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- W3103800282 abstract "The management of pulmonary atresia with intact ventricular septum (PAIVS) can result in univentricular, biventricular or a “1.5 ventricle” circulation. There is increasing consideration of catheter-based therapy as part of the treatment algorithm. The management strategies utilised and outcomes of infants with PAIVS at our centre have not been examined. A retrospective case series was undertaken including all infants with PAIVS born January 2009 until July 2019 (follow-up until January 2020) managed at The Children’s Hospital at Westmead. Demographic features, anatomical substrate, management pathway and subsequent clinical outcomes were examined. Fifty-two infants were included (male n=28, 53.8%). Antenatal diagnosis had been made in 45 (86.5%). Based on initial echocardiogram, the tricuspid valve annulus z-score (Detroit 2008) measured <-3 in 19 (36.5%) infants. Right ventricle dependent coronary circulation was suspected in 9 (17.3%) infants. Thirty-six (69.2%) infants underwent an initial surgical intervention. Sixteen (30.8%) underwent an initial catheter based intervention, including an attempted pulmonary valve perforation in 12. Four infants had a pulmonary valve perforation at a subsequent procedure. A catheter-based intervention (pulmonary valve intervention, patent ductus arteriosus Stent, balloon atrial septostomy) was more likely in the second half of the study period. Median follow-up was 62 months (range 3-119 months). Final circulation status was known in 38 infants (biventricular n=14, 26.9%, “1.5 ventricles” n=5, 9.6%, single ventricle n=19, 36.5%). Two infants were lost to follow-up prior to final circulation status being known. There were 5 deaths during the study period. PAIVS is a relatively rare condition with variation in underlying anatomy and subsequent management at our centre. There was increasing use of catheter-based therapy over the study period. Further analysis is planned." @default.
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- W3103800282 date "2020-01-01" @default.
- W3103800282 modified "2023-09-26" @default.
- W3103800282 title "680 Pulmonary Atresia With Intact Ventricular Septum – A Single Centre Experience" @default.
- W3103800282 doi "https://doi.org/10.1016/j.hlc.2020.09.687" @default.
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