title: Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum.
abstract: OBJECTIVE
  To review the outcome of patients with pulmonary atresia with intact ventricular septum after interventional perforation of the pulmonary valve, to assess the capability of this procedure to avoid neonatal or late intervention and to obtain a long-term biventricular repair.
  
  
  METHODS
  Retrospective interventional study and clinical follow-up study.
  
  
  METHODS
  Tertiary referral centre.
  
  
  METHODS
  Between November 1994 and December 2007, 40 neonates underwent radiofrequency perforation. Median age at pulmonary valvotomy was 28 hours (range 1-147 hours) and median weight was 2925 g (range 1900-4400 g).
  
  
  METHODS
  Procedural success and complication rates; early-term and long-term follow-up results.
  
  
  RESULTS
  The procedure was successful in 39 patients but 16 of them needed neonatal surgery. The overall mortality was 7.5%. At a median follow-up of 82 months, four patients underwent a bidirectional Glenn procedure, whereas all the other patients achieved a biventricular circulation without any further intervention in 19 of them. Patients who died or needed additional intervention with or without biventricular circulation failure had a higher incidence of bipartite right ventricular (65% vs 15.8% of those not needing additional intervention; p = 0.004) and a lower median tricuspid Z value (-2 (range -3.5 to 1) vs -0.5 (range -2 to 1); p = 0.004)).
  
  
  CONCLUSIONS
  The results confirm that percutaneous interventional perforation is an effective first-stage procedure in patients with pulmonary atresia with intact ventricular septum. The right heart appeared to be adequate to maintain a long-term biventricular circulation in the large majority of cases.
authors.affiliation: Department of Pediatric Cardiology and Cardiac Surgery, Giannina Gaslini Istitute, Genova, Italy. mauriziomarasini@ospedale-gaslini.ge.it
authors.forename: M
authors.initials: M
authors.lastname: Marasini
authors.forename: P F
authors.initials: PF
authors.lastname: Gorrieri
authors.forename: G
authors.initials: G
authors.lastname: Tuo
authors.forename: L
authors.initials: L
authors.lastname: Zannini
authors.forename: P
authors.initials: P
authors.lastname: Guido
authors.forename: M
authors.initials: M
authors.lastname: Pellegrini
authors.forename: S
authors.initials: S
authors.lastname: Bondanza
authors.forename: M G
authors.initials: MG
authors.lastname: Calevo
authors.forename: G
authors.initials: G
authors.lastname: Pongiglione
mesh_terms: D000293:Adolescent; D017115:Catheter Ablation / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007902:Length of Stay; D008297:Male; D018633:Pulmonary Atresia / Q000401:mortality / Q000601:surgery*; D011664:Pulmonary Valve / Q000601:surgery*; D012086:Reoperation; D012189:Retrospective Studies; D016896:Treatment Outcome; D054088:Ventricular Septum