Management of a Patient with Double Aortic Arch and Severe Tracheal Compression

Vascular ring presents less than 1% of all congenital cardiovascular anomalies and may cause respiratory symptoms and feeding problems, double aortic arch (DAA) is the most common vascular ring (40%). The anomaly is due to a failure of regression of both the right and the left fourth branchial arches, resulting in right and left aortic arches which completely encircle and compress the trachea and esophagus, producing respiratory distress and feeding problems in early infancy (1, 2, 3, 4). The first post-mortem description of double aortic arch was in 1737 by Hommell. In 1945 Gross accomplished the first successful surgical intervention for a DAA (3). Double aortic arch is commonly an isolated anomaly but is occasionally associated with a variety of congenital heart defects (ventricular septal defect, transposition of the great arteries, tetrology of Fallot, coarctation of the aorta and chromosomal abnormalities as microdeletion of 22q11.2. The clinical course of the disease and its manifestation depends on the compression of the esophagus and trachea. The most common symptoms are persistent cough (78%), dyspnoe (75%), recurrent respiratory infections (56%), feeding problems (25-40%), and failure to thrive (5%). Symptomatic tracheobronchial compression varies inversely with the severity of compression. Tracheal compression causes airflow obstruction and decreased mucociliary clearance of secretions, leading to recurrent bronchopulmonary infections. Reflex apnoe is hypothesized to be a type of respiratory arrest that occurs when vagal afferent nerves are stimulated. Respiratory distress and a history of recurrent pulmonary infections and apneic spells are indications for surgical intervention (1, 2, 3). Division of the smaller of the two arches (usually the left) is performed through the left thoracotomy. There are three successful operations in the cases of vascular ring done in the Clinics for Pediatric cardiology and Cardiac surgery of University hospital for Children in Riga. We present the one of the cases with successful treatment of severe tracheal compression.