Situs Inversus Totalis: Single-Stage Anatomic Repair of Complex Congenital Heart Disease.

Transposition of the great arteries occurs rarely in patients with mirror image dextrocardia and situs inversus, while the combination with severe coarctation of the descending aorta (CoA) makes the anatomy even more unusual. Therefore, it is not surprising that a case with such unusual and complicated anatomy presents unique problems when a primary definitive correction is attempted. We report a patient with situs inversus totalis and complex congenital heart disease including transposition of the great arteries and severe CoA who underwent successful complete, single-stage, anatomic correction.

Operating the blues.

A 63-year-old man bearing most signs and symptoms (facial pigmentation, degenerative arthritis, and dark urine) pertinent to his known history of alkaptonuria underwent aortic valve replacement for critical aortic stenosis. Although rare, aortic stenosis is the most common cardiac manifestation of alkaptonuric ochronosis.