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Perimyocarditis Complicated by Early Development of Constrictive Pericarditis
Canadian Journal of Cardiology 32 (2016) 395.e11e395.e12 www.onlinecjc.ca
Images in Cardiology
Perimyocarditis Complicated by Early Development of Constrictive Pericarditis Robert Dennert, MD, PhD,a Sebastian A.F. Streukens, MD,a Suzanne Gommers, MD,b Stephane Heymans, MD, PhD,a and Sebastiaan C.A.M. Bekkers, MD, PhDa a
Department of Cardiology, Maastricht University Medical Center, Maastricht, The Netherlands
b
Department of Radiology, Maastricht University Medical Center, Maastricht, The Netherlands
A 54-year-old man with a history of type 1 diabetes mellitus and hyperthyroidism presented with typical symptoms of pericarditis after an episode of flu-like symptoms. His electrocardiogram revealed diffuse ST-T segment elevation not confined to an arterial territory. Plasmatic levels of cardiac troponin remained within normal values. Treatment with nonsteroidal anti-inflammatory drugs was initiated, but he presented with recurrent symptoms and progressive dyspnea several weeks later. Further evaluation revealed a history of frequent purulent nasal discharge. Urine analysis demonstrated microhematuria. Echocardiography revealed a thickened pericardium without effusion, augmented respiratory variation of mitral inflow (Fig. 1A), normal medial peak E0 velocities (Fig. 1B), and abnormal septal motion during diastole (septal bounce). Cardiac magnetic resonance imaging showed marked pericardial thickening on cine, T1-, T2-weighted, and delayed enhancement (DE) imaging (Fig. 1, D-F), with diastolic septal bounce on cine imaging (see Videos 1 and 2; view videos online) compatible with constriction.1 On T2weighted and DE imaging, the hyperintense visceral and parietal pericardium were clearly discernable. Midwall linear hyperintensity in the basal inferolateral segments complemented the diagnosis of acute perimyocarditis. Endomyocardial biopsies did not show active myocarditis or granuloma formation on histology. Additional immunohistological analysis revealed borderline leukocyte
Received for publication March 13, 2015. Accepted June 4, 2015. Corresponding author: Dr Robert Dennert, Department of Cardiology, Maastricht University Medical Center, P. Debyelaan 25, 6229 HX, Maastricht, The Netherlands. Tel.: þ31-43-3882949; fax: þ31-43-3882952. E-mail: [email protected] See page 395.e12 for disclosure information.
infiltration (13 CD45-positive inflammatory cells per mm2; Fig. 1C) and an increased parvovirus-B19 viral load (319 copies per microgram DNA) in polymerase chain reaction assay. A serology test showed the presence of perinuclearantineutrophil cytoplasmic antibody (ANCA) (titer: 1/ 128) confirmed with proteinase 3-capture enzyme-linked immunosorbent assay and a marked increased soluble interleukin-2 (5587 pg/mL), consistent with granulomatosis with polyangiitis (formerly known as Wegener granulomatosis). Cardiac involvement is not uncommon in systemic inflammatory diseases.2 Pericardial involvement has been especially described in patients with granulomatosis with polyangiitis.3 Evolution to constrictive pericarditis is a feared and rare complication; it has been attributed to recurrent or incessant episodes of pericarditis.4 Development of constrictive pericarditis after a first episode of peri(myo) carditis has not previously been described in patients with granulomatosis with polyangiitis. Our case suggests a parvovirus B-19 infection triggered perimyocarditis. It highlights a secondary exaggerated inflammatory response possibly due to an underlying ANCA vasculitis resulting in early evolution to constrictive pericarditis. Antibodies directed against proteinase 3 (proteinase 3-ANCA) and myeloperoxidase (myeloperoxidase-ANCA) are highly specific for the diagnosis of systemic vasculitis. However, they have been found as an epiphenomenon of acute parvovirus B-19 infection.5 Although we do not have repeat antibody titers to confirm its biochemical diagnosis, clinical symptoms are highly suspect for a systemic autoimmune disease in this case of granulomatosis with polyangiitis. After immunosuppressive therapy with mycophenolate mofetil and corticosteroids his symptoms subsided with improvement of constrictive physiology on echocardiography. Nonetheless, the bouncing septum and increased pericardial thickening on cardiac magnetic resonance remained.
http://dx.doi.org/10.1016/j.cjca.2015.06.004 0828-282X/Ó 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
395.e12
Canadian Journal of Cardiology Volume 32 2016
Figure 1. (A) Pulsed wave Doppler signal of mitral inflow with > 25% respiratory variation of peak E velocity; (B) tissue Doppler signal with normal medial peak E0 velocities; (C) CD45 immunostaining shows borderline myocardial leukocyte infiltration (arrows); (D) thickened pericardium on diastolic still frame on cine; (E) T2-weighted black-blood turbo spin-echo with fat suppression; and (F) delayed enhancement (DE) images. Visceral (arrows) and parietal pericardium (arrows) show increased signal intensity on (E) T2-weighted and (F) DE images, with focal linear, midwall hyperintensity on the DE images, compatible with acute perimyocarditis.
Disclosures The authors have no conflicts of interest to disclose.
4. Haley JH, Tajik AJ, Danielson GK, et al. Transient constrictive pericarditis: causes and natural history. J Am Coll Cardiol 2004;43:271-5.
References
5. Hermann J, Demel U, Stunzer D, et al. Clinical interpretation of antineutrophil cytoplasmic antibodies: parvovirus B19 infection as a pitfall. Ann Rheum Dis 2005;64:641-3.
1. Cosyns B, Plein S, Nihoyanopoulos P, et al. European Association of Cardiovascular Imaging (EACVI) position paper: multimodality imaging in pericardial disease. Eur Heart J Cardiovasc Imaging 2015;16:12-31. 2. Imazio M. Pericardial involvement in systemic inflammatory diseases. Heart 2011;97:1882-92. 3. Imazio M, Brucato A, Maestroni S, et al. Risk of constrictive pericarditis after acute pericarditis. Circulation 2011;124:1270-5.
Supplementary Material To access the supplementary material accompanying this article, visit the online version of the Canadian Journal of Cardiology at www.onlinecjc.ca and at http://dx.doi.org/10. 1016/j.cjca.2015.06.004.
Images in Cardiology
Perimyocarditis Complicated by Early Development of Constrictive Pericarditis Robert Dennert, MD, PhD,a Sebastian A.F. Streukens, MD,a Suzanne Gommers, MD,b Stephane Heymans, MD, PhD,a and Sebastiaan C.A.M. Bekkers, MD, PhDa a
Department of Cardiology, Maastricht University Medical Center, Maastricht, The Netherlands
b
Department of Radiology, Maastricht University Medical Center, Maastricht, The Netherlands
A 54-year-old man with a history of type 1 diabetes mellitus and hyperthyroidism presented with typical symptoms of pericarditis after an episode of flu-like symptoms. His electrocardiogram revealed diffuse ST-T segment elevation not confined to an arterial territory. Plasmatic levels of cardiac troponin remained within normal values. Treatment with nonsteroidal anti-inflammatory drugs was initiated, but he presented with recurrent symptoms and progressive dyspnea several weeks later. Further evaluation revealed a history of frequent purulent nasal discharge. Urine analysis demonstrated microhematuria. Echocardiography revealed a thickened pericardium without effusion, augmented respiratory variation of mitral inflow (Fig. 1A), normal medial peak E0 velocities (Fig. 1B), and abnormal septal motion during diastole (septal bounce). Cardiac magnetic resonance imaging showed marked pericardial thickening on cine, T1-, T2-weighted, and delayed enhancement (DE) imaging (Fig. 1, D-F), with diastolic septal bounce on cine imaging (see Videos 1 and 2; view videos online) compatible with constriction.1 On T2weighted and DE imaging, the hyperintense visceral and parietal pericardium were clearly discernable. Midwall linear hyperintensity in the basal inferolateral segments complemented the diagnosis of acute perimyocarditis. Endomyocardial biopsies did not show active myocarditis or granuloma formation on histology. Additional immunohistological analysis revealed borderline leukocyte
Received for publication March 13, 2015. Accepted June 4, 2015. Corresponding author: Dr Robert Dennert, Department of Cardiology, Maastricht University Medical Center, P. Debyelaan 25, 6229 HX, Maastricht, The Netherlands. Tel.: þ31-43-3882949; fax: þ31-43-3882952. E-mail: [email protected] See page 395.e12 for disclosure information.
infiltration (13 CD45-positive inflammatory cells per mm2; Fig. 1C) and an increased parvovirus-B19 viral load (319 copies per microgram DNA) in polymerase chain reaction assay. A serology test showed the presence of perinuclearantineutrophil cytoplasmic antibody (ANCA) (titer: 1/ 128) confirmed with proteinase 3-capture enzyme-linked immunosorbent assay and a marked increased soluble interleukin-2 (5587 pg/mL), consistent with granulomatosis with polyangiitis (formerly known as Wegener granulomatosis). Cardiac involvement is not uncommon in systemic inflammatory diseases.2 Pericardial involvement has been especially described in patients with granulomatosis with polyangiitis.3 Evolution to constrictive pericarditis is a feared and rare complication; it has been attributed to recurrent or incessant episodes of pericarditis.4 Development of constrictive pericarditis after a first episode of peri(myo) carditis has not previously been described in patients with granulomatosis with polyangiitis. Our case suggests a parvovirus B-19 infection triggered perimyocarditis. It highlights a secondary exaggerated inflammatory response possibly due to an underlying ANCA vasculitis resulting in early evolution to constrictive pericarditis. Antibodies directed against proteinase 3 (proteinase 3-ANCA) and myeloperoxidase (myeloperoxidase-ANCA) are highly specific for the diagnosis of systemic vasculitis. However, they have been found as an epiphenomenon of acute parvovirus B-19 infection.5 Although we do not have repeat antibody titers to confirm its biochemical diagnosis, clinical symptoms are highly suspect for a systemic autoimmune disease in this case of granulomatosis with polyangiitis. After immunosuppressive therapy with mycophenolate mofetil and corticosteroids his symptoms subsided with improvement of constrictive physiology on echocardiography. Nonetheless, the bouncing septum and increased pericardial thickening on cardiac magnetic resonance remained.
http://dx.doi.org/10.1016/j.cjca.2015.06.004 0828-282X/Ó 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
395.e12
Canadian Journal of Cardiology Volume 32 2016
Figure 1. (A) Pulsed wave Doppler signal of mitral inflow with > 25% respiratory variation of peak E velocity; (B) tissue Doppler signal with normal medial peak E0 velocities; (C) CD45 immunostaining shows borderline myocardial leukocyte infiltration (arrows); (D) thickened pericardium on diastolic still frame on cine; (E) T2-weighted black-blood turbo spin-echo with fat suppression; and (F) delayed enhancement (DE) images. Visceral (arrows) and parietal pericardium (arrows) show increased signal intensity on (E) T2-weighted and (F) DE images, with focal linear, midwall hyperintensity on the DE images, compatible with acute perimyocarditis.
Disclosures The authors have no conflicts of interest to disclose.
4. Haley JH, Tajik AJ, Danielson GK, et al. Transient constrictive pericarditis: causes and natural history. J Am Coll Cardiol 2004;43:271-5.
References
5. Hermann J, Demel U, Stunzer D, et al. Clinical interpretation of antineutrophil cytoplasmic antibodies: parvovirus B19 infection as a pitfall. Ann Rheum Dis 2005;64:641-3.
1. Cosyns B, Plein S, Nihoyanopoulos P, et al. European Association of Cardiovascular Imaging (EACVI) position paper: multimodality imaging in pericardial disease. Eur Heart J Cardiovasc Imaging 2015;16:12-31. 2. Imazio M. Pericardial involvement in systemic inflammatory diseases. Heart 2011;97:1882-92. 3. Imazio M, Brucato A, Maestroni S, et al. Risk of constrictive pericarditis after acute pericarditis. Circulation 2011;124:1270-5.
Supplementary Material To access the supplementary material accompanying this article, visit the online version of the Canadian Journal of Cardiology at www.onlinecjc.ca and at http://dx.doi.org/10. 1016/j.cjca.2015.06.004.