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The pulmonary vascular complications of hereditary haemorrhagic telangiectasia

¹ Keenan Research Centre and Li Ka Shing Knowledge Institute, St Michael's Hospital, ² Dept of Medicine, Division of Respirology, St Michael’s Hospital, University of Toronto, and ³ Dept of Medicine, Division of Respirology, University Health Network, University of Toronto, Toronto, ON, Canada, ⁴ Dept of Medicine, Division of Cardiovascular Medicine, Yale Medical School, Yale University, New Haven, CT, USA.

CORRESPONDENCE: M. E. Faughnan, St Michael’s Hospital, 30 Bond Street, Toronto, ON, Canada, M5B-1W8. Fax: 1 4168645922. E-mail: faughnanm{at}smh.toronto.on.ca

Keywords: Hereditary haemorrhagic telangiectasia, pulmonary arterial hypertension, pulmonary arteriovenous malformation, pulmonary hypertension

Received: April 21, 2008
Accepted November 21, 2008

Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder, characterised by the presence of vascular malformations. The pulmonary vascular complications of HHT include pulmonary arteriovenous malformations, pulmonary hypertension associated with high-output heart failure and liver vascular malformations and, finally, pulmonary arterial hypertension secondary to HHT. In the present review, the authors describe the clinical presentation, diagnosis and management of all three pulmonary vascular presentations of HHT, as well as the underlying genetics and pathophysiology.