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Pulmonary arteriovenous malformations emerge from the shadows
  1. Claire L Shovlin1,2,
  2. Robin Condliffe3,4,
  3. James W Donaldson5,
  4. David G Kiely3,4,
  5. Stephen J Wort1,6
  1. 1 NHLI Vascular Science, Imperial College London, London, UK
  2. 2 Respiratory Medicine, and VASCERN HHT European Reference Centre, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK
  3. 3 Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK
  4. 4 Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK
  5. 5 Respiratory Medicine, Derby Teaching Hospitals NHS Foundation Trust, Derby, UK
  6. 6 Pulmonary Hypertension Department, Royal Brompton Hospital, London, UK
  1. Correspondence to Professor Claire L Shovlin, Professor of Practice (Clinical and Molecular Medicine), NHLI Vascular Science, Imperial College London, Imperial Centre for Translational and Experimental Medicine, Hammersmith Campus, Du Cane Road, London W12 0NN, UK; c.shovlin{at}imperial.ac.uk

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For many years, pulmonary arteriovenous malformations (PAVMs) have been an underdiagnosed and poorly understood aspect of respiratory medicine. Recent increases in the published literature have improved our understanding of their diagnosis and management. The British Thoracic Society Clinical Statement on PAVMs published in Thorax aims to drive an improvement in the care provided to this often-neglected patient group.1 The Statement is the first example of a new method of summarising data and providing an expert view of best practice in a given clinical area. 

Three major issues are associated with poor health outcomes in patients with PAVMs: lack of clinician awareness and education, an absence of standardised approaches to patient management and an urgent need for service development for a condition estimated to affect more than 10 000 people in the UK.2

Clinician awareness and education

PAVMs are under-represented in medical school syllabuses and, more importantly, not mentioned in UK or US specialist respiratory training curriculae.3 These abnormal blood vessels connect pulmonary arteries directly to pulmonary veins, allowing a proportion of systemic venous blood to bypass the pulmonary capillary bed and return ‘unprocessed’ to the left heart and systemic arterial tree (figure 1). Although shunting via PAVMs impairs gas exchange, patients usually compensate very well for hypoxaemia and the exuberant ventilatory requirements by physiological mechanisms such as secondary erythrocytosis and changes in haemodynamics to maintain normal oxygen delivery (figure 2). In contrast, there are no compensatory mechanisms that enable restoration of the pulmonary capillary ‘filter’ to prevent …

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