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CLINICAL SPOTLIGHT
Heart, Lung and Circulation (2014) xx, 1–4 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2014.03.004
Combined Mechanism of Refractory Shock in Primary Cardiac Lymphoma: a Rare Dilemma Pairoj Chattranukulchai, MD a,c*, Sarinya Puwanant, MD a,c, Voravut Rungpradubvong, MD a,c, Seri Singhatanadgige, MD Smonporn Boonyaratavej, MD a,c
b,c
,
a
Division of Cardiology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand Division of Cardiovascular and Thoracic Surgery Department of Surgery, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand Cardiac Center, King Chulalongkorn Memorial Hospital, Bangkok, Thailand
b c
Received 31 August 2013; received in revised form 27 January 2014; accepted 2 March 2014; online published-ahead-of-print xxx
Primary cardiac lymphoma is very rare, and usually manifests after the fifth decade of life. The lack of typical manifestations makes it difficult to diagnose at an early stage that can be discovered only by echocardiography. The location of the tumour often results in cardiac compromise, which prevents the delivery of potentially curative therapies. Clinical presentations may depend on flow obstruction, infiltration of adjacent tissues, tumour embolisation, and atrioventricular (AV) disturbances. We report a rare case of primary cardiac lymphoma that presented with clinical signs of shock from two distinct mechanisms. The first mechanism was intermittent complete AV block that was caused by disruption of the electrical conduction system from tumour infiltration in addition to direct mechanical compression of the atrioventricular node by the tumour. The second mechanism, subtotal RV inflow obstruction from the bulky mass contributed to compromising venous return, which played a major role of refractory shock in this case. Keywords
Complete atrioventricular block Shock Primary cardiac lymphoma Echocardiography
Introduction Primary cardiac lymphoma is very rare, and usually manifests after the fifth decade of life [1]. It has been reported in only 1% of primary cardiac tumours and 0.5% of extranodal lymphoma [2]. The lack of typical manifestation makes it difficult to diagnose in the early stage [3] that can be initially discovered only by echocardiography. The delay in diagnosis results in the poor prognosis despite chemotherapy [4]. Clinical presentations may depend on flow obstruction [5], infiltration of adjacent tissues, tumour embolisation, and atrioventricular disturbances [6,7] where the right atrium (RA) is the commonest site involved. The location of the tumour often results in cardiac compromise,
which prevents the delivery of potentially curative therapies. In some rare cases, the patient can present with solitary restrictive cardiomyopathy from extensive infiltration of atypical lymphocytes without demonstrable intracardiac mass [8]. We report a case of primary cardiac lymphoma presented with clinical signs of shock from two distinct mechanisms.
Clinical Presentation A 70 year-old male presented with acute onset of lightheadedness. He reported two-month progressive anorexia and weight loss. He had a history of status post right
*Corresponding author at: Division of Cardiology, Department of Medicine, Faculty of Medicine, Chulalongkorn University and Cardiac Center, King Chulalongkorn Memorial Hospital, Bangkok 10330, THAILAND. Tel.: +6681-617-8138., Email: [email protected] © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
Please cite this article in press as: Chattranukulchai P, et al. Combined Mechanism of Refractory Shock in Primary Cardiac Lymphoma: a Rare Dilemma. Heart, Lung and Circulation (2014), http://dx.doi.org/10.1016/j.hlc.2014.03.004
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Figure 1 (A) Initial electrocardiogram (ECG) demonstrating complete AV block with junctional escape of 52 beats/min. (B) ECG showing the downgrading of complete AV block to Mobitz-I second degree AV block of 92 beats/min. (C) Transthoracic echocardiography unveiling a huge mass (arrow) occupying the right atrium which extending into the right ventricle causing a near total obstruction of tricuspid inflow. LA indicates left atrium; RA, right atrium; LV, left ventricle and RV, right ventricle. (D) Transoesophageal echocardiography revealing a large echogenic mass involving the interatrial septum and protruding in and out between the tricuspid valve (arrow). (E) Computerised tomography demonstrating a non-enhanced hypodensity mass infiltrating from epicardium through endocardium of the right-sided cardiac chamber with marked thickening of the interatrial septum (arrow shows right coronary stent). (F) Intraoperative finding (above) showing the impacted tumour (arrow) in right atrium from which the vast majority of the friable tumour was excised (below). (G) Histological examination demonstrating diffuse proliferation of abnormal large lymphoid cells (hematoxylin and eosin stain). (H) Positive immunohistochemical staining of CD20 (B-cell marker), consistent with B-cell lymphoma.
Please cite this article in press as: Chattranukulchai P, et al. Combined Mechanism of Refractory Shock in Primary Cardiac Lymphoma: a Rare Dilemma. Heart, Lung and Circulation (2014), http://dx.doi.org/10.1016/j.hlc.2014.03.004
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coronary stent implantation due to unstable angina six months ago. His previous echocardiogram at that time was normal. Upon arrival, he was conscious and appeared dehydrated. His blood pressure (BP) was 70/40 mmHg. An electrocardiogram (ECG) showed complete atrioventricular (AV) block (Fig. 1A) with junctional escape of 52 beat/min. Laboratory studies revealed mild anaemia with normal leukocyte series. He had creatinine of 1.4 mg/dl and serum bicarbonate of 16 mmol/L. He was administered one litre of intravenous saline and a full dose of atropine with a trivial improvement of BP to 79/50 mmHg and downgrading of complete AV block to Mobitz-I second degree AV block (Fig. 1B) while junctional rate increased to 92 beat/min. Subsequent central venous pressure (CVP) monitoring discovered an elevation to 28 mmHg. As a result, a dopamine infusion was then administered which did not significantly raise the BP. According to his clinical signs of dehydration, the physician had challenged more intravenous fluid until his systolic BP was eventually maintained above 100 mmHg. A total of 3.5 litres of saline was infused and yet the CVP remained steady at 30 mmHg. While the initial ECG revealed complete AV block that could account for his initial BP and clinical symptoms, it could not explain the persistent hypotension after ventricular rate was raised. It also is unable to clarify the relatively high CVP in a clinically dehydrated patient. To further assess the aetiology of shock in a patient with elevated CVP, transthoracic echocardiography was performed which unexpectedly unveiled a huge mass occupying the RA extending into the right ventricle (RV) causing a near total obstruction of tricuspid inflow (Fig. 1C, supplementary 1). Transoesophageal echocardiography demonstrated a large echogenic mass involving the interatrial septum (IAS) and protruding in and out between the tricuspid valve (Fig. 1D, supplementary 2). Small and underfilled RV with mildly impaired contraction was observed. Computerised tomography demonstrated a non-enhancing hypodensity mass infiltrating the right-sided cardiac chamber from epicardium through endocardium (Fig. 1E). Neither hepatosplenomegaly nor lymphadenopathy was detected. Based on all imaging findings, the oncologist suggested primary cardiac lymphoma; hence dexamethasone was administered intravenously. On the following day, his BP was decreased and difficult to be maintained again. As a consequence, the patient underwent surgery to relieve the RV inflow obstruction. Surgical findings revealed impacted, friable tumour in RA (Fig. 1F) extending into RV where majority of the mass was excised (Fig. 1F). Unfortunately, post-operative echocardiography showed severely impaired RV systolic function leading to profound RV failure that could not be resuscitated. Histological examination showed diffuse proliferation of large lymphoid cells (Fig. 1G), which were positive for B-cell markers (Fig. 1H) compatible with B-cell lymphoma. The
bone marrow study revealed no evidence of lymphoma. This finding together with the absence of peripheral lymphadenopathy and organomegaly on imaging are consistent with a primary cardiac lymphoma.
Discussion According to the literature, the vast majority of primary cardiac lymphoma patients have nonspecific symptoms with a variety of severities, such as progressive shortness of breath, arrhythmia, cardiac tamponade, and symptom of flow obstruction. This patient manifested clinical signs of shock from a combination of two distinct mechanisms. The first one was bradyarrhythmia presenting as an intermittent complete AV block, caused by disruption of electrical conduction system from tumour infiltration in addition to direct mechanical compression to AV node by the bulky tumour. The second mechanism, which played a major role in creating a refractory shock, the subtotal RV inflow obstruction, contributed to compromise venous return that partially responded to a large volume of saline infusion. With regards to the management of this case, even though the systemic corticosteroid was administered rather early in the hospital course, it was apparently ineffective due to biologic characteristics of the mass and the extent of tumour infiltration. The patient underwent surgical removal of the tumour when he became haemodynamically unstable again; unfortunately, it led to the undesirable result of overt postoperative RV failure. Physicians should be aware of right heart obstruction as one of the aetiology of shocks in a clinically dehydrated patient who has discordantly high CVP where the level does not reflect the actual preload condition.
Disclosures We declare no conflict of interest for all authors.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.hlc. 2014.03.004.
References [1] Hsueh SC, Chung MT, Fang R, Lu HF. Primary cardiac lymphoma. J Chin Med Assoc 2006;69:169–74. [2] Patel J, Melly L, Sheppard MN. Primary cardiac lymphoma: B- and T-cell cases at a specialist UK centre. Ann Oncol 2010;21:1041–5. [3] Rolla G, Bertero MT, Pastena G, Tartaglia N, Corradi F, Casabona R. Primary lymphoma of the heart. A case report and review of the literature. Leuk Res 2002;26:117–20. [4] Yuh DD, Kubo SH, Francis GS, Bank A, McDonald KM, Jessurun J. Primary cardiac lymphoma treated with orthotopic heart transplantation: a case report. J Heart Lung Transplant 1994;13:538–42.
Please cite this article in press as: Chattranukulchai P, et al. Combined Mechanism of Refractory Shock in Primary Cardiac Lymphoma: a Rare Dilemma. Heart, Lung and Circulation (2014), http://dx.doi.org/10.1016/j.hlc.2014.03.004
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P. Chattranukulchai et al.
[5] Zakynthinos E, Tassopoulos G, Haritos C, Kitsanta P, Pyros J, Roussos C. Huge biatrial primary cardiac B-cell lymphoma resulting in bilateral atrioventricular valve obstruction. Leuk Lymphoma 2004;45:2339–42. [6] Hwang YY, Fan K, Lam YM, Chan WS, Cheung S, Wang EP. Primary cardiac lymphoma presenting with right heart mass and bradycardia. Ann Hematol 2007;86:685–6.
[7] Miguel CE, Bestetti RB. Primary Cardiac Lymphoma. Int J Cardiol 2011; 149:358–63. [8] Lee GY, Kim WS, Ko YH, Choi JO, Jeon ES. Primary cardiac lymphoma mimicking infiltrative cardiomyopathy. Eur J;1; Heart Fail 2013;15: 589–91.
Please cite this article in press as: Chattranukulchai P, et al. Combined Mechanism of Refractory Shock in Primary Cardiac Lymphoma: a Rare Dilemma. Heart, Lung and Circulation (2014), http://dx.doi.org/10.1016/j.hlc.2014.03.004
CLINICAL SPOTLIGHT
Heart, Lung and Circulation (2014) xx, 1–4 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2014.03.004
Combined Mechanism of Refractory Shock in Primary Cardiac Lymphoma: a Rare Dilemma Pairoj Chattranukulchai, MD a,c*, Sarinya Puwanant, MD a,c, Voravut Rungpradubvong, MD a,c, Seri Singhatanadgige, MD Smonporn Boonyaratavej, MD a,c
b,c
,
a
Division of Cardiology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand Division of Cardiovascular and Thoracic Surgery Department of Surgery, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand Cardiac Center, King Chulalongkorn Memorial Hospital, Bangkok, Thailand
b c
Received 31 August 2013; received in revised form 27 January 2014; accepted 2 March 2014; online published-ahead-of-print xxx
Primary cardiac lymphoma is very rare, and usually manifests after the fifth decade of life. The lack of typical manifestations makes it difficult to diagnose at an early stage that can be discovered only by echocardiography. The location of the tumour often results in cardiac compromise, which prevents the delivery of potentially curative therapies. Clinical presentations may depend on flow obstruction, infiltration of adjacent tissues, tumour embolisation, and atrioventricular (AV) disturbances. We report a rare case of primary cardiac lymphoma that presented with clinical signs of shock from two distinct mechanisms. The first mechanism was intermittent complete AV block that was caused by disruption of the electrical conduction system from tumour infiltration in addition to direct mechanical compression of the atrioventricular node by the tumour. The second mechanism, subtotal RV inflow obstruction from the bulky mass contributed to compromising venous return, which played a major role of refractory shock in this case. Keywords
Complete atrioventricular block Shock Primary cardiac lymphoma Echocardiography
Introduction Primary cardiac lymphoma is very rare, and usually manifests after the fifth decade of life [1]. It has been reported in only 1% of primary cardiac tumours and 0.5% of extranodal lymphoma [2]. The lack of typical manifestation makes it difficult to diagnose in the early stage [3] that can be initially discovered only by echocardiography. The delay in diagnosis results in the poor prognosis despite chemotherapy [4]. Clinical presentations may depend on flow obstruction [5], infiltration of adjacent tissues, tumour embolisation, and atrioventricular disturbances [6,7] where the right atrium (RA) is the commonest site involved. The location of the tumour often results in cardiac compromise,
which prevents the delivery of potentially curative therapies. In some rare cases, the patient can present with solitary restrictive cardiomyopathy from extensive infiltration of atypical lymphocytes without demonstrable intracardiac mass [8]. We report a case of primary cardiac lymphoma presented with clinical signs of shock from two distinct mechanisms.
Clinical Presentation A 70 year-old male presented with acute onset of lightheadedness. He reported two-month progressive anorexia and weight loss. He had a history of status post right
*Corresponding author at: Division of Cardiology, Department of Medicine, Faculty of Medicine, Chulalongkorn University and Cardiac Center, King Chulalongkorn Memorial Hospital, Bangkok 10330, THAILAND. Tel.: +6681-617-8138., Email: [email protected] © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
Please cite this article in press as: Chattranukulchai P, et al. Combined Mechanism of Refractory Shock in Primary Cardiac Lymphoma: a Rare Dilemma. Heart, Lung and Circulation (2014), http://dx.doi.org/10.1016/j.hlc.2014.03.004
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P. Chattranukulchai et al.
Figure 1 (A) Initial electrocardiogram (ECG) demonstrating complete AV block with junctional escape of 52 beats/min. (B) ECG showing the downgrading of complete AV block to Mobitz-I second degree AV block of 92 beats/min. (C) Transthoracic echocardiography unveiling a huge mass (arrow) occupying the right atrium which extending into the right ventricle causing a near total obstruction of tricuspid inflow. LA indicates left atrium; RA, right atrium; LV, left ventricle and RV, right ventricle. (D) Transoesophageal echocardiography revealing a large echogenic mass involving the interatrial septum and protruding in and out between the tricuspid valve (arrow). (E) Computerised tomography demonstrating a non-enhanced hypodensity mass infiltrating from epicardium through endocardium of the right-sided cardiac chamber with marked thickening of the interatrial septum (arrow shows right coronary stent). (F) Intraoperative finding (above) showing the impacted tumour (arrow) in right atrium from which the vast majority of the friable tumour was excised (below). (G) Histological examination demonstrating diffuse proliferation of abnormal large lymphoid cells (hematoxylin and eosin stain). (H) Positive immunohistochemical staining of CD20 (B-cell marker), consistent with B-cell lymphoma.
Please cite this article in press as: Chattranukulchai P, et al. Combined Mechanism of Refractory Shock in Primary Cardiac Lymphoma: a Rare Dilemma. Heart, Lung and Circulation (2014), http://dx.doi.org/10.1016/j.hlc.2014.03.004
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3
coronary stent implantation due to unstable angina six months ago. His previous echocardiogram at that time was normal. Upon arrival, he was conscious and appeared dehydrated. His blood pressure (BP) was 70/40 mmHg. An electrocardiogram (ECG) showed complete atrioventricular (AV) block (Fig. 1A) with junctional escape of 52 beat/min. Laboratory studies revealed mild anaemia with normal leukocyte series. He had creatinine of 1.4 mg/dl and serum bicarbonate of 16 mmol/L. He was administered one litre of intravenous saline and a full dose of atropine with a trivial improvement of BP to 79/50 mmHg and downgrading of complete AV block to Mobitz-I second degree AV block (Fig. 1B) while junctional rate increased to 92 beat/min. Subsequent central venous pressure (CVP) monitoring discovered an elevation to 28 mmHg. As a result, a dopamine infusion was then administered which did not significantly raise the BP. According to his clinical signs of dehydration, the physician had challenged more intravenous fluid until his systolic BP was eventually maintained above 100 mmHg. A total of 3.5 litres of saline was infused and yet the CVP remained steady at 30 mmHg. While the initial ECG revealed complete AV block that could account for his initial BP and clinical symptoms, it could not explain the persistent hypotension after ventricular rate was raised. It also is unable to clarify the relatively high CVP in a clinically dehydrated patient. To further assess the aetiology of shock in a patient with elevated CVP, transthoracic echocardiography was performed which unexpectedly unveiled a huge mass occupying the RA extending into the right ventricle (RV) causing a near total obstruction of tricuspid inflow (Fig. 1C, supplementary 1). Transoesophageal echocardiography demonstrated a large echogenic mass involving the interatrial septum (IAS) and protruding in and out between the tricuspid valve (Fig. 1D, supplementary 2). Small and underfilled RV with mildly impaired contraction was observed. Computerised tomography demonstrated a non-enhancing hypodensity mass infiltrating the right-sided cardiac chamber from epicardium through endocardium (Fig. 1E). Neither hepatosplenomegaly nor lymphadenopathy was detected. Based on all imaging findings, the oncologist suggested primary cardiac lymphoma; hence dexamethasone was administered intravenously. On the following day, his BP was decreased and difficult to be maintained again. As a consequence, the patient underwent surgery to relieve the RV inflow obstruction. Surgical findings revealed impacted, friable tumour in RA (Fig. 1F) extending into RV where majority of the mass was excised (Fig. 1F). Unfortunately, post-operative echocardiography showed severely impaired RV systolic function leading to profound RV failure that could not be resuscitated. Histological examination showed diffuse proliferation of large lymphoid cells (Fig. 1G), which were positive for B-cell markers (Fig. 1H) compatible with B-cell lymphoma. The
bone marrow study revealed no evidence of lymphoma. This finding together with the absence of peripheral lymphadenopathy and organomegaly on imaging are consistent with a primary cardiac lymphoma.
Discussion According to the literature, the vast majority of primary cardiac lymphoma patients have nonspecific symptoms with a variety of severities, such as progressive shortness of breath, arrhythmia, cardiac tamponade, and symptom of flow obstruction. This patient manifested clinical signs of shock from a combination of two distinct mechanisms. The first one was bradyarrhythmia presenting as an intermittent complete AV block, caused by disruption of electrical conduction system from tumour infiltration in addition to direct mechanical compression to AV node by the bulky tumour. The second mechanism, which played a major role in creating a refractory shock, the subtotal RV inflow obstruction, contributed to compromise venous return that partially responded to a large volume of saline infusion. With regards to the management of this case, even though the systemic corticosteroid was administered rather early in the hospital course, it was apparently ineffective due to biologic characteristics of the mass and the extent of tumour infiltration. The patient underwent surgical removal of the tumour when he became haemodynamically unstable again; unfortunately, it led to the undesirable result of overt postoperative RV failure. Physicians should be aware of right heart obstruction as one of the aetiology of shocks in a clinically dehydrated patient who has discordantly high CVP where the level does not reflect the actual preload condition.
Disclosures We declare no conflict of interest for all authors.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.hlc. 2014.03.004.
References [1] Hsueh SC, Chung MT, Fang R, Lu HF. Primary cardiac lymphoma. J Chin Med Assoc 2006;69:169–74. [2] Patel J, Melly L, Sheppard MN. Primary cardiac lymphoma: B- and T-cell cases at a specialist UK centre. Ann Oncol 2010;21:1041–5. [3] Rolla G, Bertero MT, Pastena G, Tartaglia N, Corradi F, Casabona R. Primary lymphoma of the heart. A case report and review of the literature. Leuk Res 2002;26:117–20. [4] Yuh DD, Kubo SH, Francis GS, Bank A, McDonald KM, Jessurun J. Primary cardiac lymphoma treated with orthotopic heart transplantation: a case report. J Heart Lung Transplant 1994;13:538–42.
Please cite this article in press as: Chattranukulchai P, et al. Combined Mechanism of Refractory Shock in Primary Cardiac Lymphoma: a Rare Dilemma. Heart, Lung and Circulation (2014), http://dx.doi.org/10.1016/j.hlc.2014.03.004
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P. Chattranukulchai et al.
[5] Zakynthinos E, Tassopoulos G, Haritos C, Kitsanta P, Pyros J, Roussos C. Huge biatrial primary cardiac B-cell lymphoma resulting in bilateral atrioventricular valve obstruction. Leuk Lymphoma 2004;45:2339–42. [6] Hwang YY, Fan K, Lam YM, Chan WS, Cheung S, Wang EP. Primary cardiac lymphoma presenting with right heart mass and bradycardia. Ann Hematol 2007;86:685–6.
[7] Miguel CE, Bestetti RB. Primary Cardiac Lymphoma. Int J Cardiol 2011; 149:358–63. [8] Lee GY, Kim WS, Ko YH, Choi JO, Jeon ES. Primary cardiac lymphoma mimicking infiltrative cardiomyopathy. Eur J;1; Heart Fail 2013;15: 589–91.
Please cite this article in press as: Chattranukulchai P, et al. Combined Mechanism of Refractory Shock in Primary Cardiac Lymphoma: a Rare Dilemma. Heart, Lung and Circulation (2014), http://dx.doi.org/10.1016/j.hlc.2014.03.004
