Isolation of the Left Subclavian Artery with Right Aortic Arch in Association with Bilateral Ductus Arteriosus and Ventricular Septal Defect

Abstract

Right aortic arch with isolation of the left subclavian artery is a rare anomaly. The incidence of bilateral ductus arteriosus is sporadic, and a right aortic arch with isolation of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare. Since the symptoms and signs of isolation of the left subclavian artery can include the absence or underdevelopment of the left arm, subclavian steal syndrome, or pulmonary artery steal syndrome, the proper therapeutic approach is controversial. We report a case in which surgical reconstruction was used to treat isolation of the left subclavian artery with right aortic arch in association with bilateral ductus arteriosus and a ventricular septal defect.

Right Aortic Arch and Isolated Left Subclavian Artery: Prenatal and Postnatal Echocardiographic and Tomographic Imaging

“…Alternatively, the LSCA may be also supplied by mediastinal or thoracic collateral vessels. 2 , 12

Figure 3 Diagram of theoretical double aortic arch, showing involution of left fourth aortic arch and left descending aorta (DAo), leaving the isolated LSCA supplied by the left ductus arteriosus. AAo , Ascending aorta.

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“…2 , 12 Patients can present with symptoms related to decreased perfusion of the left arm, such as pain, weakness, paresthesia, coldness, 2 or even underdevelopment of the limb. 12 On the contrary, they can also present with subclavian steal phenomenon, with retrograde runoff of vertebral artery flow, causing vertebrobasilar insufficiency. 1 , 2 , 13 This can manifest with vertigo, headache, or syncope.…”

Clues to echocardiographic diagnosis of isolation of right subclavian artery in a patient with DiGeorge syndrome and its transcatheter management with its associated anomalies

“…In the embryo, the right subclavian artery gets isolated from the aorta when the seventh inter-segmental artery separates from the right fourth arch and connects to the right sixth arch through the arterial duct. 7 Surgical re-implantation of the isolated vessel to aorta or ipsilateral carotid artery is a therapeutic option, which needs long mobilisation, division of ductal remnants, and may stretch the vessel leading to stenosis. 8 Transcatheter duct closure prevents the second steal, thereby improving the arm perfusion.…”

“…While other complex arch anomalies associated with tracheo-oesophageal compression will need a pre-interventional tomographic imaging, ducts in isolated subclavian artery are not known to compress airways. 7 The challenging echocardiographic identification of this anomaly is guided by three findings: 1) the absence of branching of the first aortic branch suggesting abnormal subclavian origin; 2) colour Doppler flows at an unusual location into the branch pulmonary artery due to ductal flows; and 3) reversed blood flows on transcranial Doppler in vertebral artery. Precise echocardiographic recognition of these three findings serves in diagnosis of this rare anomaly.…”

Anomalous Origin of the Right Subclavian Artery in a Patient with D-transposition of the Great Arteries

“…However, to our knowledge, no previous studies have described an anomalous origin of the subclavian artery combined with d-TGA in South Korea. Generally, ‘isolation’ should not be confined to a subclavian artery that does not connect to the aorta, but rather to the homolateral pulmonary artery by the ductus arteriosus, whether closed or patent [5,6]. In situations where pulmonary vascular resistance is high, there may be antegrade flow from the pulmonary artery to the RSCA, resulting in differential cyanosis with diminished oxygen saturation.…”