Diseases of the Hepatopulmonary Axis1
Cris A. Meyer, MD,
Charles S. White, MD and
Kenneth E. Sherman, MD, PhD
1 From the Departments of Radiology (C.A.M.) and Medicine (K.E.S.), University of Cincinnati, 234 Goodman St, ML 0742, Cincinnati, OH 45219-2316; and the Department of Radiology, University of Maryland Medical Center, Baltimore (C.S.W.). Received May 18, 1999; revision requested July 26 and received August 31; accepted September 7. Address reprint requests to C.A.M. (e-mail: meyerca@healthall.com).
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Figure 1. Intrapulmonary arteriovenous shunt in a 30-year-old man with cirrhosis and dyspnea. Posterior whole-body image obtained after injection of Tc-99m macroaggregated albumin into the venous circulation shows activity in the brain. The presence of radiotracer on the left side of the circulation confirms that there is a right-to-left shunt bypassing the normal filtering mechanism of the pulmonary capillary beds.
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Figure 2. Hepatopulmonary syndrome diagnosed with contrast (microbubble) echocardiography in a 48-year-old man with end-stage alcoholic liver disease and hypoxia. Chest CT scan (lung window) shows dilated peripheral pulmonary arterioles (arrow). Note the gynecomastia, which is another manifestation of chronic liver disease.
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Figure 3a. Right-sided hepatic hydrothorax in a 55-year-old woman with primary biliary cirrhosis. Posteroanterior (a) and lateral (b) chest radiographs show blunting of the right costophrenic angle, a finding compatible with effusion.
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Figure 3b. Right-sided hepatic hydrothorax in a 55-year-old woman with primary biliary cirrhosis. Posteroanterior (a) and lateral (b) chest radiographs show blunting of the right costophrenic angle, a finding compatible with effusion.
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Figure 4a. Plexogenic pulmonary hypertension in a 38-year-old woman with primary biliary cirrhosis and end-stage liver disease. (a) Posteroanterior chest radiograph shows enlarged central pulmonary arteries. (b) Coronal T1-weighted fast spoiled gradient-echo magnetic resonance (MR) image shows a cirrhotic liver with splenomegaly, periesophageal varices (arrow), and an enlarged right pulmonary artery (arrowheads).
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Figure 4b. Plexogenic pulmonary hypertension in a 38-year-old woman with primary biliary cirrhosis and end-stage liver disease. (a) Posteroanterior chest radiograph shows enlarged central pulmonary arteries. (b) Coronal T1-weighted fast spoiled gradient-echo magnetic resonance (MR) image shows a cirrhotic liver with splenomegaly, periesophageal varices (arrow), and an enlarged right pulmonary artery (arrowheads).
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Figure 5. Pulmonary edema in a 32-year-old man with primary sclerosing cholangitis who developed fulminant hepatic failure secondary to hepatitis B. Anteroposterior chest radiograph shows diffuse noncardiogenic pulmonary edema. The patient underwent intubation for respiratory failure. After clearing of the hepatitis B infection, the patient underwent liver transplantation and recovered uneventfully, with resolution of the radiographic findings.
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Figure 6a. Esophageal varices in a 57-year-old man with cirrhosis and severe portal hypertension. (a) Posteroanterior chest radiograph shows widening of the right paraspinal interface. (b) Contrast material-enhanced chest CT scan shows multiple enhancing periesophageal tubular structures compatible with varices. (Courtesy of H. Page McAdams, MD, Duke University Medical Center, Durham, NC.)
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Figure 6b. Esophageal varices in a 57-year-old man with cirrhosis and severe portal hypertension. (a) Posteroanterior chest radiograph shows widening of the right paraspinal interface. (b) Contrast material-enhanced chest CT scan shows multiple enhancing periesophageal tubular structures compatible with varices. (Courtesy of H. Page McAdams, MD, Duke University Medical Center, Durham, NC.)
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Figure 7a. Enlarged pulmonary veins in a 36-year-old woman with protein C deficiency who underwent splenopneumopexy for portal hypertension. (a) Posteroanterior chest radiograph shows enlarged pulmonary vessels in the left lower lobe (arrow). (b) CT scan (lung window) obtained just above the diaphragm shows multiple tortuous vascular structures in the left lower lobe, which are engorged pulmonary veins due to the surgically created portosystemic shunt.
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Figure 7b. Enlarged pulmonary veins in a 36-year-old woman with protein C deficiency who underwent splenopneumopexy for portal hypertension. (a) Posteroanterior chest radiograph shows enlarged pulmonary vessels in the left lower lobe (arrow). (b) CT scan (lung window) obtained just above the diaphragm shows multiple tortuous vascular structures in the left lower lobe, which are engorged pulmonary veins due to the surgically created portosystemic shunt.
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Figure 8a. Emphysema and bronchiectasis in a 52-year-old woman with  1-antitrypsin deficiency. (a) Posteroanterior chest radiograph shows findings consistent with diffuse emphysema. (b) High-resolution CT scan (lung window) shows basilar predominant panlobular emphysema and fusiform bronchiectasis (arrow).
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Figure 8b. Emphysema and bronchiectasis in a 52-year-old woman with 1-antitrypsin deficiency. (a) Posteroanterior chest radiograph shows findings consistent with diffuse emphysema. (b) High-resolution CT scan (lung window) shows basilar predominant panlobular emphysema and fusiform bronchiectasis (arrow).
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Figure 9a. Bronchiectasis and biliary cirrhosis in a 17-year-old boy with cystic fibrosis. (a) Posteroanterior chest radiograph shows irregular tubular and nodular markings, findings consistent with bronchiectasis and enlarged pulmonary arteries. (b) Contrast-enhanced abdominal CT scan shows a nodular contour of the liver with adjacent gastric varices (arrow), findings consistent with multilobular biliary cirrhosis. (c) Contrast-enhanced abdominal CT scan shows small-bowel fold thickening (arrowhead) and fatty atrophy of the pancreas (arrow).
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Figure 9b. Bronchiectasis and biliary cirrhosis in a 17-year-old boy with cystic fibrosis. (a) Posteroanterior chest radiograph shows irregular tubular and nodular markings, findings consistent with bronchiectasis and enlarged pulmonary arteries. (b) Contrast-enhanced abdominal CT scan shows a nodular contour of the liver with adjacent gastric varices (arrow), findings consistent with multilobular biliary cirrhosis. (c) Contrast-enhanced abdominal CT scan shows small-bowel fold thickening (arrowhead) and fatty atrophy of the pancreas (arrow).
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Figure 9c. Bronchiectasis and biliary cirrhosis in a 17-year-old boy with cystic fibrosis. (a) Posteroanterior chest radiograph shows irregular tubular and nodular markings, findings consistent with bronchiectasis and enlarged pulmonary arteries. (b) Contrast-enhanced abdominal CT scan shows a nodular contour of the liver with adjacent gastric varices (arrow), findings consistent with multilobular biliary cirrhosis. (c) Contrast-enhanced abdominal CT scan shows small-bowel fold thickening (arrowhead) and fatty atrophy of the pancreas (arrow).
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Figure 10a. Pulmonary arteriovenous malformation in a 33-year-old woman with orthodeoxia. (a) Posteroanterior chest radiograph shows bibasilar well-defined nodules (arrows). (b) CT scan (lung window) shows the feeding artery and large draining vein of a pulmonary arteriovenous malformation.
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Figure 10b. Pulmonary arteriovenous malformation in a 33-year-old woman with orthodeoxia. (a) Posteroanterior chest radiograph shows bibasilar well-defined nodules (arrows). (b) CT scan (lung window) shows the feeding artery and large draining vein of a pulmonary arteriovenous malformation.
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Figure 11. Hepatic telangiectases in a 79-year-old patient with hereditary hemorrhagic telangiectasia. Contrast-enhanced abdominal CT scan shows multiple intrahepatic telangiectases.
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Figure 12a. Nodular organ involvement in a 38-year-old woman with sarcoidosis. (a) CT scan (lung window) obtained at the level of the right upper lobe bronchus shows multiple pulmonary nodules and peribronchovascular nodular thickening. (b) Contrast-enhanced CT scan of the upper abdomen shows multiple hypoattenuating nodules in the liver and spleen secondary to sarcoidosis.
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Figure 12b. Nodular organ involvement in a 38-year-old woman with sarcoidosis. (a) CT scan (lung window) obtained at the level of the right upper lobe bronchus shows multiple pulmonary nodules and peribronchovascular nodular thickening. (b) Contrast-enhanced CT scan of the upper abdomen shows multiple hypoattenuating nodules in the liver and spleen secondary to sarcoidosis.
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Figure 13a. Drug reaction in a 68-year-old woman receiving amiodarone for dilated cardiomyopathy and severe arrhythmias. (a) Anteroposterior chest radiograph shows multifocal peripheral consolidations of high opacity. (b) Chest CT scan (mediastinal window) shows a hyperattenuating peripheral consolidation in the right upper lobe. (c) Nonenhanced abdominal CT scan shows high attenuation of the liver (78 HU) relative to that of the spleen (44 HU).
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Figure 13b. Drug reaction in a 68-year-old woman receiving amiodarone for dilated cardiomyopathy and severe arrhythmias. (a) Anteroposterior chest radiograph shows multifocal peripheral consolidations of high opacity. (b) Chest CT scan (mediastinal window) shows a hyperattenuating peripheral consolidation in the right upper lobe. (c) Nonenhanced abdominal CT scan shows high attenuation of the liver (78 HU) relative to that of the spleen (44 HU).
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Figure 13c. Drug reaction in a 68-year-old woman receiving amiodarone for dilated cardiomyopathy and severe arrhythmias. (a) Anteroposterior chest radiograph shows multifocal peripheral consolidations of high opacity. (b) Chest CT scan (mediastinal window) shows a hyperattenuating peripheral consolidation in the right upper lobe. (c) Nonenhanced abdominal CT scan shows high attenuation of the liver (78 HU) relative to that of the spleen (44 HU).
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Copyright © 2000 by the Radiological Society of North America.
