Partial anomalous hepatic venous drainage into left-sided at
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Right isomerism, also known as Ivemark syndrome, is an unusual degree of symmetry of some of the abdominothoracic viscera reflecting bilateral right-sidedness. We report an exceedingly rare occurrence of anomalous drainage of the left hepatic vein to the left-sided atrium in a patient of right isomerism. A 3-month-old infant presented to the pediatric clinic of our institute with chief complaints of respiratory distress since birth. There was no history of bluish discoloration of the skin, fever, or a cough. Transthoracic echocardiography revealed mild enlargement of the right atrium and right ventricle with double outlet right ventricle (DORV), atrioventricular canal defect, and partial anomalous pulmonary venous return. long with other findings detected on CTA, a diagnosis of heterotaxy syndrome (DORV, AV canal defect, PAPVD, and asplenia with abnormal subdiaphragmatic venous drainage) was offered

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