title: Recombinant Activated Factor VII (Eptacog Alfa Activated, NovoSeven®) in Patients with Rare Congenital Bleeding Disorders. A Systematic Review on its Use in Surgical Procedures.
abstract: In the absence of definite guidelines in the area, we have carried a systemic review to provide a thorough overview concerning the efficacy and safety of recombinant activated factor VII (rFVIIa, NovoSeven®, Novo Nordisk A/S, Bagsværd, Denmark) in patients with Glanzmann's thrombasthenia (GT) and FVII deficiency, undergoing surgical procedures. PubMed, Web of Science, Scopus and EMBASE databases was employed for the search. Three multicenter registries were identified: the Glanzmann's Thrombasthenia Registry (GTR), the Seven Treatment Evaluation Registry (STER), and a German post-marketing surveillance registry (the WIRK study). In addition, data from 10 case-series and/or single-center experiences have been summarized. We have found that the following; perioperatively, the hemostatic effectiveness of rFVIIa was high in GT patients and in those with FVII deficiency undergoing both minor and major surgical procedures. Moreover, in all studies, rFVIIa was well tolerated. Thus, the current evidence shows an optimal perioperative safety/efficacy profile of rFVIIa in the setting of these rare bleeding disorders, and provides the rationale for further studies aimed at evaluating the optimal perioperative anti-hemorrhagic prophylaxis with rFVIIa in GT and in FVII deficient patients.
authors.affiliation: Department of Advanced Biomedical Sciences, Division of Cardiology, Federico II University, Naples, Italy.
authors.forename: Matteo Nicola Dario
authors.initials: MND
authors.lastname: Di Minno
authors.affiliation: Department of Clinical Medicine and Surgery, Federico II University, Naples, Italy.
authors.forename: Pasquale
authors.initials: P
authors.lastname: Ambrosino
authors.affiliation: Unit of Atherosclerosis Prevention, Centro Cardiologico Monzino IRCCS, Via Carlo Parea 4, 20138, Milan, Italy.
authors.forename: Veronika
authors.initials: V
authors.lastname: Myasoedova
authors.affiliation: Centro Cardiologico Monzino, IRCCS, Milan, Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: Amato
authors.affiliation: Department of Clinical Medicine and Surgery, Federico II University, Naples, Italy.
authors.forename: Itala
authors.initials: I
authors.lastname: Ventre
authors.affiliation: Centro Cardiologico Monzino, IRCCS, Milan, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Tremoli
authors.affiliation: Centro Cardiologico Monzino, IRCCS, Milan, Italy.
authors.forename: Alessandro Di
authors.initials: AD
authors.lastname: Minno
mesh_terms: D001778:Blood Coagulation Disorders / Q000188:drug therapy* / Q000601:surgery*; D015942:Factor VIIa / Q000627:therapeutic use*; D006470:Hemorrhage / Q000188:drug therapy*; D006801:Humans; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa
keywords: Glanzmann's thrombasthenia; NovoSeven; factor VII deficiency; recombinant activated factor VII