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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 11  |  Issue : 1  |  Page : 42-45
Anomalous connection of superior vena cava to the left atrium masquerading as epilepsy: A case report with review of literature

Department of Cardiothoracic and Vascular Surgery, GB Pant Hospital, New Delhi, India

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An 11-year-old girl who presented with recurrent epileptic fits was eventually found to have a superior vena cava draining into the left atrium. There was no atrial septal defect. The patient underwent a successful surgical correction.

Keywords: Epilepsy, heart defects, congenital, brain abscess, cardiac surgery

How to cite this article:
Singh S, Geelani MA, Modi P, Niwaria Y, Shahi S, Banerjee A. Anomalous connection of superior vena cava to the left atrium masquerading as epilepsy: A case report with review of literature. Ann Card Anaesth 2008;11:42-5

How to cite this URL:
Singh S, Geelani MA, Modi P, Niwaria Y, Shahi S, Banerjee A. Anomalous connection of superior vena cava to the left atrium masquerading as epilepsy: A case report with review of literature. Ann Card Anaesth [serial online] 2008 [cited 2020 Oct 1];11:42-5. Available from:

The drainage of the superior vena cava (SVC) into the left atrium (LA) is a rare anomaly. Very few cases have been reported in literature till date. [1],[2],[3],[4],[5],[6],[7] We report a case of anomalous connection of SVC to LA without atrial septal defect (ASD) in a young female who presented with cerebral abscess and seizures. The uniqueness of the case lay in the manner in which her neurological symptoms completely diverted attention from the intracardiac lesion.

   Case Report Top

An 11-year-old girl was referred to us for surgical correction of an echocardiographically proven case of the SVC draining into LA. She was admitted a year ago in the neurosurgical ward of the authors' institute with a history of sudden onset of headache, fever with chills, rigours and recurrent syncopal attacks. Examination revealed weakness of the left upper and lower limbs. Computed tomography of brain enabled the diagnosis of right parietooccipital abscess; this was subsequently drained surgically. The postoperative course was uneventful and she was discharged. Apparently, her cardiac lesion went unnoticed.

A year later, she presented with a history of several episodes of left-sided partial seizures. Each such episode of seizure lasted for up to approximately 20 s. For one and half months prior to her presentation to us, the fits became generalised and more frequent. She also complained of a staggering gait and history of objects slipping from her grasp. There was no history of fall, though she had occasional nausea/vomiting. Examination revealed central cyanosis that was not found in her earlier case details. During general examination, grade 2/6 mid diastolic murmur was noted at the apex of the heart. Haemogram, liver function and renal function tests were within the normal limits. Chest X-ray revealed the enlargement of LA and left ventricle (LV). Two-dimensional echocardiography with colour Doppler studies revealed SVC draining into LA. The diagnosis was confirmed with the help of saline contrast. When saline was injected into a vein on each forearm, the contrast was observed in the LA, LV and aorta without the visualisation of the right ventricle (RV). On injection of agitated saline in the lower extremity veins, the right atrium (RA), RV and pulmonary artery were visualised. This suggested the normal drainage of inferior vena cava (IVC) and anomalous connection of the SVC to the LA with intact interatrial septum.

Neurological treatment was commenced with orally administered phenytoin and lamotrigine. In spite of regular treatment with anticonvulsants for 2 months, convulsions were not controlled, and they used to occur whenever she would wake up in the morning. She was scheduled for the correction of the cardiac anomaly with a plan to anastomose the SVC to the RA along with ligation or division of the SVC-LA connection without cardiopulmonary bypass (CPB). However, the readiness to institute CPB was ensured. After inserting an arterial line, peripheral venous lines were placed both in femoral and brachial veins and decision was taken not to cannulate or use the right internal jugular vein because of its proximity to the anomaly. Left internal jugular venous line was put to monitor the pressure during clamp period of SVC. Transoesophageal echocardiographic (TOE) probe was inserted as part of institutional protocol. The absence of an ASD was confirmed by TOE. Surgery was performed through median sternotomy. On opening the pericardium, there was no left SVC; the pulmonary venous connections were normal. The right and left brachiocephalic veins joined to form the SVC that was draining into the LA posterior to the atrial septal groove superiorly [Figure - 1]. The superior portion of the RA was blind, and IVC was found to be normally connected. No other associated congenital anomaly was observed. The SVC was observed passing anterior to the right pulmonary artery and then joining the LA immediately posterior to the interatrial groove in its superior aspect. An umbilical tape was passed around the junction of LA and SVC. The RA appendage (RAA) was anastomosed to SVC using side-biting clamps. The use of such a clamp, assisted by the large caliber of the SVC, ensured that there were neither deleterious pressure variations in the venous system nor the possibility of venous brain oedema. SVC and LA junction was ligated with umbilical tape [Figure - 2] without transecting the SVC.

A marked improvement was immediately observed in the O 2 saturation, and TOE demonstrated flow across the newly constructed SVC-RA anastomosis with no flow across the ligated SVC-LA junction.

The patient was transferred to the intensive care unit on a ventilator and was extubated the same evening. Her intraoperative behaviour and recovery from anaesthesia were uneventful. She made a smooth postoperative recovery till the detection of right-sided pleural effusion on the seventh postoperative day. The effusion responded to diuretics and resolved in 3 days.

   Discussion Top

The congenital anomalies of the systemic venous connection of the heart represent a rather wide and heterogeneous group of malformations, whose physiological consequences may vary being inconsequential to manifesting with severe systemic arterial desaturation. [8] Several variations of these malformations have been documented. [9],[10],[11],[12]

Van Praagh et al . [13] have advocated a shorthand method to designate the organisational pattern of the venoatrial connection, the pattern of ventricular looping and the ventriculoarterial connection. By this method, the situs of each of these segments is recorded in the sequence {venoatrial connection, ventricular looping, ventriculoarterial connection}. Situs solitus indicates the usual or normal pattern, situs inversus is the mirror-image of the normal pattern and situs ambiguus denotes an ambiguous or indeterminate pattern. The situs of the venoatrial connection can either be solitus, inversus or ambiguus .

Venoatrial situs solitus is the normal arrangement in which the morphological RA is right-sided and the morphological LA is left-sided. Situs solitus is present when, on a plain chest film, the liver shadow is right-sided and the stomach bubble is left-sided. Under these circumstances, the morphological RA is almost always right-sided and the morphological LA is left-sided.

Venoatrial situs inversus is the mirror image or inverted type of venoatrial situs in which the RA is left-sided and the LA is right-sided. Situs inversu s is almost certainly present when the liver shadow is left-sided and the stomach bubble is right-sided, and under these circumstances, the RA is almost always left-sided and the LA is almost always right-sided. In venoatrial situs solitus or situs inversus , the inferior vena cava or the suprahepatic inferior vena cava in the presence of an interrupted inferior vena cava is a highly reliable diagnostic marker of the location of the morphological RA. The suprahepatic cava is therefore a useful diagnostic marker with important clinical ramifications. In contrast, the SVC is not at all a reliable diagnostic marker of the RA since it may return to the LA where it referred to as the laevoatrial cardinal vein.

The drainage of the SVC into the LA is uncommon. [1],[5],[6] In this case, there was a single SVC that was right-sided. Kabbani et al . have reported a case with single SVC draining into the LA that was left-sided. [14] The drainage of the SVC into the LA leads to the right-to-left shunt that can cause cyanosis and left ventricular hypertrophy, [15] although left ventricular hypertrophy was not observed in the this case. The symptoms depend upon the amount of the right-to-left shunt and complications of systemic embolisation. This patient presented with symptoms attributable to the cerebral abscess of left parietooccipital lobes of brain because of the right-to-left shunt. The anomalous connection of SVC to LA was detected by echocardiography. The diagnosis was arrived at on the basis of characteristic findings on contrast echocardiography alone, Apart from the additional options of cardiac catheterisation and cineangiography, the diagnosis of such an anomaly can also be confirmed by radionuclide angiocardiography, as reported by Park and associates. [16]

A few similar cases that were surgically treated without CPB (including SVC-RAA anastomosis) are also documented. [17],[18],[19] Several modifications such as the insertion of SVC-RA shunt or an extracardiac approach [20] have also been described. The issue of interest in the present case is her presentation with predominantly neurological features, including cerebral abscess. A logical explanation would be the possibility of systemic embolisation of air or infective material during some injection administered into the upper limb veins.

No technical problems were encountered in this case. The patient showed features of the right-sided failure, probably because of the right-to-left shunt and required diuretic therapy. We conclude that a rare anomaly of SVC draining into LA can be encountered with symptoms unrelated to cardiovascular system; this can be repaired without CPB.

   References Top

1.Bharati S, Lev M. Direct entry of the right superior vena cava into the left atrium with aneurysmal dilatation and stenosis at its entry into the right atrium with stenosis of the pulmonary veins: A rare case. Pediatr Cardiol 1984;5:123-6.  Back to cited text no. 1  [PUBMED]  
2.Truman AT, Rao PS, Kulangara RJ. Use of contrast echocardiography in diagnosis of anomalous connection of right superior vena cava to left atrium. Br Heart J 1980;44:718-23.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Rosenkranz S, Stablein A, Deutsch HJ, Verhoeven HW, Erdmann E. Anomalous drainage of the right superior vena cava into the left atrium in a 61-year-old woman. Int J Cardiol 1998;64:285-91.  Back to cited text no. 3    
4.Kakadekar AP, McKay R,Tyrrell MJ. Isolated connection of the right superior caval vein to the left atrium: Non-invasive neonatal diagnosis. Cardiol Young 1999;9:310-4.  Back to cited text no. 4  [PUBMED]  
5.Park HM, Summerer MH, Preuss K, Armstrong WF, Mahomed Y, Hamilton DJ. Anomalous drainage of the right superior vena cava into the left atrium. J Am Coll Cardiol 1983;2:358-62.  Back to cited text no. 5  [PUBMED]  
6.Vaquez-Perez J, Frontera-Izquierdo P. Anomalous drainage of the right superior vena cava into the left atrium as an isolated anomaly: Rare case report. Am Heart J 1979;97:89-91.  Back to cited text no. 6    
7.Braudo M, Beanlands DS, Trusler G. Anomalous drainage of the right superior vena cava into the left atrium. Can Med Assoc J 1968;99:715-9.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Mazzucco A, Bortolotti U, Stellin G, Gallucci V. Anomalies of the systemic venous return: A review. J Card Surg 1990;5:122-33.  Back to cited text no. 8  [PUBMED]  
9.Raghib G, Ruttenberg HD, Anderson RC, Amplatz K, Adams P Jr, Edwards Je. Termination of left superior vena cava in left atrium, atrial septal defect and absence of coronary sinus: A developmental complex. Circulation 1965;31:906-18.  Back to cited text no. 9  [PUBMED]  
10.Okumori M, Hyuga M, Ogata S, Akamatsu T, Otomi S, Ota S. Raghib's syndrome: A report of two cases. Jpn J Surg 1982;12:356-61.  Back to cited text no. 10  [PUBMED]  
11.Fujimura Y, Katoh T, Koizumi S. Raghib's syndrome associated with cor triatriatum: A rare surgical case report. Nippon Kyobu Geka Gakkai Zasshi 1989;37:2417-21.  Back to cited text no. 11  [PUBMED]  
12.Honnekeri ST, Tendolkar AG, Lokhandwala YY. Double-orifice mitral and tricuspid valves in association with the Raghib complex. Ann Thorac Surg 1993;55:1001-2.  Back to cited text no. 12  [PUBMED]  
13.Truman AT, Rao PS, Kulangara RJ. Use of contrast echocardiography in diagnosis of anomalous connection of right superior vena cava to left atrium. Br Heart J 1980;44:718-23.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]
14.Kabbani SS, Feldmann M, Angelini P, Leachman RD, Cooley DA. Single (left) superior vena cava draining into the left atrium: Surgical repair. Ann Thorac Surg 1973;16:518-25.  Back to cited text no. 14    
15.Brown JF, Crumpton CW, Huston JH, Rowe GG, Tuchman H, Weinstein AB. Superior vana cava draining into left atrium: Another cause for left ventricular hypertrophy with cyanotic congenital heart disease. Am J Med 1956;21:481-4.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]
16.Park HM, Smith ET, Silberstein EB. Isolated right superior vena cava draining into left atrium diagnosed by radionuclide angiocardiography. J Nucl Med 1973;14:240-2.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]
17.Shapiro EP, Al-Sadir J, Campbell NP, Thilenius OG, Anagnostopoulos CE, Hays P. Drainage of right superior vena cava into both atria. Review of the literature and description of a case presenting with polycythemia and paradoxical embolization. Circulation 1981;63:712-7.  Back to cited text no. 17    
18.De Geest B, Vandommele J, Herregods MC, Vanhaecke J, Gewillig M, Daenen W, et al . Isolated left-sided superior vena cava draining into the left atrium associated with recurring intra-cerebral abscesses: A case report. Acta Cardiol 1994;49:175-82.  Back to cited text no. 18  [PUBMED]  
19.Nazem A, Sell JE. Closed technique for repair of right superior vena cava draining to left atrium. Ann Thorac Surg 1993;55:1568-70.  Back to cited text no. 19  [PUBMED]  
20.Takach TJ, Cortelli M, Lonquist JL, Cooley DA. Correction of anomalous systemic venous drainage: Transposition of left SVC to left PA. Ann Thorac Surg 1997;63:228-30.  Back to cited text no. 20  [PUBMED]  [FULLTEXT]

Correspondence Address:
Amit Banerjee
Department of Cardiothoracic and Vascular Surgery, GB Pant Hospital, New Delhi - 110 002
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9784.38449

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  [Figure - 1], [Figure - 2]

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