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When abdominal and thorasic organs are inversed it is called situs inversus and when most of the these organs are positioned abnormally and heterogenic It is called situs ambigus (Heterotoxy syndrome) which is a rare congenital defect (Incidence 1.44 in 100/000 live birth) In these, abnormal organs shift which could be to the Right side or left side (Right Isomerism, Left Isomerism). One of the Heterotoxy main subgroups is Right Isomerism, asplenia which is accompanied with centrally located liver, asplenia, two morphologic Right side lungs. Our case is 19 years old gril with central and peripheral cyanosis and clubbing from several years ago who had several recurrent pulmonary infection from childhood with history of PA banding surgery. She had dextrocardia, asplenia and cardiac abnormality were detected in CXR, echocardiography and sonography. Management of this abnormality include surgical correction and prevention of infection with vaccination and antibiotic prophylaxis due to asplenia.

Keywords: Situs ambigus, heterotoxy syndrome, Right Isomerism, asplenia syndrom

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