[Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries].
BACKGROUND
In order to establish the best strategy of treatment and predictors of outcome in infants with pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries, we reviewed our institutional experience concerning 47 infants.


METHODS
Inclusion criteria included an angiographic diagnosis of pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries with or without central pulmonary arteries and a repair by the same surgeon. Thirty-one patients had confluent (type III) and 16 absent (type IV) central pulmonary arteries. Pulmonary arteries were considered to be adequate when they measured > or = 4 mm.


RESULTS
There were 8 deaths (17%). Complete repair was performed in 24 patients (18 in group III and 6 in group IV) with 79.3% had a right/left ventricular pressure <0.5, 16; 5%<1 and 4.2%>1. Eleven patients are waiting for complete repair and 4 will be operated on pulmonary arteries. One stage complete repair was performed in 3 patients, two and third stage repair (after unifocalisation or right ventricle to pulmonary arteries conduit) was attained in 21 patients. Among patients having had a complete repair and showing a right/left ventricular pressure <0.5, 88.9% had a pulmonary atresia with ventricular septal defect type III and 50% a pulmonary atresia with ventricular septal defect type IV. Only 56% of type III patients with a right/left ventricular pressure <0.5 had adequate central pulmonary arteries.


CONCLUSIONS
In our study, the small size and the absence of central pulmonary arteries do not prevent a positive outcome.
Bonnet
C
C
Service de cardiologie pédiatrique, groupe hospitalier Necker-Enfants malades, Paris. caroline.bonnet@chu-dijon.fr
Agnoletti
G
G
Boudjemline
Y
Y
Beyler
C
C
Sidi
D
D
Vouhé
P
P
Bonnet
D
D
D001011:Aorta; D013504:Cardiovascular Surgical Procedures / Q000379:methods*; D003097:Collateral Circulation; D017023:Coronary Angiography; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000209:etiology* / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D011651:Pulmonary Artery; D018633:Pulmonary Atresia / Q000150:complications* / Q000601:surgery*; D016896:Treatment Outcome; D016277:Ventricular Function, Left