The persistence of left superior vena cava (PLSVC) is the most common congenital anomaly of the venous return system to the heart. Because of the increasing number of patients referred for cardiac resynchronization therapy (CRT) devices implantations, it is expected to encounter this venous anatomic variation. Left ventricular lead placement at an appropriate site is an integral and technically challenging part of successful CRT. In case of cardiac abnormalities could be difficult to achieve an optimal cardiac rhythm management devices implantation. Previous reports in patients with PLSVC highlighted the challenges to achieve an optimal cardiac rhythm device implantation. Recently, a new quadripolar active fixation left ventricular lead is available for CRT device implantation. Hereby we report a case of a device upgrading from dual-chamber pacemaker to CRT with defibrillator backup using the active fixation left ventricular quadripolar lead in a patient with PLSVC and right superior vena cava atresia.
The persistence of left superior vena cava (PLSVC) is the most common congenital anomaly of the venous return system to the heart. Because of the increasing number of patients referred for cardiac resynchronization therapy (CRT) devices implantations, it is expected to encounter this venous anatomic variation. Left ventricular lead placement at an appropriate site is an integral and technically challenging part of successful CRT. The final position of the lead depends on the anatomy of the cardiac veins, the stability of the lead, and the quality of pacing parameters. Previous reports of cardiac rhythm device implantation in patients with PLSVC highlighted the challenges to achieve a stable of the leads inside the heart chambers. Improvements in materials and techniques over the years allowed the successful implantation of cardiac rhythm management devices including CRT devices even in case of unfavorable anatomies. Recently, a new quadripolar active fixation left ventricular lead has been developed and available for CRT device implantation. This new technology combines the advantages of the multipolar technology and the stability of the active fixation leads and it allows a more precise and stable lead placement, particularly helpful in case of abnormal or unfavorable anatomy of the coronary sinus.
A 77-year-old man suffering from congestive heart failure (NYHA class II) and post-ischemic dilated cardiomyopathy was referred to our Centre for an upgrading to a cardiac resynchronization therapy device with defibrillator backup (CRT-D). Patient had a dual-chamber pacemaker (PM) implantation due to symptomatic bradycardia 7 years before. During PM implantation, a persistent left superior vena cava (PLSVC) with right superior vena cava (SVC) atresia was documented. For this reason, a dual-chamber PM was implanted using the left-side approach and the leads were advanced through the PLSVC and coronary sinus (CS) and placed into the right atrial appendage and the right ventricular apex. Current baseline ECG revealed sinus rhythm, left bundle branch clock with a QRS complex duration of 180 ms. Transthoracic echocardiography showed a dilated left ventricle and a left ventricular ejection fraction of 30%.
The procedure was performed in fasting state and in the usual sterile fashion. Cannulation of the left subclavian vein was performed. Venography of the left subclavian vein confirmed patency of the left subclavian vein and showed PLSVC draining into a hugely dilated CS. We decide to explore the CS with a standard 6F pig-tail catheter and contrast injections pulling back the diagnostic catheter from the CS ostium to the PLSVC. At first, there was no evidence of vein branches taking off from the main body of the CS. We decided to explore the CS drawing back and rotating a subselector catheter (Attain SelectTM II, Medtronic, MN, USA). A small and sharp-angle vein was selectively cannulated with an inferior-to-superior direction (
active-fixation (Attain StabilityTM, Medtronic, MN, USA) was advanced into the posterolateral vein (
The persistence of left superior vena cava (LSVC) is the most common congenital anomaly of the venous return system to the heart. PLSVC can be isolated or associated with other congenital heart diseases and it has been reported in about 0.5% of the general population and up to 10% in patients with congenital heart disease [
Briefly, development of the SVC system begins with the sinus venosus, in which 3 pairs of veins (cardinal, umbilical, and vitelline) drain. The right cardinal vein is transformed into right SVC, the left cardinal vein into LSVC, and the left part of the sinus venosus into the coronary sinus (CS). During the normal fetal development, there is progressive involution and disappearance of the left-sided anterior venous cardinal system, leaving the CS and the ligament of Marshall. Failure of the closure of the left anterior cardinal vein results in PLSVC [
Recently, a new quadripolar active fixation left ventricular lead has been developed and available for CRT device implantation. Combining together the advantages of the multipolar technology and the stability of the active fixation leads, this new technology adds an important tool in the portfolio of the electrophysiology operators. It allows a more precise lead placement and stability, particularly helpful in case of abnormal or unfavorable anatomies of the CS. To the best of our knowledge, this is the first report of Attain StabilityTM implantation in a patient with a PLSVC and right SVC atresia. The Attain StabilityTM left ventricular lead is an innovative active fixation lead with a helix attached before the proximal electrode to help fix the lead in any desired position. The lead could be easily maneuvered and it could be repositioned at the desired location if required. This lead has the potential to be used in all types of CS anatomies.
Left ventricular lead placement at an appropriate site is an integral and technically challenging part of successful CRT. The final position of the lead depends on the anatomy of the cardiac veins and on the stability of the lead. Challenging anatomical variants, often discovered at the time of the procedure, can pose difficulties and complications during central venous cannulation or device implantation such as arrhythmia, cardiogenic shock, cardiac tamponade, CS dissection and thrombosis. However, improvements in materials and techniques over the years allowed the successful implantation of cardiac rhythm management devices including CRT devices. Although the procedure was technically challenging, currently available technologies enabled the implantation of CRT devices by a totally transvenous approach even in patients with PLSVC and right SVC atresia.
The authors declare no conflicts of interest regarding the publication of this paper.
Conti, S., Taormina, A., Bonomo, V., Giordano, U. and Sgarito, G. (2018) CRT-D Upgrading in a Patient with Persistent Left Superior Vena Cava and Right Superior Vena Cava Atresia Using the Novel Active-Fixation Quadripolar Left Ventricular Lead. World Journal of Cardiovascular Diseases, 8, 462-466. https://doi.org/10.4236/wjcd.2018.89045