Pulmonary Arteriovenous Malformations (PAVMs) in Hereditary Haemorrhagic Telangiectasia (HHT)

Civil Hospices of Lyon

Status: Completed

Conditions: Cerebral Disorder; Pulmonary Arteriovenous Malformation; Hereditary Haemorrhagic Telangiectasia

Treatments: Other: Data collection from standard follow up

Study type: Observational

Funder types: Other

Identifiers

NCT03940014

PAVM

Details and patient eligibility

About

Computed tomography (CT) is the modality of choice to characterize pulmonary arteriovenous malformations (PAVMs) in patients with hereditary haemorrhagic telangiectasia (HHT). The objective of this study was to determine if CT findings were associated with frequency of brain abscess and ischaemic stroke.

This retrospective study included patients with HHT-related PAVMs. CT results, PAVM presentation (unique, multiple, disseminated or diffuse), the number of PAVMs and the largest feeding artery size, were correlated to prevalence of ischaemic stroke and brain abscess.

Enrollment: 170 patients

Sex: All

Volunteers: No Healthy Volunteers

Inclusion criteria

• Hereditary Haemorrhagic Telangiectasia (HHT) diagnosis
• Pulmonary Arteriovenous Malformations (PAVMs) related with HHT

Exclusion criteria

• Clinical follow-up not available in the database

Trial design

170 participants in 1 patient group

Pulmonary arteriovenous malformations

Description:

Patients with hereditary haemorrhagic telangiectasia (HHT)-related Pulmonary Arteriovenous Malformations (PAVMs). For all patients, the final diagnosis of certain HHT the diagnosis can be made depending on the presence of four criteria known as the Curaçao criteria: 1) Spontaneous recurrent epistaxis 2) Multiple telangiectasias in typical locations 3) Proven visceral Arteriovenous Malformations (AVM) (lung, liver, brain, spine) 4) First-degree family member with HHT. If conditions three or four are met, a patient has "definite HHT", while condition two is considered as "possible HHT". All patients had a molecular diagnosis and all follow-up clinical assessments were available in the database.

Treatment:

Other: Data collection from standard follow up