Intended for healthcare professionals

Clinical Review Lesson of the week

Right sided aortic arch in children with persistent respiratory symptoms

BMJ 2000; 321 doi: (Published 16 September 2000) Cite this as: BMJ 2000;321:687

This article has a correction. Please see:

  1. Donald N R Payne, research fellowa,
  2. Christopher Lincoln, consultant cardiac surgeonb,
  3. Andrew Bush, reader (
  1. a Department of Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Trust, London SW3 6NP
  2. b Department of Cardiothoracic Surgery, Royal Brompton and Harefield NHS Trust
  1. Correspondence to: A Bush
  • Accepted 13 December 1999

A chest x ray film may point to an alternative diagnosis in children with persistent asthma

In most children with asthma, symptoms can be controlled with low dose inhaled steroids. Failure to respond should prompt other diagnostic considerations, including poor adherence to treatment, an unsatisfactory environment, or psychological factors. Diagnoses other than asthma should also be considered; often, extensive investigations are performed to exclude other diseases. We report on three children referred with persistent respiratory symptoms. Two of the children had been diagnosed as having asthma, which was insensitive to steroids. One of the children was thought to have interstitial lung disease, but because a radiological sign was missed on his chest x ray film he received inappropriate treatment and unnecessary investigations.

Case reports

Case 1— A 12 year old boy complained of shortness of breath on exercise, worsening over a year, with no other symptoms. Examination was normal. Bronchodilators and inhaled steroids had not helped. Previous investigations included chest radiography, computed tomography of the chest, echocardiography, exercise testing, full lung function tests, measurement of immunoglobulin concentrations, and skin prick testing for allergies. Interstitial lung disease had been suggested on the basis of reduced total lung capacity and residual volume. Review of the original chest x ray film, six months after his initial presentation, showed a right sided aortic arch, with tracheal compression. Repeat spirometry yielded reduced inspiratory and expiratory flow rates, with flattened inspiratory and expiratory flow volume loops. A second echocardiogram along with angiography confirmed a right sided aortic arch with an aberrant left subclavian artery. Bronchoscopy showed narrowing of the distal trachea and right main bronchus. The patient underwent surgery. Constriction of the airway was caused by a ligamentum arteriosum, arising from the first …

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