title: Acquired von Willebrand syndrome in a child following Berlin Heart EXCOR Pediatric Ventricular Assist Device implantation: case report and concise literature review.
abstract: The development of acquired von Willebrand syndrome (AVWS) after placement of a pulsatile-flow left ventricular assist device (LVAD) is rare and only recently recognized. We report the case of a young infant who was diagnosed with ventricular assist device (VAD)-related AVWS following implantation of a Berlin Heart EXCOR Pediatric Ventricular Assist Device (Berlin Heart Inc., The Woodlands, Texas, USA) for treatment of severe heart failure. Despite significant bleeding, the patient was successfully managed with von Willebrand factor-containing concentrate until VAD explantation led to definitive resolution of the AVWS. This case demonstrates that the possibility of this diagnosis should be considered in pediatric patients when extensive, nonsurgical bleeding is encountered after pulsatile-flow VAD implantation.
authors.affiliation: Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA The Sheikh Zayed Institute for Pediatric Surgical Innovation, Children's National Health System, Washington, DC, USA.
authors.forename: John P
authors.initials: JP
authors.lastname: Costello
authors.affiliation: Department of Hematology, Children's National Health System, Washington, DC, USA.
authors.forename: Yaser A
authors.initials: YA
authors.lastname: Diab
authors.affiliation: Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Philippe-Auguste
authors.affiliation: Department of Critical Care Medicine, Children's National Health System, Washington, DC, USA.
authors.forename: Melissa B
authors.initials: MB
authors.lastname: Jones
authors.affiliation: Department of Critical Care Medicine, Children's National Health System, Washington, DC, USA.
authors.forename: Venkat
authors.initials: V
authors.lastname: Shankar
authors.affiliation: Hemostasis Reference Laboratory, Blood Center of Wisconsin, Milwaukee, WI, USA.
authors.forename: Kenneth D
authors.initials: KD
authors.lastname: Friedman
authors.affiliation: Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA dnath@childrensnational.org.
authors.forename: Dilip S
authors.initials: DS
authors.lastname: Nath
mesh_terms: D020878:Device Removal; D005260:Female; D006333:Heart Failure / Q000601:surgery*; D006353:Heart-Assist Devices / Q000009:adverse effects*; D006801:Humans; D007223:Infant; D019106:Postoperative Hemorrhage / Q000209:etiology / Q000628:therapy; D014842:von Willebrand Diseases / Q000209:etiology* / Q000628:therapy; D014841:von Willebrand Factor / Q000008:administration & dosage
chemical_list: D014841:von Willebrand Factor
keywords: blood; circulatory assist devices; heart failure; hematology