Telangiectasia, Hereditary HemorrhagicULD OSLERNCT04108052

Diagnostic Value of Ultra-low Dose Thoracic Scanner for the Pulmonary Arteriovenous Malformation Detection in HHT Patient

A study testing Low CT scanner without injection dose and Ultra-low dose CT scanner without injection in people with Telangiectasia, Hereditary Hemorrhagic and Rendu Osler Disease, run by Hospices Civils de Lyon at 1 site in France.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record

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Trial summary

Who is it for?: Ages 18 years and older
Conditions the study is for: Telangiectasia, Hereditary Hemorrhagic, Rendu Osler Disease
What is being tested?: Low CT scanner without injection dose and Ultra-low dose CT scanner without injection
Type of treatment: Injection or infusion, Surgery or procedure
Could I receive a placebo?: No, everyone receives an active treatment
How many people: 45 participants
Where: 1 siteFrance
Study dates: 28 Nov 2019to 28 May 2021
Phase: Interventional

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

Ages 18 years and older
All sexes
No healthy volunteers
Adult, Older adult

Who may be able to join

Patient monitored for a clinically confirmed Rendu-Osler disease and / or confirmed by molecular biology, seen for an initial assessment or for a reassessment of PAVM (after treatment or without treatment)
Patient who agreed to participate in the study and signed the written informed consent
Patient affiliated to a social security scheme or similar

Who cannot join

Women who are pregnant, nursing (lactating) or at risk of pregnancy (verified by a urine test before performing the scanner)
Patient who is protected adults under the terms of the law (French Public Health Code).
Patient who has not or poorly understand French

Trial locations and contacts

1 site in 1 country.

France1 site

Hospices Civils de Lyon - Hopital Louis Pradel
Bron, 69500

Other trials in France

About this study

Hereditary hemorrhagic telangiectasia (HHT) is linked to a dysregulation of angiogenesis leading to the formation of arteriovenous malformations (AVM): cutaneo-mucous telangiectasia and visceral shunts. The diagnosis is clinical and based on Curaçao criteria: recurrent epistaxis, cutaneo-mucous telangiectasia, hereditary signs and presence of visceral AVM.

Pulmonary AVMs (PAVM) expose patients to many potentially life-threatening complications, such as strokes or brain abscesses due to the right-left shunt created and the lack of filtration barrier of the pulmonary capillary within the AVM. These patients should therefore have regular monitoring throughout their life by a chest CT scanner every 5 to 10 years in the absence of PAVM at the initial scan or more often if PAVMs are present. The management of PAVMs is based on their early detection and embolization in interventional radiology during which is set up within the afferent artery of the PAVM an embolizing agent, the coil.

However, the risk of cumulative irradiation exposure from thoracic scanners and repeated thoracic embolizations over time could be reduces by a decrease of X-rays dose.

A new thoracic CT imaging protocol validated in the United States in the primary screening of lung cancer, the ultra-low dose protocol, is a CT scanner acquired at an irradiation dose equivalent to that of a frontal chest x-ray and in profile. The dose reduction is of 40 times the usual dose of a chest CT scanner.

The lung parenchyma has a high natural contrast on thoracic CT images and there are few adjacent attenuating structures allowing a drastic reduction of dose. However, from this dose, the image quality is degraded with an increase of the image noise. The diagnostic performances have to be confirmed with qualitative and quantitative measurements.

Thus, the objective of this study is to compare the sensitivity and the specificity of the current scanner and the ultra-low dose scanner to reduce the exposure to X-rays.

Conditions: Telangiectasia, Hereditary Hemorrhagic, Rendu Osler Disease
Being tested: Low CT scanner without injection dose, Ultra-low dose CT scanner without injection
Keywords: Hereditary Hemorrhagic Telangiectasia (HHT), diagnosis,, arteriovenous malformation, interventional radiology, pulmonary arteriovenous fistulas, embolization
Medical subject headings: Telangiectasia, Hereditary Hemorrhagic, Disease, Arteriovenous Malformations, Pulmonary Arteriovenous Fistulas

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

Low CT scanner without injection doseRadiation
CT scan acquisition will be performed at 140 kVp (kilovolt peak), 10 mAs(milliampere), CTDI 1.3 mGy with an irradiation dose 0.51 mSv (milliSievert). The CT image will be reconstructed and analyzed in parenchyma window. The CT images will be blinded and re-read by two independent senior radiologists 3 to 6 weeks apart between each re-reading.
Ultra-low dose CT scanner without injectionRadiation
CT acquisition will be performed at 80 kVp for patient with a body mass index<30, and 100 kVp with a body mass index>30, 10 mAs, CTDI 0.3 and 0.6 mGy with an irradiation dose 0.15-0.30 mSv. The CT images will be reconstructed and analyzed in mediastinal window. The CT images will be blinded and re-read by two independent senior radiologists 3 to 6 weeks apart between each re-reading.

Study groups in NCT04108052
Study group	Role	What they receive
Low dose CT scanner and Ultra low dose CT Scan	Other	Thoracic low dose CT acquisition and Thoracic ultra-low dose CT acquisition Receives: Radiation: Low CT scanner without injection dose; Radiation: Ultra-low dose CT scanner without injection

What is being measured

The primary outcome is the main question the study is designed to answer.

Comparison of diagnostic performances (specificity and sensitivity) between ultra-low dose and low dose CT scanner
calculation of the sensibility, specificity and the predictive values using the Pearson's method after judgment of the presence or absence of pulmonary AVM to treat on each acquisition (low dose and ultra-low dose).
Measured: Day one

Secondary outcomes (8)

the effective radiation dose
The effective dose was estimated from the product of the dose length product (DLP) using a conversion coefficient of 0.017 mSv ( milliSievert ) mGy (milligray )-1 cm-1 reported in the European Guidelines for chest examinations.
Measured: at thoracic CT acquisition - day one
the computed tomography dose index (CTDI)
The volume of CTDI was recorded in mGy.
Measured: at thoracic CT acquisition - day one
the dose length product (DLP)
The DLP was recorded in mGy.cm.
Measured: at thoracic CT acquisition - day one
the size of afferent artery
measurement in millimeter
Measured: At the two re-readings - 3 months
the size of efferent vein
measurement in millimeter
Measured: At the two re-readings - 3 months
the number of afferent artery
measurement in millimeter
Measured: At the two re-readings - 3 months
the quality of images
a grading score is used from "insufficient" to "good" to assess the quality of images produced by standard, iDose, and MRI reconstruction protocol
Measured: At the two re-readings - 3 months
the confidence of the diagnosis
A confidence score is used (1 = no diagnostic confidence, 2 = average diagnostic confidence, 3 = good diagnostic confidence, 4 = excellent diagnostic confidence)
Measured: At the two re-readings - 3 months

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

28 Nov 201928 May 2021

First posted27 Sep 2019
Study start28 Nov 2019
Primary completion28 May 2021
Study completion28 May 2021

Resources

Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
Publications (1)Papers the sponsor has linked to this study.
Other trials for Telangiectasia, Hereditary HemorrhagicStudies of the same condition, open and under way.
More trials run by Hospices Civils de LyonThe sponsor's other registered studies.
What the trial phases meanHow a treatment moves from first tests in people to approval.

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design

Study type: Interventional
Phase: Phase N/A
Primary purpose: Diagnosis
Allocation: Not applicable
Intervention model: Single group
Masking: None (open label)
Enrolment: 45 participants (actual)

IDs, dates and publications

ClinicalTrials.gov ID: NCT04108052
Sponsor's protocol ID: 69HCL19_0336
Other IDs: ID-RCB
Acronym: ULD OSLER
Status last verified: Sep 2025
First submitted: 26 Sep 2019
Last updated: 10 Sep 2025
Results posted: No
Data monitoring committee: No
FDA-regulated drug: No
FDA-regulated device: No
Individual data sharing: No

Publications

Delpon JE, Greffier J, Lacombe H, Barbe A, Bouin M, De Oliveira F, Mansuy A, Delagrange L, Fargeton AE, Beregi JP, Cottin V, Dupuis-Girod S, Si-Mohamed SA. Ultra-low dose chest CT for the diagnosis of pulmonary arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia. Diagn Interv Imaging. 2024 Oct;105(10):364-370. doi: 10.1016/j.diii.2024.03.006. Epub 2024 Apr 10.
PubMed 38604894

Want to know more?

The sponsor has not listed a contact. The official record may have more details.

Talk to your doctor first and take this page with you. Only the study team can confirm whether you can join.

Questions to ask the study team

What is the study trying to find out?
What would I need to do, and how often would I visit?
Could I receive a placebo instead of the treatment?
What are the possible risks and benefits for me?
Will it cost me anything? Are travel or time paid for?
Can I keep seeing my own doctor during the study?
What happens when the study ends?

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