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Despite being a hemorrhagic condition, patients with hereditary hemorrhagic telangiectasia (HHT) are at increased thromboembolic risk. We designed an observational retrospective cohort study of HHT patients who required anticoagulation therapy, described their indications and adverse events. From January 2006 until August 2019 28 out of 347 adult HHT patients received anticoagulant drugs for a median of 0.5 years (IQR 0.2–4.1). Median age was 64 years (IQR 51–70), all of the patients had epistaxis and at least one arteriovenous malformation. Fourteen (50%) patients received anticoagulants due to venous thromboembolic disease and 6 (21.4%) for atrial fibrillation. Nine patients (32.1%) received low-molecular-weight heparin and 19 (67.9%) vitamin-K antagonists. Seven (25%) withdrew treatment due to increased bleeding frequency from usual sites and 5 (17.9%) had a major bleeding event. Ten (35.7%) patients completed short length anticoagulation without significant adverse events.

In August, 2011, an 83-year-old woman presented to our emergency department with several days of diff use persistent abdominal pain. She also reported an episode of lower gastrointestinal bleeding with symptoms of orthostasis. She had a history of cardiac failure, pulmonary hypertension, and epistaxis of un known cause. On initial physical examination she was haemodynamically stable without signs of acute ab dom inal disease. She had oral mucocutaneous telangiectases. Gastric lavage was done, and no evidence of upper gastrointestinal bleeding was seen. Blood tests showed a haematocrit concentration reduction from 25% to 20%, and a haemoglobin of 69 g/L. She was admitted for blood transfusion and assessment. After 5 days, she developed acute abdominal pain, tachycardia (135 beats per min), and hypotension (80/40 mm Hg). Sonography of the abdomen showed a large amount of free abdominal fl uid. Multi-detector computed tomography (MDCT) of the abdomen and pelvis confi rmed intra-abdominal fl uid and also showed liver arteriovenous malformations. CT-guided paracentesis confi rmed a diagnosis of haemo peritoneum. An emergency surgical exploration of the abdominal cavity was done because of her poor clinical condition. Explor ation showed haemo peritoneum and a portal vein branch with active bleeding due to the vascular malformations (fi gure). Haemostatic suturing was done to stop bleeding, and the abdominal cavity was drained. She had an uneventful postoperative course and was discharged at postoperative day 10. At last follow-up in Lancet 2013; 381: 962