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Congenitally Corrected Transposition of the Great Arteries (L-TGA) with Situs Inversus

Imaging findings

Chest radiograph shows increased pulmonary vascularity, a dilated main pulmonary artery, and complete situs inversus (stomach bubble on the right). Chest CT demonstrates an I,D,D configuration representing congenitally corrected transposition of the great arteries (L-TGA) with complete situs inversus, alongside a previously repaired total anomalous pulmonary venous return (TAPVR) with dilated pulmonary arteries.

Key takeaways

Congenitally corrected transposition of the great arteries (L-TGA) with complete situs inversus represents a discordant atrioventricular and ventriculoarterial connection in the setting of mirrored organs (I,D,D configuration), which physically corrects blood flow pathways but leaves the morphological right ventricle serving the systemic circulation. Patients are prone to developing pulmonary arterial hypertension due to long-standing shunts (such as TAPVR or VSD) and eventual systemic (morphological right) ventricular failure.

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