Acta Cardiologica

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Pacemaker insertion in a patient with persistent left superior vena cava Barbara Bosch, Anneleen Stockman & Roel Beelen To cite this article: Barbara Bosch, Anneleen Stockman & Roel Beelen (2013) Pacemaker insertion in a patient with persistent left superior vena cava, Acta Cardiologica, 68:6, 642-644, DOI: 10.1080/AC.68.6.8000014 To link to this article:

Published online: 23 May 2017.

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Date: 12 January 2018, At: 14:10


Acta Cardiol 2013; 68(6): 642-644

doi: 10.2143/AC.68.6.8000014

Pacemaker insertion in a patient with persistent left superior vena cava Barbara BOSCH; Anneleen STOCKMAN, MD; Roel BEELEN, MD Downloaded by [Gothenburg University Library] at 14:10 12 January 2018

Dept. of Cardiovascular and Thoracic Surgery, OLVrouw Hospital, Aalst, Belgium.

Abstract A persistent left superior vena cava (PLSVC) is a rare and usually unnoticed congenital anomaly. However, clinicians must be conscious of its existence as a PLSVC can be associated with other anomalies of cardiovenous morphology with significant clinical implications for transvenous procedures. We report a case of a double superior vena cava with absent innominate vein found during catheterization for cardiac resynchronization pacemaker therapy implantation (CRT-P). We also describe a technique used for successful positioning of the leads into the right atrium and right ventricle.

Keywords Persistent left superior vena cava – pacemaker implantation – stylets – congenital heart disease.



In recent decades there has been a steady growth in the number of transvenous procedures such as insertion of a permanent pacemaker. Given the aging population and the improved survival of patients with significant medical comorbidities, a further rise in device implantation (pacemaker/defibrillator) can be anticipated1. This trend means that clinicians will be increasingly confronted with variations in thoracic venous anatomy and the technical challenges such constitutions pose. Persistence of the left superior vena cava (PLSVC) is a rare, usually asymptomatic variation in the cardiac venous return reported in 0.3% of the general population2. Nevertheless major clinical difficulties may arise when these patients’ right heart is approached via the left thoracic venous system3. In this case report a patient with double superior venae cavae and an absent bridging innominate vein is discussed. We also describe a technique that can be used to successfully implant pacemaker leads in patients with this challenging anatomy.

A 58-years-old female presented to the emergency department with pocket infection of a right-sided implanted CRT-P. Recently she had undergone mastectomy with axillary clearance for a carcinoma of the breast. Blood tests showed signs of inflammation (CRP 139 mg/l) and blood cultures confirmed Staphylococcus aureus sepsis probably originating from an infected right-sided pacemaker following a wound infection at the mastectomy site. Her medical history also included heart failure and severe cardiomyopathy with congenital aortic and pulmonary stenosis and a ventricular septum defect. Coronary artery bypass grafting and aortic valve replacement were performed at the age of 54. Four years ago the patient had a pacemaker implantated for a third-degree AV block. Due to the infection the right CRT-P system and its three leads were removed and a new CRT-P device was implanted on the left side. As the patient was pacemaker-dependent, a temporary endovenous pacing catheter was introduced by the right groin through the inferior caval vein into the right ventricle allowing wound bed preparation and placing of a left-sided CRT-P. For left ventricular pacing an epicardial screw-in lead was inserted by mini-thoracotomy and tunnelled towards a newly created left subpectoral pocket. During subsequent introduction of a guide wire under fluoroscopic control a PLSVC was noticed. Angiography confirmed a PLSVC with an absent innominate

Address for correspondence: Barbara Bosch, Hoogstraat 2F, 1730 Asse, Belgium. E-mail: [email protected] Received 24 June 2013, revision accepted for publication 10 September 2013.

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CRT-P implantation in a PLSVC patient

vein and a partially unroofed coronary sinus. This made insertion of the new device technically challenging. A long pacemaker lead was advanced transvenously via the left vena cava and coronary sinus. A wide loop was created so the screw-in lead could deflect against the atrial wall in a curve and could be guided through the tricuspid valve and secured in the apex of the right ventricle. For implantation of the atrial lead a curved stylet that had been bent to a 90 degree angle so that the lead could be implanted perpendicular on the right atrial roof, was used (figure 1). Finally, the leads were secured to the pectoral muscle fascia (figure 2). All leads had a low threshold (0.91.9 V), acceptable P-wave sensing (2.7mV) and acceptable R-wave sensing (9.4 mV).

Fig. 1 The curved atrial and ventricular stylets used for successful pacemaker implantation.

DISCUSSION Pathogenesis

Persistence of the left superior vena cava (PLSVC) originates from an aberrant embryological development4. By day 22 an embryo has bilateral cardiac inflow consisting of left and right anterior and posterior cardinal veins draining blood into the left and right sinus horns of the sinus venosus and eventually into the developing heart. Also the pacemaking tissue is duplicated at this stage and located on each side of the sinus horns near the cardinal veins. In the following weeks, this originally symmetric venous inflow tract is normally remodelled to the right. The left coronary sinus stops growing; its remnants form the coronary sinus and lose contact with the left anterior cardinal vein. The left pacemaker tissue disappears. Around day 50, branches of the left anterior cardinal vein (the thymic and thyroid veins) fuse with their rightsided counterparts and establish the innominate vein. The anterior cardinal vein then becomes obliterated. In embryos where this shift to the right does not occur, the left anterior cardinal vein persists as a left vena cava and the innominate vein may not develop. The left pacemaker area may survive as well causing ectopic pacemaker activity5. Depending on what step in this process goes wrong, a different type of PLSVC develops. Four distinct types of PLSVC have been described by Nandy and Blair6. In the type with double venae cavae, the bridging innominate can be present (type 1) or absent (type 2). In this patient no vena horizontalis could be found. The right superior vena cava was fully patent explaining the initial right-sided routine insertion of the CRT-P leads. Clinical and practical implications

Fig. 2 Chest X-ray taken after implantation of the left-sided CRT-P (cardiac resynchronization therapy – pacemaker).

Identifying a PLSVC is important as the anomaly has significant clinical and practical implications. Clinicians must be aware that PLSVC is associated with other cardiac anomalies such as bicuspid aortic valves, an atrioventricular defect, cor triatriatum or mitral atresia7. Disturbances in the sinus node and AV junction formation cause arrhythmias such as atrial fibrillation5 and bradycardia requiring permanent pacing8. These findings can be explained by the disrupted embryological development discussed above. In patients diagnosed with PLSVC active screening for additional structural or functional variations is thus recommended. Practical issues arise during transvenous interventions. Lack of awareness of the existence of PLSVC may lead to misplacement of central lines or guide wires and to life-threatening complications such as laceration of vessels, cardiogenic shock and cardiac arrest9. However,


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B. Bosch et al.

the persistence of a left vena cava should not contraindicate inserting a permanent pacemaker via the PLSVC. Pacing may be necessary for the PLSVC associated arrhythmias and successful placement has been described on several occasions with a low complication rate2,3,8,9. Before inserting a pacemaker device, it is key to analyse the patient’s anatomy thoroughly. Like in our case, a PLSVC is currently often noted by chance during an endovascular procedure. The need for a long catheter or difficult left-sided central venous catheterization can indicate an underlying venous anomaly. However, a PLSVC can already be suspected on preoperative imaging: on a chest X-ray a wide aortic shadow (figure 2) and paramediastinal bulging should raise suspicion2. Goyal et al.3 proposed screening for PLSVC by echocardiography. A dilated coronary sinus without signs of elevated right-sided filling pressures should warn the echographist that a PLSVC can be present. This diagnosis can then be confirmed by saline echocardiography. A contrast-enhanced CT of the thorax or magnetic resonance venography can be performed allowing exact analysis of the thoracic venous anatomy10. Correct placement of cardiac resynchronization devices, for example a CRT-P via the left subclavian approach is technically difficult in PLSCV patients. In Nandy and Blair type 2 subjects, inserting the leads is even more challenging as they lack the bridging innominate vein that can be used for accessing the right side of the heart via the right vena cava superior in type 1 PLSVC. Different techniques for manipulating the right leads into the accurate place have been described: Biffi et al.

used pre-shaped stylets8. We achieved successful insertion by forming a wide loop with long leads in the right atrium and establishing lead stability by active fixation via screw-in leads (figure 1). We opted also for a minimally invasive left ventricular epicardial lead placement (St Jude epicardial lead) thereby eliminating potential problems during the alternative of third lead insertion by the coronary sinus9.

CONCLUSION Persistence of the left superior vena cava is a rare and usually asymptomatic congenital anomaly. However, cardiologists, surgeons, radiologists and intensivists must be aware of its existence as a PLSVC can be associated with other variations in cardiovenous morphology and function with significant clinical implications in transvenous procedures. Diagnosis can be made before an intervention using echocardiography. The detailed anatomy can then be clarified by a contrast-enhanced CT thorax. Technical difficulties faced during implantation of cardiac resynchronization devices via the left subclavian approach can be overcome by using shaped stylets or forming a wide loop in the right atrium. Screwin leads guarantee active and stable lead fixation in these anatomically challenging conditions.

CONFLICT OF INTEREST: none declared.

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Larsen’s human embryology, 4th edition. Elsevier/Churcill Livingstone, Philadephia, 2009: 355-7. 5. Hsu LF, Jaïs P, Keane D, Wharton JM, Deisenhofer I, Hocini M, Shah DC, Sanders P, Scavée C, Weerasooriya R, Clémenty J, Haïssaguerre M. Atrial fibrillation originating from persistent left superior vena cava. Circulation 2004; 109: 828-32. 6. Nandy K, Blair CB Jr. Double superior venae cavae with completely paired azygos veins. Anat Rec 1965; 151: 1-9. 7. Nasah EN, Moore GW, Hutchins GM. Pathogenesis of persistent left superior vena cava with a coronary sinus connection. Pediatr Pathol 1991; 11: 261-9.

8. 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. Chest 2001; 120: 139-44. 9. Ratliff HL, Yousufuddin M, Lieving WR, Watson BE, Malas A, Rosencrance G, McCowan RJ. Persistent left superior vena cava: case reports and clinical implications. Int J Card 2006; 113: 242-6. 10. Peltier J, Destrieux C, Desme J, Renard C, Remond A, Velut S. The persistent left superior vena cava: anatomical study, pathogenesis and clinical considerations. Surg Radiol Anat 2006; 28: 206-10.

Pacemaker insertion in a patient with persistent left superior vena cava.

A persistent left superior vena cava (PLSVC) is a rare and usually unnoticed congenital anomaly. However, clinicians must be conscious of its existenc...
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