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

Vikas S Gupta1, Elizabeth C Popp1, Ashley H Ebanks1

  • 1Department 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.

Pediatric Surgery International
|December 29, 2022
PubMed

Insights

Infants with congenital diaphragmatic hernia (CDH) and isolated aortic arch anomalies (IAAA) face higher mortality. This increased risk appears linked to overall defect severity rather than specific aortic pathology, as few receive aortic interventions.

Area of Science:

  • Pediatric Surgery
  • Cardiovascular Surgery
  • Neonatal Medicine

Background:

  • Congenital diaphragmatic hernia (CDH) is a severe condition often associated with cardiovascular anomalies.
  • Isolated aortic arch anomalies (IAAA) are frequently suspected in CDH patients, necessitating further investigation into their impact.

Purpose of the Study:

  • To determine the incidence of isolated aortic arch anomalies (IAAA) in infants with congenital diaphragmatic hernia (CDH).
  • To describe the clinical outcomes, including intervention rates and mortality, for CDH patients with coexisting IAAAs.

Main Methods:

  • A retrospective analysis of cardiovascular data from 6357 infants with CDH (2007-2019) was performed.
  • Isolated aortic arch anomalies (IAAA) were identified via echocardiography, excluding major cardiac malformations or chromosomal abnormalities.
  • Outcomes assessed included aortic intervention rates, extracorporeal life support (ECLS) utilization, and mortality.

Main Results:

  • 7% of CDH infants (432/6357) had thoracic aortic anomalies; 165 (7% of CDH infants) were diagnosed with IAAA.
  • CDH + IAAA patients exhibited lower birthweights and Apgar scores, and underwent diaphragm repair less frequently.
  • Mortality was significantly higher in CDH + IAAA infants (58% vs. 24%), with IAAA independently associated with increased mortality (OR 3.3).
  • Only 17% of CDH + IAAA patients received aortic intervention.

Conclusions:

  • Isolated aortic arch anomalies (IAAA) are present in a notable percentage of congenital diaphragmatic hernia (CDH) cases and are associated with increased mortality.
  • The higher mortality in CDH + IAAA patients likely reflects the overall severity of the defect and thoracic anatomy rather than unique aortic pathology.
  • The low rate of aortic intervention suggests that management focuses on the primary CDH and associated systemic issues rather than specific aortic repair in most cases.
Abstract

Keywords:
Aortic anomaliesCDH study groupCongenital diaphragmatic herniaRegistryVascular malformation

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