title: Successful mechanical circulatory support for 251 days in a child with intermittent severe neutropenia due to Barth syndrome.
abstract: Barth syndrome is an X-linked recessive disorder that is characterized by cardiomyopathy, variable neutropenia, skeletal myopathy, growth delay, and organic aciduria. The cardiac involvement typically results in a high risk of severe heart failure in infancy or early childhood. While Berlin Heart EXCOR is widely accepted as ventricular assistance in pediatric patients with end-stage cardiac failure, infections remain a frequent and potentially severe complication. Therefore, the extended use of the device in the setting of intermittent or severe neutropenia is challenging. We present the case of a three-yr child with Barth syndrome who was bridged successfully to transplant with a Berlin Heart EXCOR assist device for eight months (251 days) without major infectious complication, despite several episodes of severe neutropenia. This case demonstrates that prolonged mechanical circulatory support for a patient with neutropenia is feasible without important morbidity, with careful monitoring and a multidisciplinary approach. G-CSF provides an excellent support in managing neutropenia.
authors.affiliation: Pediatric Cardiology, Cardiothoracic Unit, Great Ormond Street Hospital, London, UK. nathaliededieu@hotmail.com
authors.forename: Nathalie
authors.initials: N
authors.lastname: Dedieu
authors.forename: Alessandro
authors.initials: A
authors.lastname: Giardini
authors.forename: Colin G
authors.initials: CG
authors.lastname: Steward
authors.forename: Matthew
authors.initials: M
authors.lastname: Fenton
authors.forename: Ann
authors.initials: A
authors.lastname: Karimova
authors.forename: T Y
authors.initials: TY
authors.lastname: Hsia
authors.forename: Michael
authors.initials: M
authors.lastname: Burch
mesh_terms: D056889:Barth Syndrome / Q000150:complications / Q000601:surgery*; D002675:Child, Preschool; D006353:Heart-Assist Devices; D006801:Humans; D008297:Male; D009503:Neutropenia / Q000209:etiology*