RESTING TL-201 SCINTIGRAPHY IN THE EVALUATION OF CORONARY-ARTERY DISEASE IN CHILDREN WITH HURLER-SYNDROME

Severe coronary artery disease may occur early in the course of mucopo lysaccharidosis. The authors present radiologic, scintigraphic, and pa thologic findings in five patients with Hurler syndrome. Thallium-201 myocardial scintigraphy and selective coronary angiography obtained wi thin 2 days to 6 months were correlated in five patients. Postmortem e xamination of the coronary arteries was obtained in one patient within hours of angiography. Interobserver agreement on grading of scintigra phic abnormalities was poor. Nonspecific findings on TI-201 studies in cluded septal abnormalities in all five patients. Scintigraphic findin gs were corroborated by angiography and postmortem results in only 3 o f 5 patients. Our findings suggest that resting TI-201 scintigraphy ha s limited value in the detection of coronary artery disease in patient s with Hurler syndrome.