An unusual case of duplicated left brachiocephalic vein with right sided aortic arch and aberrant origin of LSCA in a patient undergoing implantable cardioverter defibrillator (ICD) implantation

Abstract

Background

Anomalous left brachiocephalic vein (ALBCV) is a rare venous anomaly. Double Left brachiocephalic vein is the rarest type of ALBCV anomaly.

Case report

Here we report a case of gentleman with post myocardial infarction ventricular tachycardia who underwent ICD implantation, where we could not place the lead initially through left side. CT angiography revealed presence of a duplicated circumaortic left BCV. It's cranial limb coursing normally anterior to arch and compressed at its confluence with RBCV and the caudal limb with a subaortic course draining into the RSVC. We report this first case of double LBCV along with right sided aortic arch and aberrant origin of LSCA arising from Kommerel's diverticulum.

Conclusion

This case highlights that interventional cardiologists should be aware of these venous anomalies for proper planning and implantation of CIED successfully via transvenous approach.

1. Introduction

Anomalies of venous system of thorax are rare. The most common anomaly of the left brachiocephalic vein is the Anomalous left brachiocephalic vein (ALBCV) which is present in individuals. It is usually associated with presence of congenital heart disease. The rarest type of anomaly of the BCV is the duplication or the circumaortic ring. Double left BCV has been described in medical literature but is exceedingly rare. Most of the reports have been incidental finding of this anomaly in an otherwise healthy individuals. As the number of CIED device implantation increases, there is a possibility of encountering this anomaly more frequently. The presence of a double BCV presents unique challenges in placement of leads and can also be a source of inadvertent bleeding during cardiac surgeries. Therefore knowledge of this anomaly beforehand is crucial for avoiding complications and problems during CIED procedures. Here we report, according to the author's knowledge the first case of a duplicated left BCV and also right sided aortic arch with aberrant left subclavian artery arising from Kommerel's diverticulum where Implantable Cardioverter Defibrillator (ICD) implantation was done successfully from the left side.

2. Case report

We report a case of 48 year old gentleman with hypertension, type 2 diabetes mellitus and hypothyroidism who suffered an inferior wall ST elevation myocardial infarction and he was managed medically. He developed monomorphic ventricular tachycardia associated with presyncope after 3 days. He was given DC cardioversion in view of hemodynamically unstable VT and he reverted to normal sinus rhythm. After 2 weeks, he again had an episode of hemodynamically unstable VT which required DC cardioversion. Patient was referred to us for ICD implantation. Preprocedural investigations did not reveal any abnormality except acute kidney injury, secondary to ischemia. A pre procedure venous angiogram was done from left cubital vein which did not reveal any abnormality in first instance and left BCV seem to be draining into right SVC, then into right atrium (RA). Left infraclavicular pocket was made and extra-thoracic axillary venous puncture was taken under fluoroscopy guidance. An0.038 guidewire was passed under fluoroscopy guidance but the wire was seen getting stuck midway between left BCV. Another venous angiogram was done from left arm which showed presence of an duplicated left BCV with cranial limb which was not opacifying distally, the caudal part had a tortuous course and significant obstruction in its middle part (see Fig. 1). We switched to hydrophilic J tipped guidewire for navigating the obstruction. However, on repeated attempts we could not cross the narrowest part of left BCV hence the procedure was abandoned. At this point of time, as there was not much clarity regarding the venous anatomy and drainage of the left BCV, so a CT venography was performed. CT venogram demonstrated duplication of the LBCV with the cranial limb coursing in the expected location of the LBCV, anterior to arch vessels, with significant compression of its distal part (just before its confluence with RBCV) by the ostio-proximal part of the right common carotid artery. The caudal limb of LBCV had an oblique subaortic course, anterior to trachea, before draining into SVC at the level of drainage of azygous vein. No stenosis was noted at the site of drainage of the caudal limb; the stenosis noted in the intraprocedural venogram was likely due to spasm induced by the preceding attempts of ICD placement (see Fig. 2a, Fig. 2b). There was also presence of a right sided high aortic arch with aberrant origin of LSCA arising from Kommerel's diverticulum. Careful preprocedural planning was done and the length of this venous channel from left axillary vein to RV apex was measured to match the RV lead length. A 58 cms long ICD Optisure™ lead by Abbott, Inc., St. Paul, MN, USA) was selected. Another crucial information gained from CT was that this anomalous LBCV was draining into RSVC and that RSVC was draining into RA and there was no persistent LSVC. Patient was taken up for a repeat procedure. Pocket was opened and extra-thoracic axillary vein puncture was taken and guidewire was inserted. After multiple attempts the guidewire could be negotiated across the stenosed segment of caudal limb of LBCV. As the stenosis would prevent the heavy duty defibrillator lead to cross, a 8F long sheath was taken and its distal edge was kept just proximal to the site of obstruction. RV lead was inserted under fluoroscopy guidance with the support provided by the long sheath. On initial attempts, lead would not cross and buckle up, so the stylet was removed and reshaped by giving it a distal turn. The lead finally crossed the caudal limb and was placed successfully at RV apical septum (see Fig. 3, Fig. 4, Fig. 5). Lead parameters were checked and were found satisfactory. Procedure was completed successfully. Patient was observed for 3 days and later discharged without complications.

Fig. 1.

Duplicated circumaortic left brachicephalic vein with cranial and caudal limbs.

Fig. 2a.

Coronal Computed tomographic images of thorax showing a duplicated left brachicephalic vein. The cranial limb of LBCV is coursing normally anterior to.

Fig. 2b.

Coronal Computed tomographic images of thorax showing a duplicated left brachicephalic vein. The cranial limb of LBCV is coursing normally anterior to.

Fig. 3.

Course of the ICD lead placed through the accessory caudal limb of LBCV into RV apical septum.

Fig. 4.

Volume rendered CT venogram (A to D)shows duplicated LBCV with cranial limb (asterisks) compressed by right common.

Fig. 5.

Line diagram illustrating the venous anomaly.

3. Discussion

Anomalies of brachiocephalic veins have an estimated prevalence of <0.4 % in general population and up to 1 % in patients with congenital heart diseases [1,2]. Double or duplicated brachiocephalic veins are extremely rare whereby there are two separate brachiocephalic veins instead of the single vein. In addition to normal coursing vein, there is an accessory vein which may have preaortic, retro-aortic, retro-tracheal or retroesophageal course and joins SVC caudally to the normally coursing vein [3,4]. Duplicated brachiocephalic vein is typically asymptomatic; however, it may pose difficulty during central venous catheterisation and pacemaker implantations, especially when done via left subclavian transvenous approach as seen in the present case. The anomalies of brachiocephalic veins also assume importance during various surgeries involving this region including cardiothoracic and neck surgeries.

During normal human embryogenesis, bilateral upper limbs, head and neck are drained by paired anterior cardinal veins. By the 8th week of gestation, there is development of an intersupracardinal venous plexus which connects the right and left anterior cardinal veins and surrounds the primitive aorto-pulmonary system. Gradually there is shortening of aorta with resultant regression of the inferior part of intersupracardinal venous plexus and the superior part persists to form normal supra-aortic LBCV [5]. Various deviations in the fate of the intersupracardinal venous plexus have been described in literature [6]. When there is regression of superior part of this plexus along with persistence of the inferior part, it leads to the development of subaortic LBCV. It is postulated that a duplicated LBCV may develop when there is persistence of both the superior as well as inferior parts of the intersupracardinal venous plexus leading to formation of supraaortic cranial limb and subaortic caudal limb of LBCV as seen in the present case [7]. Also, as the intersupracradinal venous plexus surrounds the primitive aortopulmonary system, anomalies of development of the aorta and pulmonary artery are associated with anomalies of brachiocephalic veins. This can be corroborated by presence of aortic arch anomalies in 73 % and pulmonary artery anomalies in 93 % cases with anomalies of brachiocephalic veins [8]. Kawamura et al. described a similar case of pacemaker implantation in a duplicated LBCV with a normal superior LBCV and an accessory inferior tortuous vein which had a course similar to PLSVC, which ran inferiorly then took a rightward turn to join the right SVC [9]. Steckiewicz et al. [2] in a case report, describe a variant of double left BCV, in which the vessels run parallel to one another above the aortic arch and the patient underwent pacemaker implantation from the upper vessel which represents the embryological counterpart of normal LBCV.

Our patient had also had right sided high aortic arch with aberrant origin of LSCA arising from Kommerel's diverticulum associated with congenital duplication of left BCV, which according to the author's knowledge has not been reported before. The prevalence of right aortic arch (RAA) with anomalous origin of the left subclavian artery (LSCA)is rare (0.05–0.1 %) and is second most common cause of symptomatic vascular ring. Right-sided aortic arch with aberrant subclavian artery typically does not cause symptoms which are mainly due to the presence of a vascular ring [10]. Also, symptoms may stem from atherosclerotic changes in the abnormal blood vessels, which can lead to dissection, aneurysm formation that can compress nearby structures, resulting in dysphagia (known as dysphagia lusoria) and dyspnea [11,12]. By ligating and splitting the ligamentum arteriosum or by resecting a large diverticulum and anastomosing the left subclavian artery with the left common carotid artery, symptoms like dysphagia or dyspnea may be relieved [13].

The present case highlights the importance of awareness of these rare systemic venous anomalies for interventional cardiologist while performing CIED implantation procedures via the left transvenous approach. The incidence of these anomalies may rise in future due to increase in CIED procedures. Cardiologists should expect such an anomaly whenever they come across an accessory vessel coursing superior to arch and there is difficulty in lead placement. Proper pre-procedural imaging and planning can mitigate these challenges and avoid unforeseen complications.

Authors’ contributions

KP and NC performed the literature review and drafted the manuscript. SY provided concept and was the chief operator. NP revised and edited the manuscript. All authors read and approved the final manuscript.

Consent

Written informed consent was obtained from the patient for publication of this case Report and any accompanying images.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Abbreviations

ALBCV

Anomalous left brachiocephalic vein

LBCV

left brachiocephalic vein

SVC

superior vena cava

PLSVC

persistent left superior vena cava

RA

right atrium

LA

left atrium

LSCA

left subclavian artery

RAA

right aortic arch

ICD

Implantable Cardioverter Defibrillator

Appendix A. Supplementary data

The following is/are the supplementary data to this article: