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Surgically Corrected Congenital Heart Disease: Utility of 64-MDCT

¹ Department of Pediatrics, The Johns Hopkins Medical Institutions and Johns Hopkins Hospital, Brady 5, 600 N Wolfe St., Baltimore, MD 21287.
² Department of Medicine, The Johns Hopkins Medical Institutions and Johns Hopkins Hospital, Baltimore, MD.
³ The Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins Medical Institutions, Baltimore, MD.

View larger version (129K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A —15-month-old male infant who had previously undergone modified left Blalock-Taussig shunt and placement of central shunt. Left pulmonary artery could not be seen on echocardiography after central shunt was placed. AO = aorta. Axial volume rendering with clip plane editing from IV contrast-enhanced CT shows central shunt (arrow) arising from anterior aorta to supply mildly hypoplastic right pulmonary artery (RPA). Origin of left pulmonary artery is not well seen.

View larger version (108K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B —15-month-old male infant who had previously undergone modified left Blalock-Taussig shunt and placement of central shunt. Left pulmonary artery could not be seen on echocardiography after central shunt was placed. AO = aorta. Coronal oblique multiplanar reconstruction (MPR) shows moderate to severe narrowing (arrow) of left pulmonary artery (LPA), and good-sized, more distal left pulmonary artery. LV = left ventricle.

View larger version (131K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C —15-month-old male infant who had previously undergone modified left Blalock-Taussig shunt and placement of central shunt. Left pulmonary artery could not be seen on echocardiography after central shunt was placed. AO = aorta. Oblique color-coded volume rendering from left superior perspective shows length of left pulmonary artery (LPA) stenosis ( 8 mm). Distance is particularly important to know in determining whether stenosis can be reached from midsternotomy, or if left thoracotomy is required.

View larger version (133K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A —7-year-old girl with double-outlet right ventricle status after pulmonary artery banding followed by bilateral bidirectional Glenn shunts. Catheterization revealed acceptable hemodynamics to proceed with Fontan procedure. MDCT was conducted to assess for branch pulmonary artery distortion, assess for aorta-to-pulmonary artery or venoatrial collaterals, and review caval–pulmonary artery connections. Sagittal multiplanar reconstruction (MPR) shows aorta (AO) and main pulmonary artery (MPA) arising from right ventricle (V). Note tight pulmonary artery band (arrow).

View larger version (102K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B —7-year-old girl with double-outlet right ventricle status after pulmonary artery banding followed by bilateral bidirectional Glenn shunts. Catheterization revealed acceptable hemodynamics to proceed with Fontan procedure. MDCT was conducted to assess for branch pulmonary artery distortion, assess for aorta-to-pulmonary artery or venoatrial collaterals, and review caval–pulmonary artery connections. Coronal oblique color-coded volume-rendering with clip plane editing shows that left-sided Glenn anastomosis, from left superior vena cava (LSVC) to left pulmonary artery (LPA), is unobstructed and left pulmonary artery is of a good size. AO = aorta.

View larger version (102K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2C —7-year-old girl with double-outlet right ventricle status after pulmonary artery banding followed by bilateral bidirectional Glenn shunts. Catheterization revealed acceptable hemodynamics to proceed with Fontan procedure. MDCT was conducted to assess for branch pulmonary artery distortion, assess for aorta-to-pulmonary artery or venoatrial collaterals, and review caval–pulmonary artery connections. Coronal MPR shows that right-sided Glenn anastomosis (right superior vena cava [RSVC] to right pulmonary artery) is minimally distorted, with caval connection close to or extending across origin of right upper lobe pulmonary artery. CPA = central pulmonary artery.

View larger version (133K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3A —12-year-old girl with double-outlet right ventricle, severely malaligned complete atrioventricular canal, ipsilateral pulmonary venous return, interrupted inferior vena cava with azygous extension to left superior vena cava, and abdominal situs inversus. Kawashima procedure was performed, and subsequently conduit was placed to incorporate hepatic blood flow into pulmonary circuit in order to treat pulmonary arteriovenous malformations. In these coronal volume renderings, conduit (C) is positioned to right of aorta (AO in A) and has mild narrowing as it turns superiorly (arrow in B). No thrombus was present. LIA in A = left innominate artery.

View larger version (155K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3B —12-year-old girl with double-outlet right ventricle, severely malaligned complete atrioventricular canal, ipsilateral pulmonary venous return, interrupted inferior vena cava with azygous extension to left superior vena cava, and abdominal situs inversus. Kawashima procedure was performed, and subsequently conduit was placed to incorporate hepatic blood flow into pulmonary circuit in order to treat pulmonary arteriovenous malformations. In these coronal volume renderings, conduit (C) is positioned to right of aorta (AO in A) and has mild narrowing as it turns superiorly (arrow in B). No thrombus was present. LIA in A = left innominate artery.

View larger version (149K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3C —12-year-old girl with double-outlet right ventricle, severely malaligned complete atrioventricular canal, ipsilateral pulmonary venous return, interrupted inferior vena cava with azygous extension to left superior vena cava, and abdominal situs inversus. Kawashima procedure was performed, and subsequently conduit was placed to incorporate hepatic blood flow into pulmonary circuit in order to treat pulmonary arteriovenous malformations. In more posterior coronal volume rendering with clip plane editing, aorta (AO) is shown arising from right ventricle (RV), and subaortic conus is present. A = right atrium, CV = connecting vein.

View larger version (146K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3D —12-year-old girl with double-outlet right ventricle, severely malaligned complete atrioventricular canal, ipsilateral pulmonary venous return, interrupted inferior vena cava with azygous extension to left superior vena cava, and abdominal situs inversus. Kawashima procedure was performed, and subsequently conduit was placed to incorporate hepatic blood flow into pulmonary circuit in order to treat pulmonary arteriovenous malformations. Coronal volume rendering with clip plane editing shows sites of ipsilateral pulmonary venous return (small arrows) and connection of left superior vena cava (LSVC) with left pulmonary artery (large arrow). Note right aortic arch (AO), right-sided stomach, and left-sided liver. Multiple small spleens were seen in right upper quadrant in addition to interrupted inferior vena cava with azygous continuation (not shown), typical of polysplenic form of heterotaxy syndrome.

View larger version (149K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4A —19-year-old man with history of double-inlet left ventricle after Glenn shunt and Fontan procedure complicated by protein-losing enteropathy. MDCT was performed to assess cardiopulmonary morphology and function. Coronal color-coded volume rendering shows external conduit from inferior vena cava to right pulmonary artery. Conduit is well seen without artifact using MDCT.

View larger version (183K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4B —19-year-old man with history of double-inlet left ventricle after Glenn shunt and Fontan procedure complicated by protein-losing enteropathy. MDCT was performed to assess cardiopulmonary morphology and function. Coronal volume rendering with clip plane editing enables visualization of internal portion of conduit from inferior vena cava to right pulmonary artery, which was patent.

View larger version (172K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4C —19-year-old man with history of double-inlet left ventricle after Glenn shunt and Fontan procedure complicated by protein-losing enteropathy. MDCT was performed to assess cardiopulmonary morphology and function. Sagittal volume rendering with clip plane editing shows persistent left superior vena cava (arrows) to coronary sinus, which is dilated.

View larger version (126K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4D —19-year-old man with history of double-inlet left ventricle after Glenn shunt and Fontan procedure complicated by protein-losing enteropathy. MDCT was performed to assess cardiopulmonary morphology and function. Sagittal oblique volume rendering from inferolateral orientation depicts prominent venous collaterals along cardiac border that arose from left vena cava. Also seen is conduit from inferior vena cava to right pulmonary artery.

View larger version (135K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5A —Unexpected desaturation after Fontan procedure in 33-year-old woman. At catheterization, there was distortion of external conduit by mediastinal mass. Echocardiography (not shown) showed that mass was fluid-filled. IV contrast-enhanced MDCT was performed to better understand nature of mediastinal mass and its relationship to external conduit. From this coronal multiplanar reconstruction (MPR), conduit (C) from inferior vena cava and hepatic veins to right pulmonary artery is well visualized and is surrounded by fluid collection (F). V = left ventricle, AO = aorta.

View larger version (112K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5B —Unexpected desaturation after Fontan procedure in 33-year-old woman. At catheterization, there was distortion of external conduit by mediastinal mass. Echocardiography (not shown) showed that mass was fluid-filled. IV contrast-enhanced MDCT was performed to better understand nature of mediastinal mass and its relationship to external conduit. Delayed IV contrast–enhanced axial MDCT image shows enhancing conduit (C) from inferior vena cava and hepatic veins to right pulmonary artery, surrounded by fluid collection (F). Note that, except for thin rim, fluid collection does not enhance, suggesting that it does not communicate with vascular bed. Collection was drained percutaneously. V = left ventricle, LA = left atrium.

View larger version (114K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6 —3-month-old boy with type II truncus arteriosus after surgical anastomosis of pulmonary arteries to right ventricle using 11-mm conduit and repair of ventral septal defect. Postoperative catheterization showed minimal residual ventral septal defect and pressure gradient at origin of right (RPA) and left (LPA) pulmonary arteries. However, because of conduit orientation, pulmonary artery origins could not be visualized to determine caliber. Axial oblique volume-rendered CT image with clip plane editing shows that both proximal pulmonary arteries are narrowed, with right measuring 3.3 mm and left, 4.2 mm. Three months after CT, diminished right ventricular systolic function prompted cardiac catheterization to allow stent placement in both branch pulmonary arteries.

View larger version (121K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7A —3.5-year-old boy with bilateral superior vena cava and hypoplastic left heart after bilateral, bidirectional Glenn shunts and an initial Norwood procedure. Oxygen saturation was less than expected, and mild right ventricular dysfunction was seen at echocardiography (not shown). MDCT was performed to image aortic arch, main pulmonary artery-to-aorta anastomosis, and cava–pulmonary artery connections. Coronal color-coded volume rendering shows that anastomosis between ascending aorta (AO) and main pulmonary artery (MPA) is wide, resulting in dilated neoascending aorta.

View larger version (146K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7B —3.5-year-old boy with bilateral superior vena cava and hypoplastic left heart after bilateral, bidirectional Glenn shunts and an initial Norwood procedure. Oxygen saturation was less than expected, and mild right ventricular dysfunction was seen at echocardiography (not shown). MDCT was performed to image aortic arch, main pulmonary artery-to-aorta anastomosis, and cava–pulmonary artery connections. Coronal volume rendering with clip plane editing confirms that connection of right superior vena cava (RSVC) to right pulmonary artery (asterisk) is unobstructed. Arrow points to anastomosis between aorta (AO) and main pulmonary artery (MPA). RA = right atrium, RV = right ventricle.

View larger version (159K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7C —3.5-year-old boy with bilateral superior vena cava and hypoplastic left heart after bilateral, bidirectional Glenn shunts and an initial Norwood procedure. Oxygen saturation was less than expected, and mild right ventricular dysfunction was seen at echocardiography (not shown). MDCT was performed to image aortic arch, main pulmonary artery-to-aorta anastomosis, and cava–pulmonary artery connections. Sagittal volume rendering with clip plane editing delineates dilated neoascending aorta (NeoAO) and shows that arch is unobstructed.

View larger version (111K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8A —17-year-old girl with history of tetralogy of Fallot and pulmonary atresia, after intracardiac repair with right ventricular outflow patch, right ventricular–pulmonary artery conduit valve replacement, and stent enlargement of left pulmonary artery, followed by surgical replacement of right ventricle-to-pulmonary artery conduit. Pulmonary perfusion studies (not shown) showed 35% flow to right lung and 65% to left lung. Branch pulmonary arteries were not visualized on echocardiography (not shown) but are known to be stenotic. MDCT was performed to delineate anatomy in consideration of augmenting flow to right lung by dilatation. Axial volume rendering from superior orientation with clip plane editing delineates unifocalization of collateral (arrows) supplying blood to right lower lobe. Collateral now passes anterior to ascending aorta. Because of critical right pulmonary artery hypoplasia (shown in B), this collateral is important to right lung flow.

View larger version (88K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8B —17-year-old girl with history of tetralogy of Fallot and pulmonary atresia, after intracardiac repair with right ventricular outflow patch, right ventricular–pulmonary artery conduit valve replacement, and stent enlargement of left pulmonary artery, followed by surgical replacement of right ventricle-to-pulmonary artery conduit. Pulmonary perfusion studies (not shown) showed 35% flow to right lung and 65% to left lung. Branch pulmonary arteries were not visualized on echocardiography (not shown) but are known to be stenotic. MDCT was performed to delineate anatomy in consideration of augmenting flow to right lung by dilatation. Axial CT image shows severe hypoplasia of native right pulmonary artery. Cardiac catheterization (not shown), performed several months later, confirmed hypoplasia of right pulmonary artery and branch pulmonary artery distortion as well as collaterals from left lower lobe pulmonary artery and right internal mammary artery to right pulmonary arterial circulation, latter of which was embolized. MDCT detected extensive aortic collaterals (not shown), which were embolized at catheterization.

View larger version (109K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9A —27-day-old, 3.4-kg female infant with transposition of great vessels who underwent arterial switch procedure. Branch pulmonary arteries are draped anterior to ascending aorta (Lecompte maneuver); ductus is ligated but aortic end remains patent. Postoperatively, patient had persistent tachypnea and right lung atelectasis. MDCT was performed to evaluate branch pulmonary arteries and right lung collapse in order to determine cause of respiratory distress. Coronal oblique color-coded volume rendering shows that main pulmonary artery (MPA) anastomosis is unobstructed. MPA and left pulmonary artery (LPA) pass anterior to ascending aorta (AO). RV = right ventricle, LV = left ventricle.

View larger version (113K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9B —27-day-old, 3.4-kg female infant with transposition of great vessels who underwent arterial switch procedure. Branch pulmonary arteries are draped anterior to ascending aorta (Lecompte maneuver); ductus is ligated but aortic end remains patent. Postoperatively, patient had persistent tachypnea and right lung atelectasis. MDCT was performed to evaluate branch pulmonary arteries and right lung collapse in order to determine cause of respiratory distress. Sagittal color-coded volume rendering confirms that left main coronary artery (arrow) is unobstructed. Aortic end of ductus arteriosus (DA) is noted. LV = left ventricle, AO = aorta.

View larger version (109K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9C —27-day-old, 3.4-kg female infant with transposition of great vessels who underwent arterial switch procedure. Branch pulmonary arteries are draped anterior to ascending aorta (Lecompte maneuver); ductus is ligated but aortic end remains patent. Postoperatively, patient had persistent tachypnea and right lung atelectasis. MDCT was performed to evaluate branch pulmonary arteries and right lung collapse in order to determine cause of respiratory distress. On this coronal color-coded volume rendering from superior orientation, branch pulmonary arteries are well seen and undistorted. RPA = right pulmonary artery, LPA = left pulmonary artery.

View larger version (133K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9D —27-day-old, 3.4-kg female infant with transposition of great vessels who underwent arterial switch procedure. Branch pulmonary arteries are draped anterior to ascending aorta (Lecompte maneuver); ductus is ligated but aortic end remains patent. Postoperatively, patient had persistent tachypnea and right lung atelectasis. MDCT was performed to evaluate branch pulmonary arteries and right lung collapse in order to determine cause of respiratory distress. Coronal multiplanar reconstruction reveals bilateral atelectasis (right worse than left), accounting for patient's respiratory symptoms.

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