Maroteaux-Lamy syndrome: a rare and challenging case of mitral valve replacement.

Maroteaux-Lamy syndrome is a very rare multisystem disorder. Cardiac valve surgery is common in these patients who present a unique set of perioperative and intraoperative challenges to the surgeon and treating team. We describe successful mitral valve replacement using an inverted aortic prosthesis in a 29-year-old lady with Maroteaux-Lamy syndrome, with a good result at 10 months.