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Mid-aortic Syndrome in a Pediatric Cohort

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Abstract

Mid-aortic syndrome (MAS) is an uncommon condition characterized by severe narrowing of the abdominal aorta, usually involving visceral and renal arteries. Most patients are asymptomatic and typically present with incidental hypertension which might evolve into end-organ damage if untreated. Our aim was to review 8 new pediatric MAS cases. A retrospective observational study of all pediatric patients with MAS diagnosis (April 1992–November 2021) was conducted. Patients underwent systematic evaluation (medical and family history; 12-lead electrocardiogram; echocardiogram; angiography and/or computed tomography or magnetic resonance angiography). 8 pediatric patients with MAS were included. Median age at diagnosis was 2.6 [0.2–4.7] years; median follow-up time was 8.6 [6.6–10.0] years. 6/8 patients presented with incidental hypertension, 1/8 with heart murmur, and 1/8 with heart failure symptoms. All patients were on antihypertensive treatment. 1/8 patients underwent surgery and 7/8 an endovascular treatment. At the end of the study period, among the 6 patients that underwent a successful endovascular procedure, 2 achieved good blood pressure (BP) control, 2 acceptable BP control, 1 stage 1 hypertension and, another, stage 2 hypertension. There was 1 death during follow-up. BP monitoring in pediatric patients is crucial for early recognition of MAS. Treatment should be based on the individual clinical characteristics of patients with careful planning of surgical revascularisation, if possible, after adult growth is completed. Our study demonstrates that endovascular treatment might be a good alternative to surgery. Nevertheless, further trials with larger sample size and longer-term follow-up are required to determine the best treatment approach.

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Authors and Affiliations

  1. Department of Paediatric Cardiology, Consorci Sanitari del Maresme, Hospital de Mataró, Barcelona, Spain

    Laia Brunet-Garcia

  2. Department of Paediatric Cardiology, Hospital Sant Joan de Déu, Passeig de Sant Joan de Déu, 2, Esplugues de Llobregat, 08950, Barcelona, Spain

    Fredy Hermógenes Prada Martínez, Joan Sanchez-de-Toledo & Juan Manuel Carretero Bellon

  3. Department of Cardiology, Hospital Clínic de Barcelona, Barcelona, Spain

    Angela Lopez Sainz

  4. Department of Paediatric Cardiology, Hospital Universitari Joan XXIII, Universitat Rovira I Virgili, Tarragona, Spain

    Juan Manuel Carretero Bellon

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  1. Laia Brunet-Garcia
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  2. Fredy Hermógenes Prada Martínez
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  3. Angela Lopez Sainz
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  4. Joan Sanchez-de-Toledo
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  5. Juan Manuel Carretero Bellon
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Contributions

L-BG is the primary author, involved in participants’ recruitment, drafted the manuscript, image acquisition, and image preparation. F-HP involved in participants’ recruitment, image acquisition, and critical revision of the manuscript. A-LS involved in participants’ recruitment and critical revision of the manuscript. J-SdT involved in critical revision of the manuscript. All authors have read and approved the final manuscript. JM-CB is responsible for the overall content and involved in participants’ recruitment, image acquisition and image preparation, and critical revision of the manuscript.

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Correspondence to Juan Manuel Carretero Bellon.

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The authors have no conflicts of interest to declare.

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This study was approved by the institutional Research Board of Hospital Sant Joan de Déu and consent waived in view of the retrospective data collection.

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Brunet-Garcia, L., Prada Martínez, F.H., Lopez Sainz, A. et al. Mid-aortic Syndrome in a Pediatric Cohort. Pediatr Cardiol 44, 168–178 (2023). https://doi.org/10.1007/s00246-022-03036-2

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  • DOI: https://doi.org/10.1007/s00246-022-03036-2

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