Clinical and imaging characteristics of isolated subclavian artery in pediatric patients

Abstract

To describe clinical and imaging characteristics of an isolated subclavian artery (ISA) in pediatric patients. Background: ISA is a rare congenital aortic arch anomaly defined as a loss of connection between the subclavian artery and aorta. The clinical manifestations and complications of ISA in children are unclear. Methods: This retrospective study included clinical and imaging data of ISA patients younger than 18 years whose data were recorded in the electronic radiology database during January 2006-August 2019. Results: Of 102 enrolled patients, 59 had been diagnosed in the first year of life. The majority of the patients also had congenital heart diseases, of which tetralogy of Fallot was the most common. The vertebral artery and collateral branch of the descending aorta served as blood flow supplies in 94 and 8 patients with ISA, respectively, as confirmed using computed tomography or magnetic resonance imaging. However, the blood supply did not influence the development of ISA. Eleven patients exhibited mild or moderate stenosis of the ISA, although only two exhibited coldness or a blood pressure gradient in the upper extremities. These two symptomatic patients also presented with patent ductus arteriosus, and this association was significant (P = 0.008). Conclusion: ISA management is often determined based on symptoms and associated congenital heart diseases. The ISA is prone to stenosis in patients with ipsilateral patent ductus arteriosus. We recommend early surgical ligation or percutaneous closure of the ductus arteriosus in patients with ISA.

Right-Sided Aortic Arch With Isolated Left Subclavian Artery, Subclavian Steal Syndrome, and Right Bronchial Compression: A Case Report

“…The initial segment of the embryonic left arch transforms into the left common carotid artery, emerging as the foremost branch of the right arch, followed by the right common and right subclavian arteries sequentially [5]. The blood supply to the left subclavian will be from the pulmonary artery from a left ductus [1], which will close after birth due to reduced pulmonary resistance, after which flow can be from the ipsilateral vertebral artery in a retrograde manner or can be from a collateral branch arising from the aorta [6]. This reversal of blood flow in a vertebral artery associated with a reduction in blood supply to the brain is called 'Subclavian steal syndrome' [6].…”

“…Clinically, the majority of patients with isolated left subclavian artery are asymptomatic, with few patients presenting with shortness of breath and respiratory infections, and very rarely, they can present with coldness of the skin over the ipsilateral arm. However, later in life, as they develop atherosclerotic changes in the arteries, they can get symptoms of vertebrobasilar insufficiency [6]. With a right aortic arch, the trachea can be compressed by the main pulmonary trunk, aortic arch, and ligamentum arteriosus [7].…”