Background
Heterotaxy, in simple terminology, can be described as abnormal orientation of abdominal and thoracic organs to the left and right of the central axis of the body, along with presence of a wide variety of complex congenital cardiac as well as extracardiac lesions [1]. The patients can be classified into either right or left isomerism depending upon the morphology of the paired structures which are mirror images present bilaterally in an otherwise asymmetric anatomy [2].
While there have been case reports on heterotaxy, very few of them have documented an association with lung parenchymal anomalies or pulmonary malinosculation. Pulmonary malinosculations can have bronchial, arterial and venous components either individually, or in combination. To the best of our knowledge, there are only two case reports showing association of heterotaxy syndrome with lung anomalies one of which mentions polysplenia with congenital lobar emphysema while the other describes polysplenia with diffuse pulmonary arteriovenous malformations [3, 4]. This is the first report highlighting association of heterotaxy syndrome with mixed bronchoarteriovenous pulmonary malinosculation and the combination of imaging features eventually altered the treatment protocol for the patient significantly.