Efficacy and Safety of Bevacizumab for the Treatment Hemorrhagic Hereditary Telangiectasia (HHT) Associated With Severe Hepatic Vascular Malformations. Phase II Study

Inclusion Criteria: * General criteria: * Age ≥ 18 years and \< 70 years * Subjects must have given their free and enlightened consent and have signed the consent form. * HHT related criteria * Patients monitored for clinically confirmed HHT disease. * Patients with severe liver involvement in relationship with the HHT disease * Patients with a high cardiac output on ultrasound. * Associated disease related criteria * Blood: neutrophil ≥ 1.0x109 / L and platelets ≥ 100x109 / L. * INR (International Normalized Ratio) ≤ 1.5 (except for patients on anticoagulants) and TCA ≤ 1.5 x upper limit of the standard laboratory * Renal function: creatinine ≤ 1.25 x upper limit of the standard laboratory. Patients who proteinuria to strip ≥ 2 + will have control of the Proteinuria of 24 hours to be ≤ 1g / 24 hours Exclusion Criteria: * • General criteria * Women who are pregnant or liable to become pregnant in the course of the trial. * Patients who have reached their majority but who are protected by the terms of the law (French public health code). * Refusal to give enlightened consent. * Patients who are not affiliated to a health insurance regime • Criteria for the medical history * Patients in whom the diagnosis of HHT disease has not been confirmed. * The presence of atrial fibrillation on the electrocardiogram at the inclusion. * The presence of cerebral arteriovenous malformations on the angioMRI done in the year prior to inclusion. * Existence of diverticulitis of the colon or sigmoid * Thrombosis within 6 months before inclusion * Infectious disease treated by antibiotics and unresolved at inclusion. * Patients with blood pressure that is not being controlled at the time of inclusion (systolic blood pressure\> 150 mmHg and / or diastolic\> 100 mmHg) with or without treatment. Patients who have high blood pressure can be included when the blood pressure numbers have been standardized by appropriate medical treatment. • Surgical criteria * Major surgery (including open biopsy) or severe trauma within 28 days preceding the start of treatment. • Medical treatments * Current or recent use of non steroidal anti-inflammatory drugs or antiplatelet therapy 10 days before the first administration of Bevacizumab. * Use of oral or parenteral anticoagulants or thrombolytic agents within 28 days preceding inclusion (anticoagulants given prophylactically are permitted). * Participation in another clinical trial within 28 days preceding inclusion. * Vaccination with live vaccines or against yellow fever during the treatment period. * Administration of phenytoin (Di-hydan ® DILANTIN ®) during the treatment period. • Allergy * Hypersensitivity to the active substance or any of its excipients. * Hypersensitivity to products made from Chinese hamster ovary (CHO) cells or to any other human or humanized recombinant antibodies.