Prevalence and Complications of Aberrant Subclavian Artery in Patients With Heritable and Nonheritable Arteriopathies

An aberrant subclavian artery (ASA) (or lusoria) is the most common congenital anomaly of the aortic arch (0.5%-2.2%; female-to-male ratio 2:1 to 3:1). ASA can become aneurysmal and result in dissection, involving Kommerell’s diverticulum when present and the aorta. Data of its significance in genet...

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Published in:	Journal of the American College of Cardiology Vol. 81; no. 10; pp. 979 - 991
Main Authors:	Giuliani, Lorenzo, Di Toro, Alessandro, Urtis, Mario, Narula, Nupoor, Grasso, Maurizia, Pelenghi, Stefano, Belliato, Mirko, Bozzani, Antonio, Arici, Vittorio, Pellegrini, Carlo, Serio, Alessandra, Pilotto, Andrea, Fergnani, Viola, Antoniazzi, Elena, Magrassi, Lorenzo, Dore, Roberto, Valentini, Adele, Preda, Lorenzo, Calliada, Fabrizio, Quaretti, Pietro, Pirrelli, Stefano, Kodama, Takaide, Vricella, Luca, Cameron, Duke, Arbustini, Eloisa
Format:	Journal Article
Language:	English
Published:	United States Elsevier Inc 14-03-2023
Subjects:	aberrant subclavian artery Aorta, Thoracic dissection Diverticulum - complications Female genetic arteriopathies Heart Defects, Congenital - complications Humans Loeys-Dietz Syndrome Lusoria Male Marfan syndrome Marfan Syndrome - complications Prevalence Subclavian Artery - abnormalities Subclavian Artery - diagnostic imaging Vascular Diseases - complications ARSA ALSA aberrant subclavian artery Lusoria Marfan syndrome LAD dissection ASA Loeys-Dietz syndrome CTA EDS KD LDS MFS genetic arteriopathies
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Summary:	An aberrant subclavian artery (ASA) (or lusoria) is the most common congenital anomaly of the aortic arch (0.5%-2.2%; female-to-male ratio 2:1 to 3:1). ASA can become aneurysmal and result in dissection, involving Kommerell’s diverticulum when present and the aorta. Data of its significance in genetic arteriopathies are not available. The purpose of this study was to assess the prevalence and complications of ASA in gene-positive and -negative nonatherosclerotic arteriopathies. The series includes 1,418 consecutive patients with gene-positive (n = 854) and gene-negative arteriopathies (n = 564) diagnosed as part of institutional work-up for nonatherosclerotic syndromic and nonsyndromic arteriopathies. Comprehensive evaluation includes genetic counseling, next-generation sequencing multigene testing, cardiovascular and multidisciplinary assessment, and whole-body computed tomography angiography. ASA was found in 34 of 1,418 cases (2.4%), with a similar prevalence in gene-positive (n = 21 of 854, 2.5%) and gene-negative (n = 13 of 564, 2.3%) arteriopathies. Of the former 21 patients, 14 had Marfan syndrome, 5 had Loeys-Dietz syndrome, 1 had type-IV Ehlers-Danlos syndrome, and 1 had periventricular heterotopia type 1. ASA did not segregate with genetic defects. Dissection occurred in 5 of 21 patients with genetic arteriopathies (23.8%; 2 Marfan syndrome and 3 Loeys-Dietz syndrome), all with associated Kommerell’s diverticulum. No dissections occurred in gene-negative patients. At baseline, none of the 5 patients with ASA dissection fulfilled criteria for elective repair according to guidelines. The risk of complications of ASA is higher in patients with genetic arteriopathies and is difficult to predict. In these diseases, imaging of the supra-aortic trunks should enter baseline investigations. Determination of precise indications for repair can prevent unexpected acute events such as those described. [Display omitted]
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0735-1097 1558-3597
DOI:	10.1016/j.jacc.2023.01.005