Non-specific aortoarteritis (NSAA) in children: a prospective observational study

Author:

Gupta Himanshu,Kaur Navjyot,Saxena Anita,Jagia Priya,Kumar Sanjeev,Gupta Saurabh Kumar,Sharma Sanjeev,Kothari Shyam S,Ramakrishnan SivasubramanianORCID

Abstract

ObjectiveProspective data on clinical profile, natural history and outcomes of NSAA (non-specific aortoarteritis) in children is limited. We initiated this prospective study to evaluate the short-term and medium-term outcomes of NSAA in children.DesignProspective observational study.SettingTertiary care hospital in India.PatientsWe included 28 consecutive children (<15 years) with a diagnosis of NSAA.Main outcome measuresClinical profile, symptoms, left ventricular ejection fraction (LVEF), the pattern of vessel involvement, complications and markers of disease activity were assessed at the time of diagnosis. All the patients underwent treatment and interventions as per the current guidelines and were followed up for a mean duration of 13.5±6.7 months for disease activity and outcomes.ResultsThe mean age of the cohort was 10±2.9 years (14 boys). Twenty (71%) patients had hypertension. Half of the children presented with acute decompensated heart failure (ADHF). Only 21 patients (75%) met Sharma et al modified Ishikawa criteria for the diagnosis. The children with active disease (36%) were managed with immunosuppressive drugs. Percutaneous and surgical interventions were performed in 26 (93%) children. New York Heart Association functional class, LVEF and control of hypertension improved in most children on follow-up. Four children developed vascular restenosis requiring reinterventions. There was no death during mid-term follow-up.ConclusionsChildren with NSAA, unlike adults seldom present with classical features of the disease. ADHF and ventricular dysfunction are strikingly common in children. Appropriate immunosuppressive therapy for active disease and timely intervention improves clinical outcomes over a medium term follow-up. Future studies assessing long-term outcome are needed.

Publisher

BMJ

Subject

Pediatrics, Perinatology, and Child Health

Cited by 3 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

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2. COVID-19 associated renal artery stenosis in infancy - A report of two cases;Annals of Pediatric Cardiology;2023

3. Childhood Takayasu’s Arteritis;Tropical Cardiovascular Pathology;2022

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