Cardiol Clin 22 (2004) 375382

Radiology of pulmonary vascular disease

David L. Levin, MD, PhD*, Eric T. Goodman, MD
Department of Radiology, University of California, San Diego Medical Center, 200 West Arbor Drive, San Diego, CA 92103, USA

The evaluation of suspected pulmonary vascular disease remains a dicult task. The clinical work-up may include a variety of radiologic studies, and the clinician is presented with multiple options. It is important that the ordering physician be familiar with the indications and the specic advantages and disadvantages of each test. This article discusses the various modalities available to the clinician for the evaluation of suspected pulmonary vascular disease and the evaluation of several specic vascular diseases.

Modalities available for evaluation of pulmonary vascular disease A variety of radiographic methods are available for the evaluation of suspected pulmonary vascular disease. These methods include conventional radiographs and angiography, computed tomography (CT), nuclear medicine studies, and magnetic resonance imaging (MRI). Conventional radiographs Conventional radiographs are typically the initial studies obtained in the evaluation of suspected pulmonary disease. Although they have the advantage of being relatively inexpensive and readily available, they are comparatively insensitive and nonspecic, especially in the evaluation of pulmonary vascular disease. Conventional angiography Conventional angiography provides direct visualization of the pulmonary vasculature. It
* Corresponding author. E-mail address: dlevin@ucsd.edu (D.L. Levin).

requires the placement of a catheter into the vessel of interest. For the evaluation of pulmonary vascular disease, a catheter is typically advanced into the pulmonary artery through the systemic venous system. A large volume of contrast is injected at a high rate to opacify the pulmonary arterial system completely. A routine injection for a pulmonary arteriogram may require 40 mL of iodinated contrast material given at a rate of 20 mL/second. This rate is typically reduced in the presence of pulmonary hypertension. Although the technique is still used in the evaluation of suspected acute pulmonary embolism, it has been largely replaced by CT angiography. The continued important role of angiography in the evaluation of patients with suspected chronic pulmonary embolism (chronic thromboembolic pulmonary hypertension [CTEPH]) is discussed in detail in the article by Guillinta et al in this issue. Computed tomography CT is an extremely versatile technique that provides excellent evaluation of the pulmonary vasculature, mediastinal structures, and the lung parenchyma. CT uses an X-ray source that revolves around the patient, acquiring data from multiple angles. Conventional CT (third- and fourth-generation scanners) alternated the movement of the X-ray source and the movement of the patient. This process required relatively long scan times, and several consecutive breathholds were needed to image the chest completely. The technology has rapidly evolved with the development of helical and multidetector helical CT. With this technology, simultaneous movement of the X-ray source and the patient allows more rapid imaging with improved spatial resolution. The entire chest can now be imaged easily at high spatial resolution

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during a single breathhold, and after the intravenous injection of contrast material imaging can be timed to the peak enhancement of the pulmonary vessels. As a result, CT has become a primary modality for the evaluation of the pulmonary vascular disease. Nuclear medicine Scintigraphic techniques are also commonly used to evaluate pulmonary blood ow. The most frequent method uses the intravenous injection of microaggregated albumin that has been labeled with technetium-99m. These particles lodge within the precapillary arterioles. Their distribution, measured with a gamma camera, is directly proportional to blood ow. The images obtained are most frequently interpreted in a qualitative fashion, but quantication of pulmonary blood ow is possible. Scintigraphic ventilation/perfusion (V/Q) scans were the rst specic test ordered in the evaluation of suspected pulmonary embolism before the use of CT angiography. Magnetic resonance imaging MRI has limited utility in the evaluation of pulmonary disease, but various MR techniques, such as MR angiography, perfusion imaging, and ventilation imaging have been used in select centers to evaluate the pulmonary vasculature and cardiac structures (Fig. 1). Stein and colleagues have recently reviewed the use of MR angiography in the detection of pulmonary embolism [1]. Evaluation of specic pulmonary vascular diseases Pulmonary venous hypertension Pulmonary venous hypertension (PVH) is related to increased resistance to ow within the pulmonary veins. The more common causes are left ventricular failure and mitral valve disease. Radiologically, PVH can be divided into three grades of severity. Grade I PVH is characterized by redistribution of pulmonary blood ow and pulmonary vascular dilation. The latter nding, however, is far more common with chronic PVH [2]. Normally, there is preferential blood ow to the lower lobes because of gravitational eects. Pulmonary vascular dilation is identied primarily by vascular redistribution, with the upper lobe vessels becoming equal (balanced ow) or greater (inverted ow) in caliber compared with the lower lobe vessels [3]. Vascular redistribution should be

Fig. 1. (A) Coronal maximum intensity projection image from an MR angiogram. The arrow identies a region of absent arterial ow within the right lung. (B) An embolus within the right interlobar pulmonary artery is identied on source images (arrow).

judged only with the patient in the fully upright position [2]. Grade II PVH is characterized by evidence of interstitial edema, with or without accompanying pleural eusions (Fig. 2). Features of interstitial edema on conventional radiographs include Kerley B lines. Kerley B lines are horizontally oriented, thin, linear densities that are typically around 1 cm in length and are usually best identied at the lung periphery extending to the pleural surface. With pulmonary edema, Kerley B lines represent uid within the interlobular septa. The dierential diagnosis for Kerley B lines, however, includes lymphangitic carcinomatosis, lymphatic obstruction, and pulmonary vein occlusion. Other evidence of interstitial edema includes indistinctness of the vessel margins, subpleural thickening along the ssures, and peribronchial cung. Loss of vascular denition is subjective and is more easily

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Fig. 2. Frontal chest radiograph obtained from a patient with an acute pulmonary embolism. The left pulmonary artery is enlarged (small arrow), and a wedge-shaped peripheral opacity is present at the left costophrenic angle (large arrow).

nary hemorrhage, and infection, especially with atypical organisms (eg, pneumocystis carinii pneumonia, cytomegalovirus). Several investigators have correlated the radiographic grading of PVH and pulmonary artery wedge pressures [4,5]. Grade I PVH correlates to a wedge pressure of 12 to 19 mm Hg in acute PVH and 15 to 25 mm Hg in the setting of chronic mitral valvular disease. Grade II PVH correlates with a wedge pressure of 20 to 25 mm Hg in acute PVH or 25 to 30 mm Hg with chronic failure. Grade III correlates to a wedge pressure greater than 25 mm Hg in acute PVH or greater than 30 mm Hg in chronic PVH. This correlation is rough, at best, and is of limited practical value. This correlation is also aected by a time lag, because the radiographs primarily identify intravascularto-interstitial uid shifts within the lung and concomitant disease. Pulmonary embolism The evaluation of suspected pulmonary embolism remains a signicant clinical challenge. Both the symptoms and clinical signs are nonspecic and fail to distinguish between patients with and without pulmonary embolism. The yearly incidence of pulmonary embolism is estimated to be between 300,000 and 500,000. Although the overall mortality from pulmonary embolism has decreased during the past 30 years, the case fatality rate for untreated pulmonary embolism may be as high as 15% at 3 months [6]. Chest radiography The major value of conventional radiographs is to exclude alternate diagnoses, such as pneumothorax, pneumonia, and pulmonary edema. An abnormal radiograph is seen in 84% patients with pulmonary embolism and in 66% of patients without pulmonary embolism [7]. The most frequent ndings, however, are nonspecic and include atelectasis (seen in 68% of patients with pulmonary embolism), pleural eusion (in 48%), and focal parenchymal opacities (in 35%). These ndings are also common in patients who do not have pulmonary embolism. Several classic plainlm signs have been described. These include Hamptons hump, a peripheral wedge-shaped opacity; Westermarks sign, focal oligemia within the lung distal to the embolus; and Fleischners sign, unilateral enlargement of the central pulmonary artery (Fig. 4). None of these signs reliably

identied with serial radiographs. Some authors consider subpleural thickening of the interlobar ssures to be the most reliable nding of early interstitial edema [3]. Peribronchial thickening is best appreciated by identifying ring shadows of bronchi traveling end-on. Grade III PVH is characterized by the ooding of edema uid into the alveoli. Radiographically, alveolar edema is characterized by diuse opacication of the lungs with obscuration of the pulmonary vessels, often with air-bronchograms (Fig. 3). This nding is most pronounced in the perihilar regions and is frequently described as having a batwing appearance. The dierential diagnosis of diuse alveolar lling includes both cardiogenic and noncardiogenic edema, pulmo-

Fig. 3. Single image from a conventional angiogram. Intravascular contrast is present streaming around a lling defect caused by a pulmonary embolism (arrow).

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Fig. 4. Axial image from CT angiogram. A saddle embolus (arrow) extends into both the left and right main pulmonary arteries.

identies patients with acute pulmonary embolism, however. Nuclear medicine Until recently, ventilation/perfusion (V/Q) scanning was the mainstay in the evaluation of suspected pulmonary embolism, but V/Q scans do not provide a conclusive diagnosis in up to 80% of patients [8]. In patients with a high clinical pretest probability, a high-probability V/Q scan has a 96% positive predictive value. A lowprobability V/Q scan with a low clinical suspicion has a negative predictive value of 94%. Unfortunately, only 25% of all studies fall into one of these two categories. Data from the Prospective Investigation of Pulmonary Embolism Diagnosis study found that 15% of all patients with pulmonary embolism had a high-probability V/Q scan, 40% had an intermediate scan, 30% a had lowprobability scan, and 15% a had normal or nearly normal scan [9]. V/Q scans remain an integral part of the evaluation of patients with suspected CTEPH, as discussed in the article by Auger et al in this issue. Conventional angiography Conventional angiography has generally been considered the criterion standard, but it is infrequently ordered for the evaluation of suspected pulmonary embolism [10]. The study is associated with a low overall morbidity (2%5%) and mortality (<1%). The ndings of acute pulmonary embolism include an intraluminal lling defect and the abrupt termination of the contrast within a vessel (Fig. 5). Although conventional

Fig. 5. (A) Frontal chest radiograph obtained from a patient with grade II pulmonary edema. The pulmonary vessels are poorly dened. The cardiac silhouette is enlarged. (B) Close-up demonstrates several Kerley B lines (arrowheads).

angiography is frequently thought to provide a denitive answer, interobserver disagreement can be signicant, especially for subsegmental emboli [11]. Additionally, data from animal studies suggest that conventional angiography and CT angiography have similar sensitivity and specicity for the detection of pulmonary embolism when compared with an independent, anatomic standard. Computed tomography CT angiography is rapidly becoming the test of choice in many institutions for the evaluation of suspected pulmonary embolism. Images are obtained using a volumetric scanning protocol. As with conventional angiography, the two primary

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ndings of pulmonary embolism with CT are the identication of an intraluminal thrombus and the abrupt termination of contrast within a vessel (Fig. 6). As with conventional radiographs, ancillary ndings may be identied using CT. Peripheral wedge-shaped opacities and linear bands are both frequently seen in association with pulmonary embolism. When pulmonary embolism is not identied, CT has been shown to nd an alternate diagnosis in more than 50% of cases [12]. The sensitivity and specicity for the detection of a pulmonary embolism vary substantially between studies. Remy-Jardin et al [13] reported a sensitivity of 91% with a specicity of 78%, whereas Drucker et al [14] reported a sensitivity of 53% with a specicity of 81%. In a review of 11 published studies, Harvey [15] found an overall sensitivity for the detection of segmental or larger emboli of 74% to 81% with a specicity of 89% to 91%. An alternate method to evaluate the performance of CT in the evaluation of pulmonary embolism would be to determine the outcome in patients with a negative study. Goodman et al [16] prospectively followed patients with suspected pulmonary embolism. Of 198 patients with negative CT angiography, 2 had a documented pulmonary embolism within a 3-month period. This rate was a slightly greater percentage than for a normal V/Q scan but less than that seen with a low-probability study. Ryu et al [17] observed similar ndings in a 3-month retrospective follow

up of 951 patients with a negative CT angiogram. In their study, a 1% incidence of nonfatal pulmonary embolism and a 0.2% incidence of fatal pulmonary embolism were found. Pulmonary arterial hypertension Pulmonary arterial hypertension (PAH) and other causes of pulmonary hypertension, such as pulmonary hypertension caused by venous hypertension from left-heart failure, have both overlapping and unique attributes that may assist in the dierential diagnosis. Chest radiography The primary radiographic nding of PAH is enlargement of the central pulmonary arteries (Fig. 7). Most frequently, this determination is made on the basis of a subjective impression. On the frontal radiograph, the diameter of the right interlobar pulmonary artery can be measured where it travels just laterally to the bronchus intermedius. At this level, a diameter greater than 16 mm (15 mm in women) suggests PAH [18]. The peripheral pulmonary vessels are most frequently decreased in caliber with rapid tapering of the vessel distal to the proximal artery. The peripheral vessels, however, may be dilated with congenital left-to-right shunts. In the setting of left ventricular failure leading to pulmonary hypertension, enlargement of the cardiac silhouette and features of chronic pulmonary edema are typically present. Computed tomography As with conventional radiographs, the primary CT nding of PAH is enlargement of the central pulmonary arteries. A diameter of the main pulmonary artery greater than 29 mm suggests but is not diagnostic of PAH [19]. In the setting of CTEPH, thrombus may be within the pulmonary arteries. An additional nding of CTEPH is mosaic attenuation within the lung parenchyma, reecting heterogeneous pulmonary perfusion [20]. Pulmonary veno-occlusive disease Pulmonary veno-occlusive disease is a rare disease leading to PAH as a result of destruction of pulmonary veins. Clinically, the disease is characterized by evidence of both PAH and pulmonary edema. Pulmonary veno-occlusive disease has been reported in associated with a number of other diseases but may occur as an idiopathic process [21,22].

Fig. 6. Frontal chest radiograph obtained from a patient with grade III pulmonary edema. Diuse air-space opacities are present bilaterally with complete obscuration of vascular margins.

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Fig. 7. (A) Frontal chest radiograph obtained from a patient with primary pulmonary hypertension. There is enlargement of the main pulmonary artery (arrowheads). (B) Close-up demonstrates rapid tapering of a left upper lobe pulmonary artery (arrow).

Fig. 8. (A) Frontal chest radiograph obtained from a patient with pulmonary veno-occlusive disease. Signs of pulmonary arterial hypertension are present along with features of pulmonary edema. There is enlargement of the central pulmonary arteries and the pulmonary vessels have indistinct margins. (B) Close-up demonstrates faint Kerley B lines (arrowheads).

Radiographically, the key features of pulmonary veno-occlusive disease are those of PAH and pulmonary edema, which is present in approximately 75% of patients [22]. The left atrium is not enlarged as it is with chronic congestive failure or mitral stenosis progressing to PAH (Fig. 8). Pulmonary arteriovenous malformation Pulmonary arteriovenous malformations (AVMs) can range in size from microscopic to

several centimeters in diameter. Radiographically, pulmonary AVMs are typically round or oval in shape and are sharply dened. The key to the diagnosis is the identication of the feeding artery and draining vein. The draining vein is more readily identied because it is typically half the diameter of the AVM. Pulmonary AVMs are more common in the lower lobes and are multiple in roughly one third of cases. Helical CT is the study of choice in the evaluation of suspected pulmonary AVM and is readily able to charac-

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Fig. 9. Coronal reconstruction from helical CT. An arteriovenous malformation is present at the left lung base (arrow). Two large draining veins are present.

terize the feeding artery and draining vein (Fig. 9). This identication is important in planning therapy, especially when embolization is considered. Because the key CT features are morphologic, contrast administration is necessary. Conventional pulmonary angiography is routinely performed before therapy. Summary Several radiologic methods are available for the evaluation of suspected pulmonary vascular disease. Of these, the conventional chest radiograph and CT are the most useful. Although conventional radiographs suer from poor sensitivity and specicity, they are readily available and relatively inexpensive. They can exclude other signicant diseases and may suggest a specic vascular process. CT is the primary modality for evaluation of pulmonary vascular disease. It provides an excellent evaluation of the lung parenchyma, mediastinum, and pulmonary vasculature, and helical CT has largely replaced conventional angiography except for selective indications such as CTEPH. References
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