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Aberrant Subclavian Artery in Interrupted Aortic Arch with Severe Aortic Outlet Obstruction: Cerebral Blood Flow as a Possible Determinant of Embryonic Cardiovascular Development?

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Abstract

Aberrant subclavian artery (ASCA) is frequently observed in interrupted aortic arch (IAA) with aortic/subaortic obstruction. Developmental significance of ASCA in IAA in utero remains elusive. Newborns with prenatally diagnosed isolated IAA under continuous prostaglandin E1 infusion were studied. Cross-sectional areas of aortic valve opening (AVOCSA) and patent ductus arteriosus (PDACSA) were represented by echocardiographic measurement of (diameter)2 indexed by body surface area (m2). Types of IAA and presence of ASCA were examined in relation to sizes of AVOCSA and PDACSA. Twenty-four newborns with IAA (six type A and 18 type B) were reviewed. Male dominance was seen in type B (male 72%). Twenty-three patients had left aortic arch. No type A patients had ASCA, but 50% of type B had ASCA; AVOCSA was significantly smaller in type B than in type A (p = 0.003). In type B, PDACSA was significantly larger in those with ASCA than without (p = 0.003), but AVOCSA exhibited no significant size difference between these two subgroups. Chromosome 22q11 deletion was only seen in type B (56%) and showed no significant correlation with the presence of ASCA. In type B IAA, the presence of ASCA was associated with larger PDACSA, suggesting an adaptive enlargement of the ductus arteriosus and ASCA in response to reduced antegrade flow across small AVOCSA, which may be augmenting cerebral blood flow. Preservation of cerebral blood flow may be another important determinant affecting embryonic cardiovascular development.

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Data Availability

All data supporting the findings of this study are available from the corresponding author upon reasonable request.

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Authors and Affiliations

  1. Nemours Cardiac Center, Nemours Children’s Health, Delaware Valley, 1600 Rockland Rd, Wilmington, DE, 19803, USA

    Danilo Tadeo Jr., Abdul Bhat & Takeshi Tsuda

  2. Division of Cardiology, Nemours Children’s Health, Orlando, FL, USA

    Barham Kakavand

  3. Department of Pediatrics, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA, USA

    Abdul Bhat & Takeshi Tsuda

Authors
  1. Danilo Tadeo Jr.
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  2. Barham Kakavand
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  3. Abdul Bhat
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  4. Takeshi Tsuda
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Contributions

TT conceptualized the study. DT and BK obtained the patients' data from electrical medical record of Nemours Children's Health Delaware and Nemours Children's Health Florida, respectively. DT drafted an initial manuscript text, which was critically edited by TT and MB. DT prepared tables 1 and 2 and figures 1-3. All authors reviewed and approved the final manuscript.

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Correspondence to Takeshi Tsuda.

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The authors declare no competing interests.

Ethical Study

This retrospective study was performed in line with the principles of the Declaration of Hensinki at Nemours Children’s Health Delaware and Nemours Children’s Health Florida. Approval was granted by the Nemours Children’s Health Institutional Review Board (#1912189-4).

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Tadeo, D., Kakavand, B., Bhat, A. et al. Aberrant Subclavian Artery in Interrupted Aortic Arch with Severe Aortic Outlet Obstruction: Cerebral Blood Flow as a Possible Determinant of Embryonic Cardiovascular Development?. Pediatr Cardiol (2024). https://doi.org/10.1007/s00246-024-03476-y

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