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Pulmonary vascular complications of hereditary haemorrhagic telangiectasia

Circo, Sebastian; Gossage, James R.

Current Opinion in Pulmonary Medicine: September 2014 - Volume 20 - Issue 5 - p 421–428
doi: 10.1097/MCP.0000000000000076
DISORDERS OF THE PULMONARY CIRCULATION: Edited by Richard N. Channick and Marc Humbert

Purpose of review 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.

Recent findings 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.

Summary Patients suffering from this elusive disease should be referred to HHT specialized centres to ensure a standardized and timely approach to diagnosis and management.

Georgia Regents University, Augusta, Georgia, USA

Correspondence to James R. Gossage, Georgia Regents University, BBR5521, 1120 15th street, Augusta, GA 30912, USA. Tel: +1 706 721 6789; e-mail:

© 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins