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Approach to Dextrocardia in Adults: Review

¹ Department of Radiology, UMDNJ-NJ Medical School, University Hospital, 150 Bergen St., UH C-320, Newark, NJ 07103-2406.
² Department of Medicine, Division of Cardiology, UMDNJ-NJ Medical School, University Hospital, Newark, NJ.

View larger version (26K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1 —Diagram illustrates bending of primitive cardiac tube (top row) and possible relationships of great arteries at semilunar valve level (bottom row). Cardiac tube is depicted from anterior view. Cardiac tube comprises atrium (A), ventricle (V), bulbus cordis (B), and truncus arteriosus (T). Cardiac tube normally bends to right, forming a D-bulboventricular loop. Rarely, tube may bend leftward, forming L-bulboventricular loop. Possible relationships of great vessels are depicted axially from inferior perspective (as seen on CT images in transverse plane). In D-bulboventricular loop, possible relationships are normal or D-TGA (transposition of great arteries). With L-bulboventricular loop, possible relationships are inverted, or L-TGA. Ao = aorta, P = pulmonary artery.

View larger version (87K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2 —CT scan in 22-year-old man with Kartagener's syndrome. Image of lungs (left image) shows dextrocardia and bronchiectasis. Image of great vessels (right image) shows that main pulmonary artery (P) is to right of ascending aorta (A), an inverted relationship, as is expected with situs inversus.

View larger version (148K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3A —42-year-old woman with isolated dextroversion. Chest radiograph shows dextrocardia and discordant location of cardiac apex relative to stomach and liver shadow. Locations of gastric bubble and liver shadow are consistent with situs solitus.

View larger version (118K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3B —42-year-old woman with isolated dextroversion. Axial image from ECG-gated CT scan at level of ventricles shows that left atrium (LA) and left ventricle (LV) are anterior to right atrium (RA) and right ventricle (RV). Ventricles are in D-Loop configuration.

View larger version (149K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3C —42-year-old woman with isolated dextroversion. Oblique image in plane of outflow tract of left ventricle (LV) shows fibrous continuity between inflow mitral valve (arrow) and outflow aortic valve, confirming that left ventricle is a morphologic left ventricle. Left ventricle is located anteriorly and inferiorly to right ventricle (RV).

View larger version (125K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3D —42-year-old woman with isolated dextroversion. Volume-rendered image in anterior view provides 3D perspective of locations of right ventricle (RV), left ventricle (LV), and pulmonary outflow tract (P) of right ventricle. Muscular outflow tract confirms that right ventricle is a morphologic right ventricle. Pulmonic valve is located anteriorly and slightly to left of aortic root, as in normal patients.

View larger version (127K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4A —ECG-gated axial spin-echo T1-weighted MR images in patient with dextrocardia, situs solitus, and corrected transposition of great arteries (TGA). (Reprinted with permission from Reddy GP, Caputo GR. Diagnosis please: case 15. Radiology 1999; 211:709-710 [2]) Liver is on right and spleen is on left (bottom image), revealing situs solitus. Image through cardiac chambers (top image) shows discordant atrioventricular connections. Inferior pulmonary veins (arrowheads) drain to morphologic left atrium (LA). Left atrium is connected to morphologic right ventricle (RV), which is distinguished by presence of a moderator band (arrow). Morphologic right atrium (RA) is connected to morphologic left ventricle (LV). Ventricles are in L-loop configuration.

View larger version (117K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4B —ECG-gated axial spin-echo T1-weighted MR images in patient with dextrocardia, situs solitus, and corrected transposition of great arteries (TGA). (Reprinted with permission from Reddy GP, Caputo GR. Diagnosis please: case 15. Radiology 1999; 211:709-710 [2]) Images at progressively higher levels show muscular outflow tract or infundibulum (I, top image), which is characteristic of a morphologic right ventricle. Pulmonary artery (P) arises from outflow tract of morphologic left ventricle (LV, top image). Right-sided atrioventricular valve (arrowhead, top image) is near root of pulmonary artery because of fibrous continuity of inflow and outflow valves characteristic of a morphologic left ventricle. In lower image, aortic root (A) arises from morphologic right ventricle. Discordant atrioventricular and ventriculoarterial connections define disorder as congenitally corrected TGA. This case represents L-TGA because aorta is to left of pulmonary artery. RA = right atrium, S = superior vena cava.

View larger version (165K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5A —Images from ECG-gated CT scan of 52-year-old woman with dextrocardia, situs inversus, and congenitally corrected transposition of great arteries (TGA). Axial image through upper abdomen shows liver on left and spleen and stomach (St) on right, consistent with situs inversus.

View larger version (144K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5B —Images from ECG-gated CT scan of 52-year-old woman with dextrocardia, situs inversus, and congenitally corrected transposition of great arteries (TGA). Axial image at level of cardiac chambers shows that morphologic left atrium (LA) is connected to a morphologic right ventricle (RV), distinguished by prominent trabeculations along its septal surface. Morphologic right atrium (RA) is connected to a morphologic left ventricle (LV). Artifacts are caused by presence of pacemaker in left ventricle and prosthetic valve replacement of inflow valve of right ventricle. (Arrhythmias and tricuspid valve dysfunction are common complications of congenitally corrected TGA.) Ventricles are in D-loop configuration.

View larger version (167K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5C —Images from ECG-gated CT scan of 52-year-old woman with dextrocardia, situs inversus, and congenitally corrected transposition of great arteries (TGA). Reformatted oblique coronal image through outflow tract of posterior ventricle shows muscular infundibulum (arrows) separating inflow and outflow regions and confirming that posterior ventricle is a morphologic right ventricle (RV). Ventricle connects to aorta (A). LA = morphologic left atrium.

View larger version (112K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5D —Images from ECG-gated CT scan of 52-year-old woman with dextrocardia, situs inversus, and congenitally corrected transposition of great arteries (TGA). Volume-rendered image from anterior view provides 3D perspective of relationships of cardiac chambers and great vessels. Aortic valve is superior and to right of pulmonic valve, as expected with D-TGA. Pulmonary artery (P) is enlarged in this patient due to pulmonary arterial hypertension that is likely secondary to tricuspid valve disorder. Even though patient has situs inversus, anterior descending coronary artery (arrowheads) is supplied by left coronary artery (arrow) because ventricles are in D-loop configuration. LA = morphologic left atrium, LV = morphologic left ventricle, RA = morphologic right atrium, RV = morphologic right ventricle, SVC = superior vena cava.

View larger version (117K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6A —Images from ECG-gated CT scan of 20-year-old man with dextrocardia and polysplenia syndrome. Axial image through upper abdomen shows multiple splenules in right upper quadrant posterior to right-sided stomach (St). Liver is predominantly left-sided. Vessel (arrow) adjacent to descending thoracic aorta is enlarged hemiazygos vein from hemiazygos continuation of inferior vena cava. Note absence of intrahepatic portion of inferior vena cava.

View larger version (106K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6B —Images from ECG-gated CT scan of 20-year-old man with dextrocardia and polysplenia syndrome. Axial image through cardiac chambers shows complete endocardial cushion defect comprised of absence of atrial septum (common atrium) and upper portion of ventricular septum. Arrows indicate leaflets of common atrioventricular valve.

View larger version (126K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6C —Images from ECG-gated CT scan of 20-year-old man with dextrocardia and polysplenia syndrome. Reformatted coronal image shows bilateral hyparterial bronchi. Pulmonary arteries (P) are enlarged from chronic pulmonary artery hypertension secondary to long-standing intracardiac left-to-right shunt. A = aorta, arrow = arch of hemiazygos vein connecting to left-sided superior vena cava (not shown).

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