Anomalies of the aortic arch complex (vascular rings) occur in multiple forms, often forming rings that cause tracheal obstruction and respiratory distress. The symptoms are usually non-specific but are potentially lethal. Echocardiography with a barium swallow or 3-D computerized tomography is feasible and accurate in the early diagnosis of congenital aortic arch complex anomalies. Early management to relieve the tracheobronchael compression to prevent mental retardation is mandatory.
Though the integrated school-based health screening survey of the prevalence of congenital and acquired heart disease in 92,884 native born school children at Taichung Country, Taiwan (from June 1, 2002 to August 31, 2003), 55 patients of undiagnosed vascular ring were found by portable echocardiographic examination with Agilent opti-Go echocardiography among the children with mental retardation. After the diagnosis of a vascular ring, patients underwent spiral CT scan with 3-D reconstruction to evaluate the severity of airway compression. In compression to clinical symptoms if any and the severity of tracheobroncheal compression, 35 out of 55 patients with a vascular ring underwent surgical correction.
Thrity-five patients(20 males and 15 females; age ranged from 2 months to 45 years with mean of 9.98 years) underwent surgical correction of a vascular ring. No mortality resulted. Minor complications (3 chylothorax, 1 minor stridor, 2 with minimal shortness of breathing) were found during the follow-up (mean of 5.2 months).
Early diagnosis through health screening programs and early management of the congenital aortic arch complex anomalies will reduce the preoperative and postoperative morbidity and eliminate potential respiratory sudden death in these patients.
School children with suspected mental retardation associated with respiratory distress should undergo health screening with echocardiography. Early surgical management in patients with vascular rings to prevent t he deterioration of symptoms or sudden death is mandatory.
T.P. Tsai, None.