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Year : 2013  |  Volume : 23  |  Issue : 1  |  Page : 42-44

Persistent left superior vena cava and absent right superior vena cava: Not only an anatomic variant

Department of Cardiology, S Carlo Clinic, Paderno Dugnano, Milano, Italy

Date of Web Publication10-Sep-2013

Correspondence Address:
Giuseppe Gibelli
Via Genova 13, 20090, Settala (MI)
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Source of Support: None, Conflict of Interest: The authors have no conflict of interest to disclose.

DOI: 10.4103/2211-4122.117985

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Introduction: A 71 year old asymptomatic woman came for an echocardiogram because of a left bundle branch block. A much dilated coronary sinus (CS) with an entering large vessel was found along with a mild left ventricular systolic dysfunction. Cardiac Magnetic Resonance (CMR) showed a persistent left superior vena cava (PLSVC), and an absent right superior vena cava (ARSVC). PLSVC drained into the dilated CS. No other cardiac abnormalities were found. Any late Gadolinium enhancement was also not seen. PLSVC and ARSVC are associated with sinus node and conduction tissue maldevelopment and atrial arrhythmias, and thus clinical follow up is indicated. Conclusion: CMR is a useful addition to echocardiogram to search for further cardiac abnormalities, and outline the anatomy with precision in doubtful cases.

Keywords: Absent right superior vena cava, dilated coronary sinus, left bundle branch block, persistent left superior vena cava, systemic venous return abnormalities

How to cite this article:
Gibelli G, Biasi S. Persistent left superior vena cava and absent right superior vena cava: Not only an anatomic variant. J Cardiovasc Echography 2013;23:42-4

How to cite this URL:
Gibelli G, Biasi S. Persistent left superior vena cava and absent right superior vena cava: Not only an anatomic variant. J Cardiovasc Echography [serial online] 2013 [cited 2021 Oct 22];23:42-4. Available from: https://www.jcecho.org/text.asp?2013/23/1/42/117985

  Case Report Top

A 71 year old asymptomatic woman came for an echocardiogram, because of the electrocardiographic (EKG) finding of a complete left bundle branch block (LBBB). Mild hypertension, well controlled by angiotensin inhibitor therapy, was recorded. The echocardiogram showed markedly dilated coronary sinus (CS): 30 mm × 17 mm (n.v: <15 mm), cross sectional area 3,2 cm 2 and a confluent large vessel [Figure 1]a, b and c] and, along with mildly altered septal movement related to conduction disturbance, mildly enlarged left atrium (42 mm parasternal long axis), mild ejection fraction (EF) reduction (51%) along with altered diastolic pattern (grade 1 diastolic dysfunction). A cardiac magnetic resonance (CMR) was performed. The examination included multiple axial, coronal and long axis cine sequences and contrast enhanced inversion recovery sequences after Gadovist 10 ml injection to look for late enhancement.
Figure 1: (a) Echocardiogram, parasternal long axis view. Dilated coronary sinus is demonstrated (arrow), along with descending Aorta (head arrow). Long and short axes of CS are shown. (b) Modified parasternal short axis. CS (arrow) and PLSVC (head arrow) are shown. (c) Modified four chamber view. Cs (arrow) is seen entering RA (star), postero superiorly to left ventricle (triangle)

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Cine sequences [Figure 2] and [Figure 3] showed absence of right superior vena cava and right and left innominate veins confluence in a left superior vena cava draining into a very dilated CS (major and minor axis: 26 and 18 mm (n.v.:<15 mm) normally connected to right atrium. Left atrium was mildly dilated, and an altered movement of apical and mid interventricular septum due to LBBB without any other abnormality was found. Right and left ventricular outputs, as estimated by ventricular volumes, were find to be nearly identical (4, 5-4,8 l/min). Left and right ventricular EF was 54% and 55%, respectively. No late Gadolinium hyper enhancement was found. Holter recording and routine laboratory data were normal. Clinical follow up along with Holter and echocardiogram was planned, and no therapy was prescribed.
Figure 2: (a) CMR, cine sequence, vertical long axis (equivalent to apical two chamber echographic view), diastolic frame. The dilated CS (arrow) is shown. 1 left atrium (LA), 2 left ventricle (LV), 3 main pulmonary artery, 4 aortic arch. (b) axial view (similar to Figure 1c). CS (arrow) is seen entering RA (star), postero superiorly to left ventricle (triangle); inferior vena cava inlet in RA is shown (point arrow)

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Figure 3: CMR, cine sequence, coronal view, diastolic frame. Slice width 8 mm. Arrow points to the site where superior vena cava, if present, should be seen entering upper RA. PLSVC is seen forming the left mediastinal border and draining into the very dilated CS, in turn connecting to RA. 1 PLSVC, 2 dilated CS, 3 RA, 4 LA, 5 ascending aorta, 6 left pulmonary artery, 7 right innominate vein and overlapping innominate artery and common carotid artery, 8 left innominate vein, 9 inferior vena cava

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Persistence of Left superior vena cava (PLSVC) is the most common congenital anomaly of the systemic thoracic venous return, and is found in less than 0.5% of normal people. [1] In about 90% of cases, bilateral superior venae cavae exist; and absence of right superior vena cava (ARSVC) is uncommon. [1],[2],[3],[4] Recupero et al,[5] to the contrary, collected five patients with echographic finding of very dilated CS, four of whom had PLSVC and associated ARSVC, and only one had both venae cavae. Most probably this unexpected finding is due to selection by echocardiogram, that shows mostly dilated CS in PLSVC and associated ARSVC, in comparison to bilateral venae cavae, as CS size depends on the volume of blood flowing through it. [6] In the presence of both venae cavae, the CS is less dilated, and in some patients the size can even be normal. [6] Arguably, some patients with both venae cavae, having an inconspicuously dilated or undilated CS, may have PLSVC undetected by echocardiogram, which does not happen in the case of anatomic and surgical casistics. PLSVC in most cases drains into a dilated CS; and in a minority of cases it drains into left atrium, by unroofed coronary sinus or, more rarely, directly. PLSVC in congenital heart disease patients is seen in about 4% of cases, [1] most commonly in association with septal defects or complex malformations, and in some cases with isomerism. Atrial arrhythmias are common. [6] PLSVC with ARSVC is associated with maldevelopment of sinus node, [6],[7] and even of the conduction tissue. [7] Lacunae and fibrosis are found inside the sinus node and conduction system together with small related arteries. Cases of juvenile sudden death have been reported; tissue stretching by dilated CS can be the cause, together with maldevelopment. [7]

In the present case, relation between the left bundle branch block and conduction abnormalities associated with PLSVC and ARSVC is uncertain, but arguably these abnormalities have more chances to progress with age, thus determining clinically detectable conduction disturbances. Moreover, in the present case, the absence of late Gadolinium enhancement plays against a coronary cause of these abnormalities; it is uncertain whether the borderline low EF can be related to the congenital abnormality. This association is undescribed, and most probably is simply due to the mild dissinergy induced by the LBBB.

The role of noninvasive diagnosis of PLSVC has been recently reviewed. [5] Echographic finding of dilated CS is suggestive of PLSVC, and when associated with ARSVC, dilatation is maximal, in the range of 30 mm diameter and 3 cm 2 area (as in the present case) vs. 20 mm and 1,8 cm 2 area in the patients with both venae cavae. Further, intravenous agitated saline injected in left antecubital vein visualizing dilated coronary sinus draining into right atrium provides a rapid demonstration of the presence of PLSVC, and the same is true for right antecubital vein injection to demonstrate ARSVC. In cases of unroofed coronary sinus, left atrium is visualized first. Dilated CS may be found in other conditions, most commonly with right atrial pressure overload (RAPO). Kolski et al,[8] found that CS cross sectional area was significantly larger in PLSVC group than in RAPO group and PLSVC subjects had an asymmetrically dilated CS. With use of an eccentricity index (EI: CS minor axis diameter/major axis diameter, measured by echocardiogram in parasternal long axis), they found that CS EI index was significantly lower in PLSVC than in RAPO group. EI was the most sensitive and specific discriminator between patients with persistent LSVC vs RAPO. CS-EI was < 0.8 in all PLSVC patients and > 0.8 in all RAPO patients (sensitivity and specificity = 100%). In our patient, EI was 0.6, which is the correct classification.

Other than for arrhythmias, knowledge of the anomaly has important implications for cardiopulmonary bypass surgery, pulmonary surgery, pacemaker implant due to difficulty of negotiating leads, and any thoracic vein catheterization. [9] PLSVC with RSVCA is not only an anatomic variant, and has implications in regard to invasive procedures. When PLSVC is found, RSVCA should be looked for; patients with the two associated anomalies should be followed up because sinus node dysfunction, conduction disturbances and hyperkinetic arrhythmias are common in such patients, and even rare sudden death cases have been described.

  References Top

1.Demos TC, Posniak HV, Pierce KL, Olson MC, Muscato M. Venous anomalies of the thorax. AJR Am J Roentgenol 2004;182:1139-50.  Back to cited text no. 1
2.Mooney DP, Snyder CL, Holder TM. An absent right and persistent left superior vena cava in an infant requiring extracorporeal membrane oxygenation therapy. J Pediatric Surg 1993;28:1633-4.  Back to cited text no. 2
3.Xiong W, Shi C. Concomitant Persistent Left Superior Vena Cava and Agenesis of Right Superior Vena Cava. A Rare Congenital Anomaly. Circulation 2010;121:2329-30.   Back to cited text no. 3
4.Gologorsky E, Macedo FI, Carvalho EM, Govindaswamy R, Salerno TA. Unexpected persistent left superior vena cava and absent right superior vena cava in situs solitus patient. J Card Surg 2010;25:42-5.   Back to cited text no. 4
5.Recupero A, Pugliatti P, Rizzo F, Carerj S, Cavalli G, de Gregorio C, et al. Persistent left-sided superior vena cava: Integrated noninvasive diagnosis. Echocardiography 2007;24:982-6.  Back to cited text no. 5
6.Lenox CC, Hashida Y, Anderson RH, Hubbard JD. Conduction tissue anomalies in absence of the right superior caval vein. Int J Cardiol 1985;8:251-60.  Back to cited text no. 6
7.James TN, Marshall TK, Edwards JE. De subitaneis mortibus, XX: Cardiac electrical instability in the presence of a left superior vena cava. Circulation 1976;54:689-97.  Back to cited text no. 7
8.Kolski BC, Khadivi B, Anawati M, Daniels LB, Demaria AN, Blanchard DG. The dilated coronary sinus: Utility of coronary sinus cross-sectional area and eccentricity index in differentiating right atrial pressure overload from persistent left superior vena cava. Echocardiography 2011;28:829-32.  Back to cited text no. 8
9.Povoski SP, Khabiri H. Persistent left superior vena cava: Review of the literature, clinical implications, and relevance of alterations in thoracic central venous anatomy as pertaining to the general principles of central venous access device placement and venography in cancer patients. World J Surg Oncol 2011;9:173.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]

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