Hereditary Haemorrhagic Telangiectasia (HHT), Osler-Weber-Rendu disease HHT
Conditions
Interventions
The investigational product is the fULM imaging system, a novel medical device utilizing ultrafast ultrasound combined with intravenous microbubble contrast agents as intravascular tracers. The system
Sponsors
St. Antonius Ziekenhuis
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: Age >18 yearsConfirmed diagnosis of HHT type 1 based on clinical criteria (3 out of 4 of the following: presence of telangiectasis on typical sites, presence of visceral arteriovenous malformation, presence of spontaneous epistaxis and a first degree relative with HHT type 1) or genetic testing (positive for a pathogenic mutation in the ENG-gene). Previous brain MRI performed in adult life.For group 1: at least one untreated cerebral vascular malformation on neuroimaging, in temporal brain region, within 8 centimetres from skull. The CVM is located within an acoustic window achievable by maintaining the ultrasound probe in the mesencephalic plane with angulation of 15° superiorly or 10° inferiorly. The depth of the CVM does not exceed 8 cm from the skull.For group 2: no evidence of cerebral vascular malformations on previous neuroimaging.
Exclusion criteria
Exclusion criteria: Intracranial surgery, angiographic intervention or radiation therapy of brain.Pregnancy or breastfeeding.Contraindications for injection of contrast agent (SonoVue®) (see EMA document SonoVue) 7Allergy or hypersensitivity for SonoVue components: sulphur hexafluoride microbubbles, macrogol 4000, distearoylphosphatidylcholine, dipalmitoylphosphatidylgycerol sodium, palmitic acid. Solvent: sodium chloride 9 mg/ml (0,9%) solution for injectionSevere pulmonary hypertension (mean pulmonary artery pressure >90mmHg)Uncontrolled systemic hypertension (=200mmHg systolic or =110 mmHg diastolic)Adult respiratory distress syndrome
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| To evaluate the technical feasibility of performing fULM examinations on patients with HHT for visualization of microvasculature:Evaluation of technical quality of produced images and data.Assessment of the device’ potential of visualizing microvasculature, as defined by the diameter of the smallest detectable vessel (a diameter <100 µm is considered microvascular).Clinical applicability of device: focus on time needed for examination, patient satisfaction. | — |
Secondary
| Measure | Time frame |
|---|---|
| If the primary objectives have been positively met, an initial exploration will examine:Microvascular parameters of patients with HHT type 1 compared to normal reference values.Velocity of blood flow in the microvasculature (cm/s)Tortuosity of blood vesselsVessel diameter (in µm)Vessel density (amount of vessels per standard imaging field.Characteristics of blood flow in cerebrovascular malformations (CVMs) in patients with HHT type 1.Visual representation of the fULM outcome | — |
Countries
Netherlands
Contacts
Public ContactP.H.J. Veen
St. Antonius Ziekenhuis
Outcome results
None listed