Persistent left superior vena cava diagnosed by contrast transesophageal echocardiography

The Journal of Emergency Medicine, Vol. 38, No. 5, pp. 638 – 641, 2010 Copyright © 2010 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/$–see front matter

doi:10.1016/j.jemermed.2008.05.022

Ultrasound in Emergency Medicine

PERSISTENT LEFT SUPERIOR VENA CAVA DIAGNOSED BY BEDSIDE ECHOCARDIOGRAPHY Jeroen Walpot,

MD,*

W. Hans Pasteuning,

MD,*

and Jan van Zwienen,

MD†

*Department of Cardiology and †Intensive Care Unit, Ziekenhuis Walcheren, Vlissingen, The Netherlands Reprint Address: Jeroen Walpot, MD, Department of Cardiology, Ziekenhuis Walcheren, Koudekerkseweg 88, Postbus 3200, 4380 DD Vlissingen, The Netherlands

e Abstract—Background: Persistent left superior vena cava (PLSVC) is a congenital anomaly with an estimated incidence of 0.3– 0.5% in the normal population. Its usual discovery is often made by an abnormally positioned catheter inserted in the left subclavian or left jugular vein. In this situation, an easy bedside approach to confirm an anatomic variation in the central venous system is helpful. In the majority of cases, the PLSVC drains to the coronary sinus. Objective: To describe the contribution of bedside echocardiography in diagnosing the unstable patient in whom there is suspicion of a PLSVC. Case Report: A 29-year-old man underwent an emergent laparotomy for multiple intra-abdominal abscesses. Postoperatively, after insertion of a central line catheter through the left subclavian vein, a chest X-ray study showed the tip of the catheter in a left paramediastinal position instead of crossing the midline to the superior vena cava. A PLSVC was suspected. The patient was hemodynamically unstable; therefore, a bedside non-invasive confirmation of the diagnosis of PLSVC was preferred. A transthoracic echocardiography study was performed after injection of agitated saline (creating air-filled microbubbles by shaking saline solution in a syringe), which showed that the coronary sinus was opacified, confirming the diagnosis of a PLSVC. Conclusion: In this brief report, we describe the contribution of echocardiography to the diagnosis of a PLSVC. Echocardiography is a reliable and easy diagnostic tool that allows a bedside approach in a patient in whom there is suspicion of a PLSVC, without administration of radiographic contrast. © 2010 Elsevier Inc.

INTRODUCTION Persistent left superior vena cava (PLSVC) is a congenital anomaly that is not uncommon (1). Its discovery often follows the finding of an abnormally positioned catheter, pacemaker, or internal defibrillator lead placed into a left subclavian or left jugular vein. Most physicians, confronted with a central venous catheter with its tip in the left paramediastinal region, will think of the diagnosis of PLSVC, but probably will desire confirmation of the presence of a PLSVC to rule out catheter perforation or migration. Wall perforation of the vein, with the tip in the intrapleural cavity, should be excluded before administering intravenous fluids or medication through the catheter. Given the situation, especially in an unstable patient in the catheterization laboratory or in the intensive care unit, the treating physician will need a quick, reliable, and, if possible, a bedside answer.

CASE REPORT A 29-year-old man underwent an emergency laparatomy for intra-abdominal abscesses complicated by sepsis and prerenal failure. Postoperatively, the patient was transferred to the intensive care unit. He required mechanical ventilation, and the administration of inotropic medications. A left subclavian catheter was inserted. The chest X-ray study after insertion showed a catheter situated in the left para-

e Keywords—persistent left superior vena cava; echocardiography; bedside approach; abnormal positioned central venous catheter; agitated saline test

RECEIVED: 15 March 2008; FINAL ACCEPTED: 23 May 2008

SUBMISSION RECEIVED:

29 April 2008; 638

Persistent Left Superior Vena Cava

Figure 1. Chest X-ray study after insertion of a left subclavian catheter. The tip (ⴱ) of the catheter is aberrantly positioned in the left paramediastinal region.

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patients with other congenital heart abnormalities, with a reported incidence of 1.5–10% in these patients (2,4). Despite its low incidence, PLSVC is the most common congenital abnormality of the venous system. In the past decade, several case reports have been published describing PLSVC as an incidental finding during invasive procedures, such as left-sided insertion of central venous catheters, pacemaker wires, or internal defibrillator leads (5– 8). The PLSVC itself does not provoke physiological derangements, but it may be associated with other cardiac abnormalities, such as septal defects, Tetralogy of Fallot, and situs inversus, that need to be excluded (9). Ninety-two percent of PLSVCs drain to the right atrium via the coronary sinus or sinus venosum. These patients are asymptomatic. Eight percent of the PLSVCs drain to the left atrium, causing right-to-left shunting. These latter patients may have unexplained cyanosis, and are at risk for systemic embolization.

mediastinal area instead of crossing the midline to the superior vena cava (SVC) (Figure 1). This was likely due to a variation in the anatomy of the venous system, normally returning blood through the SVC to the right atrium. Confirmation of correct positioning was needed. Because the patient was in critical condition, a bedside diagnostic approach was preferred. A transthoracic echocardiogram study was performed. Despite the suboptimal acoustic window, it demonstrated a prominent coronary sinus with a diameter of 1.3 cm (Figure 2). Agitated saline (creating air-filled microbubbles by shaking saline solution in a syringe), used for a contrast agent, was injected through the catheter. The coronary sinus, the right atrium, and the right ventricle were opacified (Figure 3). This examination confirmed an anomaly of the venous return, consistent with a persistent left superior vena cava (PLSVC) entering the coronary sinus. Administering intravenous fluids and medication was therefore deemed to be safe. The patient slowly recovered and was discharged from the intensive care unit. As PLSVC is associated with other congenital heart diseases, additional imaging was proposed, but the patient chose not to return for follow-up. DISCUSSION PLSVC is a congenital abnormality. Its incidence is estimated to be 0.3– 0.5% of the normal population (1,2). A PLSVC is a persistent remnant of a vessel that is present as an embryological counterpart of the normal right-sided superior vena cava (3). A PLSVC is most frequently seen in

Figure 2. (A) Transthoracic echocardiography (TTE): an image obtained from the parasternal long axis view. Note the presence of a large coronary sinus (ⴱ). (B) TEE image from a suboptimal apical acoustic window (bedside TTE in a supine patient in the intensive care unit). The coronary sinus (ⴱ) presents as a channel with inflow in the right atrium (RA). LA ⴝ left atrium, LV ⴝ left ventricle, RV ⴝ right ventricle.

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Figure 3. (A) Transthoracic echocardiography, before administration of agitated saline solution, shows a prominent coronary sinus (ⴱ). The coronary sinus is localized within the pericardium. Its intra-pericardial localization allows one to make the differentiation between the coronary sinus and the descending thoracic aorta (ⴱⴱ), which is situated in the same region, but outside the pericardium. (B) Note the opacification of the coronary sinus after injection of agitated saline through the lumen of the catheter. (C) A zoom-in view of the coronary sinus (ⴱ) before (C1) and after (C2) injection of agitated saline.

Multi-slice cardiac computed tomography scan with or without three-dimensional reconstruction, cardiac magnetic resonance imaging, venography with left-sided injection of the iodinated contrast, and echocardiography are described as diagnostic tools to confirm the diagnosis of a PLSCV. This patient was hemodynamically unstable, and a bedside approach to confirm an anatomic variation in venous return to the heart was preferred. A transthoracic echocardiography (TTE) study was performed. The first echocardiographic clue to the diagnosis was the presence of a large coronary sinus. Subsequently, an agitated saline solution (with microbubbles) was injected through the lumen of the central venous catheter. Opacification of the coronary sinus and the right atrium and right ventricle proved an abnormal venous return to the right atrium. In 2006, Ghadiali et al. reported a similar case of a hemodynamically unstable patient with a central venous catheter with its tip positioned at the left parasternal border (5). They performed bedside chest radiography

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with injection of radiopaque contrast (venography without fluoroscopy). Timing was the critical element in this procedure, as film exposure had to coincide with the arrival of the contrast in the region of interest after injection. The advantages of TTE compared with the chest radiography technique are: 1) no need for nephrotoxic contrast agents in a patient with prerenal failure; 2) no radiation exposure; 3) no hemodynamic effects after injection of agitated saline (microbubbles) compared with iodinated contrast; 4) no possible anaphylaxsis to the injected saline. Ghaliadi et al. also suggested two other easy-toperform actions that may contribute to correct interpretation of an aberrantly positioned central venous catheter: 1) take a sample for blood gas analysis through the lumen of the catheter to exclude an arterial position of the catheter tip; 2) the retrograde pressure curves may provide some information; an exaggerated jugular pressure curve may be seen (5,10). They postulated that the presence of a large coronary sinus in the proximity of the right ventricle may have contributed to the unusual wave forms. However, they did not consider the pressure curves as a real clue to the diagnosis, as they also could be explained as part of the hyperdynamic circulation. CONCLUSION We report a case of a hemodynamically unstable patient who had a left subclavian catheter placement that was found to be in an abnormal position. Transthoracic echocardiography provided a non-invasive confirmation of the presence of an anomaly of the venous return from the left subclavian vein to the right atrium. REFERENCES 1. Biffi M, Boriani G, Fabretti L, Bronzetti G, Branzi A. Left superior vena cava persistence in patients undergoing pacemaker or cardiovertor-defibrillator implantation: a 10-year experience. Chest 2001; 120:139 – 44. 2. Perloff JK. Congenital anomalies of the caval connection. In: The clinical recognition of congenital heart disease, 4th edn. Philadelphia, PA: WB Saunders Company; 1994:703-14. 3. Paval J, Nayak S. A persistent left superior vena cava. Singapore Med J 2007;48:e90. 4. Nsah EN, Moore CW, Hutchins GM. Pathogenesis of persistent left superior vena cava with coronary sinus connection. Pediatr Pathol 1991;11:261–9. 5. Ghadiali N, Teo LM, Sheak K. Bedside confirmation of persistent left superior vena cava based on aberrantly positioned central venous catheter chest radiography. Br J Anaesth 2006;96:53– 6. 6. Leibowitz AB, Halpern NA, Lee MH, Iberti TJ. Left-sides superior vena cava: a not-so-unusual vascular anomaly discovered during central venous and pulmonary artery catheterization. Crit Care Med 1992;20:1191–22.

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