Extracranial Microvascular Complications of Moyamoya Disease Leading to Left Ventricular Systolic Dysfunction

Moyamoya disease (MMD) was first used as a descriptor for a steno-occlusive process that affects primarily the internal carotid arteries (ICA) in a bilateral fashion in 1969. Characterized by recurrent ischemic events in the developing brains of young patients, the process is one that often decimates the quality of life of affected individuals. The vascular changes in MMD have been demonstrated to occur in an extracranial manner, thus it is logical to assume that the same steno-occlusive mechanism could induce dysfunction and ischemia in other organ systems. Our case presents a patient with MMD with cardiac manifestations that we suspect may be related to extracranial manifestations of MMD.