Björn Chapuy

Claudia Dellas

Gerd Hasenfuß

Mark Hünlich

Stefan Schweyer

PubMed

Springer - Publisher Connector

CLINICAL CORRESPONDENCE
A rare cause of sudden cardiac arrest: primary cardiac lymphoma
Claudia Dellas Æ Bjo¨rn Chapuy Æ Stefan Schweyer Æ
Gerd Hasenfuß Æ Mark Hu¨nlich
Received: 21 April 2009 / Accepted: 20 May 2009 / Published online: 5 June 2009
 Springer-Verlag 2009
Sirs,
Sudden cardiac arrest is a leading cause of death in
developed nations and about 40% of victims present with
ventricular fibrillation at the time of first heart rhythm
analysis [4]. Acute myocardial ischemia is generally con-
sidered to be the most common factor triggering fatal
arrhythmias [5]. We report a rare case of sudden cardiac
arrest due to ventricular fibrillation in a patient in whom
diagnostic follow-up led to the diagnosis of a primary
cardiac lymphoma (PCL).
Case report
A 56-year-old Caucasian man was resuscitated at home for
25 min for ventricular fibrillation. Following the admission
to our intensive care unit, he was sedated, intubated and
mechanically ventilated. The initial laboratory measure-
ment revealed a slightly diminished potassium (3.0 mmol/
l), a positive troponin T value, but normal values for
sodium, magnesium, renal function and creatinine kinase.
The echocardiogram showed normal left and right ven-
tricular function and no valvular heart disease. However,
an approximately 4 9 3 cm mass was detected in the apex
of the right ventricle which was confirmed by contrast-
enhanced chest tomography (Fig. 1). Owing to slightly
increasing values of cardiac enzymes, hemodynamic
instability and T-wave inversion in the ECG, the patient
was investigated by coronary angiography leading to the
diagnosis of one-vessel coronary artery disease without the
need of intervention. Angiography of the right ventricle
showed an intraventricular filling defect following the
injection of contrast medium (Fig. 1). Two biopsies were
taken from the RV mass and the final diagnosis was diffuse
large B cell lymphoma (DLBCL) with extranodal mani-
festation at the right ventricular apex, stadium I EB,
international prognostic index of 1. The subsequent clinical
course was uncomplicated and the patient was treated with
six cycles of an anti-CD20/anthracycline-based poly-
chemotherapy (rituxan, cyclophosphamide, doxorubicin,
vincristine, and prednisone every 14 days) without radia-
tion. After completing the last course of chemotherapy,
echocardiography and CT could demonstrate a significant
tumor size regression (Fig. 1). After 14 months of com-
pleting chemotherapy, a residual structure in the right
ventricle of approximately 9 9 11 mm was found to be
stable by echocardiography and except for chemotherapy-
related low-grade peripheral polyneuropathia, the patient is
otherwise in good clinical shape and free of symptoms.
Discussion
Primary cardiac tumors are extremely rare and only a
quarter of all cardiac tumors are malignant; 95% of these
are sarcomas, 5% are lymphomas [10]. PCLs are defined as
C. Dellas (&)  G. Hasenfuß  M. Hu¨nlich
Department of Cardiology and Pulmonology,
Georg August University of Goettingen,
37099 Goettingen, Germany
e-mail: claudia.dellas@t-online.de
B. Chapuy
Department of Hematology and Oncology,
Georg August University of Goettingen,
37099 Goettingen, Germany
S. Schweyer
Department of Pathology,
Georg August University of Goettingen,
37099 Goettingen, Germany
123
Clin Res Cardiol (2009) 98:509–511
DOI 10.1007/s00392-009-0034-0
non-Hodgkin’s lymphomas of different histology only
involving the heart or the pericardium and usually arise in
the right chambers of the heart [3]. The majority of patients
presents with symptoms of right heart failure [6].
Arrhythmias are mostly described as atrial flatter or
fibrillation, but lymphoma infiltration can also cause con-
duction disturbances. However, ventricular fibrillation is
rarely described in the literature. Only one case of sudden
death leading to the post mortem diagnosis of PCL and one
case of ventricular tachycardia are documented in a review
Fig. 1 Upper left panel:
transthoracic echocardiogram
shows an echodense structure in
the right ventricle (arrows).
Upper right panel: right
ventricular angiography
demonstrating an
intraventricular filling defect
after injection of contrast
medium. Middle panel:
contrast-enhanced computed
tomography demonstrating an
intraventricular filling defect
after the injection of contrast
material at the time of diagnosis
(left) and after completing the
last course of chemotherapy
(right). Lower panel: histologic
examination of the cardiac
tumor biopsy showed sheets of
large lymphoid cell admixed
with small reactive lymphocytes
[hematoxylin and eosin (a) and
Giemsa (b) stains; 9400
magnification].
Immunohistochemistry
demonstrated CD20 expression
(c) on neoplastic cells. The
proliferation rate was up to 80%
(d 9400 magnification)
510 Clin Res Cardiol (2009) 98:509–511
123
of 35 patients between 1960 and 2000 [2]. In our patient,
the PCL was localized in the right ventricle. Infiltration of
the myocardium and/or conduction system most likely
induced ventricular fibrillation. Echocardiography repre-
sents a very sensitive non-invasive technique for the
identification of intracardial tumors, in particular as a first
diagnostic approach [8, 9]. Up to 60% of PCLs are found
by transthoracic echocardiography and 97–100% by the
transesophageal approach [3]. Most PCLs are of B cell
lineage and about two-third is diagnosed as DLBCLs [2, 6].
A large study-based evaluation for the optimal therapeutic
option is lacking due to the rarity and heterogeneous his-
tology of PCLs. Ceresoli suggested that PCLs should be
treated like bulky aggressive lymphomas arising in other
primary sites [1]. In a review of 40 cases from 1995 to
2002, chemotherapy alone was applied in 57.5% and in
combination with radiation in 12.5% of patients [6].
Antracycline-based chemotherapy resulted in 61% of
complete remission (mean follow-up 17 months), whereas
surgery did not seem to improve survival at all [1]. Despite
the treatment, approximately 60% of patients died within
2–4 months after diagnosis [2, 6]. Of note, to date the
standard treatment of any DLBCL is an anti-CD20/antra-
cycline-based chemotherapy. Generally, survived cardiac
arrest due to ventricular fibrillation is a class I indication
with evidence level A for an implantable cardioverter
defibrillator (ICD) [7]. However, PCLs per se are associ-
ated with a high-mortality rate within the first months after
diagnosis. On the other hand, complete remission should
abolish the arrhythmogenic substrate. In case of lymphoma
recurrence, prognosis would be very limited. Therefore, we
decided against the implantation of an ICD, even if another
episode of ventricular fibrillation may occur.
In conclusion, PCL can be the cause of sudden cardiac
arrest due to ventricular fibrillation. In our patient, an anti-
CD20/antracycline-based chemotherapy led to complete
remission (unconfirmed, CRu), and no relapse 14 months
after the initial diagnosis. This case supports that PCL
should be treated like bulky aggressive lymphomas arising
in other primary sites.
References
1. Ceresoli GL, Ferreri AJ, Bucci E, Ripa C, Ponzoni M, Villa E
(1997) Primary cardiac lymphoma in immunocompetent patients:
diagnostic and therapeutic management. Cancer 80:1497–1506
2. Chalabreysse L, Berger F, Loire R, Davouassoux G, Cordier JF,
Thivolet-Bejui F (2002) Primary cardiac lymphoma in immuno-
competent patients: a report of three cases and review of the
literature. Virchows Arch 441:456–461
3. Faganello G, Belham M, Thaman R, Blundell J, Eller T, Wilde P
(2007) A case of primary cardiac lymphoma: analysis of the role
of echocardiography in early diagnosis. Echocardiography
24:889–892
4. Handley AJ, Koster R, Monsieurs K, Perkins GD, Davies S,
Bossaert L (2005) European Resuscitation Council guidelines for
resuscitation 2005. Section 2. Adult basic life support and use
of automated external defibrillators. Resuscitation 67(Suppl 1):
S7–S23
5. Huikuri HV, Castellanos A, Myerburg RJ (2001) Sudden death
due to cardiac arrhythmias. N Engl J Med 345:1473–1482
6. Ikeda H, Nakamura S, Nishimaki H, Masuda K, Takeo T, Kasai
K, Ohashi T, Sakamoto N, Wakida Y, Itoh G (2004) Primary
lymphoma of the heart: case report and literature review. Pathol
Int 54:187–195
7. Jung W, Andresen D, Block M, Bo¨cker D, Hohnloser SH, Kuck
K-H, Sperzel J (2006) Leitlinien zur Implantation von Defibril-
latoren. Clin Res Cardiol 95:696–708
8. Kober G, Magedanz A, Mohrs O, Nowak B, Scherer D, Bug R,
Voigtla¨nder T (2007) Non-invasive diagnosis of a pedunculated
left ventricular hemangioma: tumor classification and evaluation
of relevant literature. Clin Res Cardiol 96:227–231
9. Schwab J, Haack G, Sinss D, Ba¨r I, Zahn R (2007) Diagnosis of
left ventricular myxoma with cardiac magnetic resonance imag-
ing. Clin Res Cardiol 96:189–190
10. Shapiro LM (2001) Cardiac tumours: diagnosis and management.
Heart 85:218–222
Clin Res Cardiol (2009) 98:509–511 511
123


A rare cause of sudden cardiac arrest: primary cardiac lymphoma

Dellas, Claudia

Chapuy, Bjoern

Schweyer, Stefan

Hasenfuß, Gerd

Huenlich, Mark

GRO.publications (Univ. Göttingen)

A case of primary cardiac lymphoma: analysis of the role of echocardiography in early diagnosis.

Cardiac tumours: diagnosis and management.

Diagnosis of left ventricular myxoma with cardiac magnetic resonance imaging.

European Resuscitation Council guidelines for resuscitation 2005. Section 2. Adult basic life support and use of automated external deﬁbrillators. Resuscitation 67(Suppl 1): S7–S23

Primary cardiac lymphoma in immunocompetent patients: a report of three cases and review of the literature.

Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management.

Primary lymphoma of the heart: case report and literature review.

Sudden death due to cardiac arrhythmias.

Voigtla ¨nder T

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A rare cause of sudden cardiac arrest: primary cardiac lymphoma

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