Progression of arterial toursosity syndrome to multiple aneurysms: Role of defining aortic flow and biomechanics

Abstract

Arterial tortuosity syndrome (ATS) is a rare aortopathy characterized by multiple areas of tortuosity, stenosis and aneurysms in large and mid-sized arteries. The management of this syndrome is challenging because its complexity and variability in presentation and progression require a thorough understanding of the biological and biomechanical changes that occur in the arterial system. Here we describe, for the first time, the progression of this disease diagnosed in a 3-year old girl and the use of modern imaging modalities including cardiac magnetic resonance (CMR) 4D Flow, 3D modeling, and computational fluid dynamic simulation to characterize the complex aortic flow and its biomechanics. The integration of these modalities with the clinical evaluation will help in our understanding of this disease and provide patient-specific management.