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Systemic bevacizumab for high-output cardiac failure in hereditary hemorrhagic telangiectasia: an international survey of HHT centers.


ABSTRACT: BACKGROUND:Systemic bevacizumab is a novel targeted anti-angiogenic therapy for high-output cardiac failure (HOCF) in hereditary hemorrhagic telangiectasia (HHT) but published data is limited. This survey-based study measured physician-reported safety, effectiveness and current treatment practices for systemic bevacizumab in HHT-HOCF. METHODS:A 27-item survey was sent to center directors of 31 international HHT Centers of Excellence. RESULTS:Response rate was 74% with centers reporting 150 total patients receiving systemic bevacizumab for HHT-HOCF. Approximately two-thirds of centers had treated ?5 patients. All centers utilize a 5?mg/kg dose for induction treatment and most administer 6 doses (range, 4-6) every 2 weeks, although maintenance regimens varied considerably. Center directors reported bevacizumab to be effective, with 55% reporting significant improvement in cardiac index and HOCF symptoms in most patients treated with bevacizumab, although normalization of cardiac parameters was uncommon. Adverse events were uncommon with three-quarters of centers reporting adverse event rates

SUBMITTER: Al-Samkari H 

PROVIDER: S-EPMC6857247 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature

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Systemic bevacizumab for high-output cardiac failure in hereditary hemorrhagic telangiectasia: an international survey of HHT centers.

Al-Samkari Hanny H   Albitar Hasan A HA   Olitsky Scott E SE   Clancy Marianne S MS   Iyer Vivek N VN  

Orphanet journal of rare diseases 20191114 1


<h4>Background</h4>Systemic bevacizumab is a novel targeted anti-angiogenic therapy for high-output cardiac failure (HOCF) in hereditary hemorrhagic telangiectasia (HHT) but published data is limited. This survey-based study measured physician-reported safety, effectiveness and current treatment practices for systemic bevacizumab in HHT-HOCF.<h4>Methods</h4>A 27-item survey was sent to center directors of 31 international HHT Centers of Excellence.<h4>Results</h4>Response rate was 74% with cente  ...[more]

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