title: Supraventricular Tachycardia During Status Epilepticus in Dravet Syndrome: A Link Between Brain and Heart?
abstract: BACKGROUND
  The possibility that epileptic seizures and arrhythmias are different clinical manifestations of a common channelopathy is an interesting but unproved hypothesis. Patients with Dravet syndrome show heart rate variability and affected individuals with arrhythmias have also been documented. The possibility that a genetic mutation affecting sodium channel functions may predispose to both Dravet syndrome and arrhythmogenic disorders is an interesting hypothesis.
  
  
  METHODS
  We describe a 5-month-old girl with Dravet syndrome who presented with paroxysmal supraventricular tachycardia during status epilepticus. She presented to the hospital the first time with afebrile tonic-clonic seizures and then several subsequent times with status epilepticus confirmed with electroencephalography. During two of these episodes she also exhibited paroxysmal supraventricular tachycardia. She received propofol for status epilepticus and adenosine for the arrhythmia. A clinical and genetic (denovo mutation of a sodium channel, SCN1A) diagnosis of Dravet syndrome was made.
  
  
  CONCLUSIONS
  Our patient supports the hypothesis that SCN1A mutation might have a role as a common substrate to both epilepsy and cardiac arrhythmia. More studies are needed to better assess genetic, cardiac, respiratory, and autonomic dysfunction in patients with Dravet syndrome.
authors.affiliation: Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Daverio
authors.affiliation: Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy.
authors.forename: Ornella
authors.initials: O
authors.lastname: Ciccone
authors.affiliation: Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy.
authors.forename: Clementina
authors.initials: C
authors.lastname: Boniver
authors.affiliation: Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: De Palma
authors.affiliation: Division of Cardiology, Department of Cardiac, Thoracic and Vascular Sciences, University Hospital of Padua, Padua, Italy.
authors.forename: Domenico
authors.initials: D
authors.lastname: Corrado
authors.affiliation: Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy. Electronic address: vecchi@pediatria.unipd.it.
authors.forename: Marilena
authors.initials: M
authors.lastname: Vecchi
mesh_terms: D001921:Brain / Q000503:physiopathology*; D004562:Electrocardiography; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000150:complications* / Q000235:genetics; D005260:Female; D006321:Heart / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D009154:Mutation / Q000235:genetics; D062550:NAV1.1 Voltage-Gated Sodium Channel / Q000235:genetics; D013226:Status Epilepticus / Q000209:etiology*; D013617:Tachycardia, Supraventricular / Q000209:etiology*
chemical_list: D062550:NAV1.1 Voltage-Gated Sodium Channel; C568242:SCN1A protein, human
keywords: Dravet syndrome; SCN1A; arrhythmia; channelopathy; paroxysmal supraventricular tachycardia