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

NCT04108052 | Completed

• 2 other identifiers: 69HCL19_0336ID-RCB
• Study Type: interventional
• Target: 45
• Locations: 1 country
• Sites: 1

Brief Summary

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

Keywords

Hereditary Hemorrhagic Telangiectasia (HHT); diagnosis,; arteriovenous malformation; interventional radiology; pulmonary arteriovenous fistulas; embolization

Outcome Measures

Primary Outcomes (1)

• 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).

  Day one

Secondary Outcomes (8)

• the effective radiation dose

  at thoracic CT acquisition - day one

• the computed tomography dose index (CTDI)

  at thoracic CT acquisition - day one

• the dose length product (DLP)

  at thoracic CT acquisition - day one

• the size of afferent artery

  At the two re-readings - 3 months

• the size of efferent vein

  At the two re-readings - 3 months

Study Arms (1)

Low dose CT scanner and Ultra low dose CT Scan

OTHER

Thoracic low dose CT acquisition and Thoracic ultra-low dose CT acquisition

Radiation: Low CT scanner without injection dose; Radiation: Ultra-low dose CT scanner without injection

Interventions

Low CT scanner without injection dose RADIATION

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.

Low dose CT scanner and Ultra low dose CT Scan

Ultra-low dose CT scanner without injection RADIATION

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.

Low dose CT scanner and Ultra low dose CT Scan

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• 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

You may not qualify if:

• 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

Sponsors & Collaborators

• Hospices Civils de Lyon: lead

Study Sites (1)

Hospices Civils de Lyon - Hopital Louis Pradel

Bron, 69500, France

Location

Related Publications (1)

• 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.

  PMID: 38604894 BACKGROUND

MeSH Terms

Conditions

Telangiectasia, Hereditary Hemorrhagic; Disease; Arteriovenous Malformations; Pulmonary Arteriovenous Fistulas

Interventions

Injections

Condition Hierarchy (Ancestors)

Hemostatic Disorders; Vascular Diseases; Cardiovascular Diseases; Telangiectasis; Hemorrhagic Disorders; Hematologic Diseases; Hemic and Lymphatic Diseases; Vascular Malformations; Cardiovascular Abnormalities; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Intervention Hierarchy (Ancestors)

Drug Administration Routes; Drug Therapy; Therapeutics

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: DIAGNOSTIC
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: September 26, 2019
• First Posted: September 27, 2019
• Study Start: November 28, 2019
• Primary Completion: May 28, 2021
• Study Completion: May 28, 2021
• Last Updated: September 10, 2025

Record last verified: 2025-09

Data Sharing

• IPD Sharing: Will not share

Locations

FR (1)