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Infants with pulmonary atresia intact ventricular septum who require balloon atrial septostomy have significantly higher 18-month mortality

Part of: Interventional Cardiology

Published online by Cambridge University Press:  01 March 2021

Nicole L Herrick ,
Asimina Courelli ,
Jesse W Lee ,
Kanishka Ratnayaka ,
Laith I Alshawabkeh ,
John W Moore  and
Howaida G El-Said Open the ORCID record for Howaida G El-Said [Opens in a new window]
Nicole L Herrick
Affiliation:
Department of Medicine, UC San Diego Health, San Diego, CA, USA
Asimina Courelli
Affiliation:
UC San Diego School of Medicine, San Diego, CA, USA
Jesse W Lee
Affiliation:
Division of Cardiology, Department of Pediatrics, Children’s Hospital of San Antonio and Baylor College of Medicine, San Antonio, TX, USA
Kanishka Ratnayaka
Affiliation:
Division of Cardiology, Department of Pediatrics, Rady Children’s Hospital and UC San Diego School of Medicine, San Diego, CA, USA
Laith I Alshawabkeh
Affiliation:
Division of Cardiovascular Medicine, Department of Medicine, UC San Diego School of Medicine, San Diego, CA, USA
John W Moore
Affiliation:
Division of Cardiology, Department of Pediatrics, Rady Children’s Hospital and UC San Diego School of Medicine, San Diego, CA, USA
Howaida G El-Said*
Affiliation:
Division of Cardiology, Department of Pediatrics, Rady Children’s Hospital and UC San Diego School of Medicine, San Diego, CA, USA
*
Author for correspondence: Howaida G. El-Said, MD PhD, Division of Cardiology, Rady Children’s Hospital San Diego, 3020 Children’s Way, MC 5004, San Diego, CA92123, USA. Tel: 858-966-5855; Fax: 858-966-78903. E-mail: HEL-Said@rchsd.org
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Abstract

Introduction:

Many newborns with pulmonary atresia/intact ventricular septum require intervention to establish pulmonary flow and sufficient cardiac output. The resulting haemodynamic changes are not well characterised and may have unintended consequences.

Methods:

This is a 30-year (1988–2018) retrospective study of patients with pulmonary atresia intact ventricular septum.

Results:

Eighty-nine patients were included, and median follow-up was 8 years. Fifty-five per cent had coronary sinusoids and 27% had right ventricular-dependent coronary circulation. Most patients were managed with surgical aortopulmonary or modified Blalock–Taussig shunt (73%), and 12 patients underwent balloon atrial septostomy before surgical intervention. The remaining patients (27%) underwent only transcatheter interventions; 7 required an atrial septostomy and 17 required ductal stentings. All-cause mortality was 10%, most deaths (89%) occurred before 18 months of age. Of these early deaths, 87% required a balloon atrial septostomy and 85% had right ventricular-dependent coronary sinusoids. Eighteen-month mortality was significantly higher for patients who required a balloon atrial septostomy compared to those who did not (36% versus 1.4% p < 0.0001).

Discussion:

Patients with pulmonary atresia/intact ventricular septum who require balloon atrial septostomy in the newborn period have significantly higher 18-month mortality. Quantifying the mortality difference may help guide prognostication and expectation setting. Infants who had septostomy and a surgical shunt in the newborn period fared better than those who only underwent septostomy (even when accompanied by ductal stenting). For infants with right ventricular-dependent circulation, atrial septostomy should only be performed on an urgent or emergent basis and these patients should be considered for early surgical intervention and neonatal transplant.

Keywords

Pulmonary atresia intact ventricular septumright ventricular-dependent coronary sinusoidsballoon atrial septostomy
Type
Original Article
Information
Cardiology in the Young , Volume 31 , Issue 10 , October 2021 , pp. 1613 - 1618
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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