Complex congenital heart disease with intrathoracic kidney: A rare case-report

The coexistence of congenital cardiac and renal anomalies reflects overlapping embryological development during early gestation. Although profound congenital heart diseases (CHDs) have been occasionally associated with extracardiac anomalies, a right intrathoracic kidney is exceedingly exceptional. We report a case of a 1-month-old infant evaluated for CHD in whom cardiac computed tomography (CT) demonstrated a common cardiac atrium with biventricular morphology, perimembranous ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, and persistent left superior vena cava. In addition, a right intrathoracic kidney was identified showing preserved morphology, normal enhancement, and elongated renal vessels arising from the abdominal aorta. Intrathoracic kidney represents the rarest form of renal ectopia and is more commonly left-sided; right-sided occurrence is particularly uncommon. The simultaneous presence of complex CHD and right intrathoracic kidney suggests a shared developmental disturbance occurring between the 4 th and 8 th weeks of gestation, when cardiac septation and renal ascent take place. This case highlights the importance of comprehensive cross-sectional evaluation during cardiac CT in infants and underscores the relevance of recognizing rare cardio-renal associations with potential embryological and surgical implications.