is associated with low or n o mortality in patients with uncomplicated aortic arch complex anomali es.