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

Sponsor

Hospices Civils de Lyon (Other)

Overall Status

Completed

CT.gov ID

NCT03940014

Collaborator

(none)

170

Enrollment

49.9

Actual Duration (Months)

Study Details

Study Description

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.

Condition or Disease	Intervention/Treatment	Phase
Hereditary Haemorrhagic Telangiectasia Pulmonary Arteriovenous Malformation Cerebral Disorder	Other: Data collection from standard follow up

Study Design

Study Type:

Observational

Actual Enrollment :

170 participants

Observational Model:

Cohort

Time Perspective:

Retrospective

Official Title:

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

Actual Study Start Date :

Jan 1, 2014

Actual Primary Completion Date :

Mar 1, 2017

Actual Study Completion Date :

Mar 1, 2018

Arms and Interventions

Arm	Intervention/Treatment
Pulmonary arteriovenous malformations 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.	Other: Data collection from standard follow up 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

Intervention/Treatment

Pulmonary arteriovenous malformations

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.

Other: Data collection from standard follow up

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.

Outcome Measures

Primary Outcome Measures

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.

Eligibility Criteria

Criteria

Ages Eligible for Study:

N/A and Older

Sexes Eligible for Study:

All

Accepts 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

Contacts and Locations

Locations

No locations specified.

Sponsors and Collaborators

Hospices Civils de Lyon

Investigators

Principal Investigator: Salim Si-Mohamed, MD, Hospices Civils de Lyon (Hôpital cardiologique Louis Pradel)

Study Documents (Full-Text)

None provided.

More Information

Publications

None provided.

Responsible Party:

Hospices Civils de Lyon

ClinicalTrials.gov Identifier:

NCT03940014

Other Study ID Numbers:

PAVM

First Posted:

May 7, 2019

Last Update Posted:

May 7, 2019

Last Verified:

May 1, 2019

Studies a U.S. FDA-regulated Drug Product:

Studies a U.S. FDA-regulated Device Product:

Additional relevant MeSH terms:

Hemangioma

Telangiectasis

Arteriovenous Malformations

Telangiectasia, Hereditary Hemorrhagic

Arteriovenous Fistula

Congenital Abnormalities

Study Results

No Results Posted as of May 7, 2019