Abstract
We present a case of a complicated lead extraction and reimplantation of an implantable cardioverter defibrillator (ICD) in a young woman with complete transposition of great arteries (CTGA), a cyanotic congenital heart defect in which the aorta and the pulmonary trunk are transposed. The malformation results in two parallel circulations, whereby the left ventricle is attached to the pulmonary trunk and the right ventricle is attached to the aorta. Survival depends on the mixing of these two circulations at the level of the atria or ventricles or great arteries. Balloon atrial septostomy and creation of an intra-atrial baffle are procedures that increase atrial mixing, increase systemic oxygenation, and hence improve survival. With the improved survival of patients with CTGA, there is an increasing need for permanent pacemakers (PPMs) and ICDs for rhythm disturbances. These leads and/or devices are often inserted when the patients are very young and need to be replaced or explanted in adulthood due to device or lead malfunction, device-associated infection, or generator replacement or upgrades. These procedures are often complicated by the patients' complex anatomy and/or shunts. We describe a patient with CTGA who had an intra-atrial baffle and a nonfunctioning dual-chamber PPM. The lead was extracted via the baffle and the old PPM was upgraded to an ICD. Such descriptions are rare.
As increasing numbers of adults with complex congenital heart disease survive to adulthood, there will be an expanding need for implantation of transvenous permanent pacemakers (PPMs) and implantable cardioverter defibrillator (ICDs). Lead extraction procedures are expected to increase in parallel with increasing device implantations. We describe a young patient with complete transposition of great arteries (CTGA) and a Mustard intra-atrial baffle repair for CTGA who had a laser lead extraction of a nonfunctioning dual chamber (DDD) PPM.
CASE REPORT
A 34-year-old woman with CTGA had undergone atrial septostomy immediately after birth followed by a Mustard intra-atrial baffle repair at 18 months of age. At 5 years, an abdominal epicardial DDD PPM was placed for symptomatic bradycardia. At age 20, she had a transvenous DDD PPM inserted through the right subclavian vein (the patient was left-handed). At age 26, the pulse generator reached the elective replacement indicator and was replaced.
At age 34, the patient developed fatigue, worsening dyspnea, and bradycardia with awake heart rates of <40 beats per minute. The PPM (battery voltage 2.5 V) was at the elective replacement indicator. Both the PPM and the atrial and ventricular leads were found to be functioning poorly. (These leads had been in place for over 14 years.) The atrial lead impedance was >3200 ohms, and no capture was noted on the atrial or ventricular leads at maximal output. She had an underlying escape junctional rhythm of 35 beats per minute. A chest radiograph (Figure 1) showed a right-sided pacemaker generator with a 2-lead system that went to the left atrium and the subpulmonic ventricle (morphologic left ventricle) through the Mustard baffle. A trans-thoracic echocardiogram showed severe systemic ventricular dysfunction (morphologic right ventricle) with an ejection fraction of <0.30 and normal pulmonic ventricular function (morphologic left ventricle) with mild to moderate regurgitation of the systemic atrioventricular valve. The baffles were not well visualized. Transesophageal echocardiogram showed a dilated hypokinetic systemic ventricle, normally functioning subpulmonic ventricle, mild regurgitation of the systemic and pulmonic atrioventricular valves, and a patent baffle measuring 8 mm in minimal diameter with a small fenestration (Figure 2). This measurement was confirmed with gated cardiac multislice computed tomography angiography, which showed a patent baffle measuring 7 mm in minimal diameter (Figure 3).
Figure 1.
(a) Anteroposterior and (b) lateral views of the chest radiograph with the old pacemaker in place. The atrial and ventricular leads are seen in the left atrium and the subpulmonic ventricle (morphologic left ventricle) through the Mustard baffle, as well as old leads from the previous abdominal epicardial dual-chamber pacemaker.
Figure 2.
Transesophageal echocardiography (midesophageal) demonstrating a patent baffle to the pulmonic ventricle; the pacing wire can be appreciated coursing through this baffle. A shunt from the systemic venous return to the pulmonic venous return can be seen at the 11:00 position. SV indicates systemic ventricle; PV, pulmonic ventricle.
Figure 3.
Contrast-enhanced cardiac computed tomography demonstrating (a) a highly trabeculated and dilated systemic ventricle anteriorly with a pacing wire coursing through the pulmonic ventricle posteriorly; (b) the parallel orientation of the semilunar valves (the aortic valve is anterior); (c) the coronary arteries arising from the aortic root; and (d) a patent baffle to the pulmonic ventricle measuring 7 mm. SV indicates systemic ventricle; PV, pulmonic ventricle; AoV, aortic valve; PuV, pulmonic valve; LMCA, left main coronary artery; RCA, right coronary artery; SVC, superior vena cava.
Laser lead extraction was performed with the Spectranetics laser sheath removal system (Colorado Springs, CO) in an operating room under general anesthesia. Initially, percutaneous access of the right subclavian vein was obtained and 2 guide wires were placed in the subpulmonic ventricle (morphologic left ventricle) via the baffle in anticipation of DDD ICD placement after laser lead extraction. The pocket was opened, the pulse generator was removed, and the leads were dissected and exposed. Inspection revealed outer insulation discontinuity of both leads. After the screw was retracted, locking stylets (EZ Spectranectics, Colorado Springs, CO) were introduced in the lumen and advanced to the distal tip. Manual tension was applied, but leads could not be removed. Under fluoroscopic guidance, a 12F laser sheath was telescoped over each of the leads and advanced through the baffle into the left atrium and left ventricle. When resistance to sheath advancement was encountered, laser energy (Excimer Xenon Chloride, 308 nm wavelength) was delivered to the tip of the sheath to pulverize fibrous attachments. With laser applications, manual traction, and countertraction, both the atrial and ventricular leads were removed in their entirety.
Subsequently, a right-sided DDD ICD (Medtronic D284DRG) was inserted using a single-coil ICD lead (Medtronic 6935-58). Atrial testing showed normal thresholds with no evidence of diaphragmatic stimulation. Defibrillation threshold testing was done twice successfully at 25 joules. No complications occurred during the procedure, and the total fluoroscopy time was under 1 hour. The patient was successfully discharged home the next day after a device check was within normal limits and after a chest x-ray was obtained (Figure 4).
Figure 4.
(a) Anteroposterior and (b) lateral views of the chest radiograph with the new device in place.
DISCUSSION
Sinus node dysfunction is a frequent occurrence following certain types of palliative and complete repairs for congenital heart disease. Postoperative complete heart block is often seen with operations involving the interventricular septum such as ventricular septal defects and atrioventricular canal defects. Sudden cardiac death due to ventricular tachyarrhythmia is an uncommon but devastating event in the pediatric population (1). Patients with congenital heart disease carry a disproportionate risk of sudden cardiac death that can be as high as 25 to 100 times greater than that of the general population (2), and hence there has been an increasing use of ICDs based on published guidelines (3).
We report a case demonstrating safe and successful pacemaker laser lead extraction and reimplantation of a dual-chamber ICD system through the preexisting baffle in a young woman with CTGA and prior Mustard repair. Laser lead extraction is safe and superior to conventional extraction techniques (4). It uses pulsed ultraviolet light that can dissolve fibrous tissue and allow sheath advancement without excessive force or tearing of tissues (5).
Data regarding laser lead extraction in complex congenital heart disease is limited but has been shown in selective adult patients in this population to be safe and efficacious (6–8). Similarly, there are a few cases of laser lead extraction through a baffle, mostly limited to case reports (6, 7, 9, 10) (Table), and often the leads are abandoned rather than extracted in anticipation of the difficulty associated with extraction of the leads (7).
Table.
Cases in patients with complete transposition of great arteries and a Mustard baffle
References
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