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Scimitar syndrome is a rare variant of anomalous right pulmonary vein connection to the inferior vena cava and it is associated with other cardiopulmonary anomalies. It generally requires surgery and sometimes it may go unrecognised into adulthood. We report a unique case of a scimitar syndrome variant in a young adult, who was successfully treated percutaneously, after the first misdiagnosis of arrhythmogenic ventricular cardiomyopathy. The cardiac magnetic resonance unveiled the uncommon anatomical pattern, avoiding surgical repair. Cross-sectional imaging is extremely useful in the diagnosis and treatment planning of CHD in adults.

Transposition of the great arteries combined with totally anomalous pulmonary venous connection is extremely rare outside of heterotaxy syndrome. Most reported cases have been treated by a modified atrial switch operation. We report the successful treatment of a neonate with this rare association, repaired by arterial switch operation and connection of the pulmonary venous return to the left atrium.