Hetrotaxy syndrome is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. Although it is a known condition among physicians taking care of pediatric patients, it is rarely seen in adult day-to-day medicine and most physicians involved in care of adult patients are unaware of it. It is important to recognize this anomaly based on clinical findings,due to its reported association with various medical conditions. We report a case of a 77-year-old patient diagnosed with Hetrotaxy syndrome.

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Hetrotaxy syndrome is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. Although it is a known condition among physicians taking care of pediatric patients, it is rarely seen in adult day-to-day medicine and most physicians involved in care of adult patients are unaware of it. It is important to recognize this anomaly based on clinical findings,due to its reported association with various medical conditions.

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Although isomerism of the bronchial tree is an integral part of hetrotaxy syndrome, the association of congenital tracheal stenosis is rare in this group of disorders, and it has not yet been thoroughly described in the literature. This condition is potentially life-threatening and precludes single ventricle palliation. This report presents the case of a 5-month old infant with symptomatic congenital tracheal stenosis, functionally univentricular heart and extracardiac total anomalous pulmonary venous connection (TAPVC).

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