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Table 2 HHT outcome measures

From: European Reference Network For Rare Vascular Diseases (VASCERN) Outcome Measures For Hereditary Haemorrhagic Telangiectasia (HHT)

  Target Population Estimated cases in Europe Measure Target threshold
Measure 1} All HHT- clinical or molecular diagnosis 85,000 Screen for pulmonary AVMs ≥ 90%
Measure 2} Receive nosebleed advice in writing ≥ 90%
Measure 3} Assessment of iron deficiency at each consultation ≥ 70%
Measure 4 Pulmonary AVMs (+/− HHTa) 196,000 Receive written advice on antibiotic prophylaxis prior to dental and surgical procedures 100%
Measure 5 Pregnant women with pulmonary AVMs (+/− HHTa) ~ 1000 Receive written advice on PAVM/HHT pregnancies 100%
  1. Prevalence estimates assume a European population of 510,000,000 (196,000 with HHT or PAVMs), 100,000 females with HHT or PAVMs; and an average of 1.6 children per woman accounting for 1.2 years pregnant in 84 years life expectancy. aPulmonary AVMs also occur outside HHT and are estimated to affect 1 in 2600 people [33]. HHT and non HHT-related pulmonary AVMs are currently managed in the same way