Murugu Sundara Pandiyan

Mitral valve replacement in a 19 year old lady with Maroteaux-Lamy syndrome

Mucopolysaccharidoses (MPS) are inherited lysosomal storage disorders caused by the absence of functional enzymes that contribute to the degradation of glycosaminoglycans [1]. Maroteaux-Lamy syndrome is caused by deficiency of arylsulfatase B (N-acetylgalactoseamine-4-sulfatase) and leads to increased amounts of dermatan sulfate in tissues and in urine. Infiltration in the cardiac valve tissue can cause stenosis, insufficiency or both. The affected patients typically have growth retardation and multisystem involvement. The craniofacial dysmorphism and oropharyngeal abnormalities make them difficult patients for anesthesia and surgery [1, 2]. The clinical characteristics, preoperative evaluation, intraoperative and postoperative management of a young female with such a condition are being presented.