Efficacy and Tolerance of Treatment With Bevacizumab for Severe Liver Involvement With High Cardiac Output in Hereditary Hemorrhagic Telangiectasia Within the French Hereditary Hemorrhagic Telangiectasia Network

Sponsor
Poitiers University Hospital
Study ID
NCT07445347
Status
Enrolling By Invitation

Conditions

  • Bevacizumab
  • Hereditary Hemorrhagic Telangiectasia (HHT)
  • Pulmonary Hypertension

Eligibility Criteria

Sex
ALL
Age
18 Years - N/A
Healthy Volunteers
Not accepted

Interventions

  • Bevacizumab — DRUG
    This is a retrospective observational study of patients who received Bevacizumab as part of their routine clinical care. The study will describe efficacy and tolerance outcomes based on existing data (including echocardiographic, right heart catheterization, respiratory function, biologic and clinical data before and after treatment)

Study Details

High cardiac output secondary to hepatic arteriovenous malformations may be isolated or associated with left heart failure with post-capillary pulmonary hypertension. More rarely, precapillary pulmonary hypertension develops, linked to obstructive pulmonary arterial remodeling, referred to as pulmonary arterial hypertension (PAH), which affects younger patients and is not necessarily associated with hepatic arteriovenous malformation. BEVACIZUMAB is an anti-VEGF treatment indicated under compassionate use guidelines for hereditary hemorrhagic telangiectasia in cases of symptomatic hepatic arteriovenous malformations, when complicated by isolated high cardiac output or post-capillary pulmonary hypertension, and in cases of refractory chronic bleeding. However, the efficacy of this treatment on pulmonary hypertension related to high cardiac output, isolated or associated with left heart failure, is poorly understood. In addition, this treatment is classified as a "possible association" for the development of PAH, according to the 7th World Congress Symposium on Pulmonary Hypertension. Indeed, Hlavaty et al. found, based on pharmacovigilance data and by searching for disproportionate effects using the Bayesian network method, a possible link between the use of BEVACIZUMAB and the development of PAH. This treatment is therefore not recommended in cases of PAH associated with hereditary hemorrhagic telangiectasia. The objective of this study is to investigate the efficacy and tolerability of Bevacizumab treatment in hereditary hemorrhagic telangiectasia with cardiac involvement (isolated symptomatic high cardiac output or associated with post-capillary PAH) secondary to severe liver damage, based on the experience of the French hereditary hemorrhagic telangiectasia network since the CIROCO registry was opened in 2009.

Key Dates

First listed
Mar 3, 2026
Start date
Oct 15, 2025
Status verified
Oct 2025
Primary completion
Jun 30, 2026
Completion
Nov 30, 2026

Study Design

Enrollment
111 participants (estimated)

Arms

  • Arm: hereditary hemorrhagic telangiectasia patients
    Adult patients with hereditary hemorrhagic telangiectasia who have received treatment with BEVACIZUMAB for severe liver involvement with high cardiac output (symptomatic isolated high cardiac output or associated with post-capillary pulmonary hypertension or combined pulmonary hypertension or any pulmonary hypertension) in hereditary hemorrhagic telangiectasia within the French hereditary hemorrhagic telangiectasia network and registered in the CIROCO national hereditary hemorrhagic telangiectasia registry and having hemodynamic data (cardiac ultrasound or right heart catheterization) within 12 months prior to the start of Bevacizumab treatment

Primary Outcome Measure

Cardiac impairement before and after bevacizumab [ Time Frame: 6 months ]

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