title: Heparan sulfate storage in the cardiac conduction system triggers atrioventricular block.
abstract: OBJECTIVE
  To elucidate the novel biological functions of heparan sulfate (HS) by clinic-pathologically studying a patient with paroxysmal atrioventricular (AV) block.
  
  
  METHODS
  A long-surviving male patient with Sanfilippo syndrome type A presented with paroxysmal AV block at age 33years. He then survived another 2.5years after the onset of paroxysmal AV block and pacemaker implantation.
  
  
  RESULTS
  His cardiac histopathological examination at autopsy showed HS storage in the cardiac conduction system (CCS), especially in the atrioventricular node (AVN)-His bundle branches.
  
  
  CONCLUSIONS
  HS storage in the CCS might trigger AV block, arising from below the AVN-His bundle branches. This is the first description to indicate that HS might be an essential constituent of life-long CCS plasticity and that its storage in the CCS results in AV block.
authors.affiliation: Department of Pediatrics and Child Neurology, Oita University School of Medicine, Yufu, Oita 8795593, Japan; Department of Pediatrics and Child Neurology, Beppu Developmental Medical Center for Cerebral Palsy, Mental Retardation and Severely Handicapped Children, Beppu, Oita 8740838, Japan.
authors.forename: Rie
authors.initials: R
authors.lastname: Kato
authors.affiliation: Department of Pediatrics and Child Neurology, Oita University School of Medicine, Yufu, Oita 8795593, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Miyahara
authors.affiliation: Department of Pediatrics and Child Neurology, Oita University School of Medicine, Yufu, Oita 8795593, Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Kawano
authors.affiliation: Department of Pediatrics and Child Neurology, Oita University School of Medicine, Yufu, Oita 8795593, Japan; Department of Pediatrics and Child Neurology, Beppu Developmental Medical Center for Cerebral Palsy, Mental Retardation and Severely Handicapped Children, Beppu, Oita 8740838, Japan.
authors.forename: Atsuko
authors.initials: A
authors.lastname: Matsuzuka
authors.affiliation: Department of Pediatrics and Child Neurology, Beppu Developmental Medical Center for Cerebral Palsy, Mental Retardation and Severely Handicapped Children, Beppu, Oita 8740838, Japan.
authors.forename: Kimiko
authors.initials: K
authors.lastname: Noda
authors.affiliation: Department of Pediatrics and Child Neurology, Oita University School of Medicine, Yufu, Oita 8795593, Japan; Department of Pediatrics and Child Neurology, Beppu Developmental Medical Center for Cerebral Palsy, Mental Retardation and Severely Handicapped Children, Beppu, Oita 8740838, Japan; Department of Pediatrics and Child Neurology, Nanao National Hospital, Nanao, Ishikawa 9268531, Japan. Electronic address: izumit@oita-u.ac.jp.
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Izumi
mesh_terms: D000328:Adult; D054537:Atrioventricular Block / Q000209:etiology* / Q000473:pathology*; D004562:Electrocardiography; D004569:Electroencephalography; D006329:Heart Conduction System / Q000503:physiopathology*; D006497:Heparan Sulfate / Q000378:metabolism*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D032383:Myocytes, Cardiac / Q000378:metabolism*
chemical_list: D006497:Heparan Sulfate
keywords: Atrioventricular block; Cardiac conduction system; Heparan sulfate; Long-surviving patient; Sanfilippo syndrome