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Pulmonary vasculature
BTS Clinical Statement
British Thoracic Society Clinical Statement on Pulmonary Arteriovenous Malformations
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  1. Claire L Shovlin1,2,
  2. Robin Condliffe3,
  3. James W Donaldson4,
  4. David G Kiely3,5,
  5. Stephen J Wort6,7
  6. on behalf of the British Thoracic Society
  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 Dept of Respiratory Medicine, Derby Teaching Hospitals NHS Foundation Trust, Derby, UK
  5. 5 Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK
  6. 6 Pulmonary Hypertension Department, Royal Brompton Hospital, London, UK
  7. 7 NHLI Vascular Science, Imperial College London, 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 Rd, London W12 0NN, UK; c.shovlin{at}imperial.ac.uk

Abstract

Pulmonary arteriovenous malformations (PAVMs) are structurally abnormal vascular communications that provide a continuous right-to-left shunt between pulmonary arteries and veins. Their importance stems from the risks they pose (>1 in 4 patients will have a paradoxical embolic stroke, abscess or myocardial infarction while life-threatening haemorrhage affects 1 in 100 women in pregnancy), opportunities for risk prevention, surprisingly high prevalence and under-appreciation, thus representing a challenging condition for practising healthcare professionals. The driver for the current Clinical Statement was the plethora of new data since previous hereditary haemorrhagic telangiectasia (HHT) guidelines generated in 2006 and a systematic Cochrane Review for PAVM embolisation in 2011. The British Thoracic Society (BTS) identified key areas in which there is now evidence to drive a change in practice. Due to the paucity of data in children, this Statement focused on adults over 16 years. The Statement spans the management of PAVMs already known to be present (interventional and medical), screening and diagnosis (for PAVMs and HHT) and follow-up of patients following a first diagnosis, intervention or negative screen for PAVMs. The Good Practice Points (in bold) were generated for a target audience of general respiratory, medical and specialist clinicians and were approved by the BTS Standards of Care Committee, before formal peer review and public consultation. The Statement spans embolisation treatment, accessory medical management and issues related to the likelihood of underlying HHT.

https://doi.org/10.1136/thoraxjnl-2017-210764

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    Footnotes

    • Contributors CLS was lead author and responsible for the content overall. RC, JWD, DGK and SJW reviewed , modified and approved all sections of the document. All authors agreed the final submission.

    • Disclaimer A Clinical Statement reflects the expert views of a group of specialists who are well versed on the topic concerned and who carefully examine the available evidence in relation to their own clinical practice. A Clinical Statement does not involve a formal evidence review and is not developed in accordance with clinical practice guideline methodology. Clinical Statements are not intended as legal documents or a primary source of detailed technical information. Readers are encouraged to consider the information presented and reach their own conclusions.

    • Competing interests The authors have completed declarations of interest forms in line with the BTS policy, and forms are available from BTS Head Office.

    • Provenance and peer review Not commissioned, externally peer reviewed.

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