Discussion:
The incidence of associated cardiac defects in patients with SIT is nearly 4% (range from 0 to 10%), commonly with transposition of the great vessels (1, 3, 4). The association of situs inversus totalis with PAPVC is very rare (8). Individuals with isolated SIT are often asymptomatic, and are usually diagnosed incidentally, or during routine tests (2, 9, 10).Therefore, the recognition of such rare anomaly is so important for preventing surgical mishaps such as when approaching appendicitis in a patient with SIT (9, 10). When associated with SIT, clinical presentation of PAPVC will be the same as when it is an isolated lesion, and thus varies widely based on the degree of left to right shunt and associated cardiac defects (11). The most encountered form of left-sided PAPVC is that in which the left upper pulmonary vein (or veins) drains via a vertical vein into the left innominate vein to further drain into the right atrium (11). In our patient, the left pulmonary veins were draining directly to the left-sided right atrium with normal drainage of the right pulmonary veins to the left atrium. Surgical repair is indicated for symptomatic PAPVC patients and asymptomatic individuals in whom Qp:Qs is more than 1.5, or have an evidence of right ventricular dilation (11). In such circumstances, the surgeon should be aware of special aspects regarding the anatomy of cardiac chambers to avoid any pitfalls.