DOI: 10.1148/rg.262055092
Congenital and Acquired Pulmonary Artery Anomalies in the Adult: Radiologic Overview1
Eva Castañer, MD,
Xavier Gallardo, MD,
Jordi Rimola, MD,
Yolanda Pallardó, MD,
Josep M. Mata, MD, PhD,
Joan Perendreu, MD,
Cesar Martin, MD and
Damian Gil, MD
1 From the Department of Radiology, SDI UDIAT-CD, Institut Universitari Parc Taulí–UAB, Corporació Parc Taulí, Parc Taulí s/n, Sabadell 08208, Barcelona, Spain (E.C., X.G., J.R., J.M.M., J.P., C.M., D.G.); and Department of Radiology, Hospital de la Ribera, Alzira, Spain (Y.P.). Recipient of a Certificate of Merit award for an education exhibit at the 2004 RSNA Annual Meeting. Received April 7, 2005; revision requested May 17; revision received and accepted July 11. All authors have no financial relationships to disclose.
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Figure 1. Asymptomatic unilateral proximal interruption of the right pulmonary artery in a 48-year-old man. The posteroanterior chest radiograph shows a small hemithorax, mediastinal shift (arrowheads), absence of the right pulmonary artery shadow (open arrow), and linear opacities that correspond to systemic collateral vessels (solid arrows) along the pleura and within the lung. This patient did not manifest pulmonary hypertension.
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Figure 2a. Unilateral proximal interruption of the right pulmonary artery in a 52-year-old woman with progressive shortness of breath and hemoptysis. (a) Contrast material–enhanced CT scan shows only the proximal portion of the right pulmonary artery (arrowhead) and enlargement of the main and left pulmonary arteries that indicates pulmonary hypertension. (b) Contrast-enhanced CT scan at the level of the upper lobes shows serrated thickening of the right pleura because of enlarged intercostal collateral vessels (arrowheads). (c) CT scan obtained with a lung window setting shows multiple linear opacities perpendicular to the pleural surface that correspond to transpleural systemic vessels (arrowheads).
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Figure 2b. Unilateral proximal interruption of the right pulmonary artery in a 52-year-old woman with progressive shortness of breath and hemoptysis. (a) Contrast material–enhanced CT scan shows only the proximal portion of the right pulmonary artery (arrowhead) and enlargement of the main and left pulmonary arteries that indicates pulmonary hypertension. (b) Contrast-enhanced CT scan at the level of the upper lobes shows serrated thickening of the right pleura because of enlarged intercostal collateral vessels (arrowheads). (c) CT scan obtained with a lung window setting shows multiple linear opacities perpendicular to the pleural surface that correspond to transpleural systemic vessels (arrowheads).
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Figure 2c. Unilateral proximal interruption of the right pulmonary artery in a 52-year-old woman with progressive shortness of breath and hemoptysis. (a) Contrast material–enhanced CT scan shows only the proximal portion of the right pulmonary artery (arrowhead) and enlargement of the main and left pulmonary arteries that indicates pulmonary hypertension. (b) Contrast-enhanced CT scan at the level of the upper lobes shows serrated thickening of the right pleura because of enlarged intercostal collateral vessels (arrowheads). (c) CT scan obtained with a lung window setting shows multiple linear opacities perpendicular to the pleural surface that correspond to transpleural systemic vessels (arrowheads).
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Figure 3. Diagram shows the anomalous origin of a left pulmonary artery (P.A.) that arises from the posterior aspect of the right pulmonary artery and reaches the left hilum by passing between the trachea and the esophagus.
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Figure 4a. Anomalous origin of the left pulmonary artery in a 60-year-old asymptomatic woman. (a) Posteroanterior chest radiograph shows an anomalous right paratracheal border (arrowheads). (b, c) Unenhanced CT scan (b) and MR angiogram (c) at the level of the pulmonary trunk show the abnormal course of the left pulmonary artery (arrowheads in b) between the lower portion of the trachea and the esophagus (* in b). (Fig 4b and 4c reprinted, with permission, from reference 12.)
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Figure 4b. Anomalous origin of the left pulmonary artery in a 60-year-old asymptomatic woman. (a) Posteroanterior chest radiograph shows an anomalous right paratracheal border (arrowheads). (b, c) Unenhanced CT scan (b) and MR angiogram (c) at the level of the pulmonary trunk show the abnormal course of the left pulmonary artery (arrowheads in b) between the lower portion of the trachea and the esophagus (* in b). (Fig 4b and 4c reprinted, with permission, from reference 12.)
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Figure 4c. Anomalous origin of the left pulmonary artery in a 60-year-old asymptomatic woman. (a) Posteroanterior chest radiograph shows an anomalous right paratracheal border (arrowheads). (b, c) Unenhanced CT scan (b) and MR angiogram (c) at the level of the pulmonary trunk show the abnormal course of the left pulmonary artery (arrowheads in b) between the lower portion of the trachea and the esophagus (* in b). (Fig 4b and 4c reprinted, with permission, from reference 12.)
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Figure 5a. Idiopathic dilatation of the pulmonary trunk in a 55-year-old asymptomatic woman. (a) Posteroanterior chest radiograph shows an abnormal bulge in the left mediastinal border (arrowheads), a feature suggestive of a mediastinal mass identical to that observed on radiographs obtained 6 years earlier (not shown). (b) Contrast-enhanced CT scan shows abnormal enlargement of the main pulmonary trunk, with mild dilatation of the right and left pulmonary arteries. (c) CT scan obtained with a lung window setting at the same level as b shows normal vessels and parenchyma.
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Figure 5b. Idiopathic dilatation of the pulmonary trunk in a 55-year-old asymptomatic woman. (a) Posteroanterior chest radiograph shows an abnormal bulge in the left mediastinal border (arrowheads), a feature suggestive of a mediastinal mass identical to that observed on radiographs obtained 6 years earlier (not shown). (b) Contrast-enhanced CT scan shows abnormal enlargement of the main pulmonary trunk, with mild dilatation of the right and left pulmonary arteries. (c) CT scan obtained with a lung window setting at the same level as b shows normal vessels and parenchyma.
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Figure 5c. Idiopathic dilatation of the pulmonary trunk in a 55-year-old asymptomatic woman. (a) Posteroanterior chest radiograph shows an abnormal bulge in the left mediastinal border (arrowheads), a feature suggestive of a mediastinal mass identical to that observed on radiographs obtained 6 years earlier (not shown). (b) Contrast-enhanced CT scan shows abnormal enlargement of the main pulmonary trunk, with mild dilatation of the right and left pulmonary arteries. (c) CT scan obtained with a lung window setting at the same level as b shows normal vessels and parenchyma.
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Figure 6a. Pulmonary hypertension in a 32-year-old woman with HIV infection. (a) Posteroanterior chest radiograph shows enlargement of the main pulmonary artery (arrowheads). (b) Contrast-enhanced CT scan shows an enlarged pulmonary trunk with a maximum diameter of 39 mm (black line) near its bifurcation, lateral to the ascending aorta—a diameter greater than that of the ascending aorta.
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Figure 6b. Pulmonary hypertension in a 32-year-old woman with HIV infection. (a) Posteroanterior chest radiograph shows enlargement of the main pulmonary artery (arrowheads). (b) Contrast-enhanced CT scan shows an enlarged pulmonary trunk with a maximum diameter of 39 mm (black line) near its bifurcation, lateral to the ascending aorta—a diameter greater than that of the ascending aorta.
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Figure 7. Pulmonary hypertension. CT scan obtained with a lung window setting at the level of the upper lobes in a 75-year-old man shows marked enlargement of the pulmonary arteries (arrowheads) in relation to the bronchi.
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Figure 8. Pulmonary hypertension. CT scan obtained with a lung window setting in a 65-year-old woman shows a mosaic perfusion pattern, with increased diameters of vessels in areas of hyperattenuation (arrows) and sharp tapering of peripheral vessels in areas of hypoattenuation (arrowheads).
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Figure 9. Chronic thromboembolic pulmonary hypertension in a 62-year-old man with dyspnea. Contrast-enhanced CT scan shows enlargement of left (black arrowhead) and right (white arrowhead) bronchial arteries, as well as filling defects in the right upper lobe pulmonary vessels (arrows) that correspond to new locations of acute pulmonary thromboembolism.
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Figure 10. Pulmonary hypertension in a 72-year-old man with a mitral valve abnormality. Unenhanced CT scan shows dilatation and atherosclerotic calcification of the main and right pulmonary arteries (black arrowheads) and the left interlobar artery (white arrowhead).
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Figure 11a. Right heart abnormalities secondary to pulmonary hypertension in a 56-year-old woman. (a) Contrast-enhanced CT scan shows dilatation of the right ventricle (RV), with a right ventricle/left ventricle (LV) ratio greater than 1:1; leftward septal bowing (arrowhead); thickening of the free right ventricular wall (arrow); and dilatation of the right atrium (RA). (b) Contrast-enhanced CT scan at a lower level than a shows dilatation of the right ventricle (RV) and inferior vena cava (IVC), as well as a small pericardial effusion (*).
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Figure 11b. Right heart abnormalities secondary to pulmonary hypertension in a 56-year-old woman. (a) Contrast-enhanced CT scan shows dilatation of the right ventricle (RV), with a right ventricle/left ventricle (LV) ratio greater than 1:1; leftward septal bowing (arrowhead); thickening of the free right ventricular wall (arrow); and dilatation of the right atrium (RA). (b) Contrast-enhanced CT scan at a lower level than a shows dilatation of the right ventricle (RV) and inferior vena cava (IVC), as well as a small pericardial effusion (*).
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Figure 12. Severe pulmonary hypertension and right heart disease in a 75-year-old patient. CT scan shows opacification of the inferior vena cava and suprahepatic veins because of retrograde flow of contrast material, which is often seen in patients with elevated right atrial and right ventricular pressures.
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Figure 13. Pulmonary artery aneurysm in a 50-year-old man with Behçet disease and hemoptysis. Contrast-enhanced CT scan shows aneurysmal dilatation of a left interlobar pulmonary artery (*) with small mural thrombi (arrowheads).
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Figure 14a. Septic emboli in a 20-year-old woman with osteomyelitis and hemoptysis. (a) CT scan at the level of the lower lobes shows an enhancing round lung mass with attenuation similar to that of central arteries (not shown), findings that indicate an aneurysm in the right lower lobe artery (*). Peripheral triangular opacities in the left lung and a bilateral pleural effusion also are visible. (b) CT scan obtained with a lung window setting shows a cavitated peripheral nodule in the right lung (arrowhead), enlargement of a right interlobular artery, and right-sided pneumothorax (*) due to cavitary lesions. (c) MR angiogram shows the right lower lobe pulmonary artery aneurysm. A right lower lobectomy was performed.
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Figure 14b. Septic emboli in a 20-year-old woman with osteomyelitis and hemoptysis. (a) CT scan at the level of the lower lobes shows an enhancing round lung mass with attenuation similar to that of central arteries (not shown), findings that indicate an aneurysm in the right lower lobe artery (*). Peripheral triangular opacities in the left lung and a bilateral pleural effusion also are visible. (b) CT scan obtained with a lung window setting shows a cavitated peripheral nodule in the right lung (arrowhead), enlargement of a right interlobular artery, and right-sided pneumothorax (*) due to cavitary lesions. (c) MR angiogram shows the right lower lobe pulmonary artery aneurysm. A right lower lobectomy was performed.
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Figure 14c. Septic emboli in a 20-year-old woman with osteomyelitis and hemoptysis. (a) CT scan at the level of the lower lobes shows an enhancing round lung mass with attenuation similar to that of central arteries (not shown), findings that indicate an aneurysm in the right lower lobe artery (*). Peripheral triangular opacities in the left lung and a bilateral pleural effusion also are visible. (b) CT scan obtained with a lung window setting shows a cavitated peripheral nodule in the right lung (arrowhead), enlargement of a right interlobular artery, and right-sided pneumothorax (*) due to cavitary lesions. (c) MR angiogram shows the right lower lobe pulmonary artery aneurysm. A right lower lobectomy was performed.
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Figure 15a. Rasmussen aneurysm in a 42-year-old man with active postprimary tuberculosis and massive hemoptysis. (a) Contrast-enhanced CT scan at the level of the upper lobes shows, in an area of cavitation, a small rounded bilobed enhancing lesion (arrows) that arises from a branch of the pulmonary artery (arrowhead). (b) Conventional angiogram shows contrast material filling two aneurysms (arrowheads) in a segmental branch of the right upper lobe pulmonary artery. (c) Posteroanterior chest radiograph obtained after embolization shows coils (arrowheads) in the wall of the tuberculous cavity.
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Figure 15b. Rasmussen aneurysm in a 42-year-old man with active postprimary tuberculosis and massive hemoptysis. (a) Contrast-enhanced CT scan at the level of the upper lobes shows, in an area of cavitation, a small rounded bilobed enhancing lesion (arrows) that arises from a branch of the pulmonary artery (arrowhead). (b) Conventional angiogram shows contrast material filling two aneurysms (arrowheads) in a segmental branch of the right upper lobe pulmonary artery. (c) Posteroanterior chest radiograph obtained after embolization shows coils (arrowheads) in the wall of the tuberculous cavity.
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Figure 15c. Rasmussen aneurysm in a 42-year-old man with active postprimary tuberculosis and massive hemoptysis. (a) Contrast-enhanced CT scan at the level of the upper lobes shows, in an area of cavitation, a small rounded bilobed enhancing lesion (arrows) that arises from a branch of the pulmonary artery (arrowhead). (b) Conventional angiogram shows contrast material filling two aneurysms (arrowheads) in a segmental branch of the right upper lobe pulmonary artery. (c) Posteroanterior chest radiograph obtained after embolization shows coils (arrowheads) in the wall of the tuberculous cavity.
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Figure 16. Metastatic intravascular emboli in a 55-year-old man with renal cell carcinoma. CT scan obtained with a lung window setting shows vascular dilatation and beading of subsegmental arteries (arrowheads), findings highly suggestive of metastatic intravascular emboli.
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Figure 17. Unresectable bronchial carcinoma in a 30-year-old man. Contrast-enhanced CT scan shows extensive mediastinal tumor infiltration with obliteration of fat planes and encasement of the left pulmonary artery.
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Figure 18a. Focal mediastinal fibrosis secondary to tuberculosis in a 62-year-old man. (a) Unenhanced CT scan at the level of the right hilum shows a highly calcified mass that encases the right pulmonary artery (curved arrow) and involves the left lower lobe vein (straight arrow) and left atrium (not shown). (b) CT scan obtained with a lung window setting shows multiple linear opacities perpendicular to the pleural surface (black arrowhead), enlarged septa (white arrowhead) due to systemic supply by collateral vessels (secondary to involvement of the right pulmonary artery), and enlarged septal veins (secondary to involvement of the left lower lobe vein). Note the striking contrast in appearance between the right and left lungs.
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Figure 18b. Focal mediastinal fibrosis secondary to tuberculosis in a 62-year-old man. (a) Unenhanced CT scan at the level of the right hilum shows a highly calcified mass that encases the right pulmonary artery (curved arrow) and involves the left lower lobe vein (straight arrow) and left atrium (not shown). (b) CT scan obtained with a lung window setting shows multiple linear opacities perpendicular to the pleural surface (black arrowhead), enlarged septa (white arrowhead) due to systemic supply by collateral vessels (secondary to involvement of the right pulmonary artery), and enlarged septal veins (secondary to involvement of the left lower lobe vein). Note the striking contrast in appearance between the right and left lungs.
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Figure 19a. Late-stage Takayasu arteritis with right pulmonary artery involvement in a 63-year-old woman. (a) Unenhanced CT scan shows marked stenosis of the right pulmonary artery (arrowheads), left-sided pulmonary hypertension, and wall calcification of the left pulmonary artery and the ascending and descending aorta. (b) Contrast-enhanced CT scan at the level of the supraaortic trunks shows soft tissue that surrounds the brachiocephalic trunk (straight arrows), occlusion of the left carotid artery (curved arrow), poor visibility of vessels in the right lung because of right pulmonary artery involvement, and collateral vessel development from intercostal arteries (arrowheads). (c) Contrast-enhanced CT scan shows right pulmonary artery occlusion (straight arrow), enlarged bronchial arteries (curved arrow) in the right hilum, and an enlarged right internal mammary artery (arrowhead). (Case courtesy of Jordi Andreu, MD, Hospital Vall de Hebron, Barcelona, Spain.)
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Figure 19b. Late-stage Takayasu arteritis with right pulmonary artery involvement in a 63-year-old woman. (a) Unenhanced CT scan shows marked stenosis of the right pulmonary artery (arrowheads), left-sided pulmonary hypertension, and wall calcification of the left pulmonary artery and the ascending and descending aorta. (b) Contrast-enhanced CT scan at the level of the supraaortic trunks shows soft tissue that surrounds the brachiocephalic trunk (straight arrows), occlusion of the left carotid artery (curved arrow), poor visibility of vessels in the right lung because of right pulmonary artery involvement, and collateral vessel development from intercostal arteries (arrowheads). (c) Contrast-enhanced CT scan shows right pulmonary artery occlusion (straight arrow), enlarged bronchial arteries (curved arrow) in the right hilum, and an enlarged right internal mammary artery (arrowhead). (Case courtesy of Jordi Andreu, MD, Hospital Vall de Hebron, Barcelona, Spain.)
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Figure 19c. Late-stage Takayasu arteritis with right pulmonary artery involvement in a 63-year-old woman. (a) Unenhanced CT scan shows marked stenosis of the right pulmonary artery (arrowheads), left-sided pulmonary hypertension, and wall calcification of the left pulmonary artery and the ascending and descending aorta. (b) Contrast-enhanced CT scan at the level of the supraaortic trunks shows soft tissue that surrounds the brachiocephalic trunk (straight arrows), occlusion of the left carotid artery (curved arrow), poor visibility of vessels in the right lung because of right pulmonary artery involvement, and collateral vessel development from intercostal arteries (arrowheads). (c) Contrast-enhanced CT scan shows right pulmonary artery occlusion (straight arrow), enlarged bronchial arteries (curved arrow) in the right hilum, and an enlarged right internal mammary artery (arrowhead). (Case courtesy of Jordi Andreu, MD, Hospital Vall de Hebron, Barcelona, Spain.)
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Figure 20. Acute occlusive pulmonary thromboembolism in a 58-year-old woman. Contrast-enhanced CT scan shows enlargement of an occluded artery (arrowheads) in the left upper lobe compared with the diameters of adjacent patent vessels (arrow), as well as filling defects in the right upper lobe artery.
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Figure 21a. Acute pulmonary thromboembolism in a 32-year-old woman with severe dyspnea. (a) Contrast-enhanced CT scan shows significant bilateral partial filling defects in peripheral segments of both interlobar arteries (arrowheads). In the right interlobar artery, the filling defect results in the "railway track" sign. In the left interlobar artery, the partial filling defect and surrounding area of contrast enhancement form acute angles with the arterial wall. (b) Contrast-enhanced CT scan at the level of the lower lobes shows peripheral triangular nonenhanced densities (arrows), suggestive of infarcts or hemorrhage, and some peripheral areas with enhancement (white arrowhead) suggestive of atelectasis. The short axis (black line) of the right ventricle (RV) is wider than that of the left ventricle (LV), and mild displacement of the interventricular septa (black arrowheads) is visible. These abnormalities suggest right ventricular strain.
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Figure 21b. Acute pulmonary thromboembolism in a 32-year-old woman with severe dyspnea. (a) Contrast-enhanced CT scan shows significant bilateral partial filling defects in peripheral segments of both interlobar arteries (arrowheads). In the right interlobar artery, the filling defect results in the "railway track" sign. In the left interlobar artery, the partial filling defect and surrounding area of contrast enhancement form acute angles with the arterial wall. (b) Contrast-enhanced CT scan at the level of the lower lobes shows peripheral triangular nonenhanced densities (arrows), suggestive of infarcts or hemorrhage, and some peripheral areas with enhancement (white arrowhead) suggestive of atelectasis. The short axis (black line) of the right ventricle (RV) is wider than that of the left ventricle (LV), and mild displacement of the interventricular septa (black arrowheads) is visible. These abnormalities suggest right ventricular strain.
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Figure 22. Chronic pulmonary embolism in a 62-year-old man with dyspnea (same patient as in Fig 9). Contrast-enhanced CT scan shows a large eccentric pulmonary embolus (arrowhead) in the left main pulmonary artery.
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Figure 23. Chronic pulmonary embolism in a 72-year-old woman. Contrast-enhanced CT scan shows an eccentrically located small thrombus that forms obtuse angles with the vessel wall (arrowheads) in the right lower lobe.
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Figure 24a. Chronic pulmonary embolism in a 78-year-old man. (a) Unenhanced CT scan shows calcification at the level of the right pulmonary artery (white arrowhead) and a hypoattenuating adjacent area (black arrowheads). Unlike acute pulmonary embolism, chronic pulmonary embolism may be indicated by hypoattenuating clots at unenhanced CT. (b) Contrast-enhanced CT scan shows a peripheral clot in the corresponding hypoattenuating area (arrowhead).
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Figure 24b. Chronic pulmonary embolism in a 78-year-old man. (a) Unenhanced CT scan shows calcification at the level of the right pulmonary artery (white arrowhead) and a hypoattenuating adjacent area (black arrowheads). Unlike acute pulmonary embolism, chronic pulmonary embolism may be indicated by hypoattenuating clots at unenhanced CT. (b) Contrast-enhanced CT scan shows a peripheral clot in the corresponding hypoattenuating area (arrowhead).
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Figure 25a. Chronic pulmonary artery obstruction in a 68-year-old woman with dyspnea. (a) Contrast-enhanced CT scan at the level of the left hilum shows retracted embolic material (arrow); a marked reduction in the diameter of the left lower lobe pulmonary artery; and contrast material in the central lumen, a finding suggestive of recanalization of the artery. (b) Contrast-enhanced CT scan at a level slightly higher than a shows enlargement of the bronchial arteries (arrowhead) because of a bronchial system–to–pulmonary system shunt. (c) Unenhanced CT scan obtained with a lung window setting at the level of the lower lobes shows marked stenosis of the left lower lobe arteries and dilatation of the accompanying bronchi (arrow); multiple linear opacities (arrowheads) adjacent to the pleural surface in the left lower lobe, suggestive of transpleural systemic vessels; marked dilatation of the vessels in the right lung; and a thrombus (*) in the right lower lobe artery.
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Figure 25b. Chronic pulmonary artery obstruction in a 68-year-old woman with dyspnea. (a) Contrast-enhanced CT scan at the level of the left hilum shows retracted embolic material (arrow); a marked reduction in the diameter of the left lower lobe pulmonary artery; and contrast material in the central lumen, a finding suggestive of recanalization of the artery. (b) Contrast-enhanced CT scan at a level slightly higher than a shows enlargement of the bronchial arteries (arrowhead) because of a bronchial system–to–pulmonary system shunt. (c) Unenhanced CT scan obtained with a lung window setting at the level of the lower lobes shows marked stenosis of the left lower lobe arteries and dilatation of the accompanying bronchi (arrow); multiple linear opacities (arrowheads) adjacent to the pleural surface in the left lower lobe, suggestive of transpleural systemic vessels; marked dilatation of the vessels in the right lung; and a thrombus (*) in the right lower lobe artery.
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Figure 25c. Chronic pulmonary artery obstruction in a 68-year-old woman with dyspnea. (a) Contrast-enhanced CT scan at the level of the left hilum shows retracted embolic material (arrow); a marked reduction in the diameter of the left lower lobe pulmonary artery; and contrast material in the central lumen, a finding suggestive of recanalization of the artery. (b) Contrast-enhanced CT scan at a level slightly higher than a shows enlargement of the bronchial arteries (arrowhead) because of a bronchial system–to–pulmonary system shunt. (c) Unenhanced CT scan obtained with a lung window setting at the level of the lower lobes shows marked stenosis of the left lower lobe arteries and dilatation of the accompanying bronchi (arrow); multiple linear opacities (arrowheads) adjacent to the pleural surface in the left lower lobe, suggestive of transpleural systemic vessels; marked dilatation of the vessels in the right lung; and a thrombus (*) in the right lower lobe artery.
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Figure 26. Pulmonary artery sarcoma in a 70-year-old-man with dyspnea. Contrast-enhanced CT scan shows filling defects in the main, left, and right pulmonary arteries and the right interlobar pulmonary artery. The arterial lumina are expanded, and there is extravascular invasion.
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Copyright © 2006 by the Radiological Society of North America.
