Heart Disease in the Hurler and Marfan Syndromes

Biochemical observations of connective tissue are fundamental in understanding the pathogenesis of lesions in the cardiovascular system. Since it is actually only within the past decade that some comprehension of the chemical complexity of connective tissue which composes cardiovascular structures has been achieved, it is likely that attempts to equate clinical observations of heart disease with biochemical studies of connective tissue will prove more and more fruitful. Two heritable conditions, Hurler-Pflaunder and Marfan syndromes, first characterized by diffuse skeletal involvement, are now known to be associated with severe cardiovascular disease. Despite the enlightening review by McKusick, 1 the constancy of heart disease, the progressiveness, and the variations in manifestations of cardiovascular changes in these conditions are not generally appreciated. The present studies consist primarily of anatomic and biochemical observations related to changes of the cardiovascular system occurring in individuals with these syndromes. Material Observations of the cardiac manifestations were reviewed