Background: An isolated persistent left SVC with concomittant agenesis of right SVC in adult patients is a very rare abnormality. Physician should consider it particularly in patients, in which venous acces will be performed. Our rare case deals with the importance of detailed echocardiographic examination with screening of coronary sinus dilatation before the electrophysiology study.Case: A 65-year-old woman came to outpatient clinic for a chief complaint of palpitations. Her ECG showed paroxysmal SVT with WPW syndrome. She underwent echocardiography examination before electrophysiology study and it was found that she had a dilated coronary sinus. Therefore we performed cardiac CT. It was found that she had a persistent left superior vena cava (SVC) and an absence of a right SVC with no other congenital anomaly.Conclusion: A comprehensive echocardiography examination to look for a dilation of coronary sinus is a first suggestion to screen this anomaly, eventually followed by echocardiography with agitated saline injection and/or computed tomography can help physician to anticipate the anomaly before the invasive procedure involving the thoracic vein.Keywords: echocardiography, cardiac CT angiography, vascular malformation, superiorvena cav

Dinarti, Lucia Kris

Gharini, Putrika Prastutu Ratna

Maharani, Erika

English

ACI (Acta Cardiologia Indonesiana)

86  Gharini et al., 2016Copyright©2013. Jurnal Perikanan (Journal of Fisheries Sciences) All Right ReservedIsolated Persistent Left Superior Vena Cava,Role of Echocardiography Screening and CT angiographyPutrika Gharini, Erika Maharani, Lucia KrisdinartiDepartment of Cardiology and Vascular Medicine, Faculty of Medicine Universitas Gadjah Mada - Dr. Sardjito General Hospital, Yogyakarta, IndonesiaAbstractBackground: An isolated persistent left SVC with concomittant agenesis of right SVC in adult patients is a very rare abnormality. Physician should consider it particularly in patients, in which venous acces will be performed. Our rare case deals with the importance of detailed echocardiographic examination with screening of coronary sinus dilatation before the electrophysiology study.Case: A 65-year-old woman came to outpatient clinic for a chief complaint of palpitations. Her ECG showed paroxysmal SVT with WPW syndrome. She underwent echocardiography examination before electrophysiology study and it was found that she had a dilated coronary sinus. Therefore we performed cardiac CT. It was found that she had a persistent left superior vena cava (SVC) and an absence of a right SVC with no other congenital anomaly.Conclusion: A comprehensive echocardiography examination to look for a dilation of coronary sinus is a first suggestion to screen this anomaly, eventually followed by echocardiography with agitated saline injection and/or computed tomography can help physician to anticipate the anomaly before the invasive procedure involving the thoracic vein. Keywords: echocardiography, cardiac CT angiography, vascular malformation, superiorvena cavaBACKGROUNDPersistent left superior vena cava (PLSVC) is the most common congenital malformation of the thoracic venous system and it affects about 0.3 to 0.5% of the general population.1 This incidence increases 10-fold in patients with cardiac malformations.2 PLSVC with an absent right superior vena cava (RSVC), which is also referred to as isolated PLSVC, is very uncommon, occurring in 0.07 to 0.13% of patients who have congenital heart defects with viscero-atrial situs solitus. Nearly half of the patients with isolated PLSVC have other cardiac malformations, such as atrial septal defect, endocardial cushion defect or tetralogy of Fallot.3 In this case report we present a patient with isolatedPLSVC with no other cardiac structural abnormalities. The PLSVC was diagnosed by means of echocardiography. The diagnosis was supported by cardiac CT angiography. CASEA 65-year old woman went to outpatient clinic with a chief complaint of palpitations. Her ECG showed paroxysmal supraventricular tachycardia (SVT) with Wolf-Parkinson-White (WPW) syndrome. It was decided that she should undergo electrophysiology study and ablation. The echocardiography examination revealed a dilated coronary sinus. A CT angiography was performed because of suspicion of a persistent left superior vena cava (PLSVC). The examination showed a bridging vein draining the right jugular and right subclavian veins; it joined the left brachiocephalic vein and formed thePLSVC, which descended at the left side of the mediastinum, leftward of the pulmonary artery and left atrium (LA) before draining into the right atrium (RA) via a dilated coronary sinus(CS) (Figs 1–3). The RSVC was absent and the PLSVC carriedall venous blood from 87  Acta Cardiologia Indonesiana (Vol 2 No. 2): 86-90Copyright©2013. Jurnal Perikanan (Journal of Fisheries Sciences) All Right Reservedvein and formed thePLSVC, which descended at the left side of the mediastinum, leftward of the pulmonary artery and left atrium (LA) before draining into the right atrium (RA) via a dilated coronary sinus(CS) (Figs 1–3). The RSVC was absent and the PLSVC carriedall venous blood from the head, neck and upper extremities.There was no other intra- or extracardiac pathological structur l finding.    Fig. 1. Transthoracal echocardiography showed a dilatation of coronary sinus draining to RA.     Fig 2. Multiplanar reformatted image demonstrates that the right superior vena cava is absent and there is persistent left superior vena cava (PLSVC).    Fig. 3. Multiplanar reformatted image reveals the persistent left superior vena cava (PLSVC) draining into  dil ted cor nary sinus (CS)    vein and formed thePLSVC, which descended at the left side of the mediastinum, leftward of the pulmonary artery and left atrium (LA) before draining into the right atrium (RA) via a dilated coronary sinus(CS) (Figs 1–3). The RSVC was absent and the PLSVC carriedall venous blood from the head, neck and upper extremities.There was no other intra- or extracardiac pathological structural finding.    Fig. 1. Transthoracal echocardiography showed a dilatation of coronary sinus draining to RA.     Fig 2. Multiplanar reformatted image demonstrates that the right superior vena cava is absent and there is persistent left superior vena cava (PLSVC).    Fig. 3. Multiplanar reformatted image reveals the persistent left superior vena cava (PLSVC) draining into a dilated coronary sinus (CS)    Figure 1. Transthoracal echocardiogr phy showed a dilatation of coronary sinus draining to RA. Figure 2. Multiplanar reformatted image demonstrates that the right superior vena cava is absent and there is persistent left superior vena cava (PLSVC).the head, neck and upper extremities.There was no other intra- or extracardiac pathological structural finding. DISCUSSIONPLSVC with absent RSVC (isolated PLSVC) is a very rarevenous malformation. During normal fetal development, theleft-sided anterior venous cardinal system regresses, leaving theCS and the ligament of Marshall. Failure of the closure of the leftanterior cardinal vein results in PLSVC.4 In general, PLSVC isassociated with RSVC and drains into the RA via a dilated CS.When developmental arrest occurs at an earlier stage, the CSis absent and the PLSVC drains 88  Gharini et al., 2016Copyright©2013. Jurnal Perikanan (Journal of Fisheries Sciences) All Right Reservedvein and formed thePLSVC, which descended at the left side of the mediastinum, leftward of the pulmonary artery and left atrium (LA) before draining into the right atrium (RA) via a dilated coronary sinus(CS) (Figs 1–3). The RSVC was absent and the PLSVC carriedall venous blood from the head, neck and upper extremities.There was no other intra- or extracardiac pathological structural finding.    Fig. 1. Transthoracal echocardiography showed a dilatation of coronary sinus draining to RA.     Fig 2. Multiplanar reformatted image demonstrates that the right superior vena cava is absent and there is persistent left superior vena cava (PLSVC).    Fig. 3. Multiplanar reformatted image reveals the persistent left superior vena cava (PLSVC) draining into a dilated coronary sinus (CS)    Figure 3. Multiplanar reformatted image reveals the persistent left superior vena cava (PLSVC) draining into a dilated coronary sinus (CS) into the LA. Either isolated orassociated with RSVC, this venous malformation itself causes no haemodynamic disturbance and is usually diagnosed incidentally. 5-7However, it has several clinical implications. A PLSVC cancause problems during central venous catheterisation (access tothe CS can cause hypotension, angina, perforation of the heart,tamponade and arrest),8 pacemaker implantation (due to thecircuitous path taken by the electrode, it can be difficult to obtaina stable electrode posit ion and sustained capture),9 or cardiopulcardiopulmonarybypass ( i so la ted  PLSVC impa i r s  t he  use  o f retrogradecardioplegia).I n  a d d i t i o n ,  a  h i g h e r  i n c i d e n c e o f  ar rhy thmias and conduct ionsystem abnormalities has been described in patients w i thPLSVC.  There  a re  two  p roposed mechanisms for this association:a dilated CS stretches the atrioventricular nodal tissue,which prepares a substrate for re-entrant tachycardias; or, theearly conduction tissue has close proximity to the cardinalvenous tissue and this leads to sinus node dysfunction. Lenoxet al. found sino-atrial node abnormalities in some patients withabsent RSVC and this condition may predispose to sick sinussyndrome.10-12In 10% of patients, a PLSVC may drain into the LA eitherdirectly or via an unroofed CS. This creates a right-to-left shuntand the risk of paradoxical embolism is markedly increased. Inaddition, drugs directly enter the systemic circulation when theyare applied from the left brachiocephalic vein.A final clinical implication of PLSVC (especially whenisolated) is a high incidence of accompanying congenital heartdefects, for example ventricular septal defect,  atr ial  septal defect,endocardial cushion defect or tetralogy of Fallot.3,13 Thereforeassociated congenital heart disease should be meticulouslysearched for.When PLSVC is present, the ECG often shows an abnormalP-wave axis and a normal or shortened PR interval. A geometricchange in the LA may be a possible mechanism for the 89  Acta Cardiologia Indonesiana (Vol 2 No. 2): 86-90Copyright©2013. Jurnal Perikanan (Journal of Fisheries Sciences) All Right Reservedleft-axisdeviation of the P wave.14 On chest X-ray, a crescent-shapedshadow of the PLSVC can be seen at the aortic knob or left uppermediastinum. After insertion of a pulmonary artery catheter intothe left subclavian or jugular vein, a control chest X-ray gives thefalse appearance that the catheter has passed through the vessel.The diagnosis can be confirmed by TTE, transoesophagealechocardiography (TEE), venous angiography, computed tomography(CT) or magnetic resonance imaging (MRI).On two-dimensional B-mode TTE, the characteristic finding is a dilated CS on parasternal long-axis view. The normal diameter of the CS is smaller than 1 cm and in the case of isolated PLSVC, severely increased flow can cause a truly giant CS.15,16 Other causes of dilated CS are: increased RA pressure, an anomalous systemic or pulmonary venous connection or a fistulous connection with the coronary arteries.17The next step in the echocardiographic evaluation should becontrast application with agitated saline. In normal individuals, agitated saline injection from the left or right antecubital vein results in opacification of the RA. In isolated PLSVC, as in ourcase, contrast given from the left or right arm opacifies the CS. When PLSVC is associated with an unroofed CS, contrast injection from either arm results in opacification of the LA. If RSVC accompanies the PLSVC, contrast given from the left arm first appears in the CS, whereas contrast given from the right arm first appears in the RA. On TEE, the anomalous PLSVC and absence of RSVC can be well visualised. In mid-oesophageal views, the PLSVC can be seen near to the left atrial appendage and left upper pulmonary vein. In the bicaval view, the absence of RSVC can be demonstrated.Other techniques (venous angiography, CT, MRI) directly visualise the venous anatomy and confirm the diagnosis. In the absence of an RSVC, central venous access should bemade from the femoral vein in patients with PLSVC. During right-sided open-heart surgical procedures, a PLSVC has to be drained by inserting a separate cannula into it. If the PLSVC drains into the LA and creates a large right-to-left shunt, surgical correction should be made. Again, central venous access via the femoral vein is a safer choice in this variation. When implanting permanent pacemakers, the left subclavian vein is preferred, as lead manipulation is easier. There is an acute angle between the CS ostium and the tricuspid valve, therefore the lead should belooped in the RA in order to enter the right ventricle.18 Handshapedstylets and active fixation leads are also helpful to overcometechnical difficulties.19Finally, a wide spectrum of clinicians (radiologists, sonographers,intervenists, intensivists, anaesthesiologists, cardiothoracic-surgeons) should be aware of  PLSVC and its variations inorder to avoid possible complications.Acknowledgement: We thank Mr Haryomo for assistance in handling and reconstruction of the cardiac CT scan that greatly improved the manuscript.REFERENCES1.  Danielpour PJ, Aalberg JK, El-Ramey M, Sivina M, Wodnicki H. 2005. Persistent left superior vena cava:an incidental finding during centralvenous catheterisation – a case report. Vasc Endovascular Surg 39: 109–111.2.  Palinkas A, Nagy E, Forster T, Morvai Z, Nagy E, Varga A. 2006. A case of absent right and persistent left superior vena cava. CardiovascUltrasound 4: 6.3.  Bartram U, van Praagh S, Levine JC, Hines M, Bensky AS, van Praagh R. 1997. Absent right superior vena cava in visceroatrial situs solitus. Am J Cardiol 80: 175–183.90  Gharini et al., 2016Copyright©2013. Jurnal Perikanan (Journal of Fisheries Sciences) All Right Reserved4.  Fischer DR, Zuberbuhler JR. 2002. Anomalous systemic venous return. In: Anderson RH, Baker E, Mccartney RF, eds. Paediatric Cardiology. New York: Churchill Livingstone, 851–865.5.  Shyamkumar NK, Brown R. 2007. Double superior vena cava with a persistentleft superior vena cava: an incidental finding during peripherallyinserted central catheter placement. Australas Radiol  51(SupplB): 257–259.6.  Antretter H, Cottogni M, Oberhauser A, Lhotta K, Furtwangler W. 1992. Pacemaker implantation via a persistent left superior vena cava inatresia of the true superior vena cava. Dtsch Med Wochenschr  117: 1394–1398.7.  Heye T, Wengenroth M, Schipp A, Johannes Dengler T, Grenacher L, Werner Kauffmann G. 2007. Persistent left superior vena cava with absentright superior vena cava: morphological CT features and clinical implications.Int J Cardiol 116: e103–105.8.  Azocar RJ, Narang P, Talmor D, Lisbon A, Kaynar AM. 2002. Persistent leftsuperior vena cava identified after cannulation of the right subclavianvein. Anesth Analg 95:305–307.9.  Recupero A, Pugliatti P, Rizzo F, Carerj S, Cavalli G, de Gregorio C,et al. 2007. Persistent left-sided superior vena cava: integrated noninvasivediagnosis. Echocardiography 24: 982–986.10. Camm AJ, Dymond D, Spurrel RA. 1979. Sinus node dysfunction associatedwith absence of right superior vena cava. Br Heart J 41:504–507.11.  Morgan DR, Hanratty CG, Dixon LJ, Trimble M, O’Keeffe DB. 2002. Anomalies of cardiac venous drainage associated with abnormalities ofcardiac conduction system. Europace 4: 281–287.12.  Lenox CC, Hashida Y, Anderson RH, Hubbard JD. 1985. Conduction tissueanomalies in absence of the right superior caval vein. Int J Cardiol 8: 251–260.13.  Postema PG, Rammeloo LA, van Litsenburg R, Rothuis EG, Hruda J. 2008. Left superior vena cava in pediatric cardiology associated with extracardiacanomalies. Int J Cardiol 123: 302–306.14. Choi JY, Anderson RH, Macartney FJ. 1987. Absent right superior cavalvein (vena cava) with normal atrial arrangement. Br Heart J 57:474–478.15.  Arat N, Sokmen Y, Golbasi Z. 2007. 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Isolated Persistent Left Superior Vena Cava, Role of Echocardiography Screening and CT angiography

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Isolated Persistent Left Superior Vena Cava, Role of Echocardiography Screening and CT angiography

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