Clinical Study of 11 Patients with Double Aortic Arch

Abstract

Background: Double aortic arch (DAA), a typical vascular ring, causes dyspnea and dysphasia, regardless of whether DAA is complete or incomplete (atretic arch of one side remaining as a ligament). We report clinical findings on diagnostic imaging and the perioperative course in our cases of DAA.
Method and Results: We retrospectively studied 11 patients with DAA (6 males and 5 females) between July 1975 and September 2012. Their ages ranged from 3 months to 9 years, excluding 2 fetuses.
Results：Complete DAA was diagnosed in 4 patients, and incomplete DAA was diagnosed in 7. Respiratory symptoms were present in all patients, excluding the 2 fetuses, and dysphasia was present in only 1 patient. Contrast-enhanced computed tomography and three-dimensional reconstruction imaging showed posterior and inferior distortion of the subclavian artery due to traction by an atretic arch and Kommerell diverticulum in all patients with incomplete DAA. Percent diameter tracheal stenosis (the tracheal diameter of the narrowest part divided with the one of the widest part) was measured by computed tomography images in 4 patients of the 10 operative patients. The average of percent diameter tracheal stenosis in the operation group was 48％ and a result of 59％ was recorded in one observation patient. In all of 3 patients with lower than 50％ , the symptoms worsened while waiting for operation； on the other hand, their symptoms did not worsen in 2 with the percentage higher than 50％ , and one of them has been followed up without operation. Ten patients received operation at a median age of 2 years 3 months. Respiratory symptoms improved in all patients, but mild symptoms persisted in 3 who were observed after operation for one year or shorter and 2 of 7 observed longer than one year.
Conclusion: The contrast-enhanced computed tomography and three-dimensional reconstruction imaging are useful for diagnosis of iDAA. Early surgery is desirable for patients with severe stenosis of trachea, because symptoms may worsen while waiting for operation. All patients improved after operation, but some had persistent mild symptoms, and thus long-term observation is necessary.