Open AccessPulmonary Hypertension in a Large Cohort with Hereditary Hemorrhagic Telangiectasia
Author(s) -
Veronique M.M. Vorselaars,
Sebastiaan Velthuis,
Marco W.F. van Gent,
C. J. J. Westermann,
Repke J. Snijder,
Johannes J. Mager,
Martijn C. Post
Publication year - 2017
Publication title -
respiration
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.264
H-Index - 81
eISSN - 1423-0356
pISSN - 0025-7931
DOI - 10.1159/000458447
Subject(s) - medicine , telangiectasia , pulmonary hypertension , portal hypertension , gastroenterology , cardiology , pathology , cirrhosis
Hereditary hemorrhagic telangiectasia (HHT) is a vascular disorder characterized by arteriovenous malformations in the brain, liver, and lungs. Pulmonary hypertension (PH) is increasingly recognized as a severe complication of HHT. However, there are no studies describing the prevalence of PH in HHT compared to HHT-negative controls.