title: Palliative balloon pulmonary valvuloplasty for infants with unrestrictive ventricular septal defect or single ventricle associated with severe pulmonary stenosis.
abstract: BACKGROUND
  Symptomatic infants with unrestrictive ventricular septal defect (VSD) or single ventricle with severe pulmonary stenosis (PS) are typically treated surgically. High surgical risk infants may benefit from catheter-based palliative pulmonary valvuloplasty.
  
  
  METHODS
  We retrospectively reviewed all patients with severe PS associated with unrestrictive VSD or single ventricle who underwent palliative pulmonary valvuloplasty as initial management and describe outcomes.
  
  
  RESULTS
  From 2000 to 2013, 16 patients met inclusion criteria and underwent valvuloplasty at a median age and weight of 25 (range 1-352) days and 3.65 (range 2.2-12) kg. Common diagnoses included tetralogy of Fallot (50%) and double-outlet right ventricle (25%). In all, the indication was cyanosis. Reasons to defer surgery included low weight (37.5%), preference for complex operation at older age (31.25%) and significant comorbidity (18.75%). Following valvuloplasty, significant increases in oxygen saturation (83.1±8.8 vs. 74.7±8.7%, P=0.008) and QP:QS (1.6±0.5 vs. 0.8±0.3, P=0.0005) occurred. All two ventricle patients (n=10) ultimately had complete operative repair at a median of 125 (range 27-382) days after valvuloplasty, during which time there was 20.3±9.2 g/day of weight gain without change in oxygen saturation. Single ventricle patients who underwent cavopulmonary connection (n=3) had similar weight gain and palliation time, but with a decrease in oxygen saturation. Two patients required reintervention to augment QP prior to definitive repair.
  
  
  CONCLUSIONS
  In select infants with unrestrictive VSD or single ventricle associated with severe PS, palliative pulmonary valvuloplasty provides an effective and durable method of increasing oxygen saturation until definitive surgical treatment can be performed.
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Pennsylvania.
authors.forename: Ramiro W
authors.initials: RW
authors.lastname: Lizano Santamaria
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Pennsylvania.|Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: Gillespie
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Pennsylvania.|Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Yoav
authors.initials: Y
authors.lastname: Dori
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Pennsylvania.|Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Jonathan J
authors.initials: JJ
authors.lastname: Rome
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Pennsylvania.|Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.|Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Andrew C
authors.initials: AC
authors.lastname: Glatz
mesh_terms: D063126:Balloon Valvuloplasty / Q000009:adverse effects; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D006352:Heart Ventricles / Q000002:abnormalities* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care / Q000379:methods*; D011664:Pulmonary Valve / Q000503:physiopathology*; D011666:Pulmonary Valve Stenosis / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D020127:Recovery of Function; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome
keywords: balloon valvuloplasty; congenital heart defect; cyanosis; pulmonary valve stenosis