RESUMO
Left isomerism is characterized by bilateral left-sidedness and multiple associated cardiac and visceral anomalies. The clinical manifestation of left isomerism mainly depends upon the cardiac lesions. Occasionally an individual will have a normal heart and be presented with the extracardiac anomalies. A 3-year-old girl with a diagnosis of left isomerism was presented with pulmonary hypertension and intermittent hypoglycemia. Computerized tomography of the abdomen revealed absence of the portal vein and portosystemic shunt. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained the left renal vein and hemiazygos vein. Her pulmonary hypertension was considered as a consequence of the portosystemic shunt. We report a case of left isomerism in association with absence of the portal vein and a review of literatures.