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

Table 2 HHT outcome measures

	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 (+/− HHT^a)	196,000	Receive written advice on antibiotic prophylaxis prior to dental and surgical procedures	100%
Measure 5	Pregnant women with pulmonary AVMs (+/− HHT^a)	~ 1000	Receive written advice on PAVM/HHT pregnancies	100%

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

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