Journal of Rare Cardiovascular Diseases

Background: Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder characterised by the formation of aberrant arteriovenous malformations (AVMs). Few studies have described the epidemiology and incidence of this rare disease. Aims: This study aims to identify the incidence and prevalence of HHT in the general population of Benghazi, Libya. Materials and methods: All living patients as of 30th April 2014 who have received a diagnosis of HHT in Benghazi (Libya) were identified, as well as all patients who were diagnosed with HHT between 1st May 2013 and the 30th April 2014. These patients were identified through written enquiries sent out to the cardiology, haematology, vascular medicine, and emergency departments of all hospitals in Benghazi (15 departments). All inquiries were answered. Results: The number of living patients diagnosed with HHT as of 30th April 2014 was 121. The current estimated population of Benghazi (according to the latest census in 2006) is 670,797, producing a prevalence of HHT in Benghazi of 18.0 per 100,000. The number of new HHT diagnoses in the period of 1st May 2013 – 30th April 2014 was 8, producing an incidence of 1.19 per 100,000 person-years. Conclusion: This study identifies the incidence and prevalence of HHT in Benghazi. The prevalence seen is higher than has been identified in other populations, and more must be done to identify all patients with this severe genetic disorder. JRCD 2014; 1 (8): 11–14

hereditary haemorrhagic telangiectasia, incidence, Libya

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