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Anatomic and non-anatomic substrates in infants with two ventricles undergoing aortic arch repair

Published online by Cambridge University Press:  22 November 2024

Bari Murtuza Open the ORCID record for Bari Murtuza [Opens in a new window] ,
Juan Lehoux ,
Mario Castro Medina ,
Luciana da Fonseca Da Silva ,
Melita Viegas ,
Courtney E. McCracken ,
Jon Blaine John ,
Elsa Suh ,
Jeremy Ringewald  and
Victor O. Morell
Bari Murtuza*
Affiliation:
Department of Pediatric & Congenital Cardiovascular Surgery, St Joseph’s Children’s Hospital, Tampa, FL, USA Pediatric & Congenital Cardiovascular Surgery, Heart and Vascular Institute, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
Juan Lehoux
Affiliation:
Department of Pediatric & Congenital Cardiovascular Surgery, St Joseph’s Children’s Hospital, Tampa, FL, USA Pediatric & Congenital Cardiovascular Surgery, Heart and Vascular Institute, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
Mario Castro Medina
Affiliation:
Pediatric & Congenital Cardiovascular Surgery, Heart and Vascular Institute, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
Luciana da Fonseca Da Silva
Affiliation:
Pediatric & Congenital Cardiovascular Surgery, Heart and Vascular Institute, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
Melita Viegas
Affiliation:
Pediatric & Congenital Cardiovascular Surgery, Heart and Vascular Institute, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
Courtney E. McCracken
Affiliation:
Center for Research and Evaluation, Kaiser Permanente, Atlanta, GA, USA
Jon Blaine John
Affiliation:
Pediatric Cardiology, St Joseph’s Children’s Hospital, Tampa, FL. USA
Elsa Suh
Affiliation:
Pediatric Cardiology, St Joseph’s Children’s Hospital, Tampa, FL. USA
Jeremy Ringewald
Affiliation:
Pediatric Cardiology, St Joseph’s Children’s Hospital, Tampa, FL. USA
Victor O. Morell
Affiliation:
Department of Pediatric & Congenital Cardiovascular Surgery, St Joseph’s Children’s Hospital, Tampa, FL, USA Pediatric & Congenital Cardiovascular Surgery, Heart and Vascular Institute, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
*
Corresponding author: Bari Murtuza; Email: [email protected]
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Abstract

Objectives:

We sought to examine the relative importance of surgical lesion complexity versus the presence of genetic/syndromic/extracardiac anomalies (GSAs) in determining survival, morbidity or need for reinterventions following repair for aortic arch hypoplasia.

Methods:

A single-centre, retrospective cohort study of infants undergoing biventricular aortic arch repair via sternotomy from 2010 to 2021 was conducted. Survival analysis was performed using Kaplan–Meier methods, with additional Bayesian survival modelling for subgroups. Composite morbidity comprised respiratory, renal, neurologic, or sepsis-related complications.

Results:

Of 83 included infants, n = 13/83 (15.7%) had complex repairs; 27/83 (32.5%) were GSA+. Operative mortality was significantly higher in GSA+ versus GSA− patients (18.5% vs. 1.8%; p = 0.01), though not for complex versus non-complex repairs. Overall 10-year Kaplan–Meier survival was 86.7%. Bayesian modelling suggested equivalent post-discharge attrition in non-complex/GSA+ and complex/GSA− patients, with the poorest outcomes in complex/GSA+ patients; non-complex/GSA− patients had 100% survival. GSA+ patients exhibited higher composite morbidity (44.4% vs. 7.1% in GSA− p < 0.001), with their mode of death seemingly related to a high incidence of respiratory and neurological morbidity, notably in Dandy–Walker syndrome. The 10-year freedom from arch reinterventions was 87.7%; neither complexity, GSA status, nor post-repair peak arch velocity predicted the need for arch reinterventions.

Conclusions:

Whilst anatomic complexity may have been somewhat neutralised as a risk factor for operative mortality, in contrast to GSA+ status, there is further post-discharge attrition attributable to complexity or GSA+ status, with additive risk effects. Morbidity directly related to certain syndromes underlies some of this risk. Non-anatomic substrates represent a persistent limitation to outcomes of surgical aortic arch repair in infants.

Keywords

Aortic archhypoplasiageneticcongenitalbiventricular repair
Type
Original Article
Information
Cardiology in the Young , Volume 35 , Issue 2 , February 2025 , pp. 253 - 260
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Copyright
© The Author(s), 2024. Published by Cambridge University Press

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