Findings: The chest radiograph demonstrates a normal sized heart and normal left hemithorax, including pulmonary vasculature. The angiogram confirms a small right pulmonary artery. The right main pulmonary artery is small and the right lung is hyperlucent due to a diffuse decrease in pulmonary vascularity.
A. Osler-Weber-Rendu Syndrome, also known as hereditary hemorrhagic telangiectasia, is an autosomal dominant disorder resulting in diffuse vascular dysplasia. The dysplasia is manifested as arteriovenous malformations (small ones are called telangiectasias), and aneurysms. Pulmonary arteriovenous malformations occur in 5-15% of patients. This disease does not result in diffuse hypoplasia of a vascular bed.
B. Swyer James Syndrome is the result of acute viral bronchiolitis in infancy or early childhood that prevents further normal lung development. Symptoms include cough, dyspnea on exertion, hemoptysis, and recurrent lower respiratory infections. Radiographic abnormalities may be present even in asymptomatic individuals. Pathologic findings include a small hemithorax, diffuse bronchiectasis, emphysematous changes in the lung parenchyma, and a diffusely small pulmonary arterial tree. The findings usually involve one lung, but may be restricted to a single lobe, or involve both lungs. Ventilation perfusion scanning demonstrates air trapping and diffusely decreased perfusion in the affected region. Bronchiectasis is evident on high resolution CT. The pulmonary arterial tree is intrinsically normal but is small because it subserves a decreased amount of lung parenchyma.
C. Chronic pulmonary embolism can cause the pulmonary arteries to be small and the arterial tree to have a pruned appearance. Chronic PE, however, does not affect a single lung; abnormalities will be evident in both lungs. In addition, the arteries are intrinsically abnormal; findings include abrupt occlusion of vessels, irregularity of the vessel wall, and intravascular webs. These abnormalities are the result of clot retraction and fibrosis. Finally, in chronic PE, the central pulmonary arteries can be quite large due to the pulmonary arterial outflow obstruction.
D. Isolated valvular pulmonic stenosis is one of the most common congenital heart diseases diagnosed in adulthood. It may require no treatment or may lead to clinically significant right ventricular outflow obstruction. The most common presenting symptoms are due to exercise intolerance, and may include fatigue, dyspnea, chest pain and syncope. Pathologic findings resulting from the narrowed valve orifice include right ventricular hypertrophy and post stenotic dilation of the main pulmonary artery. Because of the direction of the jet effect, the left pulmonary artery may also be dilated. The right pulmonary artery and peripheral arterial tree are usually normal. In this case, there is no evidence of right ventricular hypertrophy or enlargement of the main pulmonary artery on the chest radiograph. In addition, the right pulmonary arterial tree is clearly hypoplastic.
E. Takayasu arteritis is a granulomatous inflammation affecting the aorta, its major branches, and the pulmonary arteries. It is of unknown etiology and usually presents in patients younger than age 50. Radiographic findings include systemic arterial stenosis and occlusions, although regions of vascular dilation can occur. The pulmonary arteries may be involved. Pulmonary arterial abnormalities include multiple segmental stenoses and occlusions that mimic chronic pulmonary embolism. Because of the diffuse nature of the disease, both lungs are typically. Due to outflow obstruction, the central pulmonary arteries may be enlarged. As is seen in the systemic circulation, affected pulmonary arteries are thick-walled on cross-sectional imaging.