Clinical, radiological and functional follow-up after surgical decompression of double aortic arch

Background: Double aortic arch (DAA) is a congenital vascular anomaly that causes tracheal and oesophageal compression. It requires surgical intervention in patients with severe symptoms. Objective: To evaluate the clinical, radiological and functional follow-up after surgical relief of the compression. Materials and methods: Ten children (seven boys) with DAA were operated on at a mean age of 1.3 years (range 0.2-7.5). At a mean age of 10.1 years (range 5-18 years), a follow-up study was performed that included clinical, radiological and functional parameters. Results: Seven children reported only mild respiratory symptoms and some trouble with swallowing. Preoperative fluoroscopy with spot images showed the mean tracheal diameter at the level of stenosis to be 37+/-23% of the maximal diameter. At the time of follow-up, this was 70+/-13%. The mean of the oesophageal diameter was 39+/-20% preoperatively and 47+/-16% postoperatively. Maximal expiratory flow volume (MEFV) curves of seven children showed typical characteristics of intrathoracic upper airway obstruction. Mean peak expiratory flow was significantly reduced (77+/-10% of predicted, P < 0.0001). Bronchial hyperreactivity, tested by methacholine challenge, was found in two patients. Conclusions: There was marked relief of clinical symptoms after surgical decompression of DAA in all patients. In spite of this, radiological narrowing of trachea and oesophagus persisted and lung function results were abnormal at long-term follow-up.