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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 …
Contributors CLS was the lead author and responsible for the content overall, and Figures. RC, JWD, DGK and SJW reviewed, modified and approved the document. All authors agreed for the final submission.
Competing interests None declared.
Provenance and peer review Commissioned; externally peer reviewed.
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