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Impact on the trachea in children with prenatally diagnosed vascular ring formed by a right aortic arch

Christopher Oakley1,2, Alice Hurn1,2, Claire Suckling1,2

  • 1Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's & St Thomas' NHS Trust, London, UK.

Pediatric Pulmonology
|May 22, 2023
PubMed

Insights

Right-sided aortic arch with a left-sided arterial duct can cause tracheal compression, even in asymptomatic infants. Bronchoscopy is crucial for assessing severity, as symptoms alone are unreliable indicators of compression.

Area of Science:

  • Cardiology
  • Pediatric Surgery
  • Medical Imaging

Background:

  • Prenatal diagnosis of right-sided aortic arch (RAA) with a left-sided arterial duct (LD) is increasing.
  • This anatomical variation can form a vascular ring, potentially causing tracheobronchial compression in infants.
  • While some infants present with symptoms, many remain asymptomatic.

Purpose of the Study:

  • To evaluate the correlation between clinical symptoms and the severity of tracheobronchial compression.
  • To assess the diagnostic value of bronchoscopy in identifying compression in infants with RAA-LD.

Main Methods:

  • Retrospective review of 112 infants with isolated RAA-LD diagnosed prenatally.
  • Analysis of clinical records, fetal echocardiograms, and flexible bronchoscopy (FB) data.
  • FB was performed in 82 infants, with a median age of 11 months; no complications were reported.

Main Results:

  • Aberrant left subclavian artery (ALSA) was present in 86% and mirror image branching (MIB) in 13% of cases.
  • Moderate to severe tracheal compression was observed in 47% of ALSA cases and 60% of MIB cases undergoing FB.
  • Notably, 36% of asymptomatic infants showed moderate to severe compression, indicating symptoms are poor predictors.

Conclusions:

  • Significant tracheal compression can be present in infants with RAA-LD, irrespective of symptoms.
  • Relying solely on symptoms may lead to underappreciation of the anatomical impact of these vascular rings.
Abstract

Keywords:
bronchoscopyprenatal diagnosisright aortic archtracheavascular ring

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