An Unusual Route for Intracardiac Cardioverter-Defibrillator Ventricular Lead Implantation in a Ventricular Tachycardia Patient with Tetralogy of Fallot

INTRODUCTION

Tetralogy of Fallot (TOF) is a common form of cyanotic congenital heart disease; early operation can lead to dramatic improvements in the prognosis, but the incidence of atrial and ventricular tachyarrhythmias increases with time from surgical repair.¹ In a multicenter cross-sectional study of 566 adult patients (mean age: 36.8 years), the prevalence of ventricular arrhythmia was 14.6% and the single most common arrhythmia subtype was ventricular tachycardia (VT) (14.2%).² Ventricular arrhythmia appears to be responsible for most sudden cardiac deaths, and implantation of implantable cardioverter-defibrillators (ICDs) should be considered in patients with documented or sustained VT. Transvenous access to heart chambers is the favored technique, most commonly via the left or right subclavian vein. The supraclavicular internal jugular vein (IJV) approach for cardiac implantable electronic device (CIED) lead implantation has been used in patients with subclavian vein occlusion. We report a case of a patient with a surgically repaired TOF who presented with recurrent VT and received ICD implantation via the supraclavicular IJV approach.

CASE

A 27-year-old man had a history of TOF and had undergone an operation 20 years ago. He presented to the emergency room of another hospital due to syncope. An electrocardiogram showed VT (Figure 1A) and a chest X-ray (CXR) showed extreme cardiomegaly (Figure 1B). Holter study revealed non-sustained ventricular tachycardia and prolong sinus pause upon termination of paroxysmal atrial fibrillation. ICD implantation was attempted but an approach from the left subclavian vein failed due to persistent left superior vena cava and extremely enlarged right atrium (RA) and right ventricle (RV) (Figure 1C-E). Implantation also failed from the right side due to severe tortuosity of the right subclavian vein (Figure 1F-H). Therefore, the patient was transferred to our hospital.

Figure 1.

Panel A displays ventricular tachycardia (VT) with a left bundle branch block morphology and superior axis. Panel B presents the chest X-ray (CXR) before implantable cardioverter-defibrillator (ICD) implantation, revealing extremely enlarged cardiac chambers. Panel C reveals persistent left side superior vena cava by venogram. Panel D reveals persistent left side superior vena cava demonstrated by computed tomography (CT) angiography. Panel E displays the inability to implant a ventricular lead from the persistent left side superior vena cava. Severe tortuosity of the right subclavian vein is disclosed in panel F and panel G. Kinking of the 9Fr. sheath is demonstrated in panel H. In panel I and J, the ventricular lead was implanted from the right IJV by using supraclavicular approach. Panel K is the final CXR after ICD implantation. The ventricular lead implanted from the internal jugular vein (IJV) by using the supraclavicular approach was connected to the ICD via a subcutaneous tunnel, indicated with a star.

At our hospital, transthoracic echocardiography showed severe pulmonary regurgitation related to the enlarged RV and RA. ICD implantation was performed again. Because the left or right subclavian venous approach would be difficult, we implanted the ICD ventricular lead from the right IJV by using a supraclavicular approach. The IJV was successfully punctured by guide of vascular color duplex with an 18-gauge needle, 1 cm lateral to the lateral head of the sternocleidomastoid muscle and 1 cm cranial to the clavicle. The needle was under and close to the clavicle; the needle pointed to the suprasternal notch. After successful puncture of the vein, a 0.38-inch guidewire was inserted, and a peel-away sheath was indwelled. The ventricular lead (Medtronic 6935 m 8.6 Fr.) was introduced smoothly into the heart (Figure 1I-J) and then implanted in the right ventricular lower septum. The RA lead was implanted via the right subclavian vein (Medtronic 5076 m 6 Fr.). After the implantation, a tunnel was made to connect the ventricular lead to the ICD generator (Medtronic Ensura MRI) in the right anterior chest wall pocket. After the procedure, the cardiovascular surgeon repaired the pulmonary valve. The whole procedure was smooth. The patient was discharged uneventfully 1 week after the operation. The final CXR is presented in Figure 1K.

DISCUSSION

When one performs ICD implantation in patients with repaired TOF, one expects difficulties (including subclavian vein occlusion, extreme enlargement of heart chambers, and double superior vena cava or other anatomical variations). Several publications have reported diverse salvage strategies to avoid thoracotomy for epicardial left ventricular implants, including femoral access or veno-plastic surgery.³ However, these time-consuming procedures were associated with high rates of lead dislodgement.⁴ Subcutaneous ICD (S-ICD) has become an important alternative for patients who receive ICD therapy. It has been suggested that S-ICD can be an alternative to traditional systems in younger population, patients with hereditary arrhythmias, such as Brugada syndrome and Long-QT syndrome, and patients with congenital heart disease. However, traditional system remains a better choice than S-ICD for patients with bradycardia, atrioventricular block, ventricular tachycardia responsive to anti-tachycardia therapy, and indication for cardiac resynchronization therapy.⁵

A supraclavicular venous approach could be a practical choice to overcome subclavian vein occlusion. In a patient whose pacemaker must be upgraded to an ICD or in a cardiac resynchronization therapy patient, this approach is noteworthy because the subclavian vein could be occluded due to a previous implanted device. Regarding the supraclavicular approach, studies have proposed the brachiocephalic vein (innominate vein), subclavian vein, or IJV approach to overcome venous occlusion and severe venous tortuosity. For our patient, we chose right supraclavicular venous approach via the right IJV due to coexistence of enlarged RA and RV, persistent left side superior vena cava, and severe tortuosity of the right subclavian vein.

A study demonstrated that the right supraclavicular venous approach was not inferior to the traditional infraclavicular approach due to low misplacement rates, high success rates, and effective first approach.⁶ Possible complication of this approach included inadvertent arterial entry, hemothorax, pneumothorax, upper extremity neural injury, and injury to the recurrent laryngeal nerve. The complication rate, however, was very rare according to previous case series.⁷ The potential disadvantages of the supraclavicular IJV approach include a high chance of lead fracture due to acute bending at the venipuncture site and skin erosion due to tunneling the lead subcutaneously, although no such situation has been reported.⁸ In conclusion, the supraclavicular approach via the right IJV can be a safe, simple, and effective alternative technique for patients in whom traditional venous access would be difficult.

LEARNING POINTS

The supraclavicular IJV approach for CIED lead implantation is safe, simple, effective, and not time-consuming compared with the traditional infraclavicular approach, which could be considered in patients with venous access difficulties (including severe venous tortuosity and subclavian vein occlusion).