Abstract
Although Hereditary Hemorrhagic Telangiectasia (HHT) is characterized by an overwhelming bleeding propensity, patients with this disease may also present medical conditions that require antithrombotic therapy (AT). However, precise information on indications, dosage, duration, effectiveness, and safety of AT in HHT patients is lacking. We performed a retrospective analysis of the HHT Registry of our University Hospital and found 26 patients who received AT for a total of 30 courses (19 courses of anticoagulant therapy and 11 courses of antiplatelet therapy). Indications to treatments included: atrial fibrillation, venous thrombosis and pulmonary embolism, heart valve replacement, retinal artery occlusion, secondary prevention after either stroke or myocardial infarction, and thromboprophylaxis for surgery. The total time of exposure to antiplatelet therapy was 385 months and to anticoagulant therapy 169 months. AT was generally well tolerated, with no fatal bleedings and no significant changes in hemoglobin levels. However, we found three major bleedings, with an incidence rate of 6.5 per 100 patients per year. When only patients treated with anticoagulants were considered, the incidence rate of major bleedings increased to 21.6 per 100 patients per year. Our study indicates that major bleeding may occur in HHT patients receiving AT, with a substantially increased rate in those treated with anticoagulants. Further studies are needed to fully estimate the tolerability of antithrombotic drugs in HHT.
Highlights
Hereditary Hemorrhagic Telangiectasia (HHT) is a rare autosomal dominant disease characterized by recurrent spontaneous epistaxis, visceral arteriovenous malformations (AVMs), and mucocutaneous telangiectases [1,2,3]
This study presents data on the way antithrombotic therapy (AT) is managed in the real-world in patients with HHT
One interesting point of discussion is the variety of medical conditions requiring AT that we found in subjects with HHT
Summary
Hereditary Hemorrhagic Telangiectasia (HHT) is a rare autosomal dominant disease characterized by recurrent spontaneous epistaxis, visceral arteriovenous malformations (AVMs), and mucocutaneous telangiectases [1,2,3]. The hallmark of HHT is an overwhelming bleeding propensity, it is well known that patients with this disease are not devoid of medical conditions that require antithrombotic therapy (AT). They may have coronary artery disease (CAD), venous thromboembolism (VTE), or atrial fibrillation (AF) [4,5,6,7,8]. Precise information on the indications, dosage, duration, and effectiveness of AT in HHT patients is lacking It is not clear whether subjects with HHT are able to tolerate AT, neither is it known if bleedings associated with AT are more common in some HHT phenotypes than other. It may happen that patients are reluctant to take these drugs, since they have been advised to avoid the use of medications that may worsen their risk of bleeding
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