Congenital heart disease (CHD) represents the most common birth defect and affects approximately 1.2% of all live births and is the leading cause of birth defect-related deaths. CHD patients require frequent diagnostic testing in order to plan surgical repair, assess preoperative risk, and/or survey for important long-term complications. Bicuspid aortic valve (BAV), one of the most common forms of CHD, and single ventricle physiology (SVP), one of the most severe, are two patient cohorts which will require such longitudinal surveillance. Standard diagnostic tools, however, often involve invasive catheter-based procedures, ionizing radiation, and/or lengthy 60-90 minute Magnetic Resonance Imaging (MRI) exams, necessitating sedation or general anesthesia in pediatric patients. Recent studies suggest that general anesthesia could adversely affect neurologic, cognitive, and social development of neonates and young children. In addition, common outcome measures for CHD diseases such as BAV or SVP are coarse and rely on simplified parameters such as the diameter of the aortic root or the post-operative clinical status which do not reflect the underlyig mechanisms of disease progression. Further knowledge of which BAV and SVP patients are at risk for aortopathy or failing Fontan physiology would improve patient management and therapy planning by defining regular follow-up intervals and generating precise criteria for referral to surgical correction. Our goal is to develop and a new comprehensive 20-minute cardiovascular functional 4D MR exam that can replace the long standard MR imaging protocol and reduce or eliminate exposure to general anesthesia. Patient-specific post-hoc analysis will allow retrospective quantification of cardiac function and flow without limitation to predefined 2D scan planes. In addition, new hemodynamic biomarkers will be derived and evaluated for their potential as prognostic markers for improved outcome prediction in patients with BAV and SVP.

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We propose to develop a novel magnetic resonance imaging (MRI) protocol for the non- invasive characterization of cardiac function and three-dimensional blood flow in the heart and great vessels in patients with congenital heart disease. Our goal is to develop a new diagnostic test to achieve an improved assessment for the most common and one of the most severe forms of congenital heart disease: bicuspid aortic valve and single ventricle physiology. We plan to develop a now 20 minute MRI exam for the evaluation of cardiac function and blood flow which will help to reduce exposure to general anesthesia which is often needed in pediatric patients with congenital heart disease when standard diagnostic tests with much longer examination times are used. In addition, the new MRI protocol will provide new measures for improved outcome prediction and therapy management for patients with bicuspid aortic valve and single ventricle physiology.

National Institute of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Research Project (R01)
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Special Emphasis Panel (ZRG1-SBIB-V (82))
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Evans, Frank
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Northwestern University at Chicago
Schools of Medicine
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Bollache, Emilie; Guzzardi, David G; Sattari, Samaneh et al. (2018) Aortic valve-mediated wall shear stress is heterogeneous and predicts regional aortic elastic fiber thinning in bicuspid aortic valve-associated aortopathy. J Thorac Cardiovasc Surg 156:2112-2120.e2
Shen, Daming; Edelman, Robert R; Robinson, Joshua D et al. (2018) Single-Shot Coronary Quiescent-Interval Slice-Selective Magnetic Resonance Angiography Using Compressed Sensing: A Feasibility Study in Patients With Congenital Heart Disease. J Comput Assist Tomogr 42:739-746
Robinson, Joshua D; Rose, Michael J; Joh, Maria et al. (2018) 4-D flow magnetic-resonance-imaging-derived energetic biomarkers are abnormal in children with repaired tetralogy of Fallot and associated with disease severity. Pediatr Radiol :
Raghav, Vrishank; Barker, Alex J; Mangiameli, Daniel et al. (2018) Valve mediated hemodynamics and their association with distal ascending aortic diameter in bicuspid aortic valve subjects. J Magn Reson Imaging 47:246-254
Bollache, Emilie; Barker, Alex J; Dolan, Ryan Scott et al. (2018) k-t accelerated aortic 4D flow MRI in under two minutes: Feasibility and impact of resolution, k-space sampling patterns, and respiratory navigator gating on hemodynamic measurements. Magn Reson Med 79:195-207
Bollache, Emilie; Fedak, Paul W M; van Ooij, Pim et al. (2018) Perioperative evaluation of regional aortic wall shear stress patterns in patients undergoing aortic valve and/or proximal thoracic aortic replacement. J Thorac Cardiovasc Surg 155:2277-2286.e2
Garcia, Julio; van der Palen, Roel L F; Bollache, Emilie et al. (2018) Distribution of blood flow velocity in the normal aorta: Effect of age and gender. J Magn Reson Imaging 47:487-498
Ma, Liliana E; Vali, Alireza; Blanken, Carmen et al. (2018) Altered Aortic 3-Dimensional Hemodynamics in Patients With Functionally Unicuspid Aortic Valves. Circ Cardiovasc Imaging 11:e007915
Geiger, Julia; Rahsepar, Amir A; Suwa, Kenichiro et al. (2018) 4D flow MRI, cardiac function, and T1 -mapping: Association of valve-mediated changes in aortic hemodynamics with left ventricular remodeling. J Magn Reson Imaging 48:121-131
Schnell, Susanne; Ansari, Sameer A; Wu, Can et al. (2017) Accelerated dual-venc 4D flow MRI for neurovascular applications. J Magn Reson Imaging 46:102-114

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