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Heterotaxy syndrome with complex single-ventricle physiology and left-sided Bochdalek hernia: Multisystem
Aamir M Kanji1, Lilian Salingwa1, Tariq Aziz2
1Department of Radiology and Imaging, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania.
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.
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