A rare and challenging mitral valve replacement in a child with Hurler syndrome

İlker Mercan; Fatih Durak; Meltem Çakmak; Muhammet Akyüz; Onur Işık

doi:10.5606/e-cvsi.2022.1282

Cardiovascular Surgery and Interventions 2022, Vol 9, Num 3 Page(s): 181-184

A rare and challenging mitral valve replacement in a child with Hurler syndrome

İlker Mercan¹, Fatih Durak², Meltem Çakmak³, Muhammet Akyüz¹, Onur Işık¹

¹Department of Pediatric Heart Surgery, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Türkiye
²Department of Pediatric Intensive Care Unit, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Türkiye
³Department of Anesthesiology and Reanimation, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Türkiye

Keywords: Hurler syndrome, valvular disease, mucopolysaccharidosis

Mucopolysaccharidoses are progressive inherited lysosomal storage disorders, and early cardiac involvement is common. Valvular involvement of mucopolysaccharidoses usually causes regurgitation, most commonly affecting the left heart and mitral valve. In this case, we discuss the treatment and perioperative management of mitral valve degeneration in a child with Hurler syndrome by performing mitral valve replacement.

DOI : 10.5606/e-cvsi.2022.1282