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Interrupted Aortic Arch: Navigating Diagnosis, Management, and Beyond

Kerri Rassa1, Amy L Williford2, Amy J Jnah2

  • 1Department of Advanced Practice Nursing Practice & Education, College of Nursing, East Carolina University, Greenville, NC, USA rassak22@students.ecu.edu.

Neonatal Network : NN
|October 15, 2025
PubMed

Insights

Interrupted aortic arch (IAA), a rare cyanotic congenital heart disease (CHD), presents subtly in newborns. Early diagnosis and intervention are critical for survival, as symptoms rapidly become life-threatening.

Area of Science:

  • Pediatric Cardiology
  • Neonatal Medicine
  • Congenital Anomalies

Background:

  • Congenital heart disease (CHD) affects ~1% of births, causing circulatory issues due to structural defects.
  • Interrupted aortic arch (IAA) is a rare, cyanotic CHD subtype with initially nonspecific, elusive symptoms.
  • Delayed diagnosis of IAA can lead to severe, life-threatening complications in neonates.

Purpose of the Study:

  • To report a case of a neonate with undiagnosed Interrupted Aortic Arch, Type A.
  • To discuss the pathogenesis, diagnosis, and treatment of Interrupted Aortic Arch.
  • To highlight the importance of clinical acumen in identifying critical congenital heart defects.

Main Methods:

  • Case report presentation of a neonate with previously unrecognized Interrupted Aortic Arch, Type A.
  • Review of the literature concerning the pathophysiology of IAA.
  • Discussion of diagnostic modalities and current therapeutic strategies for IAA.

Main Results:

  • The case illustrates the challenges in diagnosing IAA in the immediate newborn period.
  • The discussion covers key aspects of IAA, from embryological origins to clinical management.
  • Timely identification and intervention are emphasized as vital for favorable outcomes.

Conclusions:

  • Interrupted Aortic Arch requires heightened clinical suspicion in neonates presenting with nonspecific symptoms.
  • Prompt diagnosis and surgical or medical intervention are essential for managing this severe form of CHD.
  • Further awareness among healthcare providers can improve outcomes for infants with IAA.

Keywords:
cardiac anomaliescongenital heart diseasediagnosticinterrupted aortic archneonatal/infantscreening

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