Imperial College London

Professor Claire Shovlin

Faculty of MedicineNational Heart & Lung Institute

Professor of Practice (Clinical and Molecular Medicine)



c.shovlin Website




534Block L Hammersmith HospitalHammersmith Campus






BibTex format

author = {Shovlin, C and Millar, C and Droege, F and Kjeldsen, A and Manfredi, G and Suppressa, P and Ugolini, S and Coote, N and Fialla, A and Geisthoff, U and Lenato, G and Mager, HJ and Pagella, F and Post, M and Sabba, C and Sure, U and Torring, P and Dupuis, Girod S and Buscarini, E and VASCERN, HHT},
doi = {10.1186/s13023-019-1179-1},
journal = {Orphanet Journal of Rare Diseases},
title = {Safety of direct oral anticoagulants in patients with hereditary hemorrhagic telangiectasia},
url = {},
volume = {14},
year = {2019}

RIS format (EndNote, RefMan)

AB - Background: Hereditary haemorrhagic telangiectasia (HHT) is a rare vascular dysplasia resulting in visceral arteriovenous malformations and smaller mucocutaneous telangiectasia. Most patients experience recurrent nosebleeds and become anaemic without iron supplementation. However, thousands may require anticoagulation for conditions such as venous thromboembolismand/or atrial fibrillation. Over decades,tolerance data has been publishedfor almost 200HHT-affected usersof warfarinand heparins, but there are no publisheddata forthe newer direct oralanticoagulants(DOACs)in HHT. Methods: To provide such data, aretrospective audit was conducted across the eight HHT centres of the European Reference Network for Rare Vascular Disorders (VASCERN),in Denmark, France, Germany, Italy, Netherlands and UK. Results: Although HHT Centreshad not specifically recommended the use of DOACs, 32treatment episodes had been initiated by other cliniciansin 28patients reviewed at the centres, at median age 65years(range 30-84). Indications were for atrial fibrillation (16 treatment episodes) and venous thromboembolism (16 episodes).The 32 treatment episodes used Apixaban (n=15), Rivaroxaban (n=14), and Dabigatran (n=3). HHT nosebleeds increased in severity in 24/32 treatment episodes (75%), leading to treatment discontinuation in 11 (34.4%). Treatment discontinuation was required for 4/15(26.7%) Apixabanepisodes and 7/14 (50%)Rivaroxaban episodes.By a 4 point scale of increasing severity,there was a trend for Rivaroxaban to be associated with a greaterbleeding riskboth including and excluding patients who had used more than one agent (age-adjusted coefficients 0.61 (95% confidence intervals 0.11, 1.20) and 0.74 (95% confidence intervals 0.12, 1.36) respectively. Associationswere maintained after adjustment for genderand treatment indication. Extreme haemorrhagic responses, worse thananything
AU - Shovlin,C
AU - Millar,C
AU - Droege,F
AU - Kjeldsen,A
AU - Manfredi,G
AU - Suppressa,P
AU - Ugolini,S
AU - Coote,N
AU - Fialla,A
AU - Geisthoff,U
AU - Lenato,G
AU - Mager,HJ
AU - Pagella,F
AU - Post,M
AU - Sabba,C
AU - Sure,U
AU - Torring,P
AU - Dupuis,Girod S
AU - Buscarini,E
DO - 10.1186/s13023-019-1179-1
PY - 2019///
SN - 1750-1172
TI - Safety of direct oral anticoagulants in patients with hereditary hemorrhagic telangiectasia
T2 - Orphanet Journal of Rare Diseases
UR -
UR -
VL - 14
ER -