Cardiovascular disease is one of the more common features of the Marfan syndrome, but it comes in many guises. Aneurysms of the great vessels (which may dissect), defects in the interatrial or interventricular septum, deformities of the aortic or mitral valves, unexplained cardiac enlargement, and arrhythmias or conduction disturbances are all examples of such cardiovascular manifestations.1,2
Even though disturbances in cardiac rhythm or conduction have been documented relatively frequently, it is reasonable to suspect that the undetected paroxysmal occurrence may be even greater. When unexpected sudden death in the Marfan syndrome is otherwise unexplained, it is often presumed attributable to a lethal disturbance in cardiac electrical mechanism.
A recent postmortem study of the conduction system of the heart in the Marfan syndrome demonstrates structural changes which may lead to disorganized function in this critical area. In the September issue of the Archives of Internal Medicine, James et al3