RESUMO
BACKGROUND: There is increasing use of catheter-based therapy as part of the neonatal treatment algorithm for pulmonary atresia with an intact ventricular septum (PAIVS). The management strategies utilised and outcomes of patients with PAIVS at our centre have not been examined. METHODS: A retrospective case series was undertaken including all infants with PAIVS born January 2009 to July 2019 (follow-up to January 2020) managed at The Children's Hospital at Westmead, New South Wales. Demographic features, anatomical substrate, management pathway and subsequent clinical outcomes were examined. RESULTS: Fifty-two (52) infants were included (male n=28, 53.8%). The right ventricular morphology was classified as normal, mildly, moderately and severely hypoplastic in 3 (5.8%), 13 (25.0%), 19 (36.5%) and 17 (32.7%) patients respectively. Thirty-seven (37) patients underwent an initial aortopulmonary (AP) shunt (surgical shunt or patent ductus arteriosus [PDA] stent). The remaining 15 patients underwent an initial intervention to decompress the right ventricle. Twenty (20) patients underwent a neonatal catheter-based intervention. An initial catheter-based intervention was more likely in the second half of the period. Sixteen (16) patients had an attempted pulmonary valve perforation, 12 as their initial procedure. Median follow-up was 62 months (range 3-119 months). Final circulation status was known in 37 patients; biventricular n=14 (37.8%), "1.5 ventricles" n=4 (10.8%), single n=19 (51.4%). There were five deaths during the period (9.6%), including two during the initial procedural admission attributed to tamponade requiring extracorporeal membrane oxygenation (ECMO) at the time of percutaneous pulmonary valve perforation. CONCLUSION: There has been an overall trend towards including catheter-based strategies in the neonatal period as part of management at our centre. Given the risk of bleeding and ECMO related to this, consideration should be given to the availability of multidisciplinary support when planning the timing of these procedures.