Arteriography Pulmonary Pulmonary arteriography is seldom required for diagnosis or surveillance of pulmonary artery dilatation which can occur in Marfan syndrome. CTA Chest CTA chest may be used for imaging surveillance to measure the aortic diameter, and to screen for involvement of the arterial tree in the chest. CTA is especially useful in the emergent setting. ECG gating is critical for accurate assessment of the aortic root. CTA Coronary Arteries CTA is the most useful modality for the evaluation of coronary ectasia or aneurysms, which are often seen in the setting of connective tissue diseases. The diagnostic utility of CTA is very useful compared to coronary MRA in terms of spatial resolution and shorter scan duration. FDG-PET/CT Heart There is no relevant literature to support the use of FDG-PET/CT heart in the evaluation of suspected aortopathy or connective tissue disorder. MRA Chest MRA is the most useful modality for aortic assessment when TTE is suboptimal for assessment of aortopathy in pediatric patients due to its ability to screen multiple territories with the same contrast injection, including the head, neck, chest, and abdomen. To avoid multiple administrations of gadolinium-based contrast agents, follow-up imaging of the aortic root may be performed with noncontrast MRA techniques like cine gradient echo and navigator respiratory gated 3-D steady-state free precession. MRA may offer further valuable information, when complemented with phase-contrast flow data in bicuspid aortic valve, about the possibility of associated valvular regurgitation and alterations in shear stress that may predispose to progressive dilation [91-93]. MRA Neck MRA neck is frequently performed at the same setting as MRA chest to screen for extracardiac vascular involvement in connective tissue disease and in all cases of aortopathy to measure vertebral artery tortuosity, which is used as a prognostic marker to determine risk of aortic dissection [94].
Congenital or Acquired Heart Disease. Arteriography Pulmonary Pulmonary arteriography is seldom required for diagnosis or surveillance of pulmonary artery dilatation which can occur in Marfan syndrome. CTA Chest CTA chest may be used for imaging surveillance to measure the aortic diameter, and to screen for involvement of the arterial tree in the chest. CTA is especially useful in the emergent setting. ECG gating is critical for accurate assessment of the aortic root. CTA Coronary Arteries CTA is the most useful modality for the evaluation of coronary ectasia or aneurysms, which are often seen in the setting of connective tissue diseases. The diagnostic utility of CTA is very useful compared to coronary MRA in terms of spatial resolution and shorter scan duration. FDG-PET/CT Heart There is no relevant literature to support the use of FDG-PET/CT heart in the evaluation of suspected aortopathy or connective tissue disorder. MRA Chest MRA is the most useful modality for aortic assessment when TTE is suboptimal for assessment of aortopathy in pediatric patients due to its ability to screen multiple territories with the same contrast injection, including the head, neck, chest, and abdomen. To avoid multiple administrations of gadolinium-based contrast agents, follow-up imaging of the aortic root may be performed with noncontrast MRA techniques like cine gradient echo and navigator respiratory gated 3-D steady-state free precession. MRA may offer further valuable information, when complemented with phase-contrast flow data in bicuspid aortic valve, about the possibility of associated valvular regurgitation and alterations in shear stress that may predispose to progressive dilation [91-93]. MRA Neck MRA neck is frequently performed at the same setting as MRA chest to screen for extracardiac vascular involvement in connective tissue disease and in all cases of aortopathy to measure vertebral artery tortuosity, which is used as a prognostic marker to determine risk of aortic dissection [94].