title: Pulmonary atresia with ventricular septal defect and multifocal pulmonary blood supply: does an intensive interventional approach improve the outcome?
abstract: BACKGROUND
  Pulmonary atresia with ventricular septal defect (VSD) continues to be associated with significant morbidity and mortality, with significant institutional variation in therapeutic strategies. This study reports a single center experience utilizing an intensive transcatheter approach to promote pulmonary vascular growth.
  
  
  METHODS
  A retrospective analysis of 20 patients undergoing surgical and transcatheter treatment for pulmonary atresia with VSD between 2002 and 2010.
  
  
  RESULTS
  The median age at initial surgical palliation was 6.3 months (8 days to 2.5 years). Eleven patients (group 1) underwent initial surgical palliation without VSD closure and nine patients (group 2) underwent an initial complete repair with fenestrated or complete VSD closure. Group 1 had a smaller Nakata index (54 mm2/m2 vs. 134 mm2/m2 , P = .04) and a smaller absolute native pulmonary artery diameter (2.7 mm vs. 4.5 mm, P = .01) than group 2. Intraoperative angiography was performed in 10 cases to evaluate if early transcatheter intervention was warranted. The median follow-up during the study period was 2.3 years (1.6 months to 8.3 years). Of the 16 patients who survived the initial early postoperative period, 15 patients (94%) went on to receive surgical (n = 11) and/or interventional (n = 25) catheterization procedures. There was improvement in the mean Nakata index from the initial presurgical evaluation to the most recent catheterization data (38.4 mm2/m2 vs. 169.7 mm2/m2, P ≤ .05). To date, two of 11 (18%) patients in group 1 ultimately underwent surgical VSD closure. Overall mortality was six of 20 (30%) with four deaths in group 1 and two deaths in group 2. There were no procedural deaths.
  
  
  CONCLUSIONS
  Combining surgical unifocalization procedures with subsequent early and intensive catheter-based pulmonary artery rehabilitation may improve vascular growth, ultimately rendering many patients suitable for fenestrated VSD closure. Risk stratification, including intraoperative exit angiography, is essential to determine the need for early transcatheter interventions.
authors.affiliation: Nationwide Children's Hospital, The Heart Center, Columbus, Ohio 43205, USA. Christopher.Learn@nationwidechildrens.org
authors.forename: Christopher
authors.initials: C
authors.lastname: Learn
authors.forename: Alistair
authors.initials: A
authors.lastname: Phillips
authors.forename: Joanne
authors.initials: J
authors.lastname: Chisolm
authors.forename: Sharon
authors.initials: S
authors.lastname: Hill
authors.forename: John
authors.initials: J
authors.lastname: Cheatham
authors.forename: Peter
authors.initials: P
authors.lastname: Winch
authors.forename: Mark
authors.initials: M
authors.lastname: Galantowicz
authors.forename: Ralf
authors.initials: R
authors.lastname: Holzer
mesh_terms: D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D004062:DiGeorge Syndrome / Q000401:mortality; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000401:mortality* / Q000534:rehabilitation / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D009017:Morbidity; D010166:Palliative Care / Q000379:methods; D018633:Pulmonary Atresia / Q000401:mortality* / Q000534:rehabilitation / Q000601:surgery*; D011652:Pulmonary Circulation / Q000502:physiology*; D019233:Retreatment; D012189:Retrospective Studies; D012307:Risk Factors; D013771:Tetralogy of Fallot / Q000401:mortality / Q000534:rehabilitation / Q000601:surgery; D016896:Treatment Outcome