PT - JOURNAL ARTICLE AU - M. E. Faughnan AU - J. T. Granton AU - L. H. Young TI - The pulmonary vascular complications of hereditary haemorrhagic telangiectasia AID - 10.1183/09031936.00061308 DP - 2009 May 01 TA - European Respiratory Journal PG - 1186--1194 VI - 33 IP - 5 4099 - http://erj.ersjournals.com/content/33/5/1186.short 4100 - http://erj.ersjournals.com/content/33/5/1186.full SO - Eur Respir J2009 May 01; 33 AB - 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. SERIES “PULMONARY HYPERTENSION: BASIC CONCEPTS FOR PRACTICAL MANAGEMENT” Edited by M.M. Hoeper and A.T. Dinh-Xuan Number 8 in this Series