Abstract

This series describes three adolescent females who presented with chest pain and ventricular dysfunction related to acute coronary ischemia secondary to Takayasu's arteritis with varied courses of disease progression leading to a diverse range of therapies including cardiac transplantation. While Takayasu's arteritis is rare in childhood, it should be strongly considered in any adolescent female presenting with systemic inflammation and chest pain consistent with myocardial infarction. A high index of suspicion can lead to early detection and aggressive management of the underlying vasculitis reducing associated morbidity and mortality. The purpose of this report is to describe the challenges in the clinical diagnosis and management of Takayasu's arteritis with myocardial infarction. We also seek to enhance awareness about unique presentations of Takayasu's arteritis within the paediatric community.

Highlights

  • Takayasu’s arteritis is a chronic granulomatous vasculitis that primarily involves the aorta and its major branches

  • While limited information is available on the prevalence of coronary arterial involvement in children with Takayasu’s arteritis, recent studies estimate an incidence of approximately 5%

  • We present our case series of three adolescent females with Takayasu’s arteritis and myocardial infarction

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Summary

Introduction

Takayasu’s arteritis is a chronic granulomatous vasculitis that primarily involves the aorta and its major branches It is a rare disease with an incidence of 1–3 per million people in the USA and Europe and is most prevalent among females between the ages of 10–40 years.[1,2] Takayasu’s arteritis has been reported in infancy, the median age of disease onset is 12 years.[3] Diagnosis requires an abnormal angiography with positive findings on physical examination (e.g., pulse deficits, blood pressure discrepancies, bruits, or hypertension) or elevated inflammatory markers.[4]. Further evaluation with cardiac CT and MRI demonstrated proximal stenosis of the right coronary artery and a moderate to severe myocardial infarction in the distribution of the right coronary artery, with significant transmural involvement within the basal inferior, inferoseptal, and mid-inferior walls (Fig 1).

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