Unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

Adriano Carotti, Sonia Albanese, Roberto Di Donato

Research output: Contribution to journalArticlepeer-review


Aim: To correlate anatomic and genetic features of paediatric patients with pulmonary atresia, ventricular septal defect (VSD) and multiple aortopulmonary collateral arteries with surgical outcome. Methods: 44 consecutive patients aged 33±40 mo underwent either primary one-stage unifocalization (n =32) or palliative right ventricular outflow tract reconstruction (n =12) followed by secondary unifocalization and repair (n =10) based on preoperative morphometric and functional evaluation of pulmonary blood sources. Chromosome 22q11.2 microdeletion occurred in 41% of cases. Combined VSD closure during one-stage procedures was guided by an intraoperative pulmonary flow study. Complete repair was accomplished in 35 cases (83%, 95% CI 72-95%). Variables examined included occurrence of confluent intrapericardial pulmonary arteries, central pulmonary arteries, confluent intraparenchymal pulmonary arteries, dominant collateral or pulmonary arteries, and chromosome 22q11.2 microdeletion. The sensitivity and specificity of the pulmonary flow study in predicting postoperative pulmonary haemodynamics were also tested. Results: Eight-year actuarial survival and freedom from reoperation were 85% and 63%, respectively. Sensitivity and specificity of the pulmonary flow study were 94% and 100%, respectively. None of the anatomical variables examined was significantly related to the outcome of treatment. The only statistically relevant association was detected between survival and occurrence of 22q11.2 microdeletion (p

Original languageEnglish
Pages (from-to)22-26
Number of pages5
JournalActa Paediatrica, International Journal of Paediatrics
Issue numberSUPPL. 452
Publication statusPublished - Jul 1 2006


  • Deletion 22q11
  • Fallot's tetralogy
  • Major aortopulmonary collateral arteries
  • Pulmonary atresia with ventricular septal defect
  • Unifocalization

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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