Independent factors associated with mortality, reintervention, and achievement of complete repair in children with pulmonary atresia with ventricular septal defect.
OBJECTIVE
We described morphologic characteristics, particularly pulmonary anatomy, and determined the prevalence of definitive end states and their determinants in children with pulmonary atresia associated with ventricular septal defect (PAVSD).


BACKGROUND
Pulmonary atresia associated with ventricular septal defect represents a broad morphologic spectrum that greatly influences management and outcomes.


METHODS
From 1975 to 2004, 220 children with PAVSD presented to our institution. Blinded angiographic review (n = 171) characterized bronchopulmonary segment arterial supply.


RESULTS
A total of 185 patients underwent surgery, and repair was definitive in 75%. Initial operations included systemic-pulmonary artery shunt in 57%, complete primary repair in 31%, or right ventricular outflow tract reconstruction in 12%. Based on angiographic review, 118 patients had simple PAVSD and 53 patients had PAVSD with major aortopulmonary collateral arteries (MAPCAs). Overall survival from initial operation was 71% at 10 years. Risk factors for death after initial operation included younger age at repair, earlier birth cohort, fewer bronchopulmonary segments supplied by native pulmonary arteries, and initial placement of a systemic-pulmonary artery shunt. Competing-risks analysis for initially palliated patients predicted that after 10 years, 68% achieved complete repair (with associated factors including later birth cohort and more bronchopulmonary segments supplied by native pulmonary arteries), 22% died without repair, and 10% remained alive without repair. Reoperations after complete repair occurred in 38 children (27%), with risk factors including older age at palliation, MAPCAs, and more segments supplied by collaterals.


CONCLUSIONS
Outcomes in children with PAVSD have improved over time, and are better in completely repaired cases. Bronchopulmonary arterial supply is an important determinant of mortality, achievement of definitive repair, and post-repair reoperation.
Department of Pediatric Cardiology, Göteborg University, The Queen Silvia Children's Hospital, Göteborg, Sweden.
Kerstin M
KM
Amark
Tara
T
Karamlou
Aoife
A
O'Carroll
Cathy
C
MacDonald
Robert M
RM
Freedom
Shi-Joon
SJ
Yoo
William G
WG
Williams
Glen S
GS
Van Arsdell
Christopher A
CA
Caldarone
Brian W
BW
McCrindle
D000293:Adolescent; D000792:Angiography; D001027:Aortography; D001980:Bronchi / Q000098:blood supply; D002648:Child; D002675:Child, Preschool; D003097:Collateral Circulation; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000000981:diagnostic imaging / Q000401:mortality* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000098:blood supply; D008297:Male; D011651:Pulmonary Artery / Q000000981:diagnostic imaging; D018633:Pulmonary Atresia / Q000150:complications / Q000000981:diagnostic imaging / Q000401:mortality* / Q000601:surgery*; D012086:Reoperation / Q000706:statistics & numerical data; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome