Isolation of arch vessels: Imaging characteristics and associations on multidetector computed tomography angiography

Abstract

Aim: To evaluate the imaging characteristics and associations in patients with isolation of arch vessels on multidetector computed tomography angiography (CTA). Materials and Methods: We retrospectively reviewed all multidetector CTA studies performed for the evaluation of congenital heart diseases (CHDs) at our institution from January 2014 to June 2020. Cases with isolation of arch vessels were identified. The isolated arch artery and its relationship with patent arterial duct, pulmonary artery, and aortic arch were characterized in addition to other associated intra-and extracardiac anomalies. Results: Isolation of arch vessels was seen in 14/3926 (0.36%) patients. Left subclavian artery (SCA) was the commonest isolated arch vessel, involved in 7/14 (50%) cases. Isolation of right SCA, left brachiocephalic artery, and left common carotid artery was seen in 4 (28.6%), 2 (14.3%), and 1 (7.1%) patient, respectively. The isolated arch vessel was seen associated with right aortic arch in 10/14 (71.4%) cases and was on the opposite side of aortic arch in all 14 (100%) patients. Right-sided nonrestrictive patent arterial duct was seen in 3/14 (21.4%) cases, left-sided nonrestrictive patent arterial duct was seen in 1/14 (7.1%) while a left-sided restrictive patent arterial duct was seen in 3/14 (21.4%) cases. Tetralogy of Fallot (ToF) was the commonest associated anomaly seen in 8/14 (57.1%) patients. Conclusion: Isolation of aortic arch branch vessels is rare, seen most commonly associated with ToF. Left SCA is the commonest involved vessel. CTA is useful not only in the diagnosis of isolation of arch vessels, but also in the presence or absence of associated anomalies which may impact the symptomatology, prognosis, and surgical management.

Prenatal diagnosis of isolation of aortic brachiocephalic artery

“…IABA is commonly associated with other intra-cardiac CHDs and chromosome abnormalities. Sinha et al reported that 57.1% (8/14) cases of ILSCA were combined with TOF, the most common intra-cardiac anomaly (4), which agrees with our results. Luetmer et al (3) reported that 59% (23/39) of cases with ILSCA were combined with CHD and TOF, accounting for 60.87% (13/24) of cases.…”

“…The ILSCA and IRSCA were reported by Barger et al in 1956 (15) and 1954 (16), respectively, while the first cases of ILIA were described by Levine et al in 1966 (17). The detection rate of the IABA in the cases of CHD was 0.15%, while Sinha et al reported that the prevalence of the IABA in the CHD population was 0.36% in a single center after birth (4). There may be two reasons for this: (i) some cases may be misdiagnosed due to a lack of awareness of IABA; and (ii) more cases with CHD were diagnosed prenatally, and some cases, which could have a more severe prognosis in comparison to cases with CHD that survive after birth, may be terminated pregnancies or die at an early age.…”

“…Männer et al (20) summarized that of 63.63% (7/11) of cases with ILIA were associated with CHD and septal defect more frequently. D-transposition of the great artery (4,5,7), DORV (21), interruption of the aortic arch with VSD (6), PA sling (22), and ductal arteriosus aneurysm (23) were also reported in cases of IABA.…”

“…Isolated aortic brachiocephalic artery (IABA) is characterized by the loss of continuity between the aorta and the brachiocephalic artery, which is continuously connected to the pulmonary artery (PA) by means of a patent or non-patent arteriosus ductus (DA) (1)(2)(3)(4). Most reports of cases of IABA were case reports (3)(4)(5)(6)(7)(8).…”

“…Isolated aortic brachiocephalic artery (IABA) is characterized by the loss of continuity between the aorta and the brachiocephalic artery, which is continuously connected to the pulmonary artery (PA) by means of a patent or non-patent arteriosus ductus (DA) (1)(2)(3)(4). Most reports of cases of IABA were case reports (3)(4)(5)(6)(7)(8). Isolated left subclavian artery (ILSCA) was the most common isolated arch artery, which accounts for only 0.8% of right aortic arch (RAA) anomalies, whose incidence in the general population itself is 0.1% (1,5) and the ILSCA occurs significantly more often than isolated right subclavian artery (IRSCA) (6).…”