Editorial
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Cardiol. May 26, 2015; 7(5): 230-237
Published online May 26, 2015. doi: 10.4330/wjc.v7.i5.230
Pulmonary hypertension in hereditary haemorrhagic telangiectasia
Veronique MM Vorselaars, Sebastiaan Velthuis, Repke J Snijder, Jan Albert Vos, Johannes J Mager, Martijn C Post
Veronique MM Vorselaars, Sebastiaan Velthuis, Martijn C Post, Department of Cardiology, St. Antonius Hospital, 3435 CM Nieuwegein, The Netherlands
Repke J Snijder, Johannes J Mager, Department of Pulmonology, St. Antonius Hospital, 3435 CM Nieuwegein, The Netherlands
Jan Albert Vos, Department of Interventional Radiology, St. Antonius Hospital, 3435 CM Nieuwegein, The Netherlands
Author contributions: Vorselaars VMM contributed to drafting the article; Vorselaars VMM, Velthuis S, Snijder RJ, Vos JA, Mager JJ and Post MC contributed to conception and design and final approval of the version to be published; Velthuis S, Snijder RJ, Vos JA, Mager JJ, Post MC contributed to revision of the article.
Conflict-of-interest: This manuscript has been seen and approved by all co-authors and the authors declare no conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Veronique MM Vorselaars, MD, Department of Cardiology, St. Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, The Netherlands. m.post@antoniusziekenhuis.nl
Telephone: +31-88-3201228 Fax: +31-30-6092274
Received: October 22, 2014
Peer-review started: October 23, 2014
First decision: November 27, 2014
Revised: December 26, 2014
Accepted: February 9, 2015
Article in press: February 11, 2015
Published online: May 26, 2015
Processing time: 210 Days and 15.8 Hours
Core Tip

Core tip: Pulmonary hypertension (PH) is increasingly recognised as a severe complication of hereditary haemorrhagic telangiectasia (HHT), but the true prevalence of PH in HHT is not known. Post-capillary PH most often results from the high cardiac output associated with hepatic arteriovenous malformations. More rarely the HHT gene mutations (ACRVL1 or ENG) result in pre-capillary pulmonary arterial hypertension (PAH). Differentiation between post-capillary PH en pre-capillary PAH can be done by right heart catheterisation, and is of importance since both entities are associated with severe morbidity and mortality and have different options for treatments.