Download Superior Vena Cava Syndrome Caused by Right Atrial

Superior vena cava syndrome caused by heart tumor
71
Superior Vena Cava Syndrome Caused by Right Atrial
Carcinosarcoma in Emergency Department:
A Case Report and Literature Review
Kuo-Hsin Lee1,2, Cheuk-Kwan Sun1,2, I-Ting Tsai1,2
Superior vena cava syndrome, which comprises a group of nonspecific signs and symptoms caused by
obstruction of the superior vena cava (SVC), is usually a complication of SVC compression by pulmonary
or mediastinal tumors. Usual manifestations including shortness of breath, dyspnea, headache, and facial
edema occur only after days to weeks after the beginning of SVC obstruction. Although a detailed medical
history and physical examination may shed light on the diagnosis, imaging studies are usually required for
establishing the diagnosis and pinpointing the causes. Herein, we report a case of facial edema resulting
from obstruction of superior vena cava caused by right atrial carcinosarcoma with initial presentation
mimicking anaphylaxis in the emergency department. Final diagnosis was made one month later after
disease progression. The patient died of distant brain metastasis six months later despite aggressive
treatment. For early diagnosis, emergency physician should have a high level of suspicion for non-specific
symptoms hinting the diagnosis. Chest x-ray and contrast-enhanced chest computed tomography should be
considered for differential diagnosis.
Key words: superior vena cava syndrome, cardiac carcinosarcoma
Introduction
Superior vena cava syndrome (SVCS) result-
ing from external compression or intra-luminal obstruction of the superior vena cava with subsequent
engorgement of the collateral vessel of the chest,
neck, and head is a group of nonspecific signs and
symptoms including facial flushing and edema,
dyspnea, and cough. In addition, strider, nasal congestion, epistaxis, tongue swelling, hoarseness, dysphagia, visual disturbances, headache, and periorbital edema have also been described(1,2). SVCS is a
complication of many disease conditions including
malignancies and hematological disorders as well as
iatrogenic causes such as placement of transvenous
pacemaker and central venous catheters(3). Early
diagnosis of SVCS might be difficult in a patient
without known risk factors. Besides, superior vena
cava obstructed by primary cardiac tumors is rare in
the literature. We report a case of SVCS caused by
right atrial carcinosarcoma with initial presentations
of facial erythematous edema and congestive heart
failure. Definite diagnosis was made one month after his first visit to the emergency department.
Case Report
A 74-year-old man presented to our emergency
Received: November 22, 2011 Accepted for publication: June 13, 2012
From the 1Department of Emergency Medicine, E-Da Hospital, Kaohsiung, Taiwan, 2I-Shou University, Kaohsiung, Taiwan
Address reprint requests and correspondence: Dr. I-Ting Tsai
Department of Emergency Medicine, E-Da Hospital
1 Yida Road, Jiaosu Village, Yanchao District, Kaohsiung City 82445, Taiwan (R.O.C.)
Tel: (07)6150011 ext 3166 Fax: (07)6150915
E-mail: [email protected]
72
J Emerg Crit Care Med. Vol. 23, No. 2, 2012
department with complaint of acute-onset fever up
to 39℃. According to his history, mild exertion
corticosteroid was administrated and epinephrine
was inhaled. He was discharged after partial im-
eyelids were noted for 2 weeks. He had visited a local hospital for initial help where upper respiratory
for intermittent recurrent facial flushing, dyspnea,
and cough. Fever up to38.6℃ was noted with a
dyspnea with intermittent noisy breathing sound,
facial flushing with edema, and bilateral puffy
tract infection was diagnosed and oral medication
was prescribed. He was borderline-hypertensive
and mildly hyperthermic with a blood pressure of
141/82 mmHg, body temperature of 37.2℃, pulse
rate of 91 beats per minute, and respiratory rate
of 18 per minute. Physical examination showed
nonspecific findings except facial edema and
flushing. Initial laboratory study showed normal
white blood cell count (9.6 × 103 per cubic millimeter), mildly elevated C-reactive protein level
(29.1 mg/L) with normal liver and renal function
tests. Under the impression of anaphylaxis and angioedema based on his facial edema, respiratory
symptoms, and history of medication, intravenous
Fig. 1
provement of dyspnea and facial edema. Seven days
later, he came back to our emergency department
blood pressure of 109/67 mmHg, pulse rate of 93
beats per minute, respiratory rate of 21 per minute.
Physical examination revealed facial swelling with
jugular vein engorgement, systolic murmur and
gallop, decreased bilateral breathing sound with
basal rales. No abdominal distension or edema
over lower extremities was found. Laboratory data
displayed only moderately elevated C-reactive pro-
tein level (79.4 mg/L). Chest x-ray showed mild
bilateral pleural effusions, borderline cardiomegaly
and superior mediastinal widening (Fig. 1). The
diagnosis was facial cellulites, highly suspicious of
congestive heart failure. He was discharged with
oral antibiotics and diuretics. One month after his
Erect chest x-ray showing widening of superior mediastinum (white
arrows) and bilateral mild pleural effusions (black arrowheads)
Superior vena cava syndrome caused by heart tumor
73
first presentation to our emergency department,
he came to our emergency department again for
ponents over right atrium. Contrast-enhanced chest
computed tomography scan showed masses in the
plete ECG showed sinus rhythm with low voltage
on limb leads. Echocardiography was arranged for
opacification of collateral veins, compatible with
superior vena cava syndrome. No space-occupying
persistent dyspnea, intermittent facial edema, and
exacerbation of bilateral pleural effusions. Com-
the impression of congestive heart failure. Echocardiography showed a huge mass with cystic com-
Fig. 2
Fig. 3
right atrium, right ventricle, superior vena cava, and
right internal jugular vein with engorgement and
lesion was noted over bilateral pulmonary arteries (Fig. 2 & Fig. 3). To prevent embolic event,
Axial contrast-enhanced CT of the chest demonstrating a right
atrium mass (black arrow), opacification of hemi-azygous vein with
dilatation (black arrowhead), bilateral pleural effusions and pericardial effusions at the atrial level
A superior vena cava mass (white arrow) together with opacification
of venous collaterals (white arrowheads) at the level of aortic arch
74
J Emerg Crit Care Med. Vol. 23, No. 2, 2012
emergent operation for removal of tumors in right
atrium, right ventricle, and superior vena cava
by contrast-enhanced chest computed tomography
scan requires at least two key findings, including
Repeated Contrast-enhanced chest computed tomography scan two months postoperatively showed
erals on the other(9).
Right atrial tumor, which was identified in
was performed. Pathological analysis of the tumor
masses confirmed the diagnosis of carcinosarcoma.
tumor recurrence in right atrium with extension into
mediastinum. The patient expired four months after operation due to increased intracranial pressure
caused by brain metastasis.
Discussion
We described a patient of SVCS with a number of apparently unrelated non-specific symptoms
and signs that posed a challenge to the emergency
physician in differential diagnosis. For instance, increased work of breathing and dilated jugular vein
on lying down resulting from the increase in venous
return and jugular venous pressure can mimic congestive heart failure or pericardial disease. Facial
and neck flushing with edema might be diagnosed
as a dermatological or otolaryngeal condition such
as idiopathic angioedema, facial cellulites, or deep
neck infection(4). On the other hand, anaphylaxis
may also present with facial flushing and edema,
periorbital edema, or stridor that is difficult to distinguish from SVCS with unknown risk factors(5).
The majority of patients with SVCS have an abnormal chest radiograph, with the most common
findings being mediastinal widening and pleural effusion(6). However, the widened upper mediastinum
without trachea deviation as well as the abnormal
cardiac contour and mass density in the plain chest
film of our patient may be interpreted as tortuous
great vessels. On the other hand, contrast-enhanced
chest computed tomography scan has the advantages of being non-invasive, rapid, and able to precisely identify the location and degree of superior vena
cava/brachiocephalic venous obstruction as well as
the extent of lesion(7,8). Clinical diagnosis of SVCS
the lack of opacification of the superior vena cava
on the one hand and opacification of venous collat-
our patient by echocardiography, is a rare cause of
SVCS (10). Moreover, since typical cardiovascular
signs and symptoms of right atrium tumor are those
of right heart failure (i.e. fatigue, peripheral edema,
hepatomegaly, ascites, and prominent "a waves" in
jugular venous pulses), its presentation initially as
SVCS but not right heart failure is uncommon(11).
Left ventricular inflow obstruction, right heart failure, pulmonary emboli, systemic embolic event,
dysrhythmia, conduction blockade have been reported to be fatal complications associated with
cardiac tumors(12). Unrecognized SVCS caused by
tumor occupation of right atrium and superior vena
cava delayed diagnosis of the underlying disease
may lead to worsened outcome of our patient. The
symptoms and signs of cardiac tumors are also
highly variable and tumor plop sound by auscultation may be the only physical finding. Cardiac carcinosarcoma was a rare but aggressive malignancy
that has only been sporadically reported. To our
best knowledge, SVCS caused by cardiac carcinosarcoma has not been reported in the literature.
As in most cases of cardiac tumor, the diagnosis of
malignancy is not made preoperatively. The aims
of operation are to relieve ventricular inflow obstruction, prevent embolism, and obtain tissue for
diagnosis. In general, sarcomas proliferate rapidly
and cause death through widespread infiltration of
the myocardium, obstruction of blood flow through
the heart, and distant metastases. Although complete
resection is the treatment of choice, most patients develop recurrent disease and eventually succumb even
after complete tumor resection. The median survival
is typically 6 to 12 months, although long-term survival has been reported with complete resection(13).
Superior vena cava syndrome caused by heart tumor
Conclusion
High level of vigilance towards typical symptoms and signs and an incidental finding on chest
radiograph such as mediastinal widening are keys to
the diagnosis of SVCS. Our report described a patient with initial presentation of a number of seemingly nonspecific signs and symptoms that delayed
the diagnosis. Suspicious picture on chest radiograph in the emergency department may warrant the
performance of contrast-enhanced chest computed
tomography scan for early differential diagnosis.
References
1. Wi l s o n L D, D e t t e r b e c k F C, Ya h a l o m J.
Clinical practice. Superior vena cava syndrome
w i t h m a l i g n a n t c a u s e s. N E n g l J M e d
2007;356:1862-9.
2. Rice TW, Rodriquez RM, Light RW. The superior vena cava syndrome: clinical characteristics and evolving etiology. Medicine (Baltimore) 2006;85:37-42.
3. Bell DR, Woods RL, Levi JA. Superior vena
cava obstruction: a 10-years experience. Med J
Aust 1986;145:566-8.
4. Kim KM, Park CS, Kim TB, Cho YS, Moon
HB. A variant of idiopathic angioedema presenting as persistent facial swelling over 1
year. Allergy 2008;63:378-9.
5. Khalili B, Boggs PB, Bahna SL, Marsala AJ,
Tapolyai M, Bryn RO. Superior vena cava syndrome: A masquerader of anaphylaxis. J Allergy
6.
7.
8.
9.
10.
11.
12.
13.
75
Clin Immunol 2007;119(Supple 1):S29. (Abst)
Rice TW, Rodriguez RM, Barnette R, Light
RW. Prevalence and characteristics of pleural
effusions in superior vena cava syndrome. Respirology 2006;11:299-305.
Kim HJ, Kim HS, Chung SH. CT diagnosis
of superior vena cava syndrome: importance
of collateral vessels. AJR Am J Roentgenol
1993;161:539-42.
Qanadli SD, El Hajjam M, Bruckert F, et
al. Helical CT phlebography of the superior vena cava: diagnosis and evaluation of
venous obstruction. AJR Am J Roentgenol
1999;172:1327-33.
Bechtold RE, Wolfman NT, Karstaedt N, Choplin RH. Superior vena caval obstruction: detection using CT. Radiology 1985;157:485-7.
Ramnarine IR, Davidson L, van Doorn CA.
Primary cardiac carcinosarcoma: a rare, aggressive tumor. Ann Thorac Surg 2001;72:927-9.
Kuon E, Kreplin M, Weiss W, Dahm JB. The
challenge presented by right atrial myxoma.
Herz 2004;29:702-9.
Sughimoto K, Shiikawa A, Ohkado A, Nanaumi M. Multiple cardiac myxomas with pulmonary arterial obstruction and acute right
heart failure. Jpn J Thorac Cardiovasc Surg
2004;52:530-3.
Zhang PJ, Brooks JS, Goldblum JR, et al. Primary cardiac sarcomas: a clinicopathologic
analysis of a series with follow-up information
in 17 patients and emphasis on long-term survival. Hum Pathol 2008;39:1385-95.
76
J Emerg Crit Care Med. Vol. 23, No. 2, 2012
延遲診斷由右心房癌肉瘤所引起的
上腔靜脈症候群：急診室個案報告
李國新1,2　孫灼均1,2　蔡易廷1,2
上腔靜脈症候群的臨床表現通常是一群非特異性的症狀其典型表現像臉部頸部紅腫，呼吸喘及咳嗽
都是急診室裡常見的主訴。上腔靜脈症候群症狀可能都持續一段時間且需要理學檢查和詳細的病史來與
其他疾病作鑑別診斷。我們報告一個由右心房癌肉瘤引起上腔靜脈症侯群的病例在急診的臨床表現類似
過敏反應，一個月後才確定診斷。儘管積極的治療病人最終半年後病人還是死於遠處腦部轉移。像這種
懷疑上腔靜脈症候群的情形，胸部X光和胸部顯影電腦段層應及早於急診室中安排作為鑑別診斷的工具
是有價值的。
關鍵字： 上腔靜脈症候群，心臟癌肉瘤
收件：100年11月22日　接受刊載：101年6月13日
1
義大醫院急診醫學部　2義守大學
通訊及抽印本索取：蔡易廷醫師　高雄市燕巢區角宿里義大路1號
義大醫院急診醫學部
電話：(07)6150011轉3166　傳真：(07)6150915
E-mail: [email protected]