title: Pulmonary vascular complications of hereditary haemorrhagic telangiectasia.
abstract: OBJECTIVE
  The purpose of this study is to present the latest advances and recommendations in the diagnosis and treatment of pulmonary vascular complications associated with hereditary haemorrhagic telangiectasia (HHT): pulmonary arteriovenous malformations (PAVMs), pulmonary arterial hypertension (PAH), pulmonary hypertension associated with high output cardiac failure or liver vascular malformations, haemoptysis, haemothorax and thromboembolic disease.
  
  
  RESULTS
  Transthoracic contrast echocardiography has been validated as a screening tool for PAVM in patients with suspected HHT. Advancements in genetic testing support its use in family members at risk as a cost-effective measure. Therapy with bevacizumab in patients with high output cardiac failure and severe liver AVMs showed promising results. PAH tends to be more aggressive in HHT type 2 patients.
  
  
  CONCLUSIONS
  Patients suffering from this elusive disease should be referred to HHT specialized centres to ensure a standardized and timely approach to diagnosis and management.
authors.affiliation: Georgia Regents University, Augusta, Georgia, USA.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Circo
authors.forename: James R
authors.initials: JR
authors.lastname: Gossage
mesh_terms: D001165:Arteriovenous Malformations / Q000209:etiology*; D005820:Genetic Testing; D006333:Heart Failure / Q000150:complications; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*