title: Long-term outcome following catheter valvotomy for pulmonary atresia with intact ventricular septum.
abstract: OBJECTIVE
  This study investigated the outcome for all patients undergoing catheter valve perforation for pulmonary atresia with intact ventricular septum (PAIVS) 21 years after the first procedure at their center.
  
  
  BACKGROUND
  Catheter perforation for PAIVS is now an established procedure. However, the management of the borderline right ventricle (RV) is controversial, and there may be a place for novel techniques such as stenting of the arterial duct.
  
  
  METHODS
  There were 37 successful valve perforations (total 39 patients). Median length of follow-up was 9.2 years (range 2.2 to 21.0 years). Seventeen patients had stenting of the arterial duct. The mean (SD) initial z-score for the tricuspid valve was -5.1 (±3.4), and a further 142 sets of measurements were taken to assess the growth of the RV of survivors.
  
  
  RESULTS
  There were 8 deaths (21%), and no deaths after the first 35 days. There were no late arrhythmias or ischemic events. Twenty-five patients (83% of survivors) have a biventricular circulation. For patients who had stenting of the arterial duct, significant reductions in early reintervention (0 vs. 7 patients, p = 0.009) and hospital stay (17.4 ± 18.1 days vs. 33.8 ± 28.6 days, p = 0.012) occurred, with no increase in mortality or morbidity. There was no catch-up growth of the RV in patients who had a biventricular outcome (z-score increase +0.08/year, p = 0.26).
  
  
  CONCLUSIONS
  Long-term survival is good, and even small RVs may be amenable to this procedure. Multiple interventions may be required to achieve biventricular circulation, but stenting of the arterial duct may reduce hospital stay and repeat procedures.
authors.affiliation: Department of Congenital Heart Disease, Evelina Children's Hospital, London, United Kingdom. henry.chubb@gstt.nhs.uk
authors.forename: Henry
authors.initials: H
authors.lastname: Chubb
authors.forename: Erkki
authors.initials: E
authors.lastname: Pesonen
authors.forename: Sivasankaran
authors.initials: S
authors.lastname: Sivasubramanian
authors.forename: Shane M
authors.initials: SM
authors.lastname: Tibby
authors.forename: John M
authors.initials: JM
authors.lastname: Simpson
authors.forename: Eric
authors.initials: E
authors.lastname: Rosenthal
authors.forename: Shakeel A
authors.initials: SA
authors.lastname: Qureshi
mesh_terms: D006328:Cardiac Catheterization; D002404:Catheterization / Q000379:methods*; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006346:Heart Septum / Q000000981:diagnostic imaging*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D018633:Pulmonary Atresia / Q000401:mortality / Q000601:surgery*; D015996:Survival Rate / Q000639:trends; D013548:Sweden / Q000453:epidemiology; D013997:Time Factors; D016896:Treatment Outcome; D006113:United Kingdom / Q000453:epidemiology