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.

An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

Right aortic arch in association with coarctation of the aorta and vascular ring is a rare anatomy. We present an infant with chromosome 22q11.2 deletion, who had the right aortic arch with retroesophageal aberrant left subclavian artery and left internal carotid artery. The left external carotid artery and right common carotid artery originated together from the ascending aorta as a bovine branch. The infant also had severe coarctation, which was prostaglandin dependent.

The right aortic arch is a congenital vascular anomaly, which may form a vascular ring. However, prenatal identification of the branching pattern of brachiocephalic vessels is often limited. In this paper, we clearly demonstrated the branching pattern of brachiocephalic vessels in a case of right aortic arch with an aberrant left subclavian artery using HDlive Flow imaging.

In this report, a unique case of a symptomatic vascular ring formed by right aortic arch, aberrant left subclavian artery, and left ligamentum arteriosus in which there is atresia of the proximal left subclavian artery is described. Imaging modalities were non-diagnostic and the patient was sent to surgery based on strong clinical suspicion. Her anatomy was delineated in the operating room and the ring was successfully repaired.

A male neonate with d-transposition of the great arteries was diagnosed with the concomitant anomaly of left circumflex aortic arch and right-sided ductus arteriosus, which formed a vascular ring. Initial postnatal echocardiography had demonstrated an obscured aortic isthmus mimicking coarctation of the aorta, which could be a diagnostic clue to circumflex aortic arch.

Pulmonary artery slings and vascular rings are very rare congenital anomalies. It is even rarer to have both anomalies in the same setting. We present a case of a toddler who was diagnosed with a left pulmonary artery sling and a vascular ring as part of the screening process for the VACTERL association – co-occurrence of vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb malformations. He underwent a successful surgical repair via median sternotomy and on cardiopulmonary bypass with an uneventful postoperative course.

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.

We present two interesting cases of isolated left subclavian artery from the pulmonary artery with symptoms of upper airway obstruction. The first patient had tetralogy of Fallot, pulmonary artery sling, bilateral superior caval veins, and left bronchial isomerism, suggesting heterotaxy syndrome. The second patient had a right aortic arch, isolated left subclavian artery, and bilateral arterial ducts. These two cases are interesting because of their rarity and uncommon presentation.

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.

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.

An 8 year old girl presents with cyanosis, exertional dyspnoea, and palpitation only. X-ray displayed a typical coeur en sabot heart and auscultsation revealed a harsh ejection systolic murmur. Echocardiography confirmed Tetralogy of Fallot (McGoon ratio 1.3) and computed tomography was requested for further investigation of pulmonary stenosis. A vascular ring was detected, presenting asymptomatically and previously missed through echocardiography. The patient underwent a double-stage repair with ligation of an aortic arch. Recovery was unremarkable. This case highlights the limitations of echocardiography in detection of extra-cardiac anomalies.