Clinical Trial to Compare the Efficacy of Celiprolol to Placebo in Patients With Vascular Ehlers-Danlos Syndrome

Inclusion Criteria:

1. Willingness to obtain magnetic resonance angiogram (MRA) image at local imagingfacility.

2. A genetic test confirming the presence of a pathogenic COL3A1 variant (classified aslikely pathogenic or pathogenic according to ACMG/AMP Guidelines.

3. Patients must be ≥ 15 years of age at the time of randomization.

4. Able and willing to discontinue use of β-blockers prior to randomization.

Exclusion Criteria:

1. Lack of a COL3A1-positive test at screening (e.g., COL3A1 benign, likely benign,variant of unknown significance [VUS] or no variant) or presence of a COL3A1 variantbut demonstration of a COL3A1 variant reported to be a haploinsufficiency variant.

2. Arterial rupture or dissection, uterine rupture, and/or intestinal rupture within 6months prior to Screening.

3. Patients unable to discontinue β-blocker treatment prior to randomization.

4. Unable or unwilling to complete the study procedures.

5. Breastfeeding, pregnancy, or planned pregnancy during the trial.

6. Any medical condition that in the opinion of the Investigator may pose a safety riskto the patient in this study, which may confound efficacy or safety assessment, ormay interfere with study participation.

7. Use of any prohibited medications