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Isolated aortic arch anomalies are associated with defect severity and outcome in patients with congenital diaphragmatic hernia

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Abstract

Purpose

Congenital diaphragmatic hernia (CDH) patients often have suspected isolated aortic arch anomalies (IAAA) on imaging. The purpose of this work was to describe the incidence and outcomes of CDH + IAAA patients.

Methods

Cardiovascular data were collected for infants from the CDH Study Group born between 2007 and 2019. IAAA were defined as coarctation of aorta, hypoplastic aortic arch, interrupted aortic arch, and aortic aneurysmal disease on early, postnatal echocardiography. Patients with major cardiac malformations and/or chromosomal abnormalities were excluded. Primary outcomes included the rate of aortic intervention, rates of extracorporeal life support (ECLS) utilization, and mortality.

Results

Of 6357 CDH infants, 432 (7%) were diagnosed with a thoracic aortic anomaly. Of these, 165 were diagnosed with IAAA, most commonly coarctation of the aorta (n = 106; 64%) or hypoplastic aortic arch (n = 58; 35%). CDH + IAAA patients had lower birthweights (3 kg vs. 2.9 kg) and Apgar scores (7 vs. 6) than patients without IAAA (both χ2 p < 0.001). CDH + IAAA were less likely to undergo diaphragm repair (72 vs. 87%, p < 0.001), and overall mortality was higher for CDH + IAAA infants (58 vs. 24%, p < 0.001). When controlling for defect size, birth weight, and Apgar, IAAA were significantly associated with mortality (OR 3.3, 95% CI 2.2–5.0; p < 0.01) but not associated with ECLS (OR 0.98, 95% CI 0.65–1.50; p = 0.90). Only 17% (n = 28) of CDH + IAAA patients underwent aortic intervention.

Conclusions

IAAA in CDH are associated with increased mortality. This often simply reflects severity of the defect and thoracic anatomic derangement, as opposed to unique aortic pathology, given few CDH + IAAA patients undergo aortic intervention.

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Abbreviations

CDH:

Congenital diaphragmatic hernia

CDHSG:

Congenital diaphragmatic hernia study group

CI:

Confidence interval

ECLS:

Extra-corporeal life support

IAAA:

Isolated aortic arch anomalies

OR:

Odds ratio

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Author information

Authors and Affiliations

  1. Department of Pediatric Surgery, McGovern Medical School at the University of Texas Health Science Center and Children’s Memorial Hermann Hospital, 6431 Fannin St, MSB 5.233, Houston, TX, 77030, USA

    Vikas S. Gupta, Elizabeth C. Popp, Ashley H. Ebanks, Kevin P. Lally & Matthew T. Harting

  2. Division of Pediatric and Congenital Heart Surgery, Department of Cardiothoracic and Vascular Surgery, McGovern Medical School at the University of Texas Health Science Center and Children’s Memorial Hermann Hospital, Houston, TX, USA

    Christopher E. Greenleaf & Damien J. LaPar

  3. Department of Pediatrics, Division of Pediatric Cardiology, McGovern Medical School at the University of Texas Health Science Center and Children’s Memorial Hermann Hospital, Houston, TX, USA

    Vidhya Annavajjhala

  4. Department of Neonatology, Royal Hospital for Children Glasgow, Glasgow, Scotland

    Neil Patel

  5. Dayton Children’s Hospital, Dayton, OH, USA

    Daniel K. Robie

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  1. Vikas S. Gupta
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for the Congenital Diaphragmatic Hernia Study Group

Contributions

V.S.G., E.C.P, and M.T.H. wrote the main manuscript text. VSG, EFP, AHE, KPL, MTH contributed to data collection and validation. CEG, VA, NP, DKR, DJL, KPL, MTH contributed to study design, methodology, critical revision and editing of the manuscript.

Corresponding author

Correspondence to Matthew T. Harting.

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Conflict of interest

Authors have no relevant conflicts of interest to disclose.

Ethical approval and consent to publish

This retrospective chart review study involving human participants was in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The CDHSG is approved by the University of Texas at Houston Center for the Protection of Human Subjects/Institutional Review Board (#HSC-MS-03-223; Ref #118886; 06/2018). Consent to publish was not obtained.

IRB approval

The University of Texas at Houston Center for the Protection of Human Subjects/Institutional Review Board (#HSC-MS-03–223; Ref #118886; 06/2018.

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Gupta, V.S., Popp, E.C., Ebanks, A.H. et al. Isolated aortic arch anomalies are associated with defect severity and outcome in patients with congenital diaphragmatic hernia. Pediatr Surg Int 39, 69 (2023). https://doi.org/10.1007/s00383-022-05354-1

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