Therapeutic Options and Outcomes in Midaortic Syndrome: A Systematic Review and Meta-analysis

Published:October 07, 2022DOI:


      Midaortic syndrome (MAS) is narrowing of the distal thoracic and or abdominal aorta with congenital, inflammatory, or idiopathic aetiology. If left untreated, the prognosis is poor due to hypertensive complications. Follow up data after treatment are sparse, contrary to aortic coarctation. This study aimed to investigate hypertension during follow up after medical, endovascular, and surgical therapy in juveniles and adults.

      Data sources

      A meta-analysis of case series and reports was performed, focusing on the incidence of hypertension during the follow up of juvenile (i.e., age 0–17 years) and adult MAS patients after medical, endovascular, or surgical therapy.

      Review methods

      Search queries were performed in PubMed, Embase, and Web of Science, and eligible articles underwent quality control. Descriptive statistics were reported based on available data, and individual patient data meta-analyses were performed using a one stage approach, accounting for clustering by case series or decades of reporting for case reports. For the meta-analysis, missing outcome and aetiology data were multiply imputed.


      The number of juveniles and adults who underwent endovascular therapy (33.7% vs. 27.3%; p = .42) and surgery (52.2% vs. 58.0%; p = .46) was similar. At baseline, 92.4% of juveniles and 87.5% of adults were hypertensive, decreasing to 23.2% and 24.1% during a follow up of 23 months (juveniles) and 18 months (adults), respectively. More hypertension was found compared with surgery in juveniles after endovascular therapy (38.1% vs. 10.8%; p = .020). Meta-analysis also demonstrated a trend for hypertension after endovascular therapy in juveniles, whereas hypertension was more prevalent following surgery in adults compared with endovascular therapy or medication.


      This review and meta-analysis investigated therapeutic options for MAS in juveniles and adults. It found that complications and hypertension during follow up were more common in juveniles after endovascular treatment, whereas surgery in adults was associated with more hypertension.


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        • Connolly J.E.
        • Wilson S.E.
        • Lawrence P.L.
        • Fujitani R.M.
        Middle aortic syndrome: distal thoracic and abdominal coarctation, a disorder with multiple etiologies.
        J Am Coll Surg. 2002; 194: 774-781
        • Sethna C.B.
        • Kaplan B.S.
        • Cahill A.M.
        • Velazquez O.C.
        • Meyers K.E.
        Idiopathic mid-aortic syndrome in children.
        Pediatr Nephrol. 2008; 23: 1135-1142
        • Porras D.
        • Stein D.R.
        • Ferguson M.A.
        • Chaudry G.
        • Alomari A.
        • Vakili K.
        • et al.
        Midaortic syndrome: 30 years of experience with medical, endovascular and surgical management.
        Pediatr Nephrol. 2013; 28: 2023-2033
        • Forman N.
        • Sinskey J.
        • Shalabi A.
        A Review of middle aortic syndromes in pediatric patients.
        J Cardiothorac Vasc Anesth. 2020; 34: 1042-1050
        • Rumman R.K.
        • Nickel C.
        • Matsuda-Abedini M.
        • Lorenzo A.J.
        • Langlois V.
        • Radhakrishnan S.
        • et al.
        Disease beyond the arch: a systematic review of middle aortic syndrome in childhood.
        Am J Hypertens. 2015; 28: 833-846
        • Monroe E.J.
        • Morray B.H.
        • Zhao Y.
        • Smith C.A.
        Middle aortic syndrome: pathogenesis, visceral manifestations, and management.
        Dig Dis Interv. 2018; 2: 231-239
        • Liberati A.
        • Altman D.G.
        • Tetzlaff J.
        • Mulrow C.
        • Gøtzsche P.C.
        • Ioannidis J.P.
        • et al.
        The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate healthcare interventions: explanation and elaboration.
        BMJ. 2009; 339: b2700
        • Page M.J.
        • McKenzie J.E.
        • Bossuyt P.M.
        • Boutron I.
        • Hoffmann T.C.
        • Mulrow C.D.
        • et al.
        The PRISMA 2020 statement: an updated guideline for reporting systematic reviews.
        BMJ. 2021; 372: n71
        • Ouzzani M.
        • Hammady H.
        • Fedorowicz Z.
        • Elmagarmid A.
        Rayyan—a web and mobile app for systematic reviews.
        Syst Rev. 2016; 5: 210
        • Moola S.
        • Munn Z.
        • Tufanaru C.
        • Aromataris E.
        • Sears K.
        • Sfetcu R.
        • et al.
        Chapter 7: Systematic reviews of etiology and risk. Appendix 7.3: Critical appraisal checklist for case series.
        in: JBI Manual for Evidence Synthesis [Internet]. 2020 (Available from:)
        Date accessed: October 22, 2022
        • Moola S.
        • Munn Z.
        • Tufanaru C.
        • Aromataris E.
        • Sears K.
        • Sfetcu R.
        • et al.
        Chapter 7: Systematic reviews of etiology and risk. Appendix 7.4: Critical appraisal checklist for case reports.
        in: JBI Manual for Evidence Synthesis [Internet]. 2020 (Available from:)
        Date accessed: October 22, 2022
        • Parodi J.C.
        • Palmaz J.C.
        • Barone H.D.
        Transfemoral intraluminal graft implantation for abdominal aortic aneurysms.
        Ann Vasc Surg. 1991; 5: 491-499
        • Makaroun M.S.
        • Dillavou E.D.
        • Kee S.T.
        • Sicard G.
        • Chaikof E.
        • Bavaria J.
        • et al.
        Endovascular treatment of thoracic aortic aneurysms: results of the phase II multicenter trial of the GORE TAG thoracic endoprosthesis.
        J Vasc Surg. 2005; 41: 1-9
        • Debray T.P.
        • Moons K.G.
        • Abo-Zaid G.M.
        • Koffijberg H.
        • Riley R.D.
        Individual participant data meta-analysis for a binary outcome: one-stage or two-stage?.
        PLoS One. 2013; 8e60650
        • Quartagno M.
        • Carpenter J.R.
        Multiple imputation for IPD meta-analysis: allowing for heterogeneity and studies with missing covariates.
        Stat Med. 2016; 35: 2938-2954
        • Nijdam M.-E.
        • Plantinga Y.
        • Hulsen H.T.
        • Bos W.J.W.
        • Grobbee D.E.
        • van der Schouw Y.T.
        • et al.
        Pulse pressure amplification and risk of cardiovascular disease.
        Am J Hyperten. 2008; 21: 388-392
        • Kim H.B.
        • Vakili K.
        • Ramos-Gonzalez G.J.
        • Stein D.R.
        • Ferguson M.A.
        • Porras D.
        • et al.
        Tissue expander-stimulated lengthening of arteries for the treatment of midaortic syndrome in children.
        J Vasc Surg. 2018; 67: 1664-1672