Persistent left superior vena cava and central venous catheter position ...

Surg Radiol Anat (2003) 25: 315–321 DOI 10.1007/s00276-003-0138-6

R AD IO L OG I C AN A TO M Y

W. Schummer Æ C. Schummer Æ R. Fro¨ber

Persistent left superior vena cava and central venous catheter position: clinical impact illustrated by four cases

Received: 19 May 2002 / Accepted: 20 October 2002 / Published online: 31 July 2003 Ó Springer-Verlag 2003

Abstract Variations in the course of the blood vessels are often incidental findings during clinical examination. A persistent left superior vena cava (LSVC) is really not rare (healthy individuals, 0.3–0.5%; patients with congenital heart disease, 4%) and serious complications have been described during catheterization in adults with LSVC (shock, cardiac arrest, angina). Therefore variations of the superior vena cava should be considered, especially when central venous catheterization via the subclavian or internal jugular vein is difficult. We describe the embryogenesis and the anatomic variations of persistent LSVC. Subsequently we suggest a classification of superior vena cava according to the positioning of a central venous catheter on the chest radiograph: type I, normal anatomy; type II, only persistent left superior vena cava; type IIIa, right and left superior vena cava with connection; type IIIb, right and left superior vena cava without connection. This classification is illustrated by four clinical cases.

persistante (LSVC) n’est pas vraiment rare (0,3 a` 0,5% des individus sains, 4% des patients pre´sentant une cardiopathie conge´nitale) et de se´rieuses complications ont de´ja` e´te´ de´crites au cours du cathe´te´risme d’adultes pre´sentant un LSVC (choc, arreˆt cardiaque, angor). C’est pourquoi les variations de la veine cave supe´rieure devraient eˆtre envisage´es, particulie`rement quand un cathe´te´risme veineux central par la veine subclavie`re ou la veine jugulaire interne s’ave`re difficile. Nous de´crivons l’embryogene`se des varie´te´s anatomiques de la veine cave supe´rieure gauche persistante. Nous sugge´rons ensuite une classification de la veine cave supe´rieure selon la position d’un cathe´ter veineux central sur la radiographie de thorax: type I, anatomie normale; type II, veine cave supe´rieure gauche persistante isole´e; type IIIa, veines caves supe´rieures droite et gauche relie´es l’une a` l’autre; type IIIb, veines caves supe´rieures droite et gauche sans connexion. Cette classification est illustre´e par 4 cas cliniques.

Veine cave supe´rieure gauche persistante et position d’un cathe´ter veineux central

Keywords Superior vena cava, variation Æ Central venous catheterization, adverse effects Æ Case report

Re´sume´ Les variations du trajet des vaisseaux sanguins sont souvent des constatations accidentelles au cours de l’examen clinique. Une veine cave supe´rieure gauche

Introduction

Electronic Supplementary Material The french version of this article is available in the form of electronic supplementary material and can be obtained by using the Springer Link server located at http://dx.doi.org/10.1007/s00276-003-0138-6. W. Schummer (&) Æ C. Schummer Clinic for Anesthesiology and Intensive Care Medicine, Friedrich-Schiller University, Bachstrasse 18, 07743 Jena, Germany E-mail: [email protected] Tel.: +49-3641-933041 Fax: +49-3641-933256 R. Fro¨ber Institute of Anatomy I, Friedrich-Schiller University, Teichgraben 7, 07740 Jena, Germany

Detailed anatomic knowledge of the great vessels of the neck and thorax is a prerequisite for safe anesthetic and critical care practice. Whilst normal anatomy and its radiological appearance is core knowledge, not every clinician is familiar with the anatomic variation of the superior vena cava known as persistent left superior vena cava (LSVC). It is reported with an incidence of about 0.3–0.5% [1, 11, 13, 26, 33] and normally remains asymptomatic [11, 33]. Nevertheless, it can create difficulties during central venous and pulmonary artery catheterization [18, 22]. We report on central venous canulation in four cases of an unknown LSVC. Moreover, the anatomic relations of such a LSVC to the neighboring anatomic structures quite often remain

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obscure. For better understanding we propose a classification of the supracardial venous system in the following way (Fig. 1): type I, normal precardial venous arrangement; type II, single LSVC; type III, doubled superior vena cava. The right superior vena cava (RSVC) is either connected to the left one by an anastomosis (type IIIa) or is without any connection (type IIIb).

Case reports Case 1 (type II) A 43-year-old woman was admitted with subarachnoid hemorrhage (Hunt and Hess grade 4). A multilumen central venous catheter (CVC) was placed via the left internal jugular vein. On the postinsertional radiograph, the catheter was seen running down the left mediastinal border. A malposition in the descending aorta was excluded by a typical intravenous waveform (a lateral chest radiograph was not taken). Nevertheless, a malposition was assumed, and a second multilumen catheter was introduced via the right internal jugular vein. During advancement of the guidewire, minor resistance was met early but could easily be passed. The vessel dilator was inserted to its full length (9 cm), then the catheter was positioned over the guidewire. Due to some resistance, this was not done so easily. After removal the guidewire was deformed. As no blood return was obtained through the distal lumen of the catheter the catheter was removed. A chest radiograph was taken which showed a massive right hemothorax (Fig. 2). Even though two chest tubes had been inserted, the bleeding could not be managed conservatively. Despite massive volume replacement and catecholamines, the patient had become hemodynamically unstable, and was taken to the operating theater for emergency thoracotomy. Before surgery a double-lumen hemodialysis catheter was inserted, again via the right internal jugular vein, by the Seldinger technique. Intraoperatively the surgeon noticed that this hemodialysis catheter had perforated the brachiocephalic (innominate) vein; no right superior vena cava could be identified. This catheter was also removed; the vessel injury was sutured. No other bleeding sources were detected. Due to the massive hemodynamic compromise encountered in this incident the cerebral situation deteriorated further. The patient died 7 days later.

Fig. 1 Variations of the superior vena cava: type I–IIIa. 1, coronary sinus; 2, right superior vena cava (RSVC); 3, left superior vena cava (LSVC); 4, internal jugular vein (IJV); 5, subclavian vein (SV); 6, right and 7, left brachiocephalic vein (*type IIIb: left brachiocephalic vein is obliterated or missing); 8, right and 9, left internal thoracic vein; 10, aortic arch; 11, main pulmonary artery. (Redrawn from various sources)

Fig. 2 Case 1 (type II).: Central venous catheter (CVC) in the LSVC. Right hemothorax with a chest tube in place after a rightsided canulation attempt Case 2 (type IIIb) After an aorto-bifemoral bypass procedure (Y-graft) a pulmonary artery catheter was placed via the left internal jugular vein in a 67-year-old woman with aorto-occlusive disease. Placing the guidewire over the right internal jugular vein had failed. Advancement of the floating pulmonary artery catheter had not been hampered by any resistance; hemodynamic data could be obtained. The postinsertional radiograph showed the catheter tip positioned in the right pulmonary artery (Fig. 3). But with the catheter itself running down the left mediastinal border, a persistent superior vena cava was presumed. During her stay in the intensive care unit other central venous catheters were placed, depicting a second, right-sided superior vena cava (RSVC) without obvious connection to the left one (Fig. 4).

Case 3 (type II) A 73-year-old woman was scheduled for implantation of a singlechamber pacemaker via the left subclavian vein. When under fluoroscopic control the pacemaker lead did not run down the right

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Fig. 3 Case 2 (type IIIb). Pulmonary artery catheter with its tip in the right pulmonary artery

Fig. 5 Case 3 (type II). Angiographic catheter inserted through the right femoral vein with the tip in the LSVC. Contrast material enhances only the LSVC and left brachiocephalic vein as well as the right atrium at about 20 cm depth. The catheter could be placed without any resistance. An intra-atrial electrocardiogram was obtained: high P-waves could be derived as in an intra-atrial catheter position. With minimal position changes of the catheter P-waves were alternating between positive and negative deflections. The CVC was retracted to 19 cm; all the lumen could easily be aspirated. On the postinsertional radiograph both lines were depicted in the left thorax (Fig. 6). The radiologist argued a malposition in the descending aorta. This had been excluded earlier by a typical venous pressure waveform. The diagnosis of a LSVC was confirmed by excluding other left-sided malpositions by a contrast-enhanced lateral chest radiograph (Fig. 7).

Fig. 4 Case 2 (type IIIb). CVC in the RSVC inserted through the right internal jugular vein, and CVC in the LSVC inserted through the left subclavian vein mediastinal border, an angiographic catheter was inserted in the right femoral vein and advanced through the inferior vena cava, right atrium and coronary sinus into a left-sided vena cava (Fig. 5). Contrast enhancement proved the absence of a right-sided superior vena cava. With no further difficulties, the pacemaker lead could be advanced through the LSVC and coronary sinus into the right atrium, and was anchored in the right ventricle.

Case 4 (type IIIb) Under general anesthesia, a multilumen catheter and a sheath introducer set were inserted through the left internal jugular vein in a 49-year-old man scheduled for multivisceral resection due to metastasizing carcinoma of the gall bladder. During advancement of the guidewire of the multilumen CVC minor resistance was felt

Fig. 6 Case 4 (type IIIb). Venous access system in the RSVC inserted through the right subclavian vein; CVC and sheath introducer system in the LSVC inserted through the left internal jugular vein

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Fig. 7 Case 4 (type IIIb). Lateral view. The arrow marks the tip of the venous access system in the RSVC: contrast dye study via the CVC in the LSVC (arrowhead marks the tip of the CVC)

Discussion Anatomy and embryology In early embryonic development (24th day of gestation), the venous blood of the head and the upper half of the body is drained by way of bilateral symmetrically arranged veins: the two anterior cardinal veins (precardinal). Complex processes of segmental fusion and regression between the fourth and eighth weeks of gestation result in the definitive formation of the superior vena cava. During the partitioning of the primitive cardiac atrium the influx of the cardinal veins shifts more to the right atrium. As a consequence the oblique ventro-aortic anastomosis between the two precardinal veins is preferred and enlarged to the left brachiocephalic (innominate) vein. The part of the left anterior cardinal vein which was situated below this anastomosis is gradually obliterated. A residue is the oblique vein of the left atrium (vein of Marshall). The persistence of a LSVC is attributed to developmental disturbances of this originally bilateral symmetric venous system and its likewise symmetrically arranged confluence into the primitive entrance of the heart: the cardiac venous sinus [9, 20, 27]. With rightsided shifting of the venous sinus the LSVC will end in the right atrium. This usually takes place via the coronary sinus, the remainder of the primitive left-sided venous confluence. Additional developmental disturbances during integration of the venous sinus into the wall of the right atrium may cause further endings of the LSVC, for example in the left atrium. In rare cases the

right superior vena cava is obliterated. Moreover, the normal left brachiocephalic vein may be absent, quite small, or rudimentary. In all cases the LSVC descends vertically in front of the aortic arch, close to and slightly lateral to the left vagus nerve [12]. It courses anterior to the left pulmonary root structures (Fig. 8) in front of the left superior pulmonary vein to enter in most cases into the right atrium by way of the coronary sinus (Fig. 1). The LSVC may also open into the left atrium of the heart or the right superior vena cava [1, 28]. The arrangement of the azygos and hemiazygos veins may also be variable under these conditions. In most cases the hemiazygos vein terminates in the lower end of the LSVC [28]. Normal central venous anatomy as depicted by the frontal chest radiograph corresponds to type I (Fig. 1). A persistent LSCV is the most common variant of abnormal venous entry into the heart, with an incidence of 0.3–0.5% in healthy individuals and 4% in patients with congenital heart disease [4, 5, 6, 13, 18, 26, 33]. In 92% of cases the LSVC drains into the right atrium through the coronary sinus [18], but in the remaining 8% it drains into the left atrium, even if the coronary sinus is normally developed, creating a right-to-left shunt [32]. Eighteen percent of individuals with persistent LSCV possess no RSVC [18]. This is consistent with type II. In most cases, however, a RSVC is present as well (type III) [33]. In about 60% of these individuals a left innominate (brachiocephalic) vein connects the two veins (type IIIa) [33]. LSVC does not provoke symptoms or other clinical signs except in rare instances of direct drainage into the left atrium. Some reports suggest a greater prevalence of

Fig. 8 CT scan of the mediastinal structures in case of doubled superior vena cava. 1, right superior vena cava (RSVC); 2, left superior vena cava (LSVC); 3, ascending aorta; 4, descending aorta; 5, main pulmonary artery; 6, right and 7, left pulmonary artery; 8, esophagus; 9, right and 10, left main bronchi; 11, internal thoracic veins

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arrhythmia in patients with this venous drainage abnormality [15]. The main problems of patients with such a venous variant are technical and occur when an attempt is made to introduce a catheter through the narrow opening of the coronary sinus to reach the right atrium. Persistence of a LSVC should be considered especially when central venous catheterization via the subclavian or internal jugular vein is difficult. Serious complications have been described during catheterization of patients with LSVC (shock, cardiac arrest, angina), probably as a result of manipulation of the catheter in the coronary sinus rather than due to the catheter itself. The abnormality can also make correct placement of a pulmonary artery catheter (see case 2), a pacemaker lead (see case 3) or the performance of transjugular approaches to the liver difficult, or impossible [19, 22].

pericardium and mediastinum, intravascular malpositions, and correct position in the LSVC respectively: – Anterior: left internal thoracic vein; – Central: LSVC, left pericardiacophrenic vein; – Posterior: left superior intercostal vein, descending aorta [3, 8, 10, 11, 18, 23, 24, 25, 29, 31]. A catheter inserted deep into the left pericardiacophrenic vein runs a characteristic course on the frontal chest radiograph along the cardiac border [10]. Other methods to differentiate left-sided catheters and venous anomalies are: transesophageal echocardiography, magnetic resonance imaging and computed tomography [2, 16, 17, 30].

Further management of left-sided catheters Persistent left superior vena cava and cardiac surgery The only clinical importance of persistent LSVC is that cardiac catheterization is difficult. In addition, at open cardiac operations it is important to recognize the presence of the LSVC and use appropriate cannulation techniques to eliminate the large amount of systemic venous blood that enters the heart through the coronary sinus. It is also important to recognize the absence of collateral vessels between the LSVC and RSVC or, rarely, the absence of the RSVC, in which case ligation of the persistent LSVC would cause venous engorgement in the head and arms [21]. Radiology and differential diagnosis of left-sided catheters The diagnosis of a LSVC on a plain chest radiograph is not easy. Certain radiological signs that reportedly indicate the presence of a LSVC are widening of the aortic shadow together with paramediastinal bulging under the aortic arch or a low-density line along the upper left margin of the heart. In a series of 30 patients with LSVC the presence of this condition was suspected on plain radiographs in only one case [33]. We are convinced that this study represents the realistic situation: Even when looking for a LSVC in particular, it is very hard to discover. If a CVC is running down the left mediastinal border on a plain chest radiograph, it is crucial to define the catheter tip position. A catheter in a LSVC will be confused with a malposition in the descending aorta, the reason for this being the LSVC running anterior to the descending aorta (Fig. 8). Nevertheless this malposition must have been ruled out by transduction of the pressure waveform beforehand. A contrast-enhanced lateral chest radiograph is the quickest and cheapest reliable method for distinguishing between left-sided malpositions: extravascular in pleura,

Accidental positioning of the CVC tip in a smaller vein significantly increases the risk of thrombosis, and extravasation by perforation of the vessel wall—especially after application of hypertonic or hyperosmolar solutions through such catheters [10, 11]. Therefore all catheters outside the central circulation have to be removed, as do all extravascular catheters [10, 29, 31]. Exploration of a perforation under controlled conditions might be necessary with the catheter left in place [7]. Catheters in the LSVC may remain, as was done in our cases 2 to 4. Case 1 demonstrates a typical action of a clinician unaware of central venous variations such as a persistent LSVC: (1) the catheter position was not verified by a lateral contrast-enhanced chest radiograph, and (2) a new catheter was ‘‘forced’’ into the presumed ‘‘correct position’’ in the superior vena cava. We therefore see the necessity to call attention to some aspects of guidewire insertion technique.

Seldinger technique Each step of central venous cannulation carries the potential for serious complications. The operator must know the complications and their precautions, and continuous attention during the complete procedure is necessary. If precautions are violated by ignorance it may result in severe complications—as case 1 demonstrates with the perforation of the right innominate (brachiocephalic) vein. In this case the dilator was railroaded over the wire, the wire buckled and the rigid combination of wire and dilator perforated the brachiocephalic (innominate) vein and passed into the pleural space (Fig. 9). It seems reasonable that the hemodialysis catheter, which was inserted subsequently, followed the path of least resistance, and entered the pleural space through the already-existing perforation. We believe that the vessel perforation was preventable.

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Fig. 9 Mechanism of perforation in case 1. The RSVC is absent. The vessel dilator is inserted to its full length by a movement straight forward, kinking the guidewire placed initially in the left brachiocephalic vein, and perforating the right brachiocephalic vein

Conclusion Venous anatomy is variable. Additional vessels may exist while other veins may be missing. Because of its clinical significance and the fact that LSVC is really not rare it should be considered, especially when central venous catheterization via the subclavian or internal jugular vein is difficult. The operator must immediately be suspicious that the dilator is not following the intended path if the guidewire hangs or resists manipulation during dilator insertion, and, hence, the risk of misplacement or vessel injury is very much increased. Diagnosis of a catheter in a persistent LSVC is not easy. On a plain chest radiograph a catheter positioned in a LSVC will run down the left mediastinal border and can be confused with intravascular malposition in the descending aorta, left internal thoracic vein, left pericardiacophrenic vein or left superior intercostal vein, and extravascular malpositions in pleura, pericardium and mediastinum. Compared with other imaging techniques, the contrast-enhanced lateral chest radiograph is a cheap and readily available method to determine the catheter’s exact position.

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