Table 2

Symptoms and signs of PAVMs at presentation in major series. Many patients will have symptoms, stigmata or a family history of HHT

Incidence
(%)
Mean (%)No. of patientsReferencesComments
Respiratory
Dyspnoea on exertion27–7147427 52, 53, 55, 56™, 57, 70, 71 *,7276™, 77
 Chest pain6–1712132 52, 70,73
 Haemoptysis4–1811413 52, 53, 55, 56™, 57,59, 70, 71 *, 7476™, 78 Particularly if systemic rather than pulmonary arterial supply79 80
 Haemothorax0–2<1129 53, 74, 76Probably increased in pregnancy62 63 81
 Asymptomatic25–5849197 52, 56™, 71 *, 7274,76™, 82
 Cyanosis9–7330209 52, 56™, 71 *, 72,74, 76™, 82
 Clubbing6–6836201 52, 55, 56™,57, 73, 74, 76™,
 Bruit25–5849197 52, 55,56™, 70, 73, 82
Neurological
Cerebral abscesses0–259302 52, 53, 58, 71 *, 7476™,77
 Clinical TIA/stroke11–5524335 5254, 70,71 *, 7376™, 77 Asymptomatic incidence possibly double78
 Migraine4–3828150 53, 74,75
  • TIA = transient ischaemic attack.

  • ™: The 32 cases reported in reference 76 and 27 cases in reference 56include a high proportion of individuals diagnosed by screening of asymptomatic HHT family members; *indicates a childhood series of 31 patients.71 In view of overlapping series, data from reference 59 were not presented as all cases were reported in reference52, and data in reference 82 were reported only where not stated in reference 75.