(Radiographics. 1999;19:965-972.)
© RSNA, 1999
Pulmonary Parenchymal Manifestations of Mitral Valve Disease1
Kevin Woolley, MD and
Paul Stark, MD
1 From the Department of Radiology, VA Palo Alto Health Care System and Stanford University School of Medicine, 3801 Miranda Ave, Palo Alto, CA 94304. Received May 26, 1998; revision requested July 20 and received September 1; accepted September 1. Address reprint requests to P.S.
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Abstract
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Pulmonary parenchymal manifestations of mitral valve disease are the result of either pulmonary venous hypertension in mitral stenosis or abnormal regurgitant flow into pulmonary veins in mitral insufficiency. Typical radiographic findings in mitral stenosis include pulmonary vascular cephalization; interstitial, perivascular, and occasionally alveolar pulmonary edema; diffuse alveolar hemorrhage; hemosiderosis; and pulmonary ossification. Signs of interstitial pulmonary edema are frequently visible and include septal lines. Radiographic findings in diffuse alveolar hemorrhage consist of diffuse, confluent acinar or ground-glass areas of increased opacity, often sparing the peripheral parenchyma and creating the so-called window frame effect. Hemosiderosis is characterized by small, ill-defined nodules or by coarse reticular areas of increased opacity with a bias for the middle and lower lung regions. Ossification manifests as densely calcified, 1–5-mm nodules, mainly in the middle and lower lungs, with a tendency for confluence and the occasional presence of trabeculae. Imaging findings in mitral regurgitation depend on the acuteness of the disease. The most common parenchymal manifestations of acute mitral regurgitation are symmetric alveolar and interstitial pulmonary edema with indistinct, engorged pulmonary vessels and cephalized blood flow. Familiarity with these manifestations can expedite diagnosis, particularly in rare cases of unsuspected mitral valve disease.
Index Terms: Hypertension, pulmonary, 534.784, 60.7121 • Lung, calcification, 60.815 • Lung, fluid, 60.7132 • Lung, hemorrhage, 60.4123, 60.7133 • Mitral valve, regurgitation, 534.841 • Mitral valve, stenosis, 534.831
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INTRODUCTION
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The cardiac radiographic features of mitral valve disease are well known. The hallmark of mitral valve disease is pulmonary venous hypertension, which frequently leads to clinical findings of left ventricular failure. Chest radiography is an important part of the evaluation of patients with this disease. Medical and surgical treatment of mitral valve disease is particularly successful early in the course of the disease; therefore, detection at initial presentation is of considerable importance. Characteristic changes in cardiac contour, particularly in long-standing mitral stenosis, are helpful in confirming the radiographic diagnosis in affected patients. The pulmonary parenchymal manifestations of mitral valve disease are less well known and relate to pulmonary vascular engorgement. These findings are frequently nonspecific but can have a characteristic appearance that facilitates recognition of unsuspected cases of mitral valve disease.
In this article, we discuss and illustrate the imaging appearances of a variety of pulmonary parenchymal manifestations of mitral valve disease including pulmonary vascular cephalization, pulmonary edema, diffuse alveolar hemorrhage, hemosiderosis, and ossification.
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MITRAL STENOSIS
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Mitral stenosis typically results from rheumatic heart disease, although it may occasionally be congenital. Rheumatic heart disease affects women more than men, is more prevalent in developing nations, and typically manifests as mitral stenosis 5–15 years after the initial episode of rheumatic fever. Rheumatic heart disease has become a rare entity in North America. Frequently, patients have no recollection of ever having had rheumatic fever. They remain asymptomatic until abrupt onset of atrial fibrillation (due to an enlarged left atrium) or of symptoms of pulmonary venous hypertension (eg, dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea). The increased left ventricular filling pressure in mitral stenosis leads to compensatory dilation of the left atrium, pulmonary venous hypertension, and, eventually, postcapillary pulmonary arterial hypertension, which may in turn lead to right ventricular failure. When left ventricular filling is compromised, cardiac output is diminished. Typical radiographic findings in mitral stenosis include left atrial enlargement (double contour sign, enlarged left atrial appendage, splaying of the mainstem bronchi); a normal- or undersized left ventricle; pulmonary vascular cephalization; and interstitial, perivascular, and occasionally alveolar pulmonary edema (1). Echocardiography is used to determine valve area and pressure gradients (2). In approximately 0.6% of cases of mitral stenosis, a coexisting atrial septal defect may relieve the left atrial hypertension and promote the formation of a left to right shunt. This combination of findings is known as Lutembacher syndrome.
Pulmonary Edema
Nonspecific features, particularly those related to left ventricular failure, are the most common lung parenchymal manifestations of mitral stenosis. Signs of interstitial pulmonary edema including septal lines (Fig 1) are frequently visible. Patients with mitral stenosis are prone to develop acute pulmonary edema after volume overload (eg, with intraoperative infusion of liquids or during pregnancy and delivery) (Figs 2, 3). In chronic mitral stenosis, septal lines may represent interstitial fibrosis and deposition of hemosiderin-laden macrophages ("brown induration") as well as edema (1).
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Figure 1. Interstitial pulmonary edema in a 45-year-old man with paroxysmal nocturnal dyspnea. Frontal chest radiograph shows interstitial pulmonary edema with a normal-sized cardiac silhouette, double contour overlying the right atrial shadow (arrowhead), effacement of the cardiac waist, enlarged central pulmonary arteries, and distended upper lobe pulmonary vessels. Note the clearly visible thickened interlobar septa forming Kerley A lines (arrow).
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Figure 2. Acute pulmonary edema in a 37-year-old woman with previously undiagnosed rheumatic mitral stenosis who experienced acute onset of dyspnea and pulmonary edema in the recovery room following a gynecologic procedure. Frontal chest radiograph shows multiple confluent acinar shadows due to hydrostatic pulmonary edema resulting from intraoperative volume overload. The cardiac silhouette shows an enlarged, convex main pulmonary artery segment and a convex left atrial appendage (arrow).
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Figure 3a. Acute pulmonary edema in a 28-year-old woman with previously undiagnosed combined mitral stenosis and regurgitation who had just given birth to a full-term neonate. (a) Frontal chest radiograph shows massive pulmonary edema with a bias for the perihilar and lower lung regions. The cardiac silhouette is moderately enlarged. Dilation of the main and central pulmonary arteries and cephalization of pulmonary vascular flow are evident. Note the splaying of the angle of tracheal bifurcation, a finding that is indicative of marked left atrial enlargement (arrow). (b) Lateral radiograph helps confirm the enlarged left atrium, which is seen displacing the left main bronchus dorsally (arrow).
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Figure 3b. Acute pulmonary edema in a 28-year-old woman with previously undiagnosed combined mitral stenosis and regurgitation who had just given birth to a full-term neonate. (a) Frontal chest radiograph shows massive pulmonary edema with a bias for the perihilar and lower lung regions. The cardiac silhouette is moderately enlarged. Dilation of the main and central pulmonary arteries and cephalization of pulmonary vascular flow are evident. Note the splaying of the angle of tracheal bifurcation, a finding that is indicative of marked left atrial enlargement (arrow). (b) Lateral radiograph helps confirm the enlarged left atrium, which is seen displacing the left main bronchus dorsally (arrow).
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Diffuse Alveolar Hemorrhage
Patients with mitral stenosis may present with hemoptysis and diffuse alveolar hemorrhage. Hemorrhage early in the course of the disease may be related to rupture of the microvasculature. As postcapillary pulmonary arterial hypertension develops, intimal hyperplasia may protect the microvasculature. Hemorrhage at this stage may relate to abnormally engorged submucosal bronchial veins that are exposed to elevated pressures through anastomoses with the pulmonary veins (1). Affected patients frequently undergo anticoagulation therapy either to lessen the risk of systemic embolization from atrial fibrillation or as part of the postoperative regimen for valve replacement. Radiographic findings consist of diffuse, confluent acinar or ground-glass areas of increased opacity, often sparing the peripheral parenchyma and creating the so-called "window frame" effect (Fig 4). Radiographic differentiation of diffuse alveolar hemorrhage from hydrostatic pulmonary edema can be difficult, although the presence of hemoptysis and air bronchograms may help.
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Figure 4a. Pulmonary hemorrhage in a 33-year-old man with rheumatic mitral and aortic stenosis who presented with hemoptysis. The patient had undergone replacement of both valves with Ionescu-Shiley bovine pericardial prostheses followed by temporary anticoagulation therapy with warfarin sodium (Coumadin; DuPont Merck, Wilmington, Del). Frontal (a) and lateral (b) chest radiographs demonstrate extensive bilateral diffuse pulmonary consolidation with sparing of the periphery of the lungs, creating the window frame effect that is suggestive of pulmonary hemorrhage.
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Figure 4b. Pulmonary hemorrhage in a 33-year-old man with rheumatic mitral and aortic stenosis who presented with hemoptysis. The patient had undergone replacement of both valves with Ionescu-Shiley bovine pericardial prostheses followed by temporary anticoagulation therapy with warfarin sodium (Coumadin; DuPont Merck, Wilmington, Del). Frontal (a) and lateral (b) chest radiographs demonstrate extensive bilateral diffuse pulmonary consolidation with sparing of the periphery of the lungs, creating the window frame effect that is suggestive of pulmonary hemorrhage.
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Pulmonary Hemosiderosis
Hemosiderosis is regularly found at pathologic analysis in chronic mitral stenosis but is less conspicuous radiographically. At pathologic analysis, hemosiderosis is characterized by the accumulation of hemosiderin in the alveolar, lobular, and perivascular interstitium, filling of the alveoli with hemosiderin-laden macrophages, and fibrosis (1). At radiography, hemosiderosis is seen in 10%–25% of patients with mitral stenosis (3). It is characterized by small (1–3 mm-diameter), ill-defined nodules or by coarse reticular areas of increased opacity with a bias for the middle and lower lung regions (1) (Figs 5, 6).
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Figures 5, 6. (5) Pulmonary hemosiderosis due to long-standing mitral stenosis in a 35-year-old man. The patient had undergone mitral valve replacement with a Hancock porcine bioprosthesis. Frontal (a) and lateral (b) chest radiographs show diffuse small, rounded, "miliary" nodular areas of increased opacity bilaterally. A left lingular calcified granuloma is noted incidentally (arrows in a). (6) Pulmonary hemosiderosis in a 7-year-old child with congenital mitral valve stenosis and a concurrent atrial septal defect (Lutembacher syndrome). Frontal chest radiograph shows diffuse micronodules scattered throughout the lungs. The cardiac silhouette is moderately enlarged. Dilation of both atria, the right ventricle, the main pulmonary artery segment, and both central pulmonary arteries is evident.
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Figures 5, 6. (5) Pulmonary hemosiderosis due to long-standing mitral stenosis in a 35-year-old man. The patient had undergone mitral valve replacement with a Hancock porcine bioprosthesis. Frontal (a) and lateral (b) chest radiographs show diffuse small, rounded, "miliary" nodular areas of increased opacity bilaterally. A left lingular calcified granuloma is noted incidentally (arrows in a). (6) Pulmonary hemosiderosis in a 7-year-old child with congenital mitral valve stenosis and a concurrent atrial septal defect (Lutembacher syndrome). Frontal chest radiograph shows diffuse micronodules scattered throughout the lungs. The cardiac silhouette is moderately enlarged. Dilation of both atria, the right ventricle, the main pulmonary artery segment, and both central pulmonary arteries is evident.
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Figures 5, 6. (5) Pulmonary hemosiderosis due to long-standing mitral stenosis in a 35-year-old man. The patient had undergone mitral valve replacement with a Hancock porcine bioprosthesis. Frontal (a) and lateral (b) chest radiographs show diffuse small, rounded, "miliary" nodular areas of increased opacity bilaterally. A left lingular calcified granuloma is noted incidentally (arrows in a). (6) Pulmonary hemosiderosis in a 7-year-old child with congenital mitral valve stenosis and a concurrent atrial septal defect (Lutembacher syndrome). Frontal chest radiograph shows diffuse micronodules scattered throughout the lungs. The cardiac silhouette is moderately enlarged. Dilation of both atria, the right ventricle, the main pulmonary artery segment, and both central pulmonary arteries is evident.
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Pulmonary Ossification
A rare, late sequela that is virtually pathognomonic for chronic mitral stenosis is parenchymal ossification, which manifests radiographically as densely calcified, 1–5-mm nodules, mainly in the middle and lower lungs, with a tendency for confluence and the occasional presence of trabeculae (1). The prevalence of bone formation ranges from 3% to 13% in reported series and may not always correlate with the degree of pulmonary hypertension or underlying hemosiderosis (4) (Fig 7).
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Figure 7a. Pulmonary ossification in a 63-year-old woman with long-standing mitral stenosis. The patient had undergone valve replacement with a St Jude mechanical prosthesis. (a) Frontal chest radiograph shows bibasilar confluent calcific areas of increased opacity, which are most conspicuous at the right lung base. (b, c) Computed tomographic (CT) scans obtained with soft-tissue window (b) and lung window (c) settings help confirm the presence of ossified acinar clusters, which are more extensive in the right lower lung than in the left lung.
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Figure 7b. Pulmonary ossification in a 63-year-old woman with long-standing mitral stenosis. The patient had undergone valve replacement with a St Jude mechanical prosthesis. (a) Frontal chest radiograph shows bibasilar confluent calcific areas of increased opacity, which are most conspicuous at the right lung base. (b, c) Computed tomographic (CT) scans obtained with soft-tissue window (b) and lung window (c) settings help confirm the presence of ossified acinar clusters, which are more extensive in the right lower lung than in the left lung.
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Figure 7c. Pulmonary ossification in a 63-year-old woman with long-standing mitral stenosis. The patient had undergone valve replacement with a St Jude mechanical prosthesis. (a) Frontal chest radiograph shows bibasilar confluent calcific areas of increased opacity, which are most conspicuous at the right lung base. (b, c) Computed tomographic (CT) scans obtained with soft-tissue window (b) and lung window (c) settings help confirm the presence of ossified acinar clusters, which are more extensive in the right lower lung than in the left lung.
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MITRAL REGURGITATION
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Mitral regurgitation occurs clinically in the setting of infective endocarditis, myxomatous degeneration of the mitral valve, acute rheumatic fever, spontaneous rupture of the chordae ten-dineae, acute papillary muscle rupture or dysfunction related to ischemia, and mitral annulus calcification. Periprosthetic valve leak can cause the physiologic equivalent of mitral regurgitation, and severe enlargement of the left ventricle can lead to secondary mitral regurgitation by dilation of the mitral annulus. Mitral regurgitation increases left ventricular preload but does not usually increase (and may even decrease) afterload; thus, mitral regurgitation favors left ventricular emptying, and in the presence of normal myocardial function, ejection fraction is elevated. Chronic mitral regurgitation is compensated by eccentric left ventricular hypertrophy and can cause significant left ventricular dilation without producing symptoms. Clinical findings are consistent with left ventricular failure and occasionally (in severe disease) with right
ventricular dysfunction. Radiographic findings depend on the acuteness of the disease. Acute regurgitation is characterized by acute left ventricular failure with pulmonary vascular engorgement and interstitial or alveolar pulmonary edema but limited cardiac enlargement. Chronic mitral regurgitation is characterized by marked left ventricular enlargement and massive left atrial dilation as well as signs of left ventricular failure. Echocardiography and left ventriculography provide a semiquantitative evaluation of the degree of regurgitation (2).
Hydrostatic Pulmonary Edema
The most common parenchymal manifestations of acute mitral regurgitation are symmetric alveolar and interstitial pulmonary edema with indistinct, engorged pulmonary vessels and cephalized blood flow.
Asymmetric Pulmonary Edema
An unusual but pathognomonic manifestation of acute mitral regurgitation is asymmetric right upper lobe pulmonary edema (5,6) (Figs 8, 9). In a historical study of 131 patients admitted with a primary diagnosis of mitral regurgitation, 12 patients (9%) had asymmetric right upper lobe edema (6). The predilection of edema formation for the right upper lobe is explained by the anatomy of the pulmonary veins in relation to the mitral valve apparatus. The plane of the mitral valve is inclined posterosuperiorly and to the right, and the regurgitant jet penetrates the origin of the pulmonary vein in the right upper lobe. Preferential flow of the regurgitant jet has been confirmed in an echocardiographic study of 40 patients with mitral regurgitation (7), and a single case report of a regurgitant jet oriented toward the origin of the right pulmonary veins has been documented by transesophageal echocardiography in a patient with asymmetric right upper lobe edema (8).
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Figure 8. Asymmetric pulmonary edema in a 77-year-old man in cardiogenic shock with acute mitral regurgitation due to a flail posterior mitral leaflet that resulted from ischemic papillary muscle dysfunction and subsequent rupture of the chordae tendineae. Supine bedside chest radiograph shows asymmetric pulmonary edema that is more extensive on the right side with slight volume loss in the right upper lobe. The right apical cap is due to pleural effusion (arrow). The cardiac silhouette is enlarged, and the rounded sweep of the Swan-Ganz catheter suggests right ventricular dilation. An intraaortic counterpulsation balloon is also seen.
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Figure 9. Asymmetric pulmonary edema in a 78-year-old woman with rupture of a papillary muscle and hyperacute mitral regurgitation due to a coronary artery bypass graft. Frontal bedside chest radiograph demonstrates asymmetric pulmonary edema with a bias for the right upper lobe. (Courtesy of Steven Weinberger, MD, Harvard Medical School, Boston, Mass.)
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CONCLUSIONS
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Lung parenchymal changes are frequently seen in patients with mitral valve disease. These changes usually facilitate the diagnosis of mitral valve disease but can occasionally hinder such a diagnosis. Familiarity with the gamut of pulmonary findings in patients with mitral valve disease is crucial for rapid diagnosis and optimal patient care.
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References
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Abstract
INTRODUCTION
