The study population will mainly consist of patient with confirmed or suspected Hereditary Haemorrhagic Telangiectasia (HHT), also called Osler-Weber-Rendu disease (ROW). Some patients will be studied because they are screened for HHT, but will prove not to have the disease. HHT
Conditions
Interventions
Participants undergo standard-of-care TTCE with agitated saline administration during rest and Valsalva maneuver. Simultaneously, the SONAS headset acquires cerebral perfusion data for 45 seconds para
the only addition is SONAS headset placement. 
Sponsors
St. Antonius Ziekenhuis
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: Patients with a suspicion or confirmed diagnosis of HHT who are screened for the presence of pulmonary right-to-left shunting by TTCE. Patients must be = 18 years.
Exclusion criteria
Exclusion criteria: Pregnancy
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 1. Device capability of detecting High Intensity Transient Signals (HITS) using SONAS in presence of RLS, as confirmed by reference method TTCE. SONAS output is expressed as maximum HITS Density Score (0-100%). TTCE outcome is assessed by RLS grade (0-3), maximum microbubble count in left ventricle, and corrected change in echo density.2. Diagnostic accuracy (sensitivity and specificity) of SONAS for detection of clinically significant RLS (=grade 2 on TTCE) at different HITS Density Score thresholds. | — |
Secondary
| Measure | Time frame |
|---|---|
| SONAS capability to distinguish intracardiac from pulmonary RLS based on time delay between contrast administration and first HITS occurrence, and difference in HITS Density Score between rest and Valsalva maneuver. 2. Test-retest reliability of SONAS for HITS assessment using Intraclass Correlation Coefficient. | — |
Countries
Netherlands
Contacts
Public ContactP.H.J. Veen
St. Antonius Ziekenhuis
Outcome results
None listed