Ferumoxytol-Enhanced Cardiac Magnetic Resonance Angiography and 4D Flow: Safety and Utility in Pediatric and Adult Congenital Heart Disease

Figures

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  Figure 1. Total Anomalous Pulmonary Venous Connection (TAPVC), supracardiac type. In this neonate, coronal reconstructions from ferumoxytol-enhanced 4D MRA (MUSIC) clearly show abnormal connection of the pulmonary veins (black asterisks) connections to a pulmonary venous confluence (PVC), rather than the left atrium. Pulmonary venous blood then drains via superior “vertical veins” (red arrows), innominate vein, and then to the dilated right superior vena cava (yellow arrow).

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  Figure 2. Pulmonary Vein Stenosis (acquired). Reformatted projections from 4D MUSIC images in an infant with hypoplastic left heart syndrome. Acquired postoperative stenosis of the left superior and inferior pulmonary veins after stage I surgical palliation is demonstrated (solid arrows). Narrowed right superior and inferior pulmonary vein ostia are also seen (open arrows).

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  Figure 3. Fontan Circulation. Breath-held and ECG-gated FE-MRA in an adult patient with single ventricle heart disease who has undergone Fontan surgical palliation (i.e., routing of the SVC and IVC to the branch pulmonary arteries). Panel (A): Coronal maximum intensity projection depicting the superior limb (Glenn shunt) of the systemic venous drainage (red arrow). Panel (B): Coronal maximum intensity projection in a more posterior position from that in Panel (A) showing the inferior limb (Fontan conduit) of the systemic venous drainage and its connection to the right branch pulmonary artery (green arrows). Panel (C): 3D volume rendered image the image dataset which clearly and homogenously depicts the Fontan circulation (green arrows). The entire gated volumetric acquisition was acquired in a single breath-hold.

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  Figure 4. Coarctation of the Aorta. Panels (A–C): 4D FE-MRA (MUSIC) in a neonate with juxtaductal coarctation of the aorta (solid arrows) and hypoplastic transverse aortic arch. Additionally, present is an aberrant right subclavian artery (open arrow). Panels (D,E): Ferumoxytol-enhanced 4D flow in a different patient with coarctation showing anatomic narrowing of the aortic isthmus (solid arrow) with red flow (plus sign) indicating acceleration of blood flow across the area of stenosis.

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  Figure 5. Aortic Root Aneurysm in Marfan Syndrome. Multiplanar reformatted breath-held FE-MRA (sagittal, axial, and coronal planes) in an adult patient with Marfan syndrome depicting an aneurysm of the aortic root (red asterisks). Additionally, homogeneous and high-resolution 3D images of the ascending, transverse (arch), descending thoracic, and upper abdominal aorta were possible in the same acquisition without the need for bolus timing.

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  Figure 6. Vascular Ring. Reformatted maximum intensity projections from a breath-held FE-MRA study (Panels (A–C)) and 3D volume rendering (Panel (D)) depicting a right aortic arch (white arrows) with aberrant left subclavian artery (black arrows) arising from a diverticulum of Kommerell. This configuration forms a vascular ring causing mild tracheal compression in this patient.

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  Figure 7. Tetralogy of Fallot with Pulmonary Atresia. Reformatted multiplanar maximum intensity projections from a 4D FE-MRA (MUSIC) study in a neonate. Continuous right and left branch pulmonary arteries are seen supplied by a patent ductus arteriosus (solid arrow) from the underside of the aortic arch. This study was used to plan transcatheter ductus arteriosus stent placement. No significant aortopulmonary collateral arteries were identified.

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  Figure 8. Double Outlet Right Ventricle with Pulmonary Stenosis (DORV/PS) in Heterotaxy Syndrome. Reformatted multiplanar maximum intensity projections from a 4D FE-MRA (MUSIC) study in an infant with DORV/PS. The aorta (solid arrow) arises from the right ventricle and is positioned anterior and rightward of the pulmonary artery (open arrow). The heart is positioned in the midline (mesocardia) with a leftward pointing apex (curved arrow). There are bilateral SVCs without a bridging vein (asterisks) and the IVC is left-sided, connecting to the leftward half of a common atrium (arrowheads). The liver is transverse.

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  Figure 9. Unbalanced “Right-dominant” Atrioventricular Canal Defect (AVCD). Multiplanar maximum intensity projections from a 4D FE-MRA (MUSCI) study in an infant with unbalanced AVCD and relatively small left ventricle (“right-dominant”). This study was performed to determine candidacy for a full surgical repair. The left LV is significantly smaller than the RV (Panel (A)). The dashed yellow line in Panel (A) indicates the plane of the short-axis image shown in Panel (B). The plane indicating where the interventricular septum should be is depicted by solid yellow line in Panel (B). From this short-axis image the asymmetry of the right and left sides of the AV valve, and the relative paucity of LAVV tissue, can be appreciated. Panels (C,D) are images from a study in a different patient. LV (red shaded area) and RV (blue shaded area) volumes and ejection fractions were calculated from diastolic (Panel (C)) and systolic (Panel (D)) 3D image data taken during steady-state enhancement by ferumoxytol. RV = right ventricle; LV = left ventricle; AV = atrioventricular; LAVV = left atrioventricular valve.

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  Figure 10. Hypoplastic Left Heart Complex (HLHC). Multiplanar maximum intensity reformatted projections from a 4D FE-MRA (MUSIC) study in a neonate with HLHC. Panels (A,B): There is a hypoplastic mitral valve (yellow arrow) and severe aortic arch hypoplasia with coarctation (white arrow). A large patent ductus arteriosus is also present (black asterisk). There is LV hypoplasia with relative enlargement of the RV. Panels (C,D): Postoperative images in the same patient using the same technique show a widely patient aortic arch after surgical repair (red asterisk). LV = left ventricle; RV = right ventricle.

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  Figure 11. Single Coronary Artery. A single coronary artery is seen arising from the left aortic sinus in this young child with surgically repaired double outlet right ventricle and atrioventricular canal defect. Multiplanar maximum intensity projections from a 4D FE-MRA (MUSIC) study show the common origin of the right (double arrow) and left coronary (single arrow) arteries from the posterior aspect of the left aortic sinus (yellow asterisk).

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  Figure 12. Absent Right Coronary Artery in Neonatal Pulmonary Atresia with Intact Ventricular Septum (PA-IVS). FE 4D MRA study (MUSIC) performed in a neonate on day 2 of life, reformatted in multiple planes, two showing normal origin of the left main coronary artery (red arrows), but right coronary ostial atresia (blue arrows). In the 3D volume rendered image, the right coronary artery (yellow stars) is seen arising directly from the right ventricular apex, consistent with right ventricle-dependent coronary circulation. Adapted with permission from reference [59]. RV = right ventricle; LV = left ventricle.