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
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|
|Study Type :||Observational|
|Actual Enrollment :||170 participants|
|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|
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.
- 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.
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
|Principal Investigator:||Salim Si-Mohamed, MD||Hospices Civils de Lyon (Hôpital cardiologique Louis Pradel)|