Persistent left superior vena cava draining into the pulmonary venous system discovered after central venous catheter placement Daniel Evans DO/MPH, Boris Elison MD, Thomas Zanders DO, Rebecca Jeanmonod MD PII: DOI: Reference:

S0735-6757(13)00901-7 doi: 10.1016/j.ajem.2013.12.042 YAJEM 54046

To appear in:

American Journal of Emergency Medicine

Received date: Accepted date:

13 December 2013 18 December 2013

Please cite this article as: Evans Daniel, Elison Boris, Zanders Thomas, Jeanmonod Rebecca, Persistent left superior vena cava draining into the pulmonary venous system discovered after central venous catheter placement, American Journal of Emergency Medicine (2013), doi: 10.1016/j.ajem.2013.12.042

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

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Authors:Daniel Evans DO/MPH (author to whom reprint requests should be sent) Boris Elison MD Thomas Zanders DO Rebecca Jeanmonod M.D. St. Luke’s University Hospital 801 Ostrum St. Bethlehem, PA 18015

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Key words: critical care, resuscitation, imaging

ACCEPTED MANUSCRIPT Persistent left superior vena cava draining into the pulmonary venous system discovered after central venous catheter placement: a case report.

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Abstract: A 54-year-old male suffering from septic shock and acute respiratory distress syndrome required central venous access for vasopressor administration. 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. Administration of vasopressors through the catheter could have proven catastrophic, 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. Case: A 54 year old male presented to the Emergency Department (ED) with dyspnea, tachypnea, and productive cough. He had been recently discharged to a rehabilitation center after inpatient amputation of his right index finger due to a progressive osteomyelitis secondary to uncontrolled diabetes. His past 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 (ICU) for respiratory management. In the ICU, 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 (IJ) central venous catheter was placed under ultrasound guidance and without complication. Prior to its use, confirmatory chest x-ray (CXR) was obtained (Figure 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 computed tomography (CT) images (Figures 2-4) from prior hospitalization, the radiologist identified a duplicate, persistent left superior vena cava 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 expired shortly after extubation. Discussion: Persistent left superior vena cava (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 two months of fetal development [2,3]. PLSVC is the most common thoracic venous anomaly and has been reported in 0.3-0.5% of the general population [4]. It is associated with absence of the left brachiocephalic vein and decreased diameter of the right SVC compared to

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the left in 65% of cases [5]. The right SVC is present in 80-90% of cases [6] but may be absent if the normally persistent right anterior cardinal vein regresses during fetal development. There are two potential draining points of the PLSVC which have been reported: the coronary sinus (8092%) 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 significant 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 echocardiography should raise suspicion for PLSVC which is draining into the coronary sinus, thus, prompting further investigation such as saline contrast (“bubble study”) echocardiography, CT/MR venography, TEE, or radionuclide angiocardiography [7]. When the PLSVC drains into the left atrium or left superior pulmonary vein, it creates a right-to-left shunt. This is usually asymptomatic 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 paradoxical 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% [14,15,16] 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 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 underscores the importance of confirmatory chest X-ray after central venous catheter placement. 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 emergency department 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 (e.g. chemotherapy port) placement. Therefore, it is important that it becomes part of the patient’s medical record. If the PLSVC is discovered after central venous catheter (CVC) placement in the left subclavian 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 central venous catheter, 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

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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 central venous catheter, it should not be used and an alternate site should be obtained, especially if it may lie in the left-sided circulation. Conclusion: PLSVC 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 utilize other central venous access sites to avoid such complications in these patients.

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References: 1. Battal B, Karaman B, Akgun V et al. “Images in vascular medicine. The persistent left superior vena cava draining into the left atrium via the left superior pulmonary vein.” Vasc Med. 2011 Dec;16(6):477-8. 2. Cormier MG, Yedlicka JW, Gray RJ et al. “Congenital anomalies of the superior vena cava: a CT study.” Semin Roentgenol 1989; 24:77–83. 3. Gaynor JW, Weinberg PM, Spray TL. “Congenital heart surgery nomenclature and database project: systemic venous anomalies.” Ann Thorac Surg 2000; 69[suppl 4]:S70– S76. 4. Wood P: “Disesase of heart and circulation.” 2nd edition. Philadelphia: JB Lippincott; 1956. 5. Webb W, Gamsu G, Speckman J et al. “Computed tomographic demonstration of mediastinal venous anomalies.” AJR Am J Roentgenol 1982, 139(1):157-161. 6. 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. 7. Albay S, Cankal F, Kocabiyik N et al. “Double superior vena cava.” Morphologie. 2006 Mar;90(288):39-42. 8. Freeman AM, Fenster BE, Weinberger HD et al. “Hypoxia caused by persistent left superior vena cava connecting to the left atrium a rare clinical entity.” Tex Heart Inst J. 2012;39(5):662-4. 9. Meadows WR, Sharp JT. “Persistent left superior vena cava draining into the left atrium without arterial oxygen unsaturation.” Am J Cardiol 1965;16:273–9. 10. Hutyra M, Skala T, Sanak D et al. “Persistent left superior vena cava connected through the left upper pulmonary vein to the left atrium: an unusual pathway for paradoxical embolization and a rare cause of recurrent transient ischaemic attack.” Eur J Echocardiogr 2010;11:E35. 11. Recto MR, Sadlo H, Sobczyk WL. “Rare case of persistent left superior vena cava to left upper pulmonary vein: pathway for paradoxical embolization and development of transient ischemic attack and subsequent occlusion with an amplatzer vascular plug.” J Invasive Cardiol. 2007 Oct;19(10):E313-6.

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12. Erol I, Cetin II, Alehan F et al. “Brain abscess associated with isolated left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect.” Cardiovasc Intervent Radiol. 2006;29:454-6. 13. Menachem JN, Sundaram SN, Rhodes JF. “Recurrent Cerebral Abscess Secondary to a Persistent Left Superior Vena Cava.” Congenit Heart Dis. 2013 May 28. 14. Buirski G, Jordan SC, Joffe HS et al. “Superior vena caval abnormalities: their occurrence rate, associated cardiac abnormalities and angiographic classification in a paediatric population with congenital heart disease.” Clin Radiol. 1986;37:131–138. 15. Sipila W, Kakkila J, Heikel PE et al. “Persistent left superior vena cava.” Ann Med Intern Fenn. 1955;44:251–261. 16. Kula S, Cevik A, Sanli C et al. “Persistent left superior vena cava: experience of a tertiary health care center.” Pediatr Int. 2011. 17. Murphy JM, Wallis F, Molloy MP. “Hickmans in unusual places.” Clin Radiol. 1999 Feb;54(2):111-7. 18. Dinasarapu CR, Adiga GU, Malik S. “Recurrent cerebral embolism associated with indwelling catheter in the presence of anomalous neck venous structures.” Am J Med Sci. 2010 Nov;340(5):421-3. 19. Demos TC, Posniak HV, Pierce KL et al. “Venous anomalies of the thorax.” AJR Am J Roentgenol. 2004;182:1139–1150.

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