The authors reports the detection of a rare and remarkably well tolerated cardiac malformation in a 40 year old woman whose clinical presentation would have suggested an ordinary mitral stenosis, had there not been a number of very unusual signs. Cardiac catheterisation and angiography showed a corrected transposition of the great arteries with stenosis of the systemic atrioventricular valve. This case is used to illustrate the principal malformations observed in association with corrected transposition of the great arteries, especially the common and very variable abnormalities of the systemic atrioventricular valve. The unusual feature of the reported case was the stenotic nature of the valvular lesion and the fact that this was the only associated intracardiac abnormality, simulating, at first sight, a rheumatic mitral stenosis.

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