A 37-Year-Old Woman with Hypertrophic Cardiomyopathy with a Dual-Chamber Implantable Cardioverter-Defibrillator Requiring Percutaneous Transvenous Lead Extraction and Multidisciplinary Management

Background

Despite the evolution of cardiac implantable electronic devices over the last few decades, lead malfunction can occur, requiring surgical or percutaneous transvenous lead extraction (TLE) [1]. TLE is not successful in all situations and may need to be aborted in favor of surgical extraction (SE). We present a case of a 37-year-old woman with history of dual-chamber implantable cardioverter-defibrillator (ICD), who required ICD lead extraction due to lead fracture [2]. This case report highlights the limitations of TLE, reasons for aborting TLE in certain intraoperative conditions, and the importance of multi-disciplinary management in such cases to minimize the risk of major complications.

Case Report

A 37-year-old woman with history of G716R mutation in MYH7 gene for hypertrophic cardiomyopathy had a primary prevention dual-chamber ICD implant in 2001. She underwent TLE and re-implantation of a new ICD lead (6947 Sprint Quattro Secure, Medtronic, Inc, Minneapolis, MN, USA) in 2009 due to ICD lead fracture (Guidant model 0147, Boston Scientific, Marlborough, MA, USA) along with a pulse generator exchange. She was referred to our hospital in 2019 when she was again found to have an ICD lead malfunction with sudden increase in lead threshold. No high-risk features or patient activities were identified that could have led to ICD lead malfunction, except that it was a retro-pectoral implant. A chest X-ray at presentation is shown in Figure 1A. Differential diagnoses of lead failure included conductor fracture, coil fracture, or an insulation breach. This patient had a sudden increase in capture threshold of ICD lead with significant noise (short V-V intervals), indicating a pace-sense conductor fracture.

ICD therapies were turned off to avoid inappropriate shocks and a LifeVest® (Zoll Medical Corporation, Pittsburgh, PA, USA) was ordered. All available options were discussed with her, including capping the malfunctioning ICD lead with re-implantation of a new lead versus lead extraction (TLE and SE, if indicated), followed by re-implantation of a new ICD lead. Given her age and anticipated needs of future lead revisions, it was decided to extract the malfunctioning ICD lead percutaneously with re-implantation of a new ICD lead.

During the extraction procedure, a 16-Fr GlideLight™ laser sheath (Philips, Amsterdam, Netherlands) and a TightRail™ (Philips, Amsterdam, Netherlands) mechanical extraction tool were used, but the innominate vein/superior vena cava junction could not be crossed due to significant lead-on-lead and lead-on-wall binding sites. During the TLE attempt, unwinding of the ICD lead was observed. The RA pace-sense lead (5076 Capsure Fix Novus [Medtronic Inc, Minneapolis, MN, USA]) also started to disintegrate; therefore, it was decided to extract the RA lead as well. RA lead extraction was attempted using a 14-Fr GlideLight™ laser sheath (Philips, Amsterdam, Netherlands) followed by a 16-Fr GlideLight™ laser sheath (Philips, Amsterdam, Netherlands), but no progress was made beyond the point where the ICD lead had significant adhesions. Since the lead-on-lead and lead-on-wall binding sites prevented any meaningful progression of extraction tools, the TLE procedure was aborted. Cardiovascular surgery was consulted for staged elective SE. A chest X-ray after the aborted TLE procedure is shown in Figure 1B. Computed tomography (CT) of the chest with contrast before TLE showed no evidence of venous stenosis or occlusion in left-sided chest veins. Despite no evidence of large-vein obstruction, CT angiography before SE demonstrated significant left-sided venous collateral vessels.

Elective SE was done the following day via a standard median sternotomy approach. The left innominate vein was noted to have a contained tear with both leads densely adherent to the vein. The tear continued back to the confluence of the left subclavian and jugular veins and began to bleed during exposure. Signs of collateralization were noted, suggestive of prior occlusion or at least compromise of the left in-nominate vein. Due to the dense adhesions, the innominate vein was completely avulsed during removal of the leads (Figure 2). Vascular surgery was consulted to assist due to significant vascular disruption. Ligation of left internal jugular vein and left subclavian vein was done. The right internal jugular vein was reconnected to the heart via pericardial patch reconstruction, with the left side left in discontinuity due to the presence of collaterals. All of this was performed on cardiopulmonary bypass support. Surgical removal of the leads and vascular reconstruction was followed by implantation of an epicardial single-chamber ICD system. Two unipolar single-coil ICD leads (6937A and SQ 6996, Medtronic, Inc, Minneapolis, MN, USA) and a single bipolar pace-sense epicardial electrode (Capsure Epi 4968, Medtronic, Inc, Minneapolis, MN, USA) were connected to a pulse generator (Visia AF MRI, Medtronic, Inc, Minneapolis, MN, USA). The patient had an uneventful postoperative recovery. A chest X-ray after the surgical procedure is shown in Figure 3.

Discussion

This case highlights the importance of early recognition of TLE limitations as well as the need to abort TLE in certain intraoperative situations. As noticed during elective SE, any further TLE attempts in this case would have resulted in a major vascular tear requiring emergent surgery. Elective involvement of a surgical team for staged SE in these situations can prevent potential catastrophic complications.

Most cases of lead malfunction require implantation of a new lead(s) while capping or extracting the malfunctioning lead(s). SE is less often favored for lead extraction [3] due to higher morbidity, as well as availability of increasingly advanced TLE tools [4]. However, TLE is not without risks. The procedural complication rate of TLE varies from less than 1% to about 8% [1]. A study done on 3258 TLE procedures showed that around 0.8% patients had major complications requiring emergent surgery [5]. According to the National Cardiovascular Data ICD Registry™, 0.36% of patients undergoing TLE required emergent surgery due to complications, and one-third of those patients requiring surgery died [6]. The American Heart Association has highlighted the fact that these procedures require a team approach, including a primary operator, a cardiothoracic surgeon (who can be immediately available if need arises), anesthesiologist, echocardiographer, and assistants who could be nurses, technicians or physicians [7]. There should be a low threshold to abort the TLE approach if no meaningful advancement is made using TLE tools. Important variables that are known to increase complication rate of TLE procedure are coagulopathy, female sex, low body mass index, cardiomyopathy, renal dysfunction, diabetes mellitus, thrombocytopenia, higher number of leads requiring extraction, presence of a dual-coil ICD lead, prior cardiac surgeries [1], and device infection [8].

A combination of TLE and minimal invasive surgery has been used as a hybrid approach in high-risk cases such as failed prior extractions, lead fractures, old age of lead, and more than 2 cm size vegetation or thrombus on leads [9–11]. The case presented above is different because a true hybrid approach was not planned. However, the Cardiac surgery team was available as a back-up for any unforeseen circumstances. The TLE approach was aborted due to the failure of meaningful progression during the procedure, and an elective sternotomy was planned the following day for SE. Benak et al reported a similar case in which TLE had to be aborted in favor of thoracotomy due to failure of progression [12].

Conclusions

Transvenous lead extraction (TLE) can be very challenging in certain cases. An experienced surgical back-up team is necessary for a safe and effective lead extraction program. Perioperative evaluation is critical to identify and make appropriate plans for high-risk cases. A multidisciplinary team approach must involve an electrophysiologist, cardiac surgeon, anesthesiologist, and cardiac radiologist. In addition, there should be a low threshold to abort the procedure if there is no meaningful progression during TLE. This is because continued attempts of TLE in some high-risk situations can potentially lead to catastrophic complications.