CASE REPORT
An unusual presentation vvit persistent lef superior vena cava
C. Ebink, L.J. Bos, E.P.A. Vonken, B.KY Velthuis, M.J.M. Cramer
Persistent left superior vena cava (LSVC) is the most common congenital systemic venous anomaly, which may give rise to several problems. We present a case in which a persistent LSVC was an unsuspected finding. A 70-year-old male presented with intracerebral empyema which may have been caused by venous septic emboli from the left arm and facilitated by a persistent LSVC daining directly into the left atrium. Visualisation of the anomaly was performed with echocardiography and magnetic resonance angiography. In addition we present a brief review ofthe literature concerning this disorder. (Neth HeartJ 2004;12: 69-71.) Key words: persistent left superior vena cava, MRA A
persistent left superior vena cava (LSVC) is the
MA most common congenital anomaly of systemic venous return. Most frequently, the persistent LSVC drains into the right atrium through a dilated coronary sinus, without clinical implications.' However, several problems may arise due to variations in anatomy. A right-to-left shunt occurs if there is direct drainage into the left atrium, which can facilitate development of systemic abscesses or emboli. We report on a case of a patient suffering from intracerebral empyema, who was later diagnosed as having a persistent LSVC draining directly into the left atrium. Besides transC. Ebink Department of Cardiology, Medisch Centrum Rinmond-Zuid, PO Box 9100, 3007 AC Rotterdam L.J. Bos M.J.M. Cramer Department of Cardiology, Heart Lung Centre Utrecht, P0 Box 85500, 3508 GA Utrecht E.P.A. Vonken B.K. Velthuls Department of Radiology, University Medical Centre Utrecht
Correspondence to: M.J.M. Cramer E-mail: [email protected]
Netherlands Heart Journal, Volume 12, Number 2, February 2004
oesophageal echocardiographic (TOE) visualisation of this anomaly, we demonstrate that magnetic resonance angiography (MRA) is a noninvasive and accurate method for showing congenital heart disorders. Case report A 70-year-old male had suffered from slowly progressive headaches and diminishing strength on the right side of his body for two days. Except for treatment with inhalation corticosteroids and P2-sympaticomimetics for chronic obstructive pulmonary disease, his medical history was unremarkable. On admission to hospital a CT scan was performed and the initial diagnosis oflacunar infarction was made. The electrocardiogram showed no abnormalities.
However, his symptoms progressed during the following days, and he also developed short periods of rightsided muscle spasms, and fever. On neurological examination the muscle spasms ofthe right arm, which were epileptical in origin, were clearly visible. Moreover, paralysis ofthe right arm and leg with augmented tendon reflexes were observed. A mild mixed dysphasia and asymmetrical facial muscle use (right side affected) was also present. An intracerebral infection, such as cerebritis or cerebral empyema, was suspected. A repeat CT scan revealed an increase in the amount of left-parieto-occipitalsided oedema, suggestive of an intracerebral abscess. The patient was treated with antibiotics and antiepileptic medication. Three weeks after the initial symptoms his clinical condition suddenly deteriorated, leading to emergency surgery. After trepanation, a leftsided subdural empyema could be removed for the most part. After the procedure, the patient's clinical condition improved. In the meantime, a vigorous search for the source of infection was conducted. In this work-up, a TOE was performed. A left arm venous approach was chosen for agitated saline contrast administration (because of right-sided spasms), in order to detect a right-to-left 69
An unusual presentation with persistent left superior vena cava
Figure 1. Transthoracic echocardiographic images (apical fourchamber view) showing directfilling of the left atrium in case of left arm venous agitated saline contrast administration. LA=left atrium, LV=left ventricle.
shunt. This surprisingly revealed direct filling of the left atrium, without shunting at the atrial level. This was confirmed by transthoracic echocardiography (TTE, figure 1). TTE also showed filling ofthe right atrium with right arm and left leg contrast administration. A right-to-left shunt through a persistent LSVC was suspected, which may well have been the cause of the intracerebral empyema since it allows venous septic emboli from the left arm direct access to the systemic circulation. However, there were no signs of left arm infection at that time, nor could our patient remember having had one in the recent past. To elucidate anatomical details ofthis congenital disorder, an MRA scan was performed. This scan, indeed, showed a persistent LSVC draining into the left atrium (figure 2). Of note, no other abnormalities were seen.
Figure 2. MRA image showinga dorsal view ofthe aorta, the right superior vena cava and the persistent left superior vena cava. LVCS=left superior vena cava, R VCS=right superior vena cava.
Embryological considerations During early embryogenesis the cardiovascular system develops in a symmetric and paired fashion from angiogenic cell clusters into a complex four-chambered organ with arteries and veins. Likewise, the sinus venosus develops symmetrically by means of a left and right sinus horn interconnected by a central unpaired transverse part of the sinus venosus. At this stage the sinus venosus is not well demarcated from both atria.
Generally, a persistent LSVC is continuous with the (dilated) coronary sinus, through which it drains into the right atrium. A minority of persistent LSVC drain into the left atrium, usually with a communication between the coronary sinus and the left atrium (the so-called 'unroofed coronary sinus') or with a coronary sinus type atrial septal defect. A direct LSVC-left atrium connection with intact interatrial septum, as in our case, is extremely rare.5
By means of the development of a deep fold, the communications between the atrium and the sinus venosus disappear in the left horn and the transverse portion. As a result of numerous anastomotic channels between the right and left systemic veins and a preferential blood flow to the right, the right common cardinal vein and vitelline vein ultimately transform into the vena cava superior and vena cava inferior respectively. Simultaneously, the right sinus horn becomes the smooth-walled posterior part ofthe right
Discussion Patency of the LSVC without additional congenital heart disorders is usually a clinically silent and haemodynamically insignificant anatomical variation. In this report, however, we describe a patient with an intracerebral empyema that may well have been facilitated by a persistent LSVC draining into the left atrium. Another situation in which a persistent LSVC becomes a relevant anatomical finding is when a left superior venous approach to the heart is required.
70
atrium, while the transverse portion and distal sinus horn form the coronary sinus.2 The distal portion of the left sinus horn and the lower portion of the left cardinal vein usually obliterate, the remnants of which form the ligament of Marshall. In case of patency of the entire left sinus horn and left cardinal vein, a persistent LSVC is the result. The incidence of this venous anomaly is relatively high, it ranges from 0.3% in the general population to 4.3% in patients with congenital heart
disease.3'4
Netherlands Heart Journal, Volume 12, Number 2, February 2004
An unusual presentation with persistent left superior vena cava
Procedures where this becomes apparent and sometimes problematic include central venous catheter placement,6 cardiopulmonary bypass surgery,7 pulmonary artery catheterisation,8 positioning of pacemaker and cardioverter-defibrillator leads9"11 and transjugular liver approaches.'2 Furthermore, some reports suggest a greater prevalence of arrhythmias with a persistent LSVC through stretching of the atrioventricular node or His bundle by the dilated coronary sinus. However, the incidence of persistent LSVC in patients undergoing implantation of pacemakers or intracardiac defibrillators equals that of the general
population.""3,4 The diagnosis is usually made by echocardiography,"5"16 either transthoracic or transoesophageal. A dilated coronary sinus is the most common finding and should trigger the investigator to look for an LSVC, as well as for other defects.'7 Echocardiography is the most widely applied noninvasive heart visualisation technique used today. It is fast, highly cost-effective and readily available. However, there are several benefits of MRA in visualising and identfying congenital heart disease. It is also a noninvasive technique, thereby circumventing the risks involved with invasive techniques. MRA is superior to echocardiography when comparing its obtainable levels of tissue contrasts, field of view and details acquired. ECG-gated MRA can even add the fourth dimension to the obtained 3D MRA images. This is what makes MRA such a valuable tool for visualising cardiac congenital disorders, such as a persistent LSVC. Nowadays, multislice spiral computed tomography is a good alternative for MRA in diagnosing this disorder.'8 Conclusion We present the case of a patient with an extremely rare congenital cardiac malformation, which gave no symptoms for 70 years. Our patient suffered from a cerebral empyema which must have been caused by venous septic emboli from the left arm entering the brain through a persistent left superior vena cava draining directly into the left atrium. This complication of persistent LSVC has not been reported before. A persistent LSVC normally drains into the right atrium through a dilated coronary sinus. We recommend avoiding left arm venous access in patients with an LSVC-left atrium connection. c
Nctherlands Heart Journal, Volume 12, Number 2, February 2004
References Mantini E, Grondin CM, Lillehei CW, Edwards JE. Congenital anomalies involving the coronary sinus. Circulation 1966;33:317-27. 2 Miraldi F, di Gioia CR, Proietti P, de Santis M, d'Amati G, Galo P. Cardinal vein isomerism: an embryological hypothesis to explain a persistent left superior vena cava draining into the roof of the left atrium in the absence of coronary sinus and atrial septal defect. Cardiovasc Pathol 2002;11:149-52. 3 Campbell M, Deuchar DC. The left sided superior vena cava. Br HeartJ 1954;16:423-33. 4 Sarodia BD, Stoller JK. Persistent left superior vena cava: case report and literature review. Respir Care 2000;45:411-6. 5 Valdez-Cruz LM, Cayre RO. Anomalies of the systemic venous connection. In: Valdez-Cruz LM, Cayre RO, eds. Echocardiographic diagnosis of congenital heart disease. Philadelphia; Lippincott-Raven, 1999:505-10. 6 Greenberg M, Raggio C. Antecubital central venous catheter placement complicated by a persistent left superior vena cava. J Neurosurg Anaesthesiol 2000;12(2):114-7. 7 Garduno C, Chew S, Forbess J, Smith PK, Grocott HP. Persistent left superiorvena cava and partial anomalous pulmonaryvenous connection: incidental diagnosis by transesophageal echocardiography during coronary artery bypass surgery. JAm Soc Echocardiogr 1999;12(8):682-5. 8 Lai YC, Goh JC, Lim SH, Seah TG. Difficult pulmonary artery catheterization in a patient with persistent left superior vena cava. Anaesth Intensive Care 1998;26(6):671-3. 9 Biffi M, Boriani G, Frabetti L, Bronzetti G, Branzi A. Left superior vena cava persistence in patients undergoing pacemaker or cardioverter-defibrillator implantation: a 10-year experience. Chest2001; 120(1):139-44. 10 Stokkers PC, Ruiter GS de, Voogt WG de. Two chamber pacemaker implantation and a persistent left superior caval vein. Neth HeartJ2002;10:286-7. 11 Sharma PP, Vidaillet HJ, Tak T. Persistent left superior vena cava diagnosed during pacemaker implantation. Neth HeartJ2002; 10:467-8. 12 Bahramipour P, Abu-Judeh HH, Miller J. Transjugular intrahepatic portosystemic shunt placement in a patient with absent right superior vena cava and persistent left superior vena cava. Am 1
JRoentgenol 1999;173(3):631-2. 13 Huang SK Persistent left superior vena cava in a man with ventricular fibrillation. Chest 1986;89(1):155-7. 14 James TN, Marshall TK, Edwards JE. Cardiac electrical instability in the presence of a left superior vena cava. Circulation 1976;54: 689-97. 15 Pai RG. Echocardiographic features of persistent left superior vena cava. Echocardiography 1999;16:435-6. 16 Dearstine M, Taylor W, Kerut EK Persistent left superior vena cava: chest x-ray and echocardiographic findings. Echocardiography
2000;17(5):453-5. 17 Tak T, Crouch E, Drake GB. Persistent left superior vena cava: incidence, significance and clinical correlates. IntJ Cardiol 2002; 82:91-3. 18 Gerber TC, Kuzo RS. Persistent left superior vena cava demonstrated with multislice spiral computed tomography. Circulation
2002;105:e79.
71
An unusual presentation vvit persistent lef superior vena cava
C. Ebink, L.J. Bos, E.P.A. Vonken, B.KY Velthuis, M.J.M. Cramer
Persistent left superior vena cava (LSVC) is the most common congenital systemic venous anomaly, which may give rise to several problems. We present a case in which a persistent LSVC was an unsuspected finding. A 70-year-old male presented with intracerebral empyema which may have been caused by venous septic emboli from the left arm and facilitated by a persistent LSVC daining directly into the left atrium. Visualisation of the anomaly was performed with echocardiography and magnetic resonance angiography. In addition we present a brief review ofthe literature concerning this disorder. (Neth HeartJ 2004;12: 69-71.) Key words: persistent left superior vena cava, MRA A
persistent left superior vena cava (LSVC) is the
MA most common congenital anomaly of systemic venous return. Most frequently, the persistent LSVC drains into the right atrium through a dilated coronary sinus, without clinical implications.' However, several problems may arise due to variations in anatomy. A right-to-left shunt occurs if there is direct drainage into the left atrium, which can facilitate development of systemic abscesses or emboli. We report on a case of a patient suffering from intracerebral empyema, who was later diagnosed as having a persistent LSVC draining directly into the left atrium. Besides transC. Ebink Department of Cardiology, Medisch Centrum Rinmond-Zuid, PO Box 9100, 3007 AC Rotterdam L.J. Bos M.J.M. Cramer Department of Cardiology, Heart Lung Centre Utrecht, P0 Box 85500, 3508 GA Utrecht E.P.A. Vonken B.K. Velthuls Department of Radiology, University Medical Centre Utrecht
Correspondence to: M.J.M. Cramer E-mail: [email protected]
Netherlands Heart Journal, Volume 12, Number 2, February 2004
oesophageal echocardiographic (TOE) visualisation of this anomaly, we demonstrate that magnetic resonance angiography (MRA) is a noninvasive and accurate method for showing congenital heart disorders. Case report A 70-year-old male had suffered from slowly progressive headaches and diminishing strength on the right side of his body for two days. Except for treatment with inhalation corticosteroids and P2-sympaticomimetics for chronic obstructive pulmonary disease, his medical history was unremarkable. On admission to hospital a CT scan was performed and the initial diagnosis oflacunar infarction was made. The electrocardiogram showed no abnormalities.
However, his symptoms progressed during the following days, and he also developed short periods of rightsided muscle spasms, and fever. On neurological examination the muscle spasms ofthe right arm, which were epileptical in origin, were clearly visible. Moreover, paralysis ofthe right arm and leg with augmented tendon reflexes were observed. A mild mixed dysphasia and asymmetrical facial muscle use (right side affected) was also present. An intracerebral infection, such as cerebritis or cerebral empyema, was suspected. A repeat CT scan revealed an increase in the amount of left-parieto-occipitalsided oedema, suggestive of an intracerebral abscess. The patient was treated with antibiotics and antiepileptic medication. Three weeks after the initial symptoms his clinical condition suddenly deteriorated, leading to emergency surgery. After trepanation, a leftsided subdural empyema could be removed for the most part. After the procedure, the patient's clinical condition improved. In the meantime, a vigorous search for the source of infection was conducted. In this work-up, a TOE was performed. A left arm venous approach was chosen for agitated saline contrast administration (because of right-sided spasms), in order to detect a right-to-left 69
An unusual presentation with persistent left superior vena cava
Figure 1. Transthoracic echocardiographic images (apical fourchamber view) showing directfilling of the left atrium in case of left arm venous agitated saline contrast administration. LA=left atrium, LV=left ventricle.
shunt. This surprisingly revealed direct filling of the left atrium, without shunting at the atrial level. This was confirmed by transthoracic echocardiography (TTE, figure 1). TTE also showed filling ofthe right atrium with right arm and left leg contrast administration. A right-to-left shunt through a persistent LSVC was suspected, which may well have been the cause of the intracerebral empyema since it allows venous septic emboli from the left arm direct access to the systemic circulation. However, there were no signs of left arm infection at that time, nor could our patient remember having had one in the recent past. To elucidate anatomical details ofthis congenital disorder, an MRA scan was performed. This scan, indeed, showed a persistent LSVC draining into the left atrium (figure 2). Of note, no other abnormalities were seen.
Figure 2. MRA image showinga dorsal view ofthe aorta, the right superior vena cava and the persistent left superior vena cava. LVCS=left superior vena cava, R VCS=right superior vena cava.
Embryological considerations During early embryogenesis the cardiovascular system develops in a symmetric and paired fashion from angiogenic cell clusters into a complex four-chambered organ with arteries and veins. Likewise, the sinus venosus develops symmetrically by means of a left and right sinus horn interconnected by a central unpaired transverse part of the sinus venosus. At this stage the sinus venosus is not well demarcated from both atria.
Generally, a persistent LSVC is continuous with the (dilated) coronary sinus, through which it drains into the right atrium. A minority of persistent LSVC drain into the left atrium, usually with a communication between the coronary sinus and the left atrium (the so-called 'unroofed coronary sinus') or with a coronary sinus type atrial septal defect. A direct LSVC-left atrium connection with intact interatrial septum, as in our case, is extremely rare.5
By means of the development of a deep fold, the communications between the atrium and the sinus venosus disappear in the left horn and the transverse portion. As a result of numerous anastomotic channels between the right and left systemic veins and a preferential blood flow to the right, the right common cardinal vein and vitelline vein ultimately transform into the vena cava superior and vena cava inferior respectively. Simultaneously, the right sinus horn becomes the smooth-walled posterior part ofthe right
Discussion Patency of the LSVC without additional congenital heart disorders is usually a clinically silent and haemodynamically insignificant anatomical variation. In this report, however, we describe a patient with an intracerebral empyema that may well have been facilitated by a persistent LSVC draining into the left atrium. Another situation in which a persistent LSVC becomes a relevant anatomical finding is when a left superior venous approach to the heart is required.
70
atrium, while the transverse portion and distal sinus horn form the coronary sinus.2 The distal portion of the left sinus horn and the lower portion of the left cardinal vein usually obliterate, the remnants of which form the ligament of Marshall. In case of patency of the entire left sinus horn and left cardinal vein, a persistent LSVC is the result. The incidence of this venous anomaly is relatively high, it ranges from 0.3% in the general population to 4.3% in patients with congenital heart
disease.3'4
Netherlands Heart Journal, Volume 12, Number 2, February 2004
An unusual presentation with persistent left superior vena cava
Procedures where this becomes apparent and sometimes problematic include central venous catheter placement,6 cardiopulmonary bypass surgery,7 pulmonary artery catheterisation,8 positioning of pacemaker and cardioverter-defibrillator leads9"11 and transjugular liver approaches.'2 Furthermore, some reports suggest a greater prevalence of arrhythmias with a persistent LSVC through stretching of the atrioventricular node or His bundle by the dilated coronary sinus. However, the incidence of persistent LSVC in patients undergoing implantation of pacemakers or intracardiac defibrillators equals that of the general
population.""3,4 The diagnosis is usually made by echocardiography,"5"16 either transthoracic or transoesophageal. A dilated coronary sinus is the most common finding and should trigger the investigator to look for an LSVC, as well as for other defects.'7 Echocardiography is the most widely applied noninvasive heart visualisation technique used today. It is fast, highly cost-effective and readily available. However, there are several benefits of MRA in visualising and identfying congenital heart disease. It is also a noninvasive technique, thereby circumventing the risks involved with invasive techniques. MRA is superior to echocardiography when comparing its obtainable levels of tissue contrasts, field of view and details acquired. ECG-gated MRA can even add the fourth dimension to the obtained 3D MRA images. This is what makes MRA such a valuable tool for visualising cardiac congenital disorders, such as a persistent LSVC. Nowadays, multislice spiral computed tomography is a good alternative for MRA in diagnosing this disorder.'8 Conclusion We present the case of a patient with an extremely rare congenital cardiac malformation, which gave no symptoms for 70 years. Our patient suffered from a cerebral empyema which must have been caused by venous septic emboli from the left arm entering the brain through a persistent left superior vena cava draining directly into the left atrium. This complication of persistent LSVC has not been reported before. A persistent LSVC normally drains into the right atrium through a dilated coronary sinus. We recommend avoiding left arm venous access in patients with an LSVC-left atrium connection. c
Nctherlands Heart Journal, Volume 12, Number 2, February 2004
References Mantini E, Grondin CM, Lillehei CW, Edwards JE. Congenital anomalies involving the coronary sinus. Circulation 1966;33:317-27. 2 Miraldi F, di Gioia CR, Proietti P, de Santis M, d'Amati G, Galo P. Cardinal vein isomerism: an embryological hypothesis to explain a persistent left superior vena cava draining into the roof of the left atrium in the absence of coronary sinus and atrial septal defect. Cardiovasc Pathol 2002;11:149-52. 3 Campbell M, Deuchar DC. The left sided superior vena cava. Br HeartJ 1954;16:423-33. 4 Sarodia BD, Stoller JK. Persistent left superior vena cava: case report and literature review. Respir Care 2000;45:411-6. 5 Valdez-Cruz LM, Cayre RO. Anomalies of the systemic venous connection. In: Valdez-Cruz LM, Cayre RO, eds. Echocardiographic diagnosis of congenital heart disease. Philadelphia; Lippincott-Raven, 1999:505-10. 6 Greenberg M, Raggio C. Antecubital central venous catheter placement complicated by a persistent left superior vena cava. J Neurosurg Anaesthesiol 2000;12(2):114-7. 7 Garduno C, Chew S, Forbess J, Smith PK, Grocott HP. Persistent left superiorvena cava and partial anomalous pulmonaryvenous connection: incidental diagnosis by transesophageal echocardiography during coronary artery bypass surgery. JAm Soc Echocardiogr 1999;12(8):682-5. 8 Lai YC, Goh JC, Lim SH, Seah TG. Difficult pulmonary artery catheterization in a patient with persistent left superior vena cava. Anaesth Intensive Care 1998;26(6):671-3. 9 Biffi M, Boriani G, Frabetti L, Bronzetti G, Branzi A. Left superior vena cava persistence in patients undergoing pacemaker or cardioverter-defibrillator implantation: a 10-year experience. Chest2001; 120(1):139-44. 10 Stokkers PC, Ruiter GS de, Voogt WG de. Two chamber pacemaker implantation and a persistent left superior caval vein. Neth HeartJ2002;10:286-7. 11 Sharma PP, Vidaillet HJ, Tak T. Persistent left superior vena cava diagnosed during pacemaker implantation. Neth HeartJ2002; 10:467-8. 12 Bahramipour P, Abu-Judeh HH, Miller J. Transjugular intrahepatic portosystemic shunt placement in a patient with absent right superior vena cava and persistent left superior vena cava. Am 1
JRoentgenol 1999;173(3):631-2. 13 Huang SK Persistent left superior vena cava in a man with ventricular fibrillation. Chest 1986;89(1):155-7. 14 James TN, Marshall TK, Edwards JE. Cardiac electrical instability in the presence of a left superior vena cava. Circulation 1976;54: 689-97. 15 Pai RG. Echocardiographic features of persistent left superior vena cava. Echocardiography 1999;16:435-6. 16 Dearstine M, Taylor W, Kerut EK Persistent left superior vena cava: chest x-ray and echocardiographic findings. Echocardiography
2000;17(5):453-5. 17 Tak T, Crouch E, Drake GB. Persistent left superior vena cava: incidence, significance and clinical correlates. IntJ Cardiol 2002; 82:91-3. 18 Gerber TC, Kuzo RS. Persistent left superior vena cava demonstrated with multislice spiral computed tomography. Circulation
2002;105:e79.
71
