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.
Keywords
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Article info
Publication history
Published online: October 13, 2021
Accepted:
August 27,
2021
Received in revised form:
August 2,
2021
Received:
January 30,
2021
Identification
Copyright
Crown Copyright © 2021 Published by Elsevier B.V. on behalf of Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.
