Ligamentous atresia of the left side of a double arch distal to the left subclavian artery is a rare form of vascular ring, which can easily be confused, on transthoracic echocardiography, with the right-sided aortic arch when there is mirror-imaged branching. Because of its rapid acquisition, computed tomographic angiography with three-dimensional reconstruction has now become the modality of choice for accurate diagnosis of the various forms of double aortic arch. It can be performed without sedation in any age group, including neonates. It provides excellent visualisation of the aortic arch and its branching pattern, thus permitting accurate diagnosis and surgical planning. We present a case series of six children with this rare vascular ring assessed using CT, highlighting their outcomes.

Tetralogy of Fallot with an aortopulmonary window and double aortic arch is very rare. This complex coexistence may be over a wide clinical spectrum. Herein, we present an asymptomatic 8-day-old infant who was diagnosed as having tetralogy of Fallot, double aortic arch, and an aortopulmonary window using transthoracic echocardiography while being examined for microcephaly.

Respiratory symptoms are prevalent in vascular ring anomalies, including double aortic arch, which is considered the most common type of vascular ring. However, respiratory failure owing to critical airway obstruction in the neonatal period is rare with few reported cases in the literature. We report a case of a newborn patient who required emergent intubation within the first few hours of life and was later discovered to have a double aortic arch.

In this article, we report a rare case of double aortic arch. The case presented initially with a foreign object in the oesophagus. The patient was a 2-year-old boy, who was referred with primary symptoms of tussis (15 days) and emesis (2 days). He had a history of ingesting a coin. Routine chest X-ray indicated a rounded, metal foreign object in the upper oesophagus. A half-Yuan coin was removed by gastroduodenoscopy. Echocardiographic imaging suggested that the patient had double aortic arch, which was subsequently diagnosed by CT angiography with three-dimensional reconstruction. The right subclavian artery arose from the right loop of the double aortic arch. The left subclavian artery as well as left and right common carotid arteries had distinct origins from the left aortic arch. Imaging also indicated atresia of the distal left arch. The patient underwent corrective surgery and made a full recovery. Despite the rarity, double aortic arch should be considered when patients present with a foreign object in the oesophagus. Echocardiography and CT angiography can inform the diagnosis.

No reports on pulmonary atresia with ventricular septal defect with the combination of double aortic arch and interruption between left and right carotid arteries have been published so far.

Crossed pulmonary arteries is a rare, benign congenital anomaly. Both pulmonary arteries cross each other on their course to each respective lung, thus forming a crisscross pattern. We report an infant with crossed pulmonary arteries and a complete vascular ring formed by double aortic arch.

Congenital heart disease is associated with congenital diaphragmatic hernia, but diagnosis by echocardiography can be difficult. We present the unusual case of a patient with a double aortic arch and congenital diaphragmatic hernia diagnosed using cardiac magnetic resonance imaging.

We present a case of double aortic arch with a predominant right and a double arterial duct detected by echocardiogram in a 28-week gestation foetus. The first evaluation revealed that both arches were perfused; the 1-month postnatal echocardiogram showed the closure of both arterial ducts and the partial obliteration of the left aortic arch between the left subclavian artery and the dorsal aorta. In our case, the postnatal obliteration of the left arch in a double aortic arch was probably due to the closure of the left-sided arterial duct.

Vascular rings are rare vascular congenital anomalies causing oesophageal and tracheal compression. An aortoesophageal fistula is a devastating, in part iatrogenic, complication of vascular rings. It is seen with increasing frequency, and can be misleading, since differential diagnosis with other causes of haematemesis and melaena is often difficult, especially in infants. We report two infants with aortoesophageal fistulas secondary to double aortic arches forming a vascular ring. In both, the diagnosis was missed, and massive haemorrhage led to death. In both cases, the fissuration on the oesophageal and aortic sides of the fistula had sharp edges, highly suggestive of an iatrogenic laceration caused by manipulation of nasogastric tubes. The key for the diagnosis of vascular rings is, therefore, clinical suspicion and awareness of this condition. Prompt identification in infants with stridor, wheezing, or respiratory distress can prevent prolonged intubation, thus avoiding the formation of an aortoesophageal fistula and hopefully preventing a fatal outcome.