title: Impact of Percutaneous Interventions for Pulmonary Artery Stenosis in Alagille Syndrome.
abstract: OBJECTIVE
  The study aims to examine acute and midterm outcomes after percutaneous interventions for treatment of pulmonary artery stenosis (PAS) in patients with Alagille Syndrome (ALGS).
  
  
  BACKGROUND
  PAS affects up to two thirds of ALGS patients. Responsiveness to transcatheter therapies may differ from other causes of PAS. To date, there has been no study to evaluate outcomes of transcatheter interventions on PAS exclusively in ALGS.
  
  
  METHODS
  In this single-center series, we reviewed procedural, hemodynamic, and angiographic data from patients with ALGS and PAS from 2007 to 2011 who underwent an interventional catheterization. Minimal luminal diameter (MLD) was assessed pre- and postintervention, and at follow-up catheterization(s) when available. Acute and midterm response to high-pressure balloon angioplasty (HBA), bare metal stent (BMS) placement, and cutting balloon angioplasty (CBA) were assessed.
  
  
  RESULTS
  Nine patients (median age 9.1 years) underwent 16 cardiac catheterizations with 34 interventions performed (20 HBA, 11 BMS, 3 CBA). There was a significant acute increase in MLD for all three modalities (42% HBA, P < .01; 91% BMS, P < .01; 58% CBA, P = .04). Follow-up data were available for 19 treated lesions at a median of 11 months. There was no significant difference in the improvement of MLD from baseline between the HBA and BMS groups, although in contrast to the BMS group, the HBA group showed continued interval vessel growth.
  
  
  CONCLUSIONS
  Transcatheter intervention for PAS in ALGS is generally safe and acutely effective. Although BMS implantation was associated with the greatest immediate improvement in MLD, HBA-treated vessels demonstrate interval growth, whereas BMS-treated lesions do not.
authors.affiliation: Division of Cardiology, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors.forename: Matthew
authors.initials: M
authors.lastname: Zussman
authors.affiliation: Division of Cardiology, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors.forename: Russel
authors.initials: R
authors.lastname: Hirsch
authors.affiliation: Division of Cardiology, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors.forename: Robert H
authors.initials: RH
authors.lastname: Beekman
authors.affiliation: Division of Cardiology, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors.forename: Bryan H
authors.initials: BH
authors.lastname: Goldstein
mesh_terms: D000293:Adolescent; D016738:Alagille Syndrome / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D000800:Angioplasty, Balloon / Q000009:adverse effects / Q000295:instrumentation; D001157:Arterial Occlusive Diseases / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic; D005260:Female; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D008297:Male; D008670:Metals; D009820:Ohio; D011474:Prosthesis Design; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology; D011859:Radiography; D012189:Retrospective Studies; D015607:Stents; D013997:Time Factors; D016896:Treatment Outcome; D014654:Vascular Patency
chemical_list: D008670:Metals
keywords: Alagille Syndrome; Angioplasty; Cardiac Catheterization; Endovascular Stent; Pulmonary Artery Stenosis