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Copyright: © 2016 Kyaw H, et al.
Journal of Heart and Circulation
Case Report
Open Access
Ginormous Coronary Sinus with Persistent Left Superior Vena Cava
Htoo Kyaw1*, Atif Z. Shaikh1,2 and Misra Deepika1,2
Division of Cardiology, Mount Sinai Beth Israel Hospital Center, 10 Nathan D Perlman Pl, New York, NY 10003, USA
2
Department of Cardiology,The Brooklyn Hospital Center, Academic Affiliate of The Icahn School of Medicine at Mount Sinai, Clinical Affiliate of The Mount
Sinai Hospital, 121 Dekalb Avenue, Brooklyn, New York 11201, USA
1
Received Date: August 20, 2016, Accepted Date: November 22, 2016, Published Date: November 30, 2016.
*Corresponding author: Htoo Kyaw, Division of Cardiology, Mount Sinai Beth Israel Hospital Center, 10 Nathan D Perlman Pl, New York, NY 10003, USA,
Tel: 323-303-7398; E-mail: [email protected].
Abstract
An echocardiogram is the most common diagnostic imaging done
in chest pain evaluation workup. Persistent left superior vena cava
is a rare thoracic congenital anomaly but commonly reported one in
the literature. Dilated coronary sinus on echocardiogram is a signal
to think of persistent left superior vena cava which can be presented
with atypical chest pain and palpitation but is often asymptomatic. As
a catheter from left subclavian approach can incidentally advance to the
right side of the heart, early awareness of PLSVC is crucial to prevent
unwanted procedure related complications.
Keywords: Chest pain; Persistent Left Superior Vena Cava;
Congenital venous anomaly
Case Report
A 54-year-old man with a history of hypertension was referred
to cardiology clinic for the evaluation of left sided atypical chest pain
and palpitation. On examination, no significant physical findings
were found with a pulse rate 65/min and blood pressure of 160/90
mmHg. Further investigation showed normal results of complete
blood count, basal metabolic profile, thyroid and liver function
test. Electrocardiogram (EKG) showed normal sinus rhythm with
left atrial enlargement (Figure 1). Transthoracic echocardiography
(TTE) demonstrated mild concentric left ventricular hypertrophy
with low-normal systolic function (EF ~ 54%) and dilated
coronary sinus (CS), approximately 2.3 cm × 2.4 cm in the posterior
atrioventricular groove in left parasternal long axis and apical
4-chamber views (Figure 2). Since dilated CS is a relatively rare
finding, the differential diagnosis should include right-sided heart
disease with pressure and volume overload, unroofed coronary
sinus, and anomalous venous drainage into the coronary sinus.
A TTE with agitated normal saline contrast injected into the left
antecubital vein verified the presence of air bubbles entering the
coronary sinus first and then in the right atrium, suggestive of a
persistent left superior vena cava (PLSVC) draining directly into the
coronary sinus (Figure 3).
Discussion
PLSVC has been recognized as a rare venous anomaly but the
most commonly reported congenital thoracic venous anomaly
with a prevalence of 0.3% to 0.5% of general population [1].
During the early stage of fetal life, the thoracic venous system is
mainly composed of anterior and posterior cardinal veins. While
the proximal part of left cardinal vein combines with right anterior
cardinal vein remaining as the SVC, the distal portion usually
dissipates, forming a ligament of Marshall. However, a failure of
the left cardinal vein degeneration leads to the persistent left
SVC. Saline agitated TTE (bubble study) is the gold standard to
confirm the diagnosis. The following TTE findings are part of
diagnostic criteria: (1) the presence of a dilated coronary sinus in
the absence of evidence of elevated right-sided filling pressures; (2)
enhancement of the dilated coronary sinus before the right atrium
Figure 1: Electrocardiogram shows normal sinus rhythm with broad and bifid P wave in all leads.
J Heart Circ
ISSN: 2470-105X
Page 1 of 3
J Heart Circ
ISSN: 2470-105X
Vol. 2. Issue. 1. 17000111
Figure 2: Transthoracic echocardiography demonstrates dilated coronary sinus in left parasternal long axis view (a) and in apical 4-chamber view
(b). (RA: Right Atrium, RV: Right Ventricle, LA: Left Atrium, LV: Left Ventricle, CS: Coronary Sinus)
Figure 3a and 3b: Pre and Post contrast echocardiography (apical 4-chamber view) shows air bubbles entering the coronary sinus first then
followed by the right atrium.
(RA) after contrast material injection into a left arm vein and (3)
earlier RA opacification than the coronary sinus after contrast
injection from the right arm [2].
As PLSVC is a congenital disorder, up to 40% of PLSVC can be
associated with other anomalies including atrial septal defect,
coarctation of aorta, bicuspid aortic valve, unroofed coronary sinus,
coronary sinusostial atresia and cor triatriatum [3,4]. It is often
asymptomatic and discovered incidentally during central venous
cannulation, devices placement such as pacemaker and implantable
cardioverter defibrillator, and cardiac catheterization. However,
PLSVC has several practical implications. A thorough understanding
of venous drainage system is imperative especially in PLSVC cases
with a planned central line insertion or catheterization because
the catheter might be inserted unexpectedly into PLSVC leading to
unintended consequences such as arrhythmia, cardiogenic shock,
and coronary sinus thrombosis.
The clinical implication of PLSVC is different based on venous
drainage systems, and it usually drains into the right atrium via the
coronary sinus without significant complications, which represent
almost 80–92% of cases [5]. In remaining 10% of cases, the PLSVC
can drain directly into the left atrium resulting right-to-left shunting
which can lead to increased risk of paradoxical thromboembolic
complications [6]. Cardiac arrhythmia especially atrial fibrillation
(AF) could be a part of PSLVC presentation, which sometimes
might be an arrhythmogenic source. Thus, LSVC isolation rather
than pulmonary vein isolation will be required to suppress AF
recurrence [7].
Fortunately, there was no evidence of AF and other
congenital anomalies in our patient and treated with a low dose
beta blocker. In the subsequent follow-up visit, he had a better
control of blood pressure without the requirement of cardiac
catheterization.
Conflict of interest
The authors have no conflicts of interest or financial
relationships to disclose.
References
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anomalies of the thorax. AJR Am J Roentgenol. 2004;182(5):1139-50.
2. Hsiao SH, Lee D, Hsu TL, Mar GY, Tseng CJ, Chiao CD, et al. Diagnosis
of an isolated persistent left side superior vena cava by contrast
echocardiography compared with invasive angiographic study. Zhonghua
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literature review. Respir Care. 2000;45(4):411-6.
4. Kong PK, Ahmad F. Unroofed coronary sinus and persistent leftsuperior
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Citation: Kyaw H, Shaikh AZ, Deepika M (2016) Ginormous Coronary Sinus with Persistent Left Superior Vena Cava. J Heart
Circ 2(1): 111.
Page 2 of 3
J Heart Circ
ISSN: 2470-105X
5. Couvreur T, Ghaye B. Left superior vena cava. In: Rémy-Jardin M, Rémy
J. Berlin, editors. Integrated Cardiothoracic Imaging with MDCT from
Medical Radiology. Diagnostic Imaging and Radiation Oncology series.
1st ed. Heidelberg: Springer-Verlag; 2009. p. 289-305.
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associated with indwelling catheter in the presence of anomalous neck
Vol. 2. Issue. 1. 17000111
venous structures. Am J Med Sci. 2010;340(5):421-3. doi: 10.1097/
MAJ.0b013e3181eed62f.
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*Corresponding author: Htoo Kyaw, Division of Cardiology, Mount Sinai Beth Israel Hospital Center, 10 Nathan D Perlman Pl, New York, NY 10003, USA,
Tel: 323-303-7398; E-mail: [email protected].
Received Date: August 20, 2016, Accepted Date: November 22, 2016, Published Date: November 30, 2016.
Copyright: © 2016 Kyaw H, et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted
use, distribution, and reproduction in any medium, provided the original work is properly cited.
Citation: Kyaw H, Shaikh AZ, Deepika M (2016) Ginormous Coronary Sinus with Persistent Left Superior Vena Cava. J Heart Circ 2(1): 111.
Citation: Kyaw H, Shaikh AZ, Deepika M (2016) Ginormous Coronary Sinus with Persistent Left Superior Vena Cava. J Heart
Circ 2(1): 111.
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