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

Pulmonary Arteriovenous Malformations (PAVMs) in Hereditary Haemorrhagic Telangiectasia (HHT): Correlations Between Computed Tomography Findings and Cerebral Complications


Lead Sponsor: Hospices Civils de Lyon

Source 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.

Overall Status Completed
Start Date 2014-01-01
Completion Date 2018-03-01
Primary Completion Date 2017-03-01
Study Type Observational
Primary Outcome
Measure Time Frame
Yearly change in chest Computed Tomography (CT) results in patients with Hereditary Haemorrhagic Telangiectasia (HHT). Every year for 10 years
Enrollment 170

Intervention Type: Other

Intervention Name: Data collection from standard follow up

Description: Annual clinical consultation with an Hereditary Haemorrhagic Telangiectasia (HHT) specialist and/or pneumologist and organ specialists when necessary (such as hepatologists, cardiologists and neurologists). Explorations (contrast echography, chest Computed Tomography and treatments performed according to international guidelines. TransCatheter Embolotherapy for each treatable Pulmonary Arteriovenous Malformations (PAVMs) and follow-up every 3 years. Chest Computed Tomography (CT) every 6-12 months.

Arm Group Label: Pulmonary arteriovenous malformations


Sampling Method:

Non-Probability Sample


Inclusion Criteria: - Hereditary Haemorrhagic Telangiectasia (HHT) diagnosis - Pulmonary Arteriovenous Malformations (PAVMs) related with HHT Exclusion Criteria: - Clinical follow-up not available in the database



Minimum Age:


Maximum Age:


Healthy Volunteers:


Overall Official
Last Name Role Affiliation
Salim Si-Mohamed, MD Principal Investigator Hospices Civils de Lyon (Hôpital cardiologique Louis Pradel)
Verification Date


Responsible Party

Type: Sponsor

Has Expanded Access No
Condition Browse
Arm Group

Label: 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.

Acronym PAVM
Study Design Info

Observational Model: Cohort

Time Perspective: Retrospective

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