Pathology of Cardiac Conduction System in Marfan's Syndrome

Abstract

Death of patients with Marfan's syndrome is often sudden and unexpected.¹ It is natural under such circumstances to suspect a cardiac arrhythmia as one possible explanation. Recently it has been our opportunity to study two young men with Marfan's syndrome who developed disturbances in cardiac rhythm or conduction during their terminal illness. The following is a report of these two patients and the pathology of their cardiac conduction system at necropsy. Report of Cases Case 1. —A 20-year-old white male student twice had operations in childhood for attempted correction of severe funnel chest. Before these operations a grapefruit could allegedly be placed in the chest excavation, and one surgeon described the sternum as being "¼ inch from the backbone." The patient was 72 inches tall, had an arm span of 76½ inches, was slender (140 lb [63.5 kb]), and exhibited arachnodactyly. On the skin of the anterior surface of