Related Experiment Video
Updated: Nov 29, 2025
Endoscopic Injection Sclerotherapy Assisted by Cyanoacrylate and Clips for Gastroesophageal Varices
Published on: June 13, 2025
Celiprolol Treatment in Patients with Vascular Ehlers-Danlos Syndrome
Hassan Baderkhan1, Anders Wanhainen1, Anna Stenborg2
1Department of Surgical Sciences, Vascular Surgery, Uppsala, Sweden.
Insights
Celiprolol treatment in vascular Ehlers-Danlos syndrome (vEDS) patients showed a 4.7% annual risk of major vascular events. These findings suggest celiprolol may offer a protective effect for vEDS, despite some fatal outcomes.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Rare Diseases
Background:
- Vascular Ehlers-Danlos syndrome (vEDS) is a rare genetic disorder characterized by COL3A1 gene variants.
- Arterial rupture is the most severe clinical manifestation of vEDS.
- Previous studies, like the BBEST trial, indicated a protective effect of the beta-blocker celiprolol.
Purpose of the Study:
- To evaluate the outcomes of celiprolol treatment in a Swedish cohort of patients diagnosed with vEDS.
- To assess the safety and efficacy of celiprolol in managing vascular events in vEDS patients.
Main Methods:
- A cohort of 40 vEDS patients with COL3A1 pathogenic variants received celiprolol treatment between 2011 and 2019.
- Patients were monitored for clinical events, medication adherence, and side effects.
- Logistic regression was employed to identify predictors of vascular events.
Main Results:
- The median follow-up was 22 months, totaling 106 patient-years.
- Five major vascular events occurred, with four being fatal (aortic rupture, cerebral aneurysm rupture, pulmonary artery rupture).
- The annual risk of major vascular events was 4.7%, comparable to the BBEST trial's treatment arm and lower than its control arm.
Conclusions:
- Celiprolol was generally well-tolerated by most vEDS patients.
- Despite some fatal events, the results suggest a potential protective role for celiprolol in reducing vascular events in vEDS.
Objective:
Vascular Ehlers-Danlos syndrome (vEDS) is a rare monogenetic disease caused by pathogenic variants in procollagen 3A1. Arterial rupture is the most serious clinical manifestation. A randomised controlled trial, the Beta-Blockers in Ehlers-Danlos Syndrome Treatment (BBEST) trial, reported a significant protective effect of the beta blocker celiprolol. The aim was to study the outcome of celiprolol treatment in a cohort of Swedish patients with vEDS.
Methods:
Uppsala is a national referral centre for patients with vEDS. They are assessed by vascular surgeons, angiologists, and clinical geneticists. Family history, previous and future clinical events, medication, and side effects are registered. Celiprolol was administered twice daily and titrated up to a maximum dose of 400 mg daily. Logistic regression was used to analyse predictors of vascular events.
Results:
Forty patients with pathogenic sequence variants in COL3A1 were offered treatment with celiprolol in the period 2011-2019. The median follow up was 22 months (range 1-98 months); total follow up was 106 patient years. In two patients, uptitration of the dose is ongoing. Of the remaining 38, 26 (65%) patients reached the target dose of 400 mg daily. Dose uptitration was unsuccessful in six patients because of side effects; one died before reaching the maximum dose, and five terminated the treatment. Five major vascular events occurred; four were fatal (ruptured ascending aorta; aortic rupture after type B dissection; ruptured cerebral aneurysm; and ruptured pulmonary artery). One bled from a branch of the internal iliac artery, which was successfully coiled endovascularly. The annual risk of a major vascular event was 4.7% (n = 5/106), similar to the treatment arm of the BBEST trial (5%) and lower than in the control arm of the same trial (12%). No significant predictor of vascular events was identified.
Conclusion:
Treatment with celiprolol is tolerated in most patients with vEDS. Despite fatal vascular events, these observations suggest that celiprolol may have a protective effect in vEDS.
More Related Videos
08:05Occlusion of the Great and Small Saphenous Vein Using Copolymeric Glue Based on N-Butyl Cyanoacrylate and Methacryloxy Sulfolane
Published on: December 9, 2022
07:24Reduction in Left Ventricular Wall Stress and Improvement in Function in Failing Hearts using Algisyl-LVR
Published on: April 8, 2013
Related Concept Videos
01:18Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
01:21Rheumatic Heart Disease III: Medical Management
01:26Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
01:27Peripheral Artery Disease III: Interprofessional Care
01:23Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
01:25ECG Interpretation of Arrhythmias II: Atrial, Junctional and Ventricular Arrhythmias