Radiol Case Rep
. 2026 Apr 16;21(7):2782-2786.
doi: 10.1016/j.radcr.2026.03.041. eCollection 2026 Jul. https://pubmed.ncbi.nlm.nih.gov/42027841/
Heterotaxy syndrome with complex single-ventricle physiology and left-sided Bochdalek hernia: Multisystem characterization by cardiothoracic CT
Aamir M Kanji 1, Lilian Salingwa 1, Tariq Aziz 2, Sibtain Moledina 3
Affiliations Expand
- PMID: 42027841
- PMCID: PMC13099462
- DOI: 10.1016/j.radcr.2026.03.041
Abstract
Heterotaxy syndrome comprises a spectrum of laterality defects frequently associated with complex congenital heart disease and extracardiac anomalies. We report a 2-year-old male who presented with persistent cyanosis, recurrent respiratory infections, and failure to thrive. Cardiac computed tomography demonstrated ambiguous situs with dextrocardia, reversed atrial arrangement, L-looped ventricles, large atrial and ventricular septal defects, hypoplastic right ventricle, absent right ventricular outflow tract, and double-outlet left ventricle. Additional findings included polysplenia, right-sided aortic arch with mirror branching, aortopulmonary collaterals, and a left posterolateral Bochdalek hernia. This case highlights the value of comprehensive cross-sectional imaging in defining complex heterotaxy anatomy and underscores the clinical implications of associated extracardiac anomalies in surgical planning and prognosis.
Keywords: Congenital diaphragmatic hernia; Congenital heart disease; Dextrocardia; Heterotaxy syndrome; Polysplenia.
© 2026 The Authors.
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