An unusual form of supracardiac total anomalous pulmonary venous return via a right-sided vertical vein in a heterotaxy syndrome case.

Heterotaxy syndromes comprise a great variety of possible cardiac defects. Anomalies of the pulmonary venous system are well recognized in heterotaxy syndromes but constitute a frequently underdiagnosed subgroup. The case report describes a girl with a rare form of supracardiac total anomalous pulmonary venous return via a right-sided vertical vein to the right innominate vein in heterotaxy syndrome with dextrocardia, unbalanced atrioventricular septal defect with severe left ventricular hypoplasia, and transposition of the great arteries with pulmonary stenosis. Careful evaluation and imaging before surgical repair is crucial for such complex cardiac anomalies.