Atrial fibrillation in patients with haemophilia: a cross‐sectional evaluation in Europe

Abstract

Atrial fibrillation (AF) is a common health problem in the general population, but data on prevalence or management in patients with haemophilia (PWH) are lacking. The aims of this study were to analyse the prevalence of AF and risk factors for stroke using a cross-sectional pan-European design and to document current anticoagulation practice. The ADVANCE Working Group consists of members from 14 European haemophilia centres. Each centre retrieved data on their PWH with AF. From the total of 3952 adult PWH, 33 had AF with a mean age of 69 years (IQR 62-76). Haemophilia was severe in seven (21%), moderate in six (18%) and mild in 20 (61%) patients. The overall AF prevalence was 0.84% and increased with age; 0.42% in patients 40-60 years and 3.4% in patients >60 years. The mean CHA2 DS2 -Vasc score was 1.3 (range 0-4), predominantly determined by age and hypertension. Hypertension was reported in 48% of PWH with AF. In 11 patients (33%), anticoagulation was started of whom nine aspirin and two vitamin K antagonists. Of these 11 patients, nine had mild haemophilia. Anticoagulation was given in 42% of patients with a CHA2 DS2 -Vasc score ≥2. During follow-up (mean 57 months), there were no thrombotic events reported, nor increases in bleeding severity. The prevalence of AF in haemophilia increases with age and is predominantly present in mild haemophilia. PWH have a low stroke risk based on their CHA2 DS2 -Vasc scores, that might be even lower considering the hypocoagulable state. Only 33% of PWH with AF receives any form of anticoagulation therapy.

How I treat patients with inherited bleeding disorders who need anticoagulant therapy

“…A cross-sectional survey from 14 European hemophilia centers found an overall prevalence of AF in patients with hemophilia of 0.84%, 32 BLOOD, 14 JULY 2016 x VOLUME 128, NUMBER 2 ANTICOAGULATION IN BLEEDING DISORDERSwhich is comparable to that found in the general population. 33 Just as in the general population, the prevalence of AF in persons with hemophilia rises with increasing age, with a prevalence of 3.4% in subjects .60 years old compared to 0.2% in patients #60 years old.…”

“…33 Just as in the general population, the prevalence of AF in persons with hemophilia rises with increasing age, with a prevalence of 3.4% in subjects .60 years old compared to 0.2% in patients #60 years old. 32 In usual medical practice, treatment decisions regarding anticoagulation in AF are determined by weighing an individual's stroke risk as calculated by the CHA 2 DS 2 -VASc score against an estimated bleeding risk occurring as a consequence of anticoagulation therapy. However, validated bleeding scores for hemophilia patients with AF do not exist; although the HAS-BLED score estimates risk of bleeding with anticoagulation for AF in the general population, concerns exist that the score underestimates the bleeding risk in hemophilia.…”

Non-vitamin K antagonist oral anticoagulants in patients with an increased risk of bleeding

“…In a report from 2014 the general prevalence of NVAF in people with hemophilia (PWH) in Europe was 0.8%, rising to 3.4% in patients older than 60 years [23]. A European consensus statement focused on the management of AF in PWH [24].…”

Management of atrial fibrillation in people with haemophilia – a consensus view by the ADVANCE Working Group

“…As the age of the haemophilia population rises, the prevalence of AF in PWH is likely to increase as well. A recent report from Europe shows the prevalence of AF in PWH to be 0.8%, increasing to 3.4% in haemophilia patients aged >60 years, which is not that different from the general population [5].…”