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

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.

Surgical myectomy: Rationale and personalized technique.

Septal myectomy is currently the gold standard treatment for symptomatic patients with hypertrophic cardiomyopathy. The procedure needs to be tailored and performed in a personalized fashion, taking into consideration the anatomical spectrum of this disease. The procedure needs to address the various components that contribute to the clinical and pathological picture of this disease including, the fibrous trigones, accessory tissues, papillary muscles, mitral valve and myocardial bridges. The operation can be performed with very low mortality and morbidity in high-volume experienced centers with predictable excellent short and long-term outcomes. There is a need for broadening the experience of this procedure to the rest of the world and for future development of new enhanced precision imaging and surgical tools.