Catheterization and Cardiovascular Diagnosis 20:18+192 (1990)

Left-Sided Inferior Vena Cava Draining Into the Coronary Sinus Via Persistent Left Superior Vena Cava: Case Report and Review of the Literature M. Elizabeth Brickner, MD, Eric J. Eichhorn, MD, Dymphna Netto, MD, Ricardo G. Cigarroa, MD, Walter C. Brogan, 111, MD, PhD, Randall L. Simonsen, MD, and Paul A. Grayburn, MD A case of a left-sided Inferior vena cava draining into the coronary sinus via a persistent lett superior vena cava is described and the pertinent literature reviewed. Key words: cardiac catheterization, contrast echocardiography, venous anomalies

INTRODUCTION

Anomalies of the systemic venous circulation are frequently seen in patients with congenital heart disease and are occasionally encountered in patients without evidence of other congenital abnormalities. Persistence of a left superior vena cava has been well described and may cause difficulty in placement of transvenous pacemakers or right heart catheterization 1-41, A left-sided inferior vena cava has also been described in the radiologic literature [5.6]. A left-sided inferior vena cava usually communicates with a normally positioned infrahepatic inferior vena cava and thus empties into the right atrium in the usual position. Occasionally. a left-sided inferior vena cava may communicate with either the azygos or hemiazygos vein [7-10]. On rare occasions. a left-sided inferior vena cava may communicate with a persistent left superior vena cava by way of the hemiazygos vein [ 5 ] . We recently encountered a case of this unusual venous anomaly in a patient undergoing cardiac catheterization for evaluation of aortic stenosis. CASE REPORT Clinical Data

A 75-year-old white man was transferred to the Dallas Veterans Affairs Medical Center with complaints of increasing chest pain. decreased exercise tolerance. fatigue, and episodes of syncope. Physical examination revealed evidence of aortic stenosis with diminished carotid upstrokes and a harsh grade 111 late-peaking systolic ejection murmur. The ECG showed AV dissociation (atrial rate, 66; ventricular rate. 72) with a left bundle branch block morphology. This clearly represented an accelerated junctional rhythm. as previous ECGs dating back to 1983 showed first-degree AV block and leftbundle branch block. Nevertheless, the admitting physi0 1990 Wiley-Liss, Inc.

cians mistakenly diagnosed complete heart block and placed a temporary transvenous pacemaker from the right subclavian vein. The following morning, echocardiography demonstrated left ventricular hypertrophy with normal systolic function. a markedly thickened aortic valve with diminished leaflet excursion, and an aortic valve area of 0.7 cm2 by the continuity equation. Catheterization Data

Right and left heart catheterization was performed from the leg. During right heart catheterization, a 7Fr Cournand catheter was advanced from the right femoral vein and was noted on fluoroscopy to ascend on the left side of the patient's vertebral column. Oxygen saturation and the pressure waveform confirmed that the catheter was in the venous system. As the catheter was further advanced. it was noted to ascend in a cephalad direction beyond the cardiac silhouette and then made a sharp bend in a caudal direction just below the patient's left clavicle. The stiff catheter could not be further advanced beyond this point and was removed. A therniodilution SwanGanz catheter then was advanced from the right femoral vein and was noted to take the same route as the previous catheter. This catheter could be further advanced into the right pulmonary artery (position confirmed by pressure wavefotnis), but an adequate wedge position could not be obtained. The course of this right heart catheter was

From the Department of Internal Medicine (Cardiology Division), University of Texas Southwestern Medical Center and Department of Veteran's Affairs Medical Center, Dallas, Texas. Received October 30. 1989: revision received March 4, 1990 Address reprint requests to Paul A . Grayburn. M.D.. Cardiology I I IA). VA Medical Center. 4500 S. Lancaster Road, Dallas, TX 752 16.

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Fig. 1. AP fluoroscopic projection demonstrating a SwanGanz catheter (closed arrow) ascending from the inferior vena cava on the left side of the spine and entering the pulmonary artery via a circuitous route. A pigtail catheter is present in the aorta (open arrow). A pacemaker wire is seen to enter the right heart from the right superior vena cava.

noted to be different from the course of the transvenous pacing wire (Fig. I ) , which was left in place until after catheterization. Fick and thermodilution cardiac outputs were measured, and the diagnosis of aortic stenosis was confirmed using simultaneous LV and central aortic pressures (aortic valve area 0.9 cm2 by Gorlin equation). The right heart catheter then was pulled back from the pulmonary artery into the inferior vena cava and contrast was injected (Fig. 2). Contrast was seen to move cephalad in the left side of the chest with a sharp bend caudally, at which point contrast could be seen to empty into a large, dilated coronary sinus, and then into the right atrium. Coronary arteriography then was performed, and the patient appeared to have separate ostia for the left anterior descending and dominant left circumflex arteries. No right coronary artery ostium could be identified. A 75% stenosis was identified in a large circumflex marginal branch. After completion of the catheterization, contrast echocardiography was performed using agitated saline injected intravenously. In the subcostal view, a small evagination of the right atrial wall was noted in the region where the inferior vena cava is normally seen to enter the right atrium. A small hepatic vein appeared to empty directly into the right atrium. Contrast injection into the right femoral vein resulted in opacification of a large coronary sinus. Clinical Course

Aortic valve replacement with coronary artery bypass grafting to the circumflex marginal artery was recommended and performed on the following day. At the time

Fig. 2. Right anterior oblique projection showing contrast injected into the inferior vena cava filling the hemiazygos vein (HV) and then draining into the coronary sinus (CS) via the left superior vena cava (LSVC).

Fig. 3. Autopsy specimen (dorsal view) showing markedly dilated coronary sinus (horizontal scissors) draining into the right atrium (vertical scissors).

of the operative procedure, the patient was found to have a small right superior vena cava which, although patent, could not be cannulated for bypass. A large, dilated persistent left superior vena cava was identified and emptied into a massively dilated coronary sinus. The inferior vena cava joined the persistent left superior vena cava via the hemiazygos vein. A small venous structure draining from the liver into the right atrium was also identified that was again too small to cannulate for bypass. A heavily calcified bicuspid aortic valve was found and replaced with a porcine bioprosthesis. No right coronary ostium could be identified. Bypass grafting to the left circumflex artery was performed without complications. His immediate postoperative course was uncomplicated, but the patient developed refractory ventricular tachycardia approximately 12 hr later and could not be resuscitated.

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Fig. 4. Contrast echocardiogram in the parasternal long-axis projection. A dilated coronary sinus (CS, left panel) in seen to opacify (right panel) following peripheral injection of agitated sallne.

Autopsy Findings An autopsy revealed a left-sided inferior vena cava communicating with a persistent left superior vena cava by means of a markedly dilated hemiazygos vein. The persistent left superior vena cava drained into a markedly dilated coronary sinus, measuring 2.7 cm in diameter (Fig. 3). The right superior vena cava wah present and of normal caliber. A large hepatic vein emptied directly into the right atrium. There was no direct connection between the inferior vena cava and the right atrium. The bioprosthetic aortic valve appeared structurally intact. A nondominant right coronary artery was identified arising I .4 cm above the sinus of Valsalva. The left anterior descending and circumflex arteries arose from a very short left main coronary artery. DISCUSSION Anomalies of the systemic venous circulation may be occasionally encountered in patients undergoing cardiac catheterization and rnay cause technical difficulties during the procedure. Persistence of the left superior vena cava has been well described and occurs in approximately 0.5%:of the general population [2.11,12]. I t results from persistence of the left anterior cardinal vein and usually drains into a dilated coronary sinus. Alternatively, it may drain directly into the right atrium or into the left atrium, thus producing a right to left shunt 1131. The right superior vena c a w may or niay not be present and, if present, may communicate with the left superior vena cava 121. In the usual circumstance of a persistent left superior vena cava emptying into a dilated coronary sinus, the latter structure may be identitied by two-dimensional (2-D) echocardiography as an echo-free chamber visualized behind the posterior mitral leaflet and left atrium in the parasternal long axis view [ 14-17]. When agitated saline is in.jected into a peripheral vein in

the left arm, opacification of the coronary sinus will occur. as shown in Figure 4. In the case of a venous anomaly such as that seen in our patient, injection of saline into the inferior vena cava via a femoral vein would also result in opacification of the coronary sinus. Anomalies of the inferior vena cava are not uncommon. and there are several variations. A left-sided inferior vena cava results from persistence of the left supracardial vein and. most commonly. will cross back to the right side after receiving the renal veins to enter the right atrium in the usual position (61. Alternatively. a left-sided inferior vena cava may communicate with the azygos vein and empty into the superior vena cava. Communication of a left-sided inferior vena cava with the hemiazygos vein is uncommon but has been previously described (51. The dilated hemiazygos vein then rnay take one of three routes to drain in the right atrium. It may I ) drain into the azygos vein, then into the superior vena cava, and thus into the right atrium: 2) drain into the accessory herniazygos vein and from there into the superior intercostal vein and thus into the right atrium; or 3) drain into a persistent left superior vena cava, as in this patient. The association o f these venous anomalies with a bicuspid aortic valve has not been previously reported and is probably coincidental. REFERENCES Schonleld AJ. McKinney RG: Abnormal position of flowdirected right h e m catheter. Chest 90:893-894.1986. Cobh RJ. Mendelson DS. Norton KI: Persistent left superior vena cava: case report and review. MI Sinai J Med 54: 17 1-173. 1987. Bashour IT. Antonini C Sr. Antonini C Jr. Duke L: Left-sided superior vena cava: A rare anonialy precluding transvenous implantation of a pernianent pacemaker (letter).Catheter Cardiovasc Dlagn 131356-357. 19x7. Dirix LY, Kersschot IE. Fierens H . Goethals MA. Van Daele G . Claissen G : Implantation of a dual chamber pacemaker in a patient with persistent left superior vena cava. Pace l1:343-345,

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5 . Abrams HL (ed): "Abrams Angiography: Vascular and Interventional Radiology." 3rd ed. Boston: Little Brown and Co., 1983. pp 905, 912, 947-950. 6. Mayo J, Gray R, St. Louis E, Grossman H, McLoughlin M. Wise D: Anomalies of the inferior vena cava. AJR 140:339-345. 1983. 7. Haswell DM, Benigan TJ: Anomalous inferior vena cava with accessory hemiazygos continuation. Radiology I 195 1-52, 1976. 8. Anderson RC, Heilig W, Novich R, Jarvis C: Anomalous inferior vena cava with azygos drainage: so-called absence of the inferior vena cava. Am Heart J 49:318-322. 1955. 9. Munechika H,Cohan RH. Baker ME, Cooper CJ. Dunnick NR: Hemiazygos continuation of a left inferior vena cava: CT appearance. J Comput Assist Tomogr 12:328-330. 1988. 10. Pallin J. Buckner B, Ferris El. Shah HR, Jones JS: Azygos continuation of the inferior vena cava masquerading as neoplasm. South Med J 82:259-261, 1989. I 1. Campbell M. Deuchar DC: The left-sided superior vena cava. Br Heart J 16:423-439, 1954. 12. Bunger PC, Neufeld DA, Moore JC, Carter GA: Persistent left

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superior vena cava and associated structural and functional considerations. Angiology 32:601-608, 1981. Soward A. ten Cate F, Fioretti P, Roelandt J. Sermys P An elusive persistent left superior vena cava draining into left atrium. Cardiology 73:368-71. 1986. Feigenbaurn H: "Echocardiography." 4th ed. Philadelphia, PA: 1986. p 367. Stewart JA. Fraker TD, Slosky DA. Wise N K , Kisslo JA: Detection of persistent left superior vena cava by two-dimensional contrast echocardiography. J Clin Ultrasound 7:357-360, 1979. Hibi N, Fukui Y,Nishimura K, Miwa A , Kambe T, Sakamoto N: Cross-sectional echocardiographic study on persistent left superior vena cava. Am Heart J 100:69-76. 1980. Ferrara N , Zarra AMF, Vigorito C, Longobardi G. Giordano A. Rengo F: Combined contrast echocardiographic and hemodynarnic evaluation of atrial septa1 defect associated with persistent left superior vena cava and partial anomalous pulmonary venous connection. J Clin Ultrasound 15x54-67, 1987.

Left-sided inferior vena cava draining into the coronary sinus via persistent left superior vena cava: case report and review of the literature.

Catheterization and Cardiovascular Diagnosis 20:18+192 (1990) Left-Sided Inferior Vena Cava Draining Into the Coronary Sinus Via Persistent Left Supe...
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