The Natural and Unnatural History of Congenital Aortic Arch Abnormalities Evaluated in an Adult Survival Cohort

Joyce E. Lodeweges, Frederik G. Dikkers, Barbara J. M. Mulder, Jolien W. Roos-Hesselink, Hubert W. Vliegen, Arie P. J. van Dijk, Gertjan T. Sieswerda, Thelma C. Konings, Rolf M. F. Berger, Dirk-Jan Slebos, Tjark Ebels, Joost P. van Melle

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Background: This study describes the different types of congenital vascular rings according to their anatomy, symptoms, and age at clinical onset and reports the surgical outcomes. Methods: A retrospective observational database study was conducted, reviewing the medical charts of 69 adult survivors with a history of a vascular ring, identified from the Dutch Congenital Cor vitia database. Results: Median age at presentation was 8.5 years (0-53.0 years). Thirty patients (43.5%) had a “left aortic arch with aberrant right subclavian artery,” 21 patients (30.4%) a “double aortic arch,” and 16 patients (23.2%) a “right aortic arch with aberrant left subclavian artery.” The main symptomatology at presentation comprised respiratory symptoms (82.9%). Almost three-quarters of patients were also diagnosed with asthma/bronchial hyperreactivity. Patients with a double aortic arch had more symptoms than patients with a left aortic arch with aberrant right subclavian artery and right aortic arch with aberrant left subclavian artery (P < 0.001), requiring surgery most often (P < 0.001). In patients with childhood onset of symptoms, preoperative spirometry (ie, peak expiratory flows) was more often abnormal as compared with adult patients (P = 0.007). Surgery was performed in 42.0% of all patients at a median age of 17 years (0-63.0 years). Twenty-four (92.3%) of the operated patients showed improvement or complete relief of symptoms shortly after surgery. Of 26 asymptomatic nonoperated patients, 3 patients (11.5%) eventually developed symptoms. Conclusions: The low incidence of vascular rings, their anatomic heterogeneity, and a wide range of common symptoms often lead to misdiagnosis. Clinical awareness is warranted as a large subset of patients could benefit from surgery, even at an adult age.
Original languageEnglish
Pages (from-to)438-445
Number of pages8
JournalCanadian Journal of Cardiology
Issue number4
Publication statusPublished - 1 Apr 2019

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