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

Completed

• Conditions: Hereditary Haemorrhagic Telangiectasia; Pulmonary Arteriovenous Malformation; Cerebral Disorder
• Interventions: Other: Data collection from standard follow up

• Registration Number: NCT03940014
• Lead Sponsor: Hospices Civils de Lyon

Brief Summary

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.

Detailed Description

Not available

Study Timeline

• Study Start: 2014-01-01
• Primary Completion: 2017-03-01
• Study Completion: 2018-03-01
• Status Verified: 2019-05
• Study First Submitted: 2019-05-02
• Study First Submitted QC: 2019-05-03
• Last Update Submitted: 2019-05-03
• Study First Posted: 2019-05-07
• Last Update Posted: 2019-05-07

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 170

Inclusion Criteria

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

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Exclusion Criteria

• Clinical follow-up not available in the database

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Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Pulmonary arteriovenous malformations	Data collection from standard follow up	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.

Primary Outcome Measures

Name	Time	Method
Yearly change in chest Computed Tomography (CT) results in patients with Hereditary Haemorrhagic Telangiectasia (HHT).	Every year for 10 years	The presence of pulmonary arteriovenous malformations (PAVMs) (unique, multiple, disseminated or diffuse, the number of PAVMs and the largest feeding artery size) upon examination by CT could be correlated to the frequency of brain abscess and ischemic stroke in patients with hereditary haemorrhagic telangiectasia (HHT). These patients underwent a yearly CT scan to check for PAVM presence.