CLINICAL PROGRESS Cardiovascular Manifestations of the Hurler Syndrome

SINCE the original report of Hunter,' cardiovascular disease has been recognized as an integral feature of the Hurler syndrome (gargoylism, lipochondrodystrophy). Hunter noted cardiomegaly and both systolic and diastolic murmurs in the older of two brothers with this syndrome who died at age 20 of "dropsy."2 Other publications3-5 have continued to record cardiac murmurs, cardiomegaly, and premature deaths secondary to cardiac disease. In several reports2' 5, 6 the murmurs were sufficiently striking to suggest congenital heart disease, particularly ventricular septal defect. Necropsies have not corroborated the presence of septal defects bu-t have stressed the frequency of valvular deformities,2' 4' 5 endocardial fibroelastosis,7 and narrowing of the coronary arteries.3' 5, 8 Emanuel,3 reviewing the literature of the Hurler syndrome in 1954, found 32 necropsies, 22 of which had evidence of cardiovascular disease. It is surprising that only four general re-