Pulmonary vascular complications of hereditary haemorrhagic telangiectasia

Sebastian Circo, James R. Gossage

Research output: Contribution to journalReview article

16 Scopus citations

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 languageEnglish (US)
Pages (from-to)421-428
Number of pages8
JournalCurrent Opinion in Pulmonary Medicine
Volume20
Issue number5
DOIs
StatePublished - Sep 2014

Keywords

  • haemoptysis
  • haemothorax
  • hereditary haemorrhagic telangiectasia
  • pulmonary arteriovenous malformation
  • pulmonary hypertension

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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