title: Pacemaker in complicated and refractory breath-holding spells: when to think about it?
abstract: BACKGROUND
  Breath-holding spells (BHS) are benign non-epileptic paroxysmal events of infancy, rarely occurring with high frequency and complicated by prolonged syncope, convulsions and even status epilepticus. In these cases response to medical treatment is often unsatisfactory. Pacemaker implantation is a possible therapeutic option, but its indications, efficacy and complications have not been clarified yet.
  
  
  OBJECTIVE
  To report a new case of BHS treated with pacemaker and to review its indications and efficacy in patients with severe BHS.
  
  
  METHODS
  We extensively searched the literature in PubMed on cardiac pacing in patients with BHS and we described a new case.
  
  
  RESULTS
  A previously healthy boy presented at the age of 4 months with frequent BHS inconstantly associated to prolonged syncope and post-anoxic non-epileptic and epileptic seizures. Parental reassurance, iron supplementation and piracetam were ineffective. After cardiac pacing at the age of 16 months, BHS and their complications disappeared. We identified 47 patients with BHS treated with pacemaker in the literature. Based on the available data, in all patients asystole or marked bradycardia were documented during BHS or stimulating maneuvers; syncope complicated BHS in 100% of cases and post-anoxic convulsions in 78.3%. Medical treatment before pacing, when administered, was ineffective or poorly tolerated. After pacing, BHS complications disappeared in 86.4% of cases, and decreased in 13.6%. Technical problems with the device were reported in 25.7% of patients and mild medical complications in 11.4%.
  
  
  CONCLUSIONS
  Pacemaker could be reasonably considered in subjects with frequent and severe BHS, poor response to medications, and demonstration of cardioinhibition during spells.
authors.affiliation: Pediatric Neurology Unit, Division of Pediatrics, University of Padua, Padua, Italy. Electronic address: stefano.sartori@unipd.it.
authors.forename: Stefano
authors.initials: S
authors.lastname: Sartori
authors.affiliation: Pediatric Neurology Unit, Division of Pediatrics, University of Padua, Padua, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Nosadini
authors.affiliation: Cardiology Division, University of Padua, Padua, Italy.
authors.forename: Loira
authors.initials: L
authors.lastname: Leoni
authors.affiliation: Pediatric Neurology Unit, Division of Pediatrics, University of Padua, Padua, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: de Palma
authors.affiliation: Pediatric Neurology Unit, Division of Pediatrics, University of Padua, Padua, Italy.
authors.forename: Irene
authors.initials: I
authors.lastname: Toldo
authors.affiliation: Pediatric Cardiology Unit, Division of Pediatrics, University of Padua, Padua, Italy.
authors.forename: Ornella
authors.initials: O
authors.lastname: Milanesi
authors.affiliation: Pediatric Cardiology Unit, Division of Pediatrics, University of Padua, Padua, Italy.
authors.forename: Alessia
authors.initials: A
authors.lastname: Cerutti
authors.affiliation: Pediatric Neurology Unit, Division of Pediatrics, University of Padua, Padua, Italy.
authors.forename: Agnese
authors.initials: A
authors.lastname: Suppiej
mesh_terms: D062485:Breath Holding; D006801:Humans; D007223:Infant; D008297:Male; D010138:Pacemaker, Artificial; D012640:Seizures / Q000209:etiology; D013575:Syncope / Q000209:etiology
keywords: Asystole; Breath-holding spells; Cardiac pacing; Pacemaker; Post-anoxic convulsions; Post-anoxic convulsive epileptic seizures; Reflex anoxic seizures; Syncope