We present a 22-year-old male patient, a history of a right bidirectional Glenn procedure (BDG), with dyspnea, cyanosis, palpitations and chest pain. Transthoracic echocardiography and computed tomography showed aneurysmal dilation of the right superior venous system. BDG takedown was performed with resection of the aneurysmal superior vena cava and reconnection of the superior vena cava to the right atrium and the creation of a central systemic-to-pulmonary shunt. The patient died from complications derived from a massive collateral circulation.

Background: The association of double aortic arch and common arterial trunk is extremely rare. The initial surgical approach depends on the patient’s clinical condition and associated cardiac anomalies. Aim: To report a rare association of common arterial trunk with double aortic arch in a 4-month-old female infant. Methods: description of case of a rare association where double aortic arch was not diagnosed initially, surgical repair was done successfully. Results and conclusions: associated cardiovascular anomalies may have an impact on management and outcome. Magnetic resonance imaging and computed tomography may be useful in assessment of this rare association. Complete repair has favorable outcome.