Article, Cardiology

Persistent left superior vena cava draining into the pulmonary venous system discovered after central venous catheter placement

Unlabelled imageAmerican Journal of Emergency Medicine 32 (2014) 943.e1-943.e3

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Case Report

Persistent left superior vena cava draining into the pulmonary Venous system discovered after Central venous catheter placement

Abstract

A 54-year-old man with septic shock and acute respiratory distress syndrome required central venous access for vasopressor adminis- tration. He was found to have a persistent left superior vena cava (PLSVC) after central venous catheter placement in his left internal jugular vein. This anatomical variant usually drains into the coronary sinus. Our patient’s PLSVC, however, directly anastomosed to the left superior pulmonary vein, with the catheter tip ending in one of the branches of the lingular vein. This anomaly is extremely rare, and this is the only documented case of such catheter placement. Adminis- tration of vasopressors through the catheter could have proven cata- strophic, potentially resulting in pulmonary vasoconstriction and infarction. In this case report, we review the epidemiology, etiology, and clinical significance of PLSVC in the emergency medicine and critical care setting.

A 54-year-old man presented to the emergency department (ED) with dyspnea, tachypnea, and productive cough. He had been recently discharged from a rehabilitation center after inpatient amputation of his right index finger due to a progressive osteomy- elitis secondary to uncontrolled diabetes. His medical history also included congestive heart failure, chronic kidney disease, multiple lower extremity digit amputations, and below-the-knee amputation. The patient was diagnosed with severe sepsis secondary to health care-associated pneumonia and transferred to the intensive care unit for respiratory management.

In the intensive care unit, the patient developed hypoxic respiratory failure requiring intubation and, subsequently, acute respiratory distress syndrome. He became increasingly hypotensive without response to fluid resuscitation and required vasopressors. Therefore, a left internal jugular Central venous catheter was placed under ultrasound guidance and without complication. Before its use, confirmatory chest x-ray was obtained (Fig. 1). It showed the catheter tip to be overlying the patient’s left lung field, and radiology was contacted. By reviewing the patient’s contrast-enhanced com- puted tomographic (CT) images (Figs. 2-4) from prior hospitalization, the radiologist identified a duplicate, persistent left superior vena cava (SVC) draining into the left pulmonary venous system, most likely the left superior pulmonary vein, an anatomical variant, which has been documented [1]. The radiologist believed that our catheter entered the left superior pulmonary vein and traveled retrograde into the lingular vein, thus ending somewhere in the venous system of the lingula.

Based on this information, we made the decision not to use the catheter, fearing that vasopressors would cause lung infarction. The catheter was withdrawn, and alternative central access was obtained using the patient’s right femoral vein. Unfortunately, the patient’s

condition continued to deteriorate, and he developed acute-on- chronic renal failure requiring hemodialysis. The patient’s family felt that hemodialysis would have been against the patient’s wishes, and the decision was made to withdraw care. The patient died shortly after extubation.

Persistent left SVC (PLSVC) is a congenital anomaly caused by the failure of the left anterior cardinal vein to regress (and go on to form the ligament of Marshall) during the first 2 months of fetal development [2,3]. Persistent left SVC is the most common thoracic venous anomaly and has been reported in 0.3% to 0.5% of the general population [4]. It is associated with absence of the left brachioce- phalic vein and decreased diameter of the right SVC compared with the left in 65% of cases [5]. The right SVC is present in 80% to 90% of cases [6] but may be absent if the normally persistent right anterior cardinal vein regresses during fetal development. There are 2 poten- tial draining points of the PLSVC, which have been reported: the coronary sinus (80%-92%) and directly into the left atrium or left superior pulmonary vein (10%-20%).

When the PLSVC drains into the coronary sinus, it is rare to have associated congenital heart defects, and generally, there are no sig- nificant clinical consequences of the anomaly. It is often diagnosed as an incidental finding after central venous access, surgery, or during radiographic imaging. An incidental finding of a dilated coronary sinus without evidence of elevated right-sided filling pressures on echo- cardiography should raise suspicion for PLSVC, which is draining into the coronary sinus, thus prompting further investigation such as saline contrast (“bubble study”) echocardiography, CT/magnetic reso- nance venography, transesophageal echocardiography, or radionu- clide angiocardiography [7].

When the PLSVC drains into the left atrium or left superior pul- monary vein, it creates a right-to-left shunt. This is usually asympto- matic due to the relatively small distribution drained by the PLSVC (the left arm, neck, and head) [6] but has been documented to present with persistent, unexplained hypoxia or cyanosis [8,9] and paradox- ical emboli causing recurrent transient ischemic attacks [10,11] and cerebral abscesses [12,13]. This variant of PLSVC is more commonly associated with absence of the right SVC and congenital heart abnormalities, most characteristically septal defects, but also aortic coarctation, atresia of the coronary sinus ostium, cor triatriatum, and tetralogy of Fallot [6,8]. The incidence of PLSVC in patients with congenital heart defects has been reported to be as high as 12% [14- 16], and the rate of congenital heart defects in patients with PLSVC is as high as 40% [7].

Our discovery of the PLSVC secondary to central venous access is a common presentation of this anatomical anomaly. However, to our

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Fig. 3. PLSVC splitting from left brachiocephalic vein.

Fig. 1. Left IJ with catheter tip overlying left lung.

knowledge, this is the only documented case of a catheter traversing a PLSVC and ending in the pulmonary venous system. This is most likely due to the extreme rarity of this embryologic variant. It under- scores the importance of confirmatory chest x-ray after CVC place- ment. Of note, review of our patient’s records showed no evidence of Cardiac abnormalities, and prior echocardiograms demonstrated normal ejection fraction, valve structure, chamber sizes, and no evidence of diastolic dysfunction.

In the instance that a PLSVC is discovered incidentally in the ED during radiographic evaluation for an unrelated medical problem, we recommend further outPatient evaluation to establish the course and drainage of the PLSVC, assess for the presence of right SVC, and screen for congenital cardiac abnormalities. Although PLSVC is by itself likely to be asymptomatic, it may interfere with future procedures such as pacemaker and central venous access device (eg, chemotherapy port) placement. Therefore, it is important that it becomes part of the patient’s medical record.

If the PLSVC is discovered after CVC placement in the left sub- clavian or internal jugular veins, we recommend withdrawing the catheter and attempting placement on the right side or in the femoral veins. If the PLSVC drains into the coronary sinus, a guide wire or catheter in such close proximity to the atria may predispose the patient to cardiac irritation and dysrhythmias [17]. As mentioned, PLSVC draining into the left superior pulmonary vein or left atrium can cause paradoxical cerebral emboli. This catastrophic risk can be further compounded with the introduction and use of a CVC,

Fig. 2. PLSVC splitting from left brachiocephalic vein.

Fig. 4. Junction of PLSVC and left superior pulmonary vein.

potentially causing an ischemic event during each use [18]. In our case, we were concerned that vasopressor administration into the small vessels of the pulmonary venous system may cause pulmonary infarction secondary to severe venoconstriction or extravasation. In general, we believe that a good rule is that, if the clinician is unsure of the location of the CVC, it should not be used and an alternate site should be obtained, especially if it may lie in the left-sided circulation. Persistent left SVC is a rare anatomical variant that usually drains into the coronary sinus. Our case demonstrates that PLSVC has the potential to anastomose with the pulmonary vasculature and left atrium. This is generally not of any clinical consequence unless the patient undergoes instrumentation of the central vasculature. In these cases, the altered anatomy may predispose to significant morbidity and mortality from complications of central access. The clinician should be aware of this potential variation in vascular anatomy and use other central venous access sites to avoid such complications in

these patients.

Boris Elison, MD Daniel Evans, DO/MPH Thomas Zanders, DO Rebecca Jeanmonod, MD

St Luke’s University Hospital, Bethlehem, PA 18015, USA E-mail address: [email protected]

http://dx.doi.org/10.1016/j.ajem.2013.12.042

B. Elison et al. / American Journal of Emergency Medicine 32 (2014) 943.e1943.e3 943.e3

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