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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| 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 |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| 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) |
| Measure | Description | Time Frame |
|---|---|---|
| Cardiac impairement before and after bevacizumab | mean pulmonary arterial pressure (right heart catheterization) or systolic pulmonary arterial pressure (cardiac ultrasound) in mmHg before (M0) and after (M6) treatment (bevacizumab) | 6 months |
| Measure | Description | Time Frame |
|---|---|---|
| Outcome | Death or hospitalization for heart failure or liver transplantation | 1 year |
| Adverse effects of bevacizumab | Adverse effects from bevacizumab such as hypertension, proteinuria, fatigue, nausea, diarrhea, joint pain, headache, dysphonia etc. |
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Inclusion Criteria:
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Adult patients with hereditary hemorrhagic telangiectasia who have received treatment with BEVACIZUMAB for severe liver involvement with high cardiac output in hereditary hemorrhagic telangiectasia within the French hereditary hemorrhagic telangiectasia network and registered in the CIROCO national hereditary hemorrhagic telangiectasia registry
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Centre hospitalier universitaire de Poitiers | Poitiers | 86000 | France |
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|
| 6 months |
| Right ventricular function | Peak S wave (in cm/s) measure in ultrasound | 6 months |
| peak tricuspid regurgitant velocity | peak tricuspid regurgitant velocity (in m/s) mesured in cardiac ultrasound | 6 months |
| Liver ultrasound before and after bevacizumab | Hepatic artery diameter (in cm) | 6 months |
| Clinical evolution before and after bevacizumab | Dyspnea in NYHA | 6 months |
| Cardiac output | Cardiac output in L/min before (M0) and after (M6) treatment (bevacizumab) | 6 months |
| right ventricular function | TAPSE (in mm) measured in cardiac ultra sound | 6 months |
| ID | Term |
|---|---|
| D013683 | Telangiectasia, Hereditary Hemorrhagic |
| D006976 | Hypertension, Pulmonary |
| ID | Term |
|---|---|
| D020141 | Hemostatic Disorders |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
| D013684 | Telangiectasis |
| D006474 | Hemorrhagic Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D054079 | Vascular Malformations |
| D018376 | Cardiovascular Abnormalities |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
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| ID | Term |
|---|---|
| D000068258 | Bevacizumab |
| ID | Term |
|---|---|
| D061067 | Antibodies, Monoclonal, Humanized |
| D000911 | Antibodies, Monoclonal |
| D000906 | Antibodies |
| D007136 | Immunoglobulins |
| D007162 | Immunoproteins |
| D001798 | Blood Proteins |
| D011506 | Proteins |
| D000602 | Amino Acids, Peptides, and Proteins |
| D012712 | Serum Globulins |
| D005916 | Globulins |
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