title: Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals.
abstract: OBJECTIVE
  Among 63 patients with pulmonary atresia and ventricular septal defect (VSD), 10 patients with extreme hypoplasia of the pulmonary arteries (PA) (mean Nakata index 20.6 mm(2)/m(2)), but with confluent arteries and a diminutive main PA, and major aorto-pulmonary collaterals (MAPCAS), have been submitted to a 'rehabilitation' of the PA with several stages: (i) connection between RV and PAs, (ii) interventional catheterizations, (iii) complete correction with or without unifocalisation. We report here the results of this approach.
  
  
  METHODS
  The RV-PA connection was direct (nine cases) or with an homograft conduit (one case), done under normothermic cardiopulmonary by-pass in patients aged 4.9 months (range 0.1-18 months). Subsequently, six underwent interventional catheterizations (dilations and stents in the PA, MAPCAS occlusion by coils). Complete correction was done in seven patients (mean age 30 months, range 8-49). One patient is awaiting correction.
  
  
  RESULTS
  One patient died after the first stage. All patients having had the third stage had a satisfactory development of the PA, had a complete closure of the VSD and a satisfactory reconstruction of the PA bifurcation. There was one death of severe pulmonary infection 6 months after repair. All other patients have been followed by catheterization and/or echocardiograms. With a follow-up of 83+/-65 months, all patients are improved, 50% have no cardiac medications, none has residual shunt, RV/LV pressure ratio is 0.6 (range 0.3-1).
  
  
  CONCLUSIONS
  The strategy of 'rehabilitation' of PA allowing: (i) antegrade flow in the PA, (ii) interventional catheterizations, (iii) growth of the PA with possible angiogenesis, (iv) complete correction, is a logical approach to be undertaken in the young patient and is a valid alternative to strategies relying more on MAPCAS for pulmonary vascular supply. The therapeutic sequences depend upon the individual anatomy.
authors.affiliation: Cardio-thoracic Unit, Children's Hospital La Timone, Marseille, France. dmetras@ap-hm.fr
authors.forename: D
authors.initials: D
authors.lastname: Metras
authors.forename: P
authors.initials: P
authors.lastname: Chetaille
authors.forename: B
authors.initials: B
authors.lastname: Kreitmann
authors.forename: A
authors.initials: A
authors.lastname: Fraisse
authors.forename: O
authors.initials: O
authors.lastname: Ghez
authors.forename: A
authors.initials: A
authors.lastname: Riberi
mesh_terms: D001013:Aorta, Thoracic / Q000002:abnormalities / Q000000981:diagnostic imaging; D006348:Cardiac Surgical Procedures / Q000379:methods; D002675:Child, Preschool; D003097:Collateral Circulation; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000601:surgery; D006352:Heart Ventricles / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D018633:Pulmonary Atresia / Q000150:complications* / Q000601:surgery; D011859:Radiography