Double aortic arch with right positioned descending thoracic aorta and coexistent aortic kinking.

To cite: Ricciuti B, Rebonato A, Capodicasa E. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/ bcr-2015-213270 DESCRIPTION An 82-year-old woman presented with fever, severe dyspnoea and pneumonia, which required noninvasive ventilation and intravenous antibiotics. She had a history of recurrent airway infections with inspiratory stridor and dysphagia. During the pneumonia work up, in order to unveil a predisposing condition for pneumonia, and rule out alternative diagnoses, a high resolution CT scan of the chest revealed the presence of a double aortic arch. Following this finding, a contrast-enhanced threedimensional CT scan confirmed the diagnosis of incomplete double aortic arch (IDAA) (figure 1) with right positioned descending thoracic aorta and coexistent aortic kinking (figure 2). IDAA is a rare congenital abnormality resulting from atresia rather than complete involution of the distal left arch in a fibrous cord between the left arch and descending thoracic aorta. This anatomic anomaly is due to the persistence of the fourth right and left arches and dorsal aortas, resulting in the abnormal formation of vascular rings encircling the trachea and oesophagus. IDAA is generally diagnosed in childhood because of the early onset of symptoms related to tracheal and oesophageal obstruction. Anecdotal cases have been reported in adult patients, who usually became symptomatic because of asthma-like symptoms or swallowing difficulties as a consequence of the compression of the trachea and oesophagus by the abnormal aortic arches. Our patient was evaluated at the cardiac surgery unit, but because of age, comorbidities and the mildness of her symptoms, medical management of potential IDAA-related complications and shortterm follow-up were chosen.