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Logo of thijTexas Heart Institute JournalSee also Cardiovascular Diseases Journal in PMCSubscribeSubmissionsTHI Journal Website
 
Tex Heart Inst J. 2007; 34(4): 492–493.
PMCID: PMC2170488

Persistent Left Superior Vena Cava with Absent Right Superior Vena Cava and Coronary Sinus Dilation Mimicking a Paracardiac Mass

Raymond F. Stainback, MD, Section Editor
Department of Adult Cardiology, Texas Heart Institute and St. Luke's Episcopal Hospital, 6624 Fannin Street, Suite 2480, Houston, TX 77030

A 54-year-old man was referred to our hospital due to atypical chest pain and a suspected paracardiac mass. Cardiac examination revealed a grade 1/6 systolic murmur in the left parasternal border. Results of surface electrocardiography and laboratory examinations were within normal limits.

Transthoracic (Fig. 1) and transesophageal echocardiography showed the absence of a right superior vena cava (SVC), the presence of a left SVC at the lateral border of the left atrium, and no other structural abnormalities. The diagnosis was confirmed on computed tomography (CT) (Fig. 2). The patient's visceral organs were normally positioned and structured. Coronary angiography and cardiac catheterization revealed normal coronary arteries and no stenosis. Upper-extremity and cranial veins drained into the coronary sinus via the persistent left SVC; therefore, the coronary sinus was approximately 5 cm in diameter and mimicked a paracardiac mass (Fig. 3).

figure 25FF1
Fig. 1 The dilated coronary sinus (arrows) resembles a paracardiac mass, as shown on transthoracic 2-dimensional echocardiography in the apical 5-chamber view. Ao = aorta; LA = left atrium; LV = left ventricle
figure 25FF2
Fig. 2 Computed tomography of the chest shows the persistent left superior vena cava (arrow) next to the aortic arch (Ao).
figure 25FF3
Fig. 3 Coronary sinus angiography shows retrograde opacification via the coronary sinus of the persistent left superior vena cava in the left lateral thorax.

Comment

Persistent left SVC, an anomaly of the venous circulation, occurs in 0.5% of the general population, 0.3% of healthy persons, and 4.3% of those with congenital heart disease.1–4 In most patients with left SVC, a right SVC is present. Persistent left SVC with absent right SVC occurs in only 0.09% to 0.13% of patients who have congenital heart defects.5 Although this rare condition is frequently associated with situs inversus, our situs solitus patient lacked a right SVC and had a persistent left SVC that drained into the right atrium via an extremely dilated coronary sinus. The sinus was even larger than that usually seen in the more common left-SVC persistence with right SVC; this was likely due to the rarer associated agenesis of the right SVC in this patient.

Persistent left SVC, and other anomalies and structures in the left atrioventricular groove, can be overlooked if they are not carefully and appropriately evaluated. Venous angiography is a widely available and low-cost imaging method for early confirmation of the presented combined venous malformation. Angiography and CT were useful in this case, because right-SVC agenesis would not necessarily be suspected.

Footnotes

Address for reprints: Nurcan Arat, MD, 9.cadde 110.sokak, Vadi 3000 sitesi, No: 2-A/24, 06400, Birlik Mahallesi, Ankara, Turkey. E-mail: moc.liamg@nacruntara

References

1. Chandra A, Reul GJ Jr. Persistent left superior vena cava discovered during placement of central venous catheter. Tex Heart Inst J 1998;25:90. [PMC free article] [PubMed]
2. Wood PH. Diseases of the heart and circulation. 2nd ed. Philadelphia: Lippincott; 1956. p. 457.
3. Higgs AG, Paris S, Potter F. Discovery of left-sided superior vena cava during central venous catheterization. Br J Anaesth 1998;81:260–1. [PubMed]
4. Winter FS. Persistent left superior vena cava; survey of world literature and report of thirty additional cases. Angiology 1954;5:90–132. [PubMed]
5. Soward A, ten Cate F, Fioretti P, Roelandt J, Serruys PW. An elusive persistent left superior vena cava draining into left atrium. Cardiology 1986;73:368–71. [PubMed]

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