Pulmonary vascular complications of hereditary haemorrhagic telangiectasia

Sebastian Circo, James R Gossage

Research output: Contribution to journalReview article

13 Citations (Scopus)

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 - Jan 1 2014

Fingerprint

Hereditary Hemorrhagic Telangiectasia
Blood Vessels
Pulmonary Hypertension
Lung
Arteriovenous Malformations
Echocardiography
Heart Failure
Hemothorax
Vascular Malformations
Hemoptysis
Liver
Genetic Testing
Costs and Cost Analysis
Therapeutics

Keywords

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

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Pulmonary vascular complications of hereditary haemorrhagic telangiectasia. / Circo, Sebastian; Gossage, James R.

In: Current Opinion in Pulmonary Medicine, Vol. 20, No. 5, 01.01.2014, p. 421-428.

Research output: Contribution to journalReview article

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