Circulation. 2008;117:1745-1749
doi: 10.1161/CIRCULATIONAHA.107.721738
(Circulation. 2008;117:1745-1749.)
© 2008 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Magnetic Resonance Assessment and Therapy Monitoring of Cardiac Involvement in Churg-Strauss Syndrome
Hannibal Baccouche, MD;
Ali Yilmaz, MD;
Dominik Alscher, MD;
Karin Klingel, MD;
Jose Fernando Val-Bernal, MD, PhD;
Heiko Mahrholdt, MD
From the Departments of Cardiology (H.B., A.Y., H.M.) and Nephrology (D.A.), Robert-Bosch-Medical Center, Stuttgart, Germany; Department of Molecular Pathology, University Hospital of Tuebingen, Germany (K.K.); and Department of Anatomical Pathology, Marqués de Valdecilla University Hospital, University of Cantabria, Santander, Spain (J.F.V.-B.).
Correspondence to Heiko Mahrholdt, MD, Robert-Bosch-Medical Center, Auerbachstrasse 110, 70376 Stuttgart, Germany. E-mail heiko.mahrholdt{at}rbk.de
A 39-year–old man, with a temperature of 38.5°C and sinus tachycardia, was admitted for work-up of chest pain. He had a history of asthma, recurrent pneumonia, sinusitis, and nasal polyposis. Clinical examinations, ECG (Figure 1) and chest x-ray (Figure 2) on admission were suspicious for perimyocarditis. Routine blood analysis revealed an elevated erythrocyte sedimentation rate (88 mm/h; normal <15 mm/h) and a normal leukocyte count (7200/mm3) with 21% eosinophilic granulocytes (normal 1% to 6%). Levels of C-reactive protein and immunoglobulin E were elevated at 14.0 mg/dL (normal 0.1 to 0.5 mg/dL) and 237 U/mL (normal <100 U/mL), respectively. Thus, Churg-Strauss syndrome with perimyocardial involvement was suspected.
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Figure 1. Twelve-lead ECG obtained at initial presentation in our emergency room, demonstrating ST-abnormalities suspicious for perimyocarditis.
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Figure 2. Frontal posterior–anterior chest x-ray on admission. Note the enlarged pulmonary hila, as well as the relation of the cardiac silhouette to the lateral distance (16 cm/32.5 cm), suggesting mild cardiac enlargement, pericardial effusion, or a combination of these conditions.
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Because echocardiography could not provide any information on myocardial involvement in this case (Figure 3; for full-motion images, see Movie I in the online-only Data Supplement), the patient was referred for cardiovascular magnetic resonance imaging (CMR; 1.5 Tesla Sonata, Siemens Medical Systems, Erlangen, Germany). Cine images were acquired using fast-gradient echo steady-state free precession sequences that demonstrated increased pericardial thickness (5 mm), as well as small amounts of pericardial effusion (Figure 4). Systolic left ventricular (LV) function was mildly impaired (LV end-diastolic volume 144 mL, LV end-systolic volume 59 mL, and LV ejection fraction 59% (Figure 4; Movie II in the online-only Data Supplement). Ten minutes after injection of 0.2-mmol/kg gadodiamide (Omniscan, Amersham-Health, Braunschweig, Germany) contrast CMR was performed using an inversion recovery gradient echo technique (IR-FLASH), constantly adjusting inversion time to null normal myocardium. Late gadolinium enhancement was present in the basal and midventricular lateral wall, as well as in the septum, and distributed in the subendocardial, intramural, and subepicardial myocardium, as demonstrated in Figure 4.
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Figure 3. Transthoracic echocardiogram (TTE) after chest x-ray. Systolic as well as diastolic images of apical long axis (4CH) and parasternal short axis (SAX) are displayed. No pericardial effusion could be detected, and no information on myocardial involvement in suspected Churg-Strauss syndrome could be obtained by echocardiogram.
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Figure 4. CMR study at initial presentation. Steady-state free precession CMR images of multiple short- and long-axis views are displayed in the upper 2 rows (diastole and systole). Note the increased pericardial thickness in combination with a small pericardial effusion (black arrows). Contrast CMR images are displayed in the bottom row. Contrast CMR (normal myocardium is black) reveals contrast enhancement, located in the basal and midventricular lateral as well as septal wall and distributed in the subendocardial, intramural, and also subepicardial myocardium (white arrows).
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Before initiating immunosuppressive therapy, coronary artery disease, as well as myocardial infection with cardiotropic viruses or bacteria, was ruled out by coronary angiography and CMR-guided endomyocardial biopsies. Histopathological work-up of biopsy samples demonstrated eosinophilic infiltrates in combination with tissue edema and myocardial necrosis (Figure 5), confirming the suspected diagnosis of Churg-Strauss syndrome with cardiac involvement as defined by American College of Rheumatology criteria. Interestingly, the subepicardial, intramural, and subendocardial pattern of late gadolinium enhancement demonstrated by contrast CMR nicely matches the pattern of scattered patches of reddish-gray discoloration representing eosinophilic infiltrates and hemorrhagic necrosis on the necropsy sample of a different patient who died suddenly from Churg-Strauss syndrome with cardiac involvement (Figure 6).1
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Figure 5. Histopathological work-up of LV endomoycardial biopsy specimen (Giemsa staining), displaying normal myocytes (A), numerous infiltrating clusters of eosinophilic granulocytes (B, revealing red cytoplasmic granules), fibrosis (C, curly collagen fibers), and myocyte necrosis (D).
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Figure 6. Short-axis contrast CMR image from the current patient compared with a necropsy sample of a different patient who died suddenly from Churg-Strauss syndrome with cardiac involvement. Interestingly, the pattern of late gadolinium enhancement, located in the basal and midventricular lateral wall, as well as in the septum, and distributed in the subendocardial, intramural, and also subepicardial myocardium (left image, white circles), nicely matches the pattern of scattered patches of reddish-gray discoloration (right image, white circles) representing eosinophilic infiltrates and hemorrhagic necrosis in the necropsy sample.
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After 2 cycles of intravenous cyclophosphamide (750 mg) and oral steroids (1.0 mg/kg daily) the patient’s symptoms had resolved. Two months after initiation of treatment, the erythrocyte sedimentation rate had decreased from 88 mm/h to 8 mm/h, and the eosinophilic count had dropped from 21% to 1.6% of granulocytes. Follow-up CMR imaging at this time still demonstrated mildly impaired LV function (LV end-diastolic volume 160 mL, LV end-systolic volume 65 mL, and LV ejection fraction 60%) but normalization of pericardial thickness (2 mm; Figure 7). As in certain patterns of viral myocarditis, late gadolinium enhancement decreased over time in good correlation to clinical improvement (Figure 7; Movie III in the online-only Data Supplement).2 A quantitative plot of the extent of late gadolinium enhancement using signal intensity detection in the American Heart Association/American College of Cardiology–recommended 17-segment model before and after treatment can be viewed in Figure 8.
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Figure 7. Follow-up CMR after 2 months of immunosuppressive therapy using the same CMR protocol as described above. After treatment, pericardial thickness (black arrows), pericardial effusion, and contrast enhancement have decreased in good correlation to clinical improvement.
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Figure 8. Quantitative plot visualizing the extent of late gadolinium enhancement before (left panel) and after 2 months of immunosuppressive treatment (right panel) as gray-scale maps representing signal intensity in the American Heart Association/American College of Cardiology–recommended 17-segment model. Note the decrease of signal intensity after treatment (right versus left panel). Signal intensity analysis was performed using the National Institutes of Health Image Analysis software package (National Institutes of Health, Bethesda, Md).
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Six months after initiation of treatment, LV function had also normalized (LV end-diastolic volume 135 mL, LV end-systolic volume 42 mL, LV ejection fraction 69%). At this time point, ECG abnormalities were decreasing. However, the ECG remained abnormal despite normalization of LV function (Figure 9).
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Figure 9. Twelve-lead ECG obtained 6 months after initiation of immunosuppressive treatment, demonstrating a decrease in ST abnormalities. However, the ECG remains abnormal, despite clinical improvement and normalization of LV function.
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To our knowledge, this is the first case demonstrating that CMR is not only an important tool for diagnosing cardiac involvement in Churg-Strauss syndrome3,4 but may also be useful for directly monitoring myocardial response to medical treatment, independent of LV ejection fraction or ECG abnormalities.
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Disclosures
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None.
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Footnotes
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The online-only Data Supplement, consisting of 3 movies, can be found with this article at http://circ.ahajournals.org/cgi/content/full/117/ 1745/DC1.
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References
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1. Val-Bernal JF, Mayorga M, Garcia-Alberdi E, Pozueta JA. Churg-Strauss syndrome and sudden cardiac death.
Cardiovasc Pathol. 2003; 12: 94–97.
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[Order article via Infotrieve]2. Mahrholdt H, Wagner A, Deluigi CC, Kispert E, Hager S, Meinhardt G, Vogelsberg H, Fritz P, Dippon J, Bock CT, Klingel K, Kandolf R, Sechtem U. Presentation, Patterns of Myocardial Damage, and Clinical Course of Viral Myocarditis. Circulation. 2006; 114: 1581–1590.[Abstract/Free Full Text]
3. Chun W, Grist TM, Kamp TJ, Warner TF, Christian TF. Infiltrative eosinophilic myocarditis diagnosed and localized by cardiac magnetic resonance imaging. Circulation. 2004; 110; e19.[Free Full Text]
4. Peterson SE, Kardos A, Neubauer S. Subendocardial and papillary muscle involvement in a patient with Churg-Strauss syndrome, detected by contrast enhanced cardiovascular magnetic resonance. Heart. 2005; 91: e9.[Free Full Text]
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Disclosures
References