Abstract
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.
Original language | English (US) |
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Pages (from-to) | 421-428 |
Number of pages | 8 |
Journal | Current Opinion in Pulmonary Medicine |
Volume | 20 |
Issue number | 5 |
DOIs | |
State | Published - Sep 2014 |
Keywords
- haemoptysis
- haemothorax
- hereditary haemorrhagic telangiectasia
- pulmonary arteriovenous malformation
- pulmonary hypertension
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine