CARDIAC INVOLVEMENT IN MUCOPOLYSACCHARIDOSES - EFFECTS OF ALLOGENEIC BONE-MARROW TRANSPLANTATION

Echocardiography was performed in 16 children undergoing bone marrow t ransplantation (BMT) for mucopolysaccharidoses. Cardiac involvement be fore BMT was detected in seven (44%). One year after BMT (11 patients/ five with cardiac involvement), left ventricular restriction resolved in 2/3 patients and hypertrophy in one. In the remainder, at mean foll ow up of 2.5 years, no progression of preexisting or development of ne w cardiac involvement was noted. It is concluded that in a significant proportion of patients with mucopolysaccharidoses, cardiac involvemen t improved after BMT.