LAUSR

Coffin–Lowry syndrome is a rare X-linked disorder that shows a varied clinical presentation. We report cardiac involvement, particularly abnormalities of mitral valve morphology, in a 14-year-old male adolescent with a known diagnosis of Coffin–Lowry syndrome, who presented with easy fatigability. Echocardiography revealed severe mitral and moderate tricuspid regurgitation, and the papillary muscles were attached to the base of the left ventricle. We performed tricuspid annuloplasty and mitral valve repair using two artificial chordae and a semi-rigid full ring. The patient's postoperative course was uneventful. Postoperative echocardiography revealed reduced mitral regurgitation, and his cardiac failure improved.