Īmong all patients with RAA, a total of 65% of them have a true vascular ring due to the presence of a retroesophageal left subclavian artery with a left ligamentum arteriosum (LLA). ![]() ![]() Less common complete VR subtypes are: RAA with mirror-image branching (MB), and retro-oesophageal course of the left- sided arterial duct between the right-sided descending aorta and the left pulmonary artery circumflex retro-oesophageal aortic arch, and left aortic arch (LAA) with aberrant right subclavian artery (rSa) when the arterial duct is right-sided (RLA). ![]() Innominate artery (IA) compression accounts 3 to 20% of cases of incomplete vascular ring, followed by left pulmonary artery (LPA) sling. Īs reported by recent studies, right aortic arch (RAA) with aberrant left subclavian artery (ALSA) is the most common complete VR, followed by double aortic arch (DAA). Vascular ringsĭue to the strong relationship of the thoracic vascular structures with the trachea and bronchi, any anatomic variation leading to complete or incomplete VR may produce several grade of EAC that appears typically pulsatile and located in characteristic positions. When prenatal diagnosis is not available and reported symptoms are suspicious for EAC by VR, a complete diagnostic workup should be considered by clinicians. Additional symptoms such as failure to thrive and dysphagia are reported in patients with oesophagus compression. Otherwise, tracheal stenosis due to complete cartilaginous rings is found in association with left pulmonary artery sling (LPA sling).Īpparent life-threatening events (ALTE), apnoea, respiratory distress during airway infection and need of intubation, recurrent respiratory infections, barky cough, stridor, wheezing, and dyspnoea on exertion are all symptoms affecting patients with airway compression, associated malacia and tracheal stenosis due to VR. Extrinsic airway compression (EAC) and malacia are common complications of vascular rings. Though most VRs are isolated, congenital heart defects and chromosomal abnormalities can be associated. Their real incidence is difficult to establish as the clinical presentations are non-specific and range from early onset of symptoms to asymptomatic clinical setting into adulthood. Vascular rings (VRs) account for < 1% of congenital cardiac defects. Early diagnosis and referral to specialized centres can prevent the long-term complications and improve the respiratory outcomes of these patients.Ībnormalities in position and/ or branching of the aortic arch can lead to a complete or incomplete vascular ring (VR) that encircles and compresses the trachea, the bronchi and/or the oesophagus. As they are nonspecific and therefore difficult to recognize, attention should be given to all children with history of respiratory distress, extubation failure, noisy breathing, and recurrent respiratory infections. Recurrent respiratory infections, wheezing, atelectasis, and hyperinflation are mostly reported. Clinical presentation depends on the severity of the tracheal lumen reduction and the presence of associated tracheomalacia. ![]() Respiratory morbidity related to external airways compression is a major concern in children affected by vascular rings. Abnormalities in position and/or branching of the aortic arch can lead to vascular rings that may cause narrowing of the tracheal lumen due to external compression, or constriction of the oesophagus, causing symptoms that vary in relation to the anatomical vascular pattern and the relationship between these structures.
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