(Radiographics. 2002;22:1111-1124.)
© RSNA, 2002
Helical CT Features of Arrhythmogenic Right Ventricular Cardiomyopathy1
Fumiko Kimura, MD,
Fumikazu Sakai, MD,
Yasunari Sakomura, MD,
Mikihiko Fujimura, MD,
Eiko Ueno, MD,
Naoki Matsuda, MD,
Hiroshi Kasanuki, MD and
Norio Mitsuhashi, MD
1 From the Department of Radiology (F.K., F.S., M.F., E.U., N. Mitsuhashi) and the Department of Cardiology in the Heart Institute of Japan (Y.S., N. Matsuda, H.K.), Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan. Recipient of a Certificate of Merit award for an education exhibit at the 2001 RSNA scientific assembly. Received February 14, 2002; revision requested March 13 and received May 8; accepted May 10. Address correspondence to F.K. (e-mail: fumikok@rad.twmu.ac.jp or fumikok@jms.cc).
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Abstract
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Arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia, is a disorder of the heart muscle of unknown origin. It is characterized by electrical instability of the heart as a result of replacement of the right ventricular myocardium with fatty or fibrous fatty tissue. Dilatation of the right ventricle; fatty tissue in conspicuous trabeculae of the right ventricle, especially in the anterior wall, apex, and inferior (diaphragmatic) wall; and a scalloped appearance (bulging) of the right ventricular wall are characteristic findings at helical computed tomography (CT) that may be used to diagnose ARVC. Fatty tissue in the left ventricle and ventricular septum is seen relatively frequently in ARVC, and fat in the ventricular septum is another useful finding for diagnosis of ARVC with helical CT. ARVC is usually diagnosed on the basis of clinical or pathologic findings, and electron-beam CT is superior to nongated helical CT in assessment of abnormal right ventricular function. However, with knowledge of the characteristic findings, standard nongated helical CT can be helpful in diagnosing ARVC.
© RSNA, 2002
Index Terms: Heart, cardiomyopathy, 51.86 • Heart, diseases, 51.86 • Heart, ventricles, 52.86 • Myocardium, CT, 51.12114, 51.12115 • Myocardium, diseases, 511.86
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LEARNING OBJECTIVES FOR TEST 3
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After reading this article and taking the test, the reader will be able to:
- Recognize the spectrum of characteristic helical CT findings of ARVC.
- Describe the abnormalities of the left ventricular wall and ventricular septum in patients with ARVC.
- Discuss the usefulness of nongated helical CT in diagnosis of ARVC.
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Introduction
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Arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia, is a disorder of the heart muscle characterized pathologically by replacement of the right ventricular myocardium with fatty or fibrous fatty tissue. Thirty percent to 50% of patients with ARVC have a family history of the disease, and the most common pattern of inheritance is autosomal dominant (1). Expression of a specific gene associated with the disease was recently reported; the pathogenesis remains unclear (1,2). The involved myocardium evokes ventricular arrhythmias originating in the right ventricle that induce syncope and sudden death in young adults.
Because histologic diagnosis of ARVC based on right ventricular endomyocardial biopsy specimens is subject to sampling error, diagnosis relies heavily on the clinical demonstration of morphologic, functional, and electrophysiologic abnormalities that are caused by or reflect the underlying histologic changes. Standardized diagnostic criteria proposed by the Study Group on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy of the Task Force of the Working Group on Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology (3) require the presence of two major criteria, one major plus two minor criteria, or four minor criteria from six specific categories for diagnosis (Table).
Recently, because of its excellent temporal resolution, electron-beam computed tomography (CT) has received much attention as a noninvasive method of diagnosing ARVC. Previously reported electron-beam CT findings for ARVC include (a) a dilated right ventricle, (b) abundant epicardial fatty tissue, (c) conspicuous trabeculae with low attenuation, (d) a scalloped appearance of the free wall of the right ventricle, and (e) reduced function of the right ventricle in combination with abnormal wall motion (4,5). Although the helical technique is a standard method of CT scanning, its usefulness in diagnosing ARVC is unknown because of its inferior temporal resolution relative to that of electron-beam CT.
In patients with ARVC, regional involvement of the right ventricle is common, but evolution to more diffuse right ventricular involvement and to abnormalities in the left ventricle, which cause heart failure, is not unusual. In recent years, reports of left ventricular involvement have increased, leading to the recognition of ARVC as a diffuse disease of the heart muscle affecting both ventricles (1,6,7). Basso and associates (8) report that the ventricular septum is rarely involved (only 20% of cases in their study); however, in concurrence with the findings of Tada et al (5), we frequently observed fatty attenuation in the ventricular septum on the CT images of patients with ARVC. In this article, we demonstrate the spectrum of helical CT features of ARVC, which include abnormalities of the left ventricular wall and ventricular septum, and describe the usefulness of helical CT in diagnosing ARVC.
From May 1995 to October 2001 at our institution, 32 patients with ARVC that was confirmed electrophysiologically and/or pathologically underwent single–detector row helical CT. Our study group comprised 22 male patients and six female patients aged 14–62 years (average, 44.9 years) for whom helical CT images of 3- or 5-mm section thickness were available. The other four patients were excluded because images of only 10-mm section thickness were available.
Radiologic-pathologic correlation was available in one case of autopsy-proved ARVC. Because only conventional CT scans with a 10-mm section thickness were obtained while the patient was alive, he was not included in our patient group. We used recently obtained helical CT and magnetic resonance (MR) images of the fixed heart in this case to correlate radiologic and pathologic findings of ARVC.
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CT Protocols
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Nongated helical CT of the heart was performed in all patients by using a single–detector row scanner with a gantry rotation speed of 0.8–1 second. Following unenhanced helical CT, a 100-mL dose of nonionic contrast material was injected intravenously at 3 mL/sec and enhanced images were acquired after a 30-second scanning delay. In 26 patients, images were obtained by using a 3-mm section thickness with a pitch of 1.5 and a reconstruction interval of 3 mm; in the other two patients, images were obtained by using a 5-mm section thickness with a pitch of 1 and a reconstruction interval of 5 mm. An 18-cm field of view and 180° helical interpolation were applied to reconstruct the images.
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Spectrum of Helical CT Features of ARVC
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Dilatation of the Right Ventricle
Dilatation of the right ventricle is one criterion for diagnosis of ARVC (Table), and it is commonly seen in patients with ARVC (Fig 1). Fatty or fibrous fatty transmural myocardial replacement may be diffuse to the entire right ventricular wall, but it is more often localized to the "triangle of dysplasia," that is, the infundibular region, inferior (diaphragmatic) wall, and anteroapical wall of the right ventricle (1,8–11). Therefore, dilatation of the infundibular region (Fig 2) as well as the right ventricular body is frequently seen.
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Figure 1a. Representative CT findings of ARVC in a 50-year-old man who first experienced arrhythmia at 21 years of age. Unenhanced (a, b) and contrast material-enhanced (c, d) CT scans obtained at the level of the right ventricular inflow region (a, c) and the inferior wall of the right ventricle (RV) (b, d) show marked dilatation of the right ventricle and conspicuous trabeculae (arrows) with low-attenuation fatty tissue in the inferior wall of the right ventricle. A scalloped appearance of the free wall of the right ventricle is also seen (arrowheads). The fatty tissue is easier to recognize on the unenhanced scans (a, b) than on the contrast-enhanced scans (c, d). RA = right atrium.
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Figure 1b. Representative CT findings of ARVC in a 50-year-old man who first experienced arrhythmia at 21 years of age. Unenhanced (a, b) and contrast material-enhanced (c, d) CT scans obtained at the level of the right ventricular inflow region (a, c) and the inferior wall of the right ventricle (RV) (b, d) show marked dilatation of the right ventricle and conspicuous trabeculae (arrows) with low-attenuation fatty tissue in the inferior wall of the right ventricle. A scalloped appearance of the free wall of the right ventricle is also seen (arrowheads). The fatty tissue is easier to recognize on the unenhanced scans (a, b) than on the contrast-enhanced scans (c, d). RA = right atrium.
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Figure 1c. Representative CT findings of ARVC in a 50-year-old man who first experienced arrhythmia at 21 years of age. Unenhanced (a, b) and contrast material-enhanced (c, d) CT scans obtained at the level of the right ventricular inflow region (a, c) and the inferior wall of the right ventricle (RV) (b, d) show marked dilatation of the right ventricle and conspicuous trabeculae (arrows) with low-attenuation fatty tissue in the inferior wall of the right ventricle. A scalloped appearance of the free wall of the right ventricle is also seen (arrowheads). The fatty tissue is easier to recognize on the unenhanced scans (a, b) than on the contrast-enhanced scans (c, d). RA = right atrium.
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Figure 1d. Representative CT findings of ARVC in a 50-year-old man who first experienced arrhythmia at 21 years of age. Unenhanced (a, b) and contrast material-enhanced (c, d) CT scans obtained at the level of the right ventricular inflow region (a, c) and the inferior wall of the right ventricle (RV) (b, d) show marked dilatation of the right ventricle and conspicuous trabeculae (arrows) with low-attenuation fatty tissue in the inferior wall of the right ventricle. A scalloped appearance of the free wall of the right ventricle is also seen (arrowheads). The fatty tissue is easier to recognize on the unenhanced scans (a, b) than on the contrast-enhanced scans (c, d). RA = right atrium.
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Figure 2a. Fatty tissue at the triangle of dysplasia in a 50-year-old man with histologically confirmed ARVC. CT scans were obtained at the levels of the infundibular (a), inflow (b), and apical and inferior (c) regions. Fatty tissue is observed in the slightly dilated infundibular region (I) (arrow in a) and in the conspicuous trabeculae of the anterior wall (arrow in b) and inferior wall (solid arrow in c) of the right ventricle (RV). These three regions, called the triangle of dysplasia, are the typical location of fatty degeneration in ARVC. Fatty tissue is also noted in the right ventricular aspect of the ventricular septum (arrowheads in b and c) and in the apex of the slightly dilated left ventricle (LV) (open arrow in c); the fat in the left ventricle is continuous with the fat in the ventricular septum.
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Figure 2b. Fatty tissue at the triangle of dysplasia in a 50-year-old man with histologically confirmed ARVC. CT scans were obtained at the levels of the infundibular (a), inflow (b), and apical and inferior (c) regions. Fatty tissue is observed in the slightly dilated infundibular region (I) (arrow in a) and in the conspicuous trabeculae of the anterior wall (arrow in b) and inferior wall (solid arrow in c) of the right ventricle (RV). These three regions, called the triangle of dysplasia, are the typical location of fatty degeneration in ARVC. Fatty tissue is also noted in the right ventricular aspect of the ventricular septum (arrowheads in b and c) and in the apex of the slightly dilated left ventricle (LV) (open arrow in c); the fat in the left ventricle is continuous with the fat in the ventricular septum.
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Figure 2c. Fatty tissue at the triangle of dysplasia in a 50-year-old man with histologically confirmed ARVC. CT scans were obtained at the levels of the infundibular (a), inflow (b), and apical and inferior (c) regions. Fatty tissue is observed in the slightly dilated infundibular region (I) (arrow in a) and in the conspicuous trabeculae of the anterior wall (arrow in b) and inferior wall (solid arrow in c) of the right ventricle (RV). These three regions, called the triangle of dysplasia, are the typical location of fatty degeneration in ARVC. Fatty tissue is also noted in the right ventricular aspect of the ventricular septum (arrowheads in b and c) and in the apex of the slightly dilated left ventricle (LV) (open arrow in c); the fat in the left ventricle is continuous with the fat in the ventricular septum.
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When evaluating dilatation of the right ventricle, it is important to rule out pulmonary hypertension. A dilated right ventricle with an ectatic pulmonary trunk may indicate pulmonary hypertension rather than ARVC (10).
Dimensions of the normal adult right ventricle are usually assessed in short and long axes with echocardiography (12,13), but not in a transverse plane, as with CT. Furthermore, because it is difficult to measure the end-diastolic volume of the right ventricle with nongated helical CT, the body of the right ventricle is considered dilated on transverse CT images when the shortest anteroposterior diameter of the right ventricular inflow region is equal to or longer than that of the left ventricular inflow region (Fig 3). However, with this method, when both ventricles are dilated, a large right ventricle that is smaller than the left ventricle could not be considered dilated.
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Figure 3a. Enlargement of the right ventricle in a 45-year-old man with ARVC. CT scans were obtained at the levels of the mitral valve (MV) (a) and tricuspid valve (TV) (b). The anteroposterior diameter of the right ventricular inflow region (two-headed arrow in b) is greater than that of the left ventricular inflow region (two-headed arrow in a), and the body of the right ventricle is interpreted as dilated. In addition, fat in the right ventricular aspect of the subendocardial portion of the ventricular septum is seen in b.
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Figure 3b. Enlargement of the right ventricle in a 45-year-old man with ARVC. CT scans were obtained at the levels of the mitral valve (MV) (a) and tricuspid valve (TV) (b). The anteroposterior diameter of the right ventricular inflow region (two-headed arrow in b) is greater than that of the left ventricular inflow region (two-headed arrow in a), and the body of the right ventricle is interpreted as dilated. In addition, fat in the right ventricular aspect of the subendocardial portion of the ventricular septum is seen in b.
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Fatty Tissue in the Right Ventricular Wall
Visualization of fatty tissue in the right ventricular wall is most important to diagnose ARVC at helical CT.
In patients with ARVC, major fatty infiltration takes place in the subepicardial myocardium of the right ventricular wall, and the remaining subendocardial layer thins (8,9). Because it is difficult to distinguish between fatty infiltration of the subepicardial myocardium and epicardial fatty tissue at CT, abundant epicardial fatty tissue was reported as a characteristic electron-beam CT finding (4). However, distinguishing between normal and abnormally increased epicardial fatty tissue is difficult because a considerable amount of epicardial fat can be observed in obese persons (Figs 4, 5). Therefore, it seems difficult to accept a finding of abundant epicardial fatty tissue as a characteristic helical CT finding of ARVC.
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Figure 4a. Slightly scalloped appearance of the right ventricle in a 59-year-old woman with ARVC. Unenhanced (a) and contrast-enhanced (b) CT scans show slight bulging of the anterior wall of the right ventricle (RV) (arrow) and abundant epicardial fatty tissue (*). The bulging of the free wall of the right ventricle is more conspicuous on the unenhanced scan (a) than the contrast-enhanced scan (b), probably as a result of a difference in the cardiac phase. In addition, fatty tissue is seen in the right ventricular aspect of the subendocardial portion of the ventricular septum (arrowheads). LV = left ventricle.
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Figure 4b. Slightly scalloped appearance of the right ventricle in a 59-year-old woman with ARVC. Unenhanced (a) and contrast-enhanced (b) CT scans show slight bulging of the anterior wall of the right ventricle (RV) (arrow) and abundant epicardial fatty tissue (*). The bulging of the free wall of the right ventricle is more conspicuous on the unenhanced scan (a) than the contrast-enhanced scan (b), probably as a result of a difference in the cardiac phase. In addition, fatty tissue is seen in the right ventricular aspect of the subendocardial portion of the ventricular septum (arrowheads). LV = left ventricle.
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Figure 5. Abundant epicardial fat in a healthy obese man with no history of arrhythmia. Contrast-enhanced CT scan shows abundant epicardial fat (*). From this finding alone, it is difficult to distinguish between normal epicardial fatty tissue and abnormally increased epicardial fatty tissue caused by ARVC.
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The right ventricular wall is thin in normal adults, usually less than 0.4 cm (regional variation between 0.2 and 0.7 cm) (12), and the right ventricular free wall becomes even thinner in patients with ARVC. Because of motion artifacts, it is difficult to demonstrate the thin free wall with nongated helical CT.
In patients with ARVC, the myocardial trabeculae are often enlarged, particularly on the anterior and inferior walls, which explains the angiographic images of deep fissures (11,14). Fat in the conspicuous trabeculae, the moderator band, or the papillary muscle is frequently seen with helical CT (Figs 1, 2), and the fat in these regions is easier to recognize than that in the thin right ventricular free wall.
Within the triangle of dysplasia, fatty tissue is most frequently seen in the inferior wall, and it is most conspicuous in this area of the three. It is commonly observed in the anteroapical wall of the right ventricle as well (Figs 1, 2). Although the infundibular region is also a part of the triangle of dysplasia, the fat in this region is sometimes difficult to recognize with helical CT because of the thin wall and the absence of trabeculae (Fig 2).
Unenhanced CT is essential to identify small amounts of fatty tissue, and contrast-enhanced CT is not necessary if the purpose of the study is only to find fat. Nevertheless, contrast-enhanced CT aids in discerning the precise location of fat and of the enlarged myocardial trabeculae, one of the characteristic findings of ARVC, and it aids in evaluating the degree of right ventricular enlargement.
Scalloped Appearance of the Right Ventricular Wall
At cine angiography, localized akinetic or dyskinetic bulges are frequently seen at the apex, infundibulum, and inferior wall (14). A scalloped appearance of the free wall of the right ventricle at helical CT corresponds to the findings of wall bulging, outpouching, or aneurysm of the right ventricular wall at cine angiography (Figs 1, 4, 6). A scalloped right ventricular wall is less frequently observed than other CT findings, tends to be seen in patients with severe fatty degeneration in the wall of a markedly dilated right ventricle, and is thought to be evidence of advanced ARVC.
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Figure 6. Scalloped appearance (aneurysm formation) of the inferior wall of the right ventricle in a 41-year-old man with histologically confirmed ARVC. Contrast-enhanced CT scan shows bulging of the inferior wall (arrow), which is compatible with aneurysm formation and is pathognomonic for ARVC. RV = right ventricle.
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Fatty Tissue in the Left Ventricular Wall
As the disease progresses, the left ventricle may progressively undergo biventricular failure. The prevalence of left ventricular involvement varies from 24% at left ventriculography (15) to 76% at histologic examination (1,6). Left ventricular involvement is not rare and, in fact, occurs relatively frequently. Tada and associates (5) reported a wedge-shaped defect of the left ventricular myocardium, which suggests subepicardial fatty replacement, as characteristic of left ventricular involvement in patients with ARVC (Fig 7). In addition to this wedge formation, linear fatty tissue is also seen in the wall of the left ventricle (Figs 2, 7). Thin-section CT enables detection of fatty tissue in the left ventricular wall and definitive diagnosis of subclinical left ventricular abnormality without left ventricular motion abnormality (5).
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Figure 7a. Involvement of the left ventricle and ventricular septum in a 44-year-old man with histologically confirmed ARVC. Unenhanced (a-c) and contrast-enhanced (d, e) CT scans show enlargement of the right ventricle (RV) with fatty tissue in the trabeculae (arrow in a, open arrow in b), as well as thick fatty tissue in the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow in b, arrow in c and e); the fatty tissue in the ventricular septum is continuous with the interventricular sulcus (c). Wedge-shaped fatty tissue (arrowhead in b, c, and e) and linear fatty tissue (arrowhead in a and d) are observed in the anterolateral wall of the left ventricle (LV).
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Figure 7b. Involvement of the left ventricle and ventricular septum in a 44-year-old man with histologically confirmed ARVC. Unenhanced (a-c) and contrast-enhanced (d, e) CT scans show enlargement of the right ventricle (RV) with fatty tissue in the trabeculae (arrow in a, open arrow in b), as well as thick fatty tissue in the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow in b, arrow in c and e); the fatty tissue in the ventricular septum is continuous with the interventricular sulcus (c). Wedge-shaped fatty tissue (arrowhead in b, c, and e) and linear fatty tissue (arrowhead in a and d) are observed in the anterolateral wall of the left ventricle (LV).
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Figure 7c. Involvement of the left ventricle and ventricular septum in a 44-year-old man with histologically confirmed ARVC. Unenhanced (a-c) and contrast-enhanced (d, e) CT scans show enlargement of the right ventricle (RV) with fatty tissue in the trabeculae (arrow in a, open arrow in b), as well as thick fatty tissue in the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow in b, arrow in c and e); the fatty tissue in the ventricular septum is continuous with the interventricular sulcus (c). Wedge-shaped fatty tissue (arrowhead in b, c, and e) and linear fatty tissue (arrowhead in a and d) are observed in the anterolateral wall of the left ventricle (LV).
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Figure 7d. Involvement of the left ventricle and ventricular septum in a 44-year-old man with histologically confirmed ARVC. Unenhanced (a-c) and contrast-enhanced (d, e) CT scans show enlargement of the right ventricle (RV) with fatty tissue in the trabeculae (arrow in a, open arrow in b), as well as thick fatty tissue in the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow in b, arrow in c and e); the fatty tissue in the ventricular septum is continuous with the interventricular sulcus (c). Wedge-shaped fatty tissue (arrowhead in b, c, and e) and linear fatty tissue (arrowhead in a and d) are observed in the anterolateral wall of the left ventricle (LV).
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Figure 7e. Involvement of the left ventricle and ventricular septum in a 44-year-old man with histologically confirmed ARVC. Unenhanced (a-c) and contrast-enhanced (d, e) CT scans show enlargement of the right ventricle (RV) with fatty tissue in the trabeculae (arrow in a, open arrow in b), as well as thick fatty tissue in the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow in b, arrow in c and e); the fatty tissue in the ventricular septum is continuous with the interventricular sulcus (c). Wedge-shaped fatty tissue (arrowhead in b, c, and e) and linear fatty tissue (arrowhead in a and d) are observed in the anterolateral wall of the left ventricle (LV).
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Fatty Tissue in the Ventricular Septum
Several authors report that involvement of the ventricular septum is rare (1,3,8). However, at electron-beam CT, Tada and associates (5) observed fatty tissue in the ventricular septum in seven of 14 patients. Fatty tissue in the ventricular septum, which is observed more frequently than fatty tissue in the left ventricular wall, was always located in the right ventricular aspect of the subendocardial portion and in most cases was continuous with fat in the interventricular sulcus (Figs 2, 7). Because fatty tissue in the smooth-contour septum (as opposed to that in the irregular inner surface of the right ventricle that results from trabeculae) may not be a substrate for re-entry circuits and may not produce ventricular arrhythmia, the septum may have been ignored clinically.
In terms of the embryogenesis of the ventricular septum, the inferior part of the bulboventricular sulcus begins to protrude into the cardiac lumen along the interface between the presumptive right and left ventricular chambers. A septum apparently forms as the growing walls of the right and left ventricles become more closely apposed to one another, and it grows to be a primitive muscular part of the ventricular septum (16). Therefore, the right ventricular aspect of the subendocardial portion of the ventricular septum can be regarded as a part of the right ventricular wall, and involvement of that portion of the septum seems to be a natural course for ARVC. In addition, finding continuity with the fat of the interventricular sulcus suggests that fatty replacement in the ventricular septum begins from the subepicardial region as well.
Because the septum is usually seen as thick bandlike myocardium at CT, it may not be susceptible to motion artifacts and its small amounts of fat may be easily detected with helical CT. We believe that finding fat in the subendocardial portion of the septum of the right ventricle also contributes to a diagnosis of ARVC at CT.
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Radiologic-Pathologic Correlation
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Histopathologic findings from the fixed heart in an autopsy-proved case of ARVC were compared with the radiologic findings of the fixed heart and helical CT findings obtained while the patient was alive.
Conventional CT images of 10-mm section thickness obtained while the patient was alive showed dilatation of the right ventricle with an area of bold bandlike fatty attenuation in the ventricular septum. Small amounts of fatty tissue, which were inconspicuous because of section thickness and motion artifacts, were also suspected in the trabeculae of the right and left ventricular walls (Fig 8).
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Figure 8a. Radiologic-pathologic correlation in a man who died at the age of 85 years as a result of autopsy-proved ARVC. LV = left ventricle, RV = right ventricle. (a, b) Unenhanced (a) and contrast-enhanced (b) conventional CT scans (10-mm section thickness) obtained while the patient was alive show enlargement of the right ventricle and apparent linear fatty tissue in the ventricular septum (arrow). Areas of fatty attenuation are also suspected in the trabeculae of the right and left ventricular walls (arrowheads). (c-e) Helical CT scan (3-mm section thickness) (c), T1-weighted MR image (d), and fat-suppressed T1-weighted MR image (e) of the fixed heart (views of the short axis of the ventricles) show fatty tissue in the right ventricular wall (arrowheads), the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow) with continuation to the interventricular sulcus, and the subepicardial portion of the left ventricular wall (open arrows). The fatty tissue appears as low attenuation on the CT scan (c), high signal intensity on the T1-weighted MR image (d), and low signal intensity on the fat-suppressed T1-weighted MR image (e). The right ventricular wall is very thin, and most of it is replaced by fatty tissue (arrowheads). (f) Low-power photomicrograph (original magnification, x20; Masson stain) of the right ventricular wall shows fatty and fibrofatty transmural myocardial replacement. (g) High-power photomicrograph (original magnification, x200; Masson stain) shows severe myocardial degeneration, interstitial fibrosis, and fatty infiltration. (h, i) Scanning electron micrographs (Masson stain) of the ventricular septum (VS) (h) and left ventricular wall (i) show fatty infiltration and fibrosis (areas stained blue). Fatty infiltration is prominent in the subepicardial regions of the right and left ventricular walls (arrows in f and i) and in the subendocardial region of the ventricular septum with continuation to the interventricular sulcus (arrowheads in h).
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Figure 8b. Radiologic-pathologic correlation in a man who died at the age of 85 years as a result of autopsy-proved ARVC. LV = left ventricle, RV = right ventricle. (a, b) Unenhanced (a) and contrast-enhanced (b) conventional CT scans (10-mm section thickness) obtained while the patient was alive show enlargement of the right ventricle and apparent linear fatty tissue in the ventricular septum (arrow). Areas of fatty attenuation are also suspected in the trabeculae of the right and left ventricular walls (arrowheads). (c-e) Helical CT scan (3-mm section thickness) (c), T1-weighted MR image (d), and fat-suppressed T1-weighted MR image (e) of the fixed heart (views of the short axis of the ventricles) show fatty tissue in the right ventricular wall (arrowheads), the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow) with continuation to the interventricular sulcus, and the subepicardial portion of the left ventricular wall (open arrows). The fatty tissue appears as low attenuation on the CT scan (c), high signal intensity on the T1-weighted MR image (d), and low signal intensity on the fat-suppressed T1-weighted MR image (e). The right ventricular wall is very thin, and most of it is replaced by fatty tissue (arrowheads). (f) Low-power photomicrograph (original magnification, x20; Masson stain) of the right ventricular wall shows fatty and fibrofatty transmural myocardial replacement. (g) High-power photomicrograph (original magnification, x200; Masson stain) shows severe myocardial degeneration, interstitial fibrosis, and fatty infiltration. (h, i) Scanning electron micrographs (Masson stain) of the ventricular septum (VS) (h) and left ventricular wall (i) show fatty infiltration and fibrosis (areas stained blue). Fatty infiltration is prominent in the subepicardial regions of the right and left ventricular walls (arrows in f and i) and in the subendocardial region of the ventricular septum with continuation to the interventricular sulcus (arrowheads in h).
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Figure 8c. Radiologic-pathologic correlation in a man who died at the age of 85 years as a result of autopsy-proved ARVC. LV = left ventricle, RV = right ventricle. (a, b) Unenhanced (a) and contrast-enhanced (b) conventional CT scans (10-mm section thickness) obtained while the patient was alive show enlargement of the right ventricle and apparent linear fatty tissue in the ventricular septum (arrow). Areas of fatty attenuation are also suspected in the trabeculae of the right and left ventricular walls (arrowheads). (c-e) Helical CT scan (3-mm section thickness) (c), T1-weighted MR image (d), and fat-suppressed T1-weighted MR image (e) of the fixed heart (views of the short axis of the ventricles) show fatty tissue in the right ventricular wall (arrowheads), the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow) with continuation to the interventricular sulcus, and the subepicardial portion of the left ventricular wall (open arrows). The fatty tissue appears as low attenuation on the CT scan (c), high signal intensity on the T1-weighted MR image (d), and low signal intensity on the fat-suppressed T1-weighted MR image (e). The right ventricular wall is very thin, and most of it is replaced by fatty tissue (arrowheads). (f) Low-power photomicrograph (original magnification, x20; Masson stain) of the right ventricular wall shows fatty and fibrofatty transmural myocardial replacement. (g) High-power photomicrograph (original magnification, x200; Masson stain) shows severe myocardial degeneration, interstitial fibrosis, and fatty infiltration. (h, i) Scanning electron micrographs (Masson stain) of the ventricular septum (VS) (h) and left ventricular wall (i) show fatty infiltration and fibrosis (areas stained blue). Fatty infiltration is prominent in the subepicardial regions of the right and left ventricular walls (arrows in f and i) and in the subendocardial region of the ventricular septum with continuation to the interventricular sulcus (arrowheads in h).
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Figure 8d. Radiologic-pathologic correlation in a man who died at the age of 85 years as a result of autopsy-proved ARVC. LV = left ventricle, RV = right ventricle. (a, b) Unenhanced (a) and contrast-enhanced (b) conventional CT scans (10-mm section thickness) obtained while the patient was alive show enlargement of the right ventricle and apparent linear fatty tissue in the ventricular septum (arrow). Areas of fatty attenuation are also suspected in the trabeculae of the right and left ventricular walls (arrowheads). (c-e) Helical CT scan (3-mm section thickness) (c), T1-weighted MR image (d), and fat-suppressed T1-weighted MR image (e) of the fixed heart (views of the short axis of the ventricles) show fatty tissue in the right ventricular wall (arrowheads), the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow) with continuation to the interventricular sulcus, and the subepicardial portion of the left ventricular wall (open arrows). The fatty tissue appears as low attenuation on the CT scan (c), high signal intensity on the T1-weighted MR image (d), and low signal intensity on the fat-suppressed T1-weighted MR image (e). The right ventricular wall is very thin, and most of it is replaced by fatty tissue (arrowheads). (f) Low-power photomicrograph (original magnification, x20; Masson stain) of the right ventricular wall shows fatty and fibrofatty transmural myocardial replacement. (g) High-power photomicrograph (original magnification, x200; Masson stain) shows severe myocardial degeneration, interstitial fibrosis, and fatty infiltration. (h, i) Scanning electron micrographs (Masson stain) of the ventricular septum (VS) (h) and left ventricular wall (i) show fatty infiltration and fibrosis (areas stained blue). Fatty infiltration is prominent in the subepicardial regions of the right and left ventricular walls (arrows in f and i) and in the subendocardial region of the ventricular septum with continuation to the interventricular sulcus (arrowheads in h).
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Figure 8e. Radiologic-pathologic correlation in a man who died at the age of 85 years as a result of autopsy-proved ARVC. LV = left ventricle, RV = right ventricle. (a, b) Unenhanced (a) and contrast-enhanced (b) conventional CT scans (10-mm section thickness) obtained while the patient was alive show enlargement of the right ventricle and apparent linear fatty tissue in the ventricular septum (arrow). Areas of fatty attenuation are also suspected in the trabeculae of the right and left ventricular walls (arrowheads). (c-e) Helical CT scan (3-mm section thickness) (c), T1-weighted MR image (d), and fat-suppressed T1-weighted MR image (e) of the fixed heart (views of the short axis of the ventricles) show fatty tissue in the right ventricular wall (arrowheads), the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow) with continuation to the interventricular sulcus, and the subepicardial portion of the left ventricular wall (open arrows). The fatty tissue appears as low attenuation on the CT scan (c), high signal intensity on the T1-weighted MR image (d), and low signal intensity on the fat-suppressed T1-weighted MR image (e). The right ventricular wall is very thin, and most of it is replaced by fatty tissue (arrowheads). (f) Low-power photomicrograph (original magnification, x20; Masson stain) of the right ventricular wall shows fatty and fibrofatty transmural myocardial replacement. (g) High-power photomicrograph (original magnification, x200; Masson stain) shows severe myocardial degeneration, interstitial fibrosis, and fatty infiltration. (h, i) Scanning electron micrographs (Masson stain) of the ventricular septum (VS) (h) and left ventricular wall (i) show fatty infiltration and fibrosis (areas stained blue). Fatty infiltration is prominent in the subepicardial regions of the right and left ventricular walls (arrows in f and i) and in the subendocardial region of the ventricular septum with continuation to the interventricular sulcus (arrowheads in h).
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Figure 8f. Radiologic-pathologic correlation in a man who died at the age of 85 years as a result of autopsy-proved ARVC. LV = left ventricle, RV = right ventricle. (a, b) Unenhanced (a) and contrast-enhanced (b) conventional CT scans (10-mm section thickness) obtained while the patient was alive show enlargement of the right ventricle and apparent linear fatty tissue in the ventricular septum (arrow). Areas of fatty attenuation are also suspected in the trabeculae of the right and left ventricular walls (arrowheads). (c-e) Helical CT scan (3-mm section thickness) (c), T1-weighted MR image (d), and fat-suppressed T1-weighted MR image (e) of the fixed heart (views of the short axis of the ventricles) show fatty tissue in the right ventricular wall (arrowheads), the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow) with continuation to the interventricular sulcus, and the subepicardial portion of the left ventricular wall (open arrows). The fatty tissue appears as low attenuation on the CT scan (c), high signal intensity on the T1-weighted MR image (d), and low signal intensity on the fat-suppressed T1-weighted MR image (e). The right ventricular wall is very thin, and most of it is replaced by fatty tissue (arrowheads). (f) Low-power photomicrograph (original magnification, x20; Masson stain) of the right ventricular wall shows fatty and fibrofatty transmural myocardial replacement. (g) High-power photomicrograph (original magnification, x200; Masson stain) shows severe myocardial degeneration, interstitial fibrosis, and fatty infiltration. (h, i) Scanning electron micrographs (Masson stain) of the ventricular septum (VS) (h) and left ventricular wall (i) show fatty infiltration and fibrosis (areas stained blue). Fatty infiltration is prominent in the subepicardial regions of the right and left ventricular walls (arrows in f and i) and in the subendocardial region of the ventricular septum with continuation to the interventricular sulcus (arrowheads in h).
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Figure 8g. Radiologic-pathologic correlation in a man who died at the age of 85 years as a result of autopsy-proved ARVC. LV = left ventricle, RV = right ventricle. (a, b) Unenhanced (a) and contrast-enhanced (b) conventional CT scans (10-mm section thickness) obtained while the patient was alive show enlargement of the right ventricle and apparent linear fatty tissue in the ventricular septum (arrow). Areas of fatty attenuation are also suspected in the trabeculae of the right and left ventricular walls (arrowheads). (c-e) Helical CT scan (3-mm section thickness) (c), T1-weighted MR image (d), and fat-suppressed T1-weighted MR image (e) of the fixed heart (views of the short axis of the ventricles) show fatty tissue in the right ventricular wall (arrowheads), the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow) with continuation to the interventricular sulcus, and the subepicardial portion of the left ventricular wall (open arrows). The fatty tissue appears as low attenuation on the CT scan (c), high signal intensity on the T1-weighted MR image (d), and low signal intensity on the fat-suppressed T1-weighted MR image (e). The right ventricular wall is very thin, and most of it is replaced by fatty tissue (arrowheads). (f) Low-power photomicrograph (original magnification, x20; Masson stain) of the right ventricular wall shows fatty and fibrofatty transmural myocardial replacement. (g) High-power photomicrograph (original magnification, x200; Masson stain) shows severe myocardial degeneration, interstitial fibrosis, and fatty infiltration. (h, i) Scanning electron micrographs (Masson stain) of the ventricular septum (VS) (h) and left ventricular wall (i) show fatty infiltration and fibrosis (areas stained blue). Fatty infiltration is prominent in the subepicardial regions of the right and left ventricular walls (arrows in f and i) and in the subendocardial region of the ventricular septum with continuation to the interventricular sulcus (arrowheads in h).
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Figure 8h. Radiologic-pathologic correlation in a man who died at the age of 85 years as a result of autopsy-proved ARVC. LV = left ventricle, RV = right ventricle. (a, b) Unenhanced (a) and contrast-enhanced (b) conventional CT scans (10-mm section thickness) obtained while the patient was alive show enlargement of the right ventricle and apparent linear fatty tissue in the ventricular septum (arrow). Areas of fatty attenuation are also suspected in the trabeculae of the right and left ventricular walls (arrowheads). (c-e) Helical CT scan (3-mm section thickness) (c), T1-weighted MR image (d), and fat-suppressed T1-weighted MR image (e) of the fixed heart (views of the short axis of the ventricles) show fatty tissue in the right ventricular wall (arrowheads), the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow) with continuation to the interventricular sulcus, and the subepicardial portion of the left ventricular wall (open arrows). The fatty tissue appears as low attenuation on the CT scan (c), high signal intensity on the T1-weighted MR image (d), and low signal intensity on the fat-suppressed T1-weighted MR image (e). The right ventricular wall is very thin, and most of it is replaced by fatty tissue (arrowheads). (f) Low-power photomicrograph (original magnification, x20; Masson stain) of the right ventricular wall shows fatty and fibrofatty transmural myocardial replacement. (g) High-power photomicrograph (original magnification, x200; Masson stain) shows severe myocardial degeneration, interstitial fibrosis, and fatty infiltration. (h, i) Scanning electron micrographs (Masson stain) of the ventricular septum (VS) (h) and left ventricular wall (i) show fatty infiltration and fibrosis (areas stained blue). Fatty infiltration is prominent in the subepicardial regions of the right and left ventricular walls (arrows in f and i) and in the subendocardial region of the ventricular septum with continuation to the interventricular sulcus (arrowheads in h).
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Figure 8i. Radiologic-pathologic correlation in a man who died at the age of 85 years as a result of autopsy-proved ARVC. LV = left ventricle, RV = right ventricle. (a, b) Unenhanced (a) and contrast-enhanced (b) conventional CT scans (10-mm section thickness) obtained while the patient was alive show enlargement of the right ventricle and apparent linear fatty tissue in the ventricular septum (arrow). Areas of fatty attenuation are also suspected in the trabeculae of the right and left ventricular walls (arrowheads). (c-e) Helical CT scan (3-mm section thickness) (c), T1-weighted MR image (d), and fat-suppressed T1-weighted MR image (e) of the fixed heart (views of the short axis of the ventricles) show fatty tissue in the right ventricular wall (arrowheads), the right ventricular aspect of the subendocardial portion of the ventricular septum (solid arrow) with continuation to the interventricular sulcus, and the subepicardial portion of the left ventricular wall (open arrows). The fatty tissue appears as low attenuation on the CT scan (c), high signal intensity on the T1-weighted MR image (d), and low signal intensity on the fat-suppressed T1-weighted MR image (e). The right ventricular wall is very thin, and most of it is replaced by fatty tissue (arrowheads). (f) Low-power photomicrograph (original magnification, x20; Masson stain) of the right ventricular wall shows fatty and fibrofatty transmural myocardial replacement. (g) High-power photomicrograph (original magnification, x200; Masson stain) shows severe myocardial degeneration, interstitial fibrosis, and fatty infiltration. (h, i) Scanning electron micrographs (Masson stain) of the ventricular septum (VS) (h) and left ventricular wall (i) show fatty infiltration and fibrosis (areas stained blue). Fatty infiltration is prominent in the subepicardial regions of the right and left ventricular walls (arrows in f and i) and in the subendocardial region of the ventricular septum with continuation to the interventricular sulcus (arrowheads in h).
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CT and MR images of the fixed heart showed that the fatty tissue replaced most of the right ventricular wall with only a small portion of the right ventricular myocardium remaining. The fatty tissue in the subepicardial region of the left ventricular wall was seen to form the wedge-shaped defect of the left ventricular wall. The fat in the ventricular septum was identified at the right ventricular aspect of the subendocardial portion of the ventricular septum and was continuous with the ventricular sulcus (Fig 8). These findings from the fixed heart are comparable with the helical CT findings of living patients with ARVC.
Microscopic sections from the free wall of the right ventricle, the ventricular septum, and the left ventricular wall revealed fatty infiltration and fibrosis. A high-power photomicrograph demonstrated severe myocardial degeneration, interstitial fibrosis, and fatty infiltration (Fig 8). These histologic findings are compatible with ARVC.
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Differential Diagnosis
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Fatty tissue is found in the myocardium of patients with ARVC and several other conditions; it has been observed in healthy subjects (17–19) and in patients with dilated cardiomyopathy (20,21), hypertrophic cardiomyopathy (21), Kawasaki disease (21), and old myocardial infarction (21).
Fontaine and associates (17) reported that fat is interspersed with right ventricular myocardial fibers without fibrosis or signs of inflammation in more than half of normal hearts. Tada and associates (5) compared electron-beam CT images from patients with ARVC, patients with right ventricular enlargement and/or dysfunction without ARVC, and a control group; intramyocardial fat deposits were observed only in the group with ARVC. However, Burke and associates (19) found that the degree of fat infiltration in patients with fibrofatty ARVC, although consistently greater than that in the control group, was significantly increased only in the anterior base and lateral wall. To our knowledge, there is no report of CT or MR imaging of fat in the normal myocardium, and finding fat in the right ventricular myocardium alone does not suggest a diagnosis of ARVC. Rather, other findings (ie, dilatation of the right ventricle, enlargement of the trabeculae, or a scalloped appearance of the right ventricular wall) must be carefully assessed at helical CT, but CT images of fat in the right ventricular wall in patients in early stages of ARVC may not differ from those of healthy subjects.
In patients with dilated cardiomyopathy, a fatty component, considered to represent fatty degeneration as a result of myocardial damage, is seen in the left ventricular wall or ventricular septum (20). The location of the fat and associated findings (eg, dilatation of the left ventricle, biventricular dilatation with left ventricle dominance, or thinning of the left ventricular wall) could be the differential points from ARVC (Fig 9). However, differentiation between advanced ARVC with biventricular involvement and dilated cardiomyopathy may be difficult.
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Figure 9. Histologically and clinically diagnosed dilated cardiomyopathy in a 45-year-old woman. Contrast-enhanced CT scan shows bandlike fatty tissue in the ventricular septum and the wall of the markedly dilated left ventricle (arrowheads). Characteristic CT findings of ARVC, such as dilatation of the right ventricle, conspicuous trabeculae in the right ventricle, or a scalloped appearance of the right ventricle, are not seen.
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In patients with old myocardial infarction, fat is sometimes seen in the affected myocardium. However, old myocardial infarction is usually easily differentiated from ARVC because the fat is usually localized in the thin left ventricular wall and/or ventricular septum (Fig 10).
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Figure 10a. Old myocardial infarction in a 71-year-old man with obstruction of the left anterior descending coronary artery, which was confirmed with angiography. Unenhanced (a) and contrast-enhanced (b) CT scans show fatty tissue (arrowheads) localized in the thin ventricular septum and the anterior wall of the left ventricle. An area of low attenuation in the right ventricle (arrow) is presumed to be an artifact because it does not appear on the unenhanced scan (a).
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Figure 10b. Old myocardial infarction in a 71-year-old man with obstruction of the left anterior descending coronary artery, which was confirmed with angiography. Unenhanced (a) and contrast-enhanced (b) CT scans show fatty tissue (arrowheads) localized in the thin ventricular septum and the anterior wall of the left ventricle. An area of low attenuation in the right ventricle (arrow) is presumed to be an artifact because it does not appear on the unenhanced scan (a).
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Future Applications of Helical CT
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Although our single–detector row helical CT scanner does not allow assessment of wall motion, electrocardiographically triggered acquisition of images minimizes cardiac motion and may facilitate evaluation of the right ventricular wall. Retrospective gating with multi–detector row CT allows reconstruction of images at various cardiac phases and functional assessment and may play a more important role in diagnosis of ARVC.
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Conclusions
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Dilatation of the right ventricle; fatty tissue in conspicuous trabeculae of the right ventricle, especially in the anterior wall, apex, and inferior wall; and a scalloped appearance (bulging) of the right ventricular wall are characteristic helical CT findings that may be used to diagnose ARVC. Fatty tissue of the left ventricle and ventricular septum is seen relatively frequently in ARVC, and fat in the ventricular septum is another useful finding for diagnosing ARVC by using helical CT.
ARVC is usually diagnosed on the basis of clinical and/or pathologic findings, and electron-beam CT is superior to nongated helical CT in assessment of abnormal right ventricular function. However, with a knowledge of the above-described characteristic findings, standard nongated helical CT can be helpful in diagnosing ARVC.
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Footnotes
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Abbreviation: ARVC = arrhythmogenic right ventricular cardiomyopathy
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References
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