EVALUATION OF CORONARY-ARTERY DISEASE IN THE HURLER SYNDROME BY ANGIOGRAPHY

Hurler syndrome (mucopolysaccharidosis type I-H) is an inherited lysosomal storage disease characterized by the widespread accumulation of mucopolysaccharide in tissues throughout the body.1 Although severe coronary artery luminal narrowing has been documented by postmortem examination of the heart of patients with Hurler syndrome,2 the only reported study in which coronary angiography was performed stated that the coronary arteries appeared normal.3 As part of an ongoing protocol at our institution, selective coronary angiography was performed in children with Hurler syndrome before bone marrow transplantation. The subsequent availability of postmortem material from 3 such patients has allowed us to perform a retrospective analysis of the usefulness of selective coronary angiography in detecting the presence of coronary artery disease in patients with the Hurler syndrome. © 1992.