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

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ClinicalTrials.gov Identifier: NCT03940014
Recruitment Status : Completed
First Posted : May 7, 2019
Last Update Posted : May 7, 2019
Sponsor:
Information provided by (Responsible Party):
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.


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

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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 : January 1, 2014
Actual Primary Completion Date : March 1, 2017
Actual Study Completion Date : March 1, 2018


Group/Cohort 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.





Primary Outcome Measures :
  1. Yearly change in chest Computed Tomography (CT) results in patients with Hereditary Haemorrhagic Telangiectasia (HHT). [ Time Frame: 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.



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Ages Eligible for Study:   Child, Adult, Older Adult
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Hereditary Haemorrhagic Telangiectasia (HHT) Patients who had a molecular diagnosis and all follow-up clinical assessments available in the database.
Criteria

Inclusion Criteria:

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

Exclusion Criteria:

  • Clinical follow-up not available in the database

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03940014


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

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Responsible Party: Hospices Civils de Lyon
ClinicalTrials.gov Identifier: NCT03940014     History of Changes
Other Study ID Numbers: PAVM
First Posted: May 7, 2019    Key Record Dates
Last Update Posted: May 7, 2019
Last Verified: May 2019

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Additional relevant MeSH terms:
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Telangiectasia, Hereditary Hemorrhagic
Vascular Diseases
Cardiovascular Diseases
Hemostatic Disorders
Hemorrhagic Disorders
Hematologic Diseases
Pathological Conditions, Anatomical
Hemangioma
Telangiectasis
Arteriovenous Malformations
Arteriovenous Fistula
Congenital Abnormalities
Vascular Malformations
Cardiovascular Abnormalities
Neoplasms, Vascular Tissue
Neoplasms by Histologic Type
Neoplasms
Vascular Fistula
Fistula