Pulmonary Hypertension in Hereditary Haemorrhagic Telangiectasia

Overview

Journal World J Cardiol

Publisher Baishideng Publishing Group

Specialty Cardiology & Vascular Diseases

Date 2015 May 28

PMID 26015855

Citations 23

Authors

Veronique Mm Vorselaars

Sebastiaan Velthuis

Repke J Snijder

Jan Albert Vos

Johannes J Mager

Martijn C Post

Affiliations

Soon will be listed here.

Abstract

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disorder characterised by vascular malformations in predominantly the brain, liver and lungs. Pulmonary hypertension (PH) is increasingly recognised as a severe complication of HHT. PH may be categorised into two distinct types in patients with HHT. Post-capillary PH most often results from a high pulmonary blood flow that accompanies the high cardiac output state associated with liver arteriovenous malformations. Less frequently, the HHT-related gene mutations in ENG or ACVRL1 appear to predispose patients with HHT to develop pre-capillary pulmonary arterial hypertension. Differentiation between both forms of PH by right heart catheterisation is essential, since both entities are associated with severe morbidity and mortality with different treatment options. Therefore all HHT patients should be referred to an HHT centre.

Citing Articles

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