Mid-aortic syndrome in a paediatric cohort

Abstract

Abstract AimsMid-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 paediatric MAS cases.MethodsA retrospective observational study of all paediatric patients with MAS diagnosis (April 1992-Novembre 2021) was conducted. Patients underwent systematic evaluation (medical and family history; 12-lead electrocardiogram; echocardiogram; angiography and/or computed tomography or magnetic resonance angiography).Results8 paediatric patients with MAS were included. Median age at diagnosis 2.6 [0.2–4.7] years; median follow-up time 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.ConclusionBP monitoring in paediatric 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.