- Email: [email protected]
A rare cause of constrictive pericarditis: Primary cardiac lymphoma
International Journal of Cardiology 123 (2008) 208 – 209 www.elsevier.com/locate/ijcard
Letter to the Editor
A rare cause of constrictive pericarditis: Primary cardiac lymphoma Hee Hwa Ho ⁎, On Hing Kwok, Wing Hung Chui, Elaine Wang, Mo Chee Chau, Wing Hing Chow Division of Cardiology, Department of Medicine, University of Hong Kong, Grantham Hospital, Wong Chuk Hang Road, Hong Kong, China Received 16 September 2006; received in revised form 14 November 2006; accepted 17 November 2006 Available online 23 March 2007
Abstract We described a patient who presented with symptoms of heart failure and was found to have diastolic heart failure. Several investigations pointed to the diagnosis of constrictive pericarditis which was later confirmed by cardiac catheterisation. The etiology turns out to be primary cardiac lymphoma which is extremely rare in immunocompetent patients. This is followed by a brief review on the clinical presentation, diagnostic approach and management of primary cardiac lymphoma. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Heart failure; Pericardium; Constrictive pericarditis; Lymphoma
A 65 years old Chinese male with previous history of hypertension, presented with acute onset of dyspnoea and mild chest discomfort. Physical examination showed presence of Kussmaul's sign, basal lung crepitations, ascites and ankle edema. Electrocardiogram showed atrial flutter with a heart rate of 130 beats/min. Chest X-ray showed pulmonary congestion with cardiomegaly. Blood tests revealed normal troponin level with markedly elevated brain natriuretic peptide level of 11,578 pg/ml. Echocardiogram showed normal left ventricular function with ejection fraction of 56%, marked concentric left ventricular hypertrophy and biatrial enlargement. Abnormal appearance of the right ventricular apex with irregular external surface was observed. The pericardium also appeared thickened with mixed echogenecity (Fig. 1A). The hepatic veins and inferior vena cava were also dilated. and showed restricted respiratory fluctuation. Cardiovascular magnetic resonance (MR) imaging showed diffuse thickening of the pericardium with intense enhancement post contrast, suggestive of an ongoing inflammatory process. Computer tomography scan of the thorax failed to demonstrate calcification of the pericardium and there was no mediastinal lymphadenopathy. ⁎ Corresponding author. Tel.: +852 2518 2619; fax: +852 2518 8558. E-mail address: [email protected] (H.H. Ho). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2006.11.159
Based on the clinical, echocardiographic and MR findings, constrictive pericarditis was suspected. Inflammatory markers (erythrocyte sedimentation rate and C reactive protein)
Fig. 1. (A) Apical four chamber view of transthoracic echocardiogram showing the abnormal surface of the right ventricle and pericardium(arrows → ←) RV— right ventricle; RA—right atrium (B) Simultaneous right and left ventricular pressure tracings during cardiac catheterisation (RV—right ventricle pressure tracing in white; LV—left ventricle pressure tracing in black).
H.H. Ho et al. / International Journal of Cardiology 123 (2008) 208–209
Fig. 2. High power microscopic examination of the pericardial biopsy.
were elevated, raising the suspicion of an infectious cause like active caseating tuberculous pericarditis. However, Mantoux test was negative after 48 h of inoculation. He did not complain of fever and had no constitutional symptoms. There was no previous history of cardiac surgery or radiation therapy to the mediastinum. Other differential diagnosis of constrictive pericarditis includes tumour infiltration or autoimmune pathology. Blood tests for tumour markers and autoimmune screen were however negative. His heart failure symptoms were relieved by diuretics and the arrhythmia was controlled with digoxin and isoptin. He subsequently underwent a cardiac catheterisation. Coronary angiogram showed normal coronary vessels. Endomyocardial biopsy from the right ventricle showed mild myocyte hypertrophy with no evidence of amyloid or iron by special stains. Hemodynamics study demonstrated equal and elevated biatrial pressures with blunted x and exaggerated y descent observed on atrial tracings. Simultaneous right and left heart catheterisation also showed equalisation and elevation of right and left ventricular end diastolic pressures (Fig. 1B) with dip and plateau sign. Respiratory discordance between the right and left ventricular peak systolic pressures was observed during inspiration and expiration, confirming the initial suspicion of constrictive pericarditis. He was subsequently referred for pericardiectomy. After median sternotomy, surgical exploration showed diffuse thickening of the anterior aspect of the pericardium with areas of nodularity causing significant compression on the heart. There were no areas of calcification seen. Pericardial biopsy together with some of the suspicious nodules were taken and sent for frozen section examination. Initial frozen section examination showed large cell infiltrates. In view of the high possibility of malignancy with extensive involvement of the pericardium, the intended pericardiectomy was aborted. Microscopic examination of the pericardial tissues showed atypical lymphoid cells of varying sizes and shapes (Fig. 2). Small mature lymphocytes were also present. Immunostaining showed diffuse positive staining for CD20,
209
compatible with the diagnosis of large Bcell lymphoma. He was subsequently referred to the hematologist for further management. Bone marrow biopsies and computer tomography of the thorax and abdomen did not show any involvement of lymphoma. Hence, he was diagnosed to have primary cardiac lymphoma. He was subsequently treated with rituximab and combination chemotherapy (cyclophosphamide, epiribucin, vincristine and prednisolone) and had shown partial response. Primary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma that is exclusively located in the heart and/ or pericardium. It is extremely rare in immunocompetent patients, and more often seen in immunocompromised patients. Antemortem diagnosis of primary cardiac lymphoma has been reported world-wide. [1–6]. Its clinical presentation can be quite variable ranging from heart failure, superior vena caval syndrome, constitutional symptoms, chest pain, arrhythmia, heart block and symptoms related to pericardial effusion/tamponade. Definitive diagnosis is obtained by histological examination of endomyocardial/ pericardial biopsies or cytological examination of pericardial fluid. The treatment of choice is combination chemotherapy and early treatment may alter the clinical course[4–6] although the overall prognosis is poor. To our knowledge, this is the first reported case of primary cardiac lymphoma masquerading as constrictive pericarditis with symptoms of heart failure. Although tumour infiltration is an uncommon cause of constrictive pericarditis, it is important to bear this possibility in mind. As illustrated by the current case, pericardial inspection and frozen section examination should be performed in suspicious cases so that the correct diagnosis can be made without delay and appropriate treatment be administered. References [1] Chalabreysse L, Berger F, Loire R, Devouassoux G, Cordier JF, Thivolet-Bejui F. Primary cardiac lymphoma in immunocompetent patients: a report of three cases and review of the literature. Virchows Arch 2002;441(5):456–61. [2] Fuzellier JF, Saade YA, Torossian PF, Baehrel B. Primary cardiac lymphoma: diagnosis and treatment. Report of 6 cases and review of the literature. Arch Mal Coeur Vaiss 2005;98(9):875–80 Sep. [3] Hirabayashi T, Tanabe M, Onishi K, Tanigawa T, Kitamura T, Yamada N, Ito M, Isaka N, Nakano T. Cardiac malignant lymphoma with atrial arrhythmias. Int J Cardiol 2007;Jan 8;114(2):E42–4. [4] Morillas P, Quiles J, Nunez D, et al. Complete regression of cardiac nonHodgkin lymphoma. Int J Cardiol 2006;106(3):426–7. [5] Kosugi M, Ono T, Yamaguchi H, Sato N, Dan K, Tanaka K, Takano T. Successful treatment of primary cardiac lymphoma and pulmonary tumor embolism with chemotherapy. Int J Cardiol 2006;111(1):172–3 Jul 28. [6] Sato Y, Matsumoto N, Kinukawa N, et al. Successful treatment of primary cardiac B-cell lymphoma: depiction at multislice computed tomography and magnetic resonance imaging. Int J Cardiol 2006;113 (1):E26–9.
Letter to the Editor
A rare cause of constrictive pericarditis: Primary cardiac lymphoma Hee Hwa Ho ⁎, On Hing Kwok, Wing Hung Chui, Elaine Wang, Mo Chee Chau, Wing Hing Chow Division of Cardiology, Department of Medicine, University of Hong Kong, Grantham Hospital, Wong Chuk Hang Road, Hong Kong, China Received 16 September 2006; received in revised form 14 November 2006; accepted 17 November 2006 Available online 23 March 2007
Abstract We described a patient who presented with symptoms of heart failure and was found to have diastolic heart failure. Several investigations pointed to the diagnosis of constrictive pericarditis which was later confirmed by cardiac catheterisation. The etiology turns out to be primary cardiac lymphoma which is extremely rare in immunocompetent patients. This is followed by a brief review on the clinical presentation, diagnostic approach and management of primary cardiac lymphoma. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Heart failure; Pericardium; Constrictive pericarditis; Lymphoma
A 65 years old Chinese male with previous history of hypertension, presented with acute onset of dyspnoea and mild chest discomfort. Physical examination showed presence of Kussmaul's sign, basal lung crepitations, ascites and ankle edema. Electrocardiogram showed atrial flutter with a heart rate of 130 beats/min. Chest X-ray showed pulmonary congestion with cardiomegaly. Blood tests revealed normal troponin level with markedly elevated brain natriuretic peptide level of 11,578 pg/ml. Echocardiogram showed normal left ventricular function with ejection fraction of 56%, marked concentric left ventricular hypertrophy and biatrial enlargement. Abnormal appearance of the right ventricular apex with irregular external surface was observed. The pericardium also appeared thickened with mixed echogenecity (Fig. 1A). The hepatic veins and inferior vena cava were also dilated. and showed restricted respiratory fluctuation. Cardiovascular magnetic resonance (MR) imaging showed diffuse thickening of the pericardium with intense enhancement post contrast, suggestive of an ongoing inflammatory process. Computer tomography scan of the thorax failed to demonstrate calcification of the pericardium and there was no mediastinal lymphadenopathy. ⁎ Corresponding author. Tel.: +852 2518 2619; fax: +852 2518 8558. E-mail address: [email protected] (H.H. Ho). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2006.11.159
Based on the clinical, echocardiographic and MR findings, constrictive pericarditis was suspected. Inflammatory markers (erythrocyte sedimentation rate and C reactive protein)
Fig. 1. (A) Apical four chamber view of transthoracic echocardiogram showing the abnormal surface of the right ventricle and pericardium(arrows → ←) RV— right ventricle; RA—right atrium (B) Simultaneous right and left ventricular pressure tracings during cardiac catheterisation (RV—right ventricle pressure tracing in white; LV—left ventricle pressure tracing in black).
H.H. Ho et al. / International Journal of Cardiology 123 (2008) 208–209
Fig. 2. High power microscopic examination of the pericardial biopsy.
were elevated, raising the suspicion of an infectious cause like active caseating tuberculous pericarditis. However, Mantoux test was negative after 48 h of inoculation. He did not complain of fever and had no constitutional symptoms. There was no previous history of cardiac surgery or radiation therapy to the mediastinum. Other differential diagnosis of constrictive pericarditis includes tumour infiltration or autoimmune pathology. Blood tests for tumour markers and autoimmune screen were however negative. His heart failure symptoms were relieved by diuretics and the arrhythmia was controlled with digoxin and isoptin. He subsequently underwent a cardiac catheterisation. Coronary angiogram showed normal coronary vessels. Endomyocardial biopsy from the right ventricle showed mild myocyte hypertrophy with no evidence of amyloid or iron by special stains. Hemodynamics study demonstrated equal and elevated biatrial pressures with blunted x and exaggerated y descent observed on atrial tracings. Simultaneous right and left heart catheterisation also showed equalisation and elevation of right and left ventricular end diastolic pressures (Fig. 1B) with dip and plateau sign. Respiratory discordance between the right and left ventricular peak systolic pressures was observed during inspiration and expiration, confirming the initial suspicion of constrictive pericarditis. He was subsequently referred for pericardiectomy. After median sternotomy, surgical exploration showed diffuse thickening of the anterior aspect of the pericardium with areas of nodularity causing significant compression on the heart. There were no areas of calcification seen. Pericardial biopsy together with some of the suspicious nodules were taken and sent for frozen section examination. Initial frozen section examination showed large cell infiltrates. In view of the high possibility of malignancy with extensive involvement of the pericardium, the intended pericardiectomy was aborted. Microscopic examination of the pericardial tissues showed atypical lymphoid cells of varying sizes and shapes (Fig. 2). Small mature lymphocytes were also present. Immunostaining showed diffuse positive staining for CD20,
209
compatible with the diagnosis of large Bcell lymphoma. He was subsequently referred to the hematologist for further management. Bone marrow biopsies and computer tomography of the thorax and abdomen did not show any involvement of lymphoma. Hence, he was diagnosed to have primary cardiac lymphoma. He was subsequently treated with rituximab and combination chemotherapy (cyclophosphamide, epiribucin, vincristine and prednisolone) and had shown partial response. Primary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma that is exclusively located in the heart and/ or pericardium. It is extremely rare in immunocompetent patients, and more often seen in immunocompromised patients. Antemortem diagnosis of primary cardiac lymphoma has been reported world-wide. [1–6]. Its clinical presentation can be quite variable ranging from heart failure, superior vena caval syndrome, constitutional symptoms, chest pain, arrhythmia, heart block and symptoms related to pericardial effusion/tamponade. Definitive diagnosis is obtained by histological examination of endomyocardial/ pericardial biopsies or cytological examination of pericardial fluid. The treatment of choice is combination chemotherapy and early treatment may alter the clinical course[4–6] although the overall prognosis is poor. To our knowledge, this is the first reported case of primary cardiac lymphoma masquerading as constrictive pericarditis with symptoms of heart failure. Although tumour infiltration is an uncommon cause of constrictive pericarditis, it is important to bear this possibility in mind. As illustrated by the current case, pericardial inspection and frozen section examination should be performed in suspicious cases so that the correct diagnosis can be made without delay and appropriate treatment be administered. References [1] Chalabreysse L, Berger F, Loire R, Devouassoux G, Cordier JF, Thivolet-Bejui F. Primary cardiac lymphoma in immunocompetent patients: a report of three cases and review of the literature. Virchows Arch 2002;441(5):456–61. [2] Fuzellier JF, Saade YA, Torossian PF, Baehrel B. Primary cardiac lymphoma: diagnosis and treatment. Report of 6 cases and review of the literature. Arch Mal Coeur Vaiss 2005;98(9):875–80 Sep. [3] Hirabayashi T, Tanabe M, Onishi K, Tanigawa T, Kitamura T, Yamada N, Ito M, Isaka N, Nakano T. Cardiac malignant lymphoma with atrial arrhythmias. Int J Cardiol 2007;Jan 8;114(2):E42–4. [4] Morillas P, Quiles J, Nunez D, et al. Complete regression of cardiac nonHodgkin lymphoma. Int J Cardiol 2006;106(3):426–7. [5] Kosugi M, Ono T, Yamaguchi H, Sato N, Dan K, Tanaka K, Takano T. Successful treatment of primary cardiac lymphoma and pulmonary tumor embolism with chemotherapy. Int J Cardiol 2006;111(1):172–3 Jul 28. [6] Sato Y, Matsumoto N, Kinukawa N, et al. Successful treatment of primary cardiac B-cell lymphoma: depiction at multislice computed tomography and magnetic resonance imaging. Int J Cardiol 2006;113 (1):E26–9.