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Ann Thorac Surg 2009;87:303-305. doi:10.1016/j.athoracsur.2008.06.014
© 2009 The Society of Thoracic Surgeons

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Case Reports

Total Transvenous Approach to Pacing and Defibrillation After Ebstein's Anomaly

J. Alberto Lopez, MD*

Department of Cardiology and Electrophysiology, Texas Heart Institute at St. Luke's Episcopal Hospital, and Baylor College of Medicine, Houston, Texas

Accepted for publication June 10, 2008.

* Address correspondence to Dr Lopez, 6624 Fannin, Suite 2780, Houston, TX 77030 (Email: jalopez{at}bcm.tmc.edu).


    Abstract
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 Abstract
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We describe the successful use of a percutaneous, transvenous endocardial approach to atrioventricular pacing and cardiac defibrillation in an adult born with Ebstein's anomaly who had undergone tricuspid valve repair. The patient has systolic left ventricular dysfunction, congestive heart failure, and sinus node dysfunction. Ventricular pacing and sensing was obtained with a bipolar lead placed in the inferolateral cardiac vein; atrial pacing was obtained at the low interatrial septum. Internal cardiac defibrillation was achieved with a coil lead placed in the middle cardiac vein and the active can in the left retro mammary pre-pectoral position. With this approach, we avoided a thoracotomy and epicardial patch in a patient whose previous tricuspid valve surgery precluded an endocardial right ventricular lead position.


    Introduction
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 Abstract
 Introduction
 Comment
 References
 
The use of transvenous pacing or defibrillation therapy is challenging in patients who have undergone surgery for Ebstein's anomaly. However, when ventricular pacing is required and there is no intracardiac shunt, ventricular stimulation may be obtained in the right ventricular (RV) outflow tract distal to the tricuspid valve, in the atrialized right ventricle [1], or by using the coronary sinus tributaries [2]. Placement of the defibrillating transvenous leads may be even more challenging; use of the optimal site for lead placement, the right ventricular apex, may not be possible if the patient has undergone valve repair or tricuspid valve replacement.

A 59-year-old woman born with moderate Ebstein's anomaly and a ventricular septal defect underwent ventricular septal defect closure and tricuspid valve repair at age 15. In 2001, she underwent percutaneous closure of a patent foramen ovale. She had congestive heart failure develop in 2005, and in March 2007, she had atrial fibrillation develop and more severe congestive heart failure symptoms. She received anticoagulation and optimal congestive heart failure therapy. An elective cardioversion procedure was also performed. Once sinus rhythm was established, sinus bradycardia and junctional rhythm indicated severe sick sinus syndrome. At our institution, transthoracic and transesophageal echocardiography showed the Ebstein's anomaly, with mild regurgitation, and a left ventricular ejection fraction of 30%. There was noncompaction of the left ventricular myocardium and 2/4 mitral regurgitation. The patient's atrial septal closure device was well seated, with residual trace left-to-right shunting. Cardiac catheterization showed no evidence of coronary occlusive disease. Atrial pacing and an implantable cardioverter defibrillator were recommended as primary preventive therapy for sudden cardiac death.

With the patient under conscious sedation, we obtained vascular access via the left axillary vein. Right atrial angiography showed that the atrialized right ventricle and the closure point of distal valve repair precluded lead placement in the right ventricular apex or the interventricular septum (Fig 1). We assessed the coronary sinus tributaries for alternative positioning of the ventricular defibrillation leads (Fig 2). An Attain bipolar lead (Model 4191 [Medtronic, Minneapolis, MN]) was placed in the inferolateral vein. The R-wave amplitude was 6.8 mV, the pacing threshold was 1.3 V at 0.5 mSec (820 mOhms), and there was no phrenic nerve stimulation. A defibrillating coil lead (Model 6949 [Medtronic]) was delivered through the sheath into the middle cardiac vein without difficulty. We successfully placed a SelectSecure bipolar lead (Model 3830 [Medtronic]) in the atrial septum, anterior to the closure device and superior to the ostium of the coronary sinus. The P-wave amplitude was 2.8 mV, and the pacing threshold was 0.8 V at 0.5 mSec (505 Ohms) (Fig 3).


Figure 1
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Fig 1. Angiogram at (a) end diastole and (b) systole in the anterior–posterior projection showing the atrialized right ventricle (RV), the coaptation point of the tricuspid valve, and the excluded RV apex. (RVOT = right ventricular outflow tract.)

 

Figure 2
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Fig 2. Selective angiograms of the (a) inferolateral left anterior oblique (LAO) projection and (b) the middle cardiac vein in the right anterior oblique (RAO) projection.

 

Figure 3
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Fig 3. Final lead configuration. Atrial lead in the septum above the ostium of the coronary sinus; bipolar pace/sense (p/s) lead in the lateral (lat) vein; defibrillating (defib) coil lead in the middle cardiac vein (MCV). (ASD = atrial septal defect.)

 
With the aid of local anesthesia, a retromammary prepectoral pocket was formed. Leads were tunneled from the infraclavicular region and connected to a Virtuoso DR pulse generator (Model D154A [Medtronic]). Using 50 H2 burst pacing, ventricular fibrillation was induced three times: it was successfully terminated using a 25-J shock and unsuccessfully terminated once using a 20-J shock (impedance, 35–37 Ohms). The patient was discharged home in stable condition 2 days later. Follow-up at 7, 30, and 90 days showed stable atrial and ventricular pacing and sensing thresholds and defibrillation lead impedance.


    Comment
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 Abstract
 Introduction
 Comment
 References
 
Ebstein's anomaly is a rare congenital heart disorder accounting for less than 0.5% of all defects [3]. After adequate repair, most patients enjoy good quality of life and an expected postoperative survival of 89.9% at 15 years [4]. Patients who present later for surgical correction (and thus have more severe cardiomegaly), those with ventricular tachycardia and fibrillation in the postoperative period, and those with persistent symptomatic arrhythmias requiring antiarrhythmic medications seem to be at an increased risk for sudden cardiac death [4, 5].

In patients with congestive heart failure symptoms and decreased left ventricular systolic function, implantation of a cardioverter has decreased total mortality more than medical therapy has with amiodarone [6]. In this patient, we positioned the defibrillating coil lead in the middle cardiac vein because of its stability and the resulting current vector, and we obtained left ventricular pacing and sensing by placing the bipolar lead in the posterolateral vein (Fig 2), thus avoiding the need for a thoracotomy and epicardial patch. Recently, coils designed for subcutaneous use have been placed in the pericardial space by a subxiphoid approach in patients with high defibrillation thresholds [7] or in children and patients with congenital heart disease [8] to avoid the use of transvenous coil leads or epicardial patches.

One significant consideration with this technique is the complexity, difficulty, and risks associated with coronary sinus lead removal should it become necessary; however, we believe the risks compare favorably with those of epicardial pacing and defibrillation with regard to morbidity and mortality and the need for reintervention due to lead failure.


    References
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 Abstract
 Introduction
 Comment
 References
 

  1. Andersen C, Oxhoj H, Justesen P. Pacing in a patient with Ebstein's anomaly Pacing Clin Electrophysiol 1989;12:1586-1588.[Medline]
  2. Lopez JA, Leachman DR. Successful use of transvenous atrial and bifocal left ventricular pacing in Ebstein's anomaly after tricuspid prosthetic valve surgery Ann Thorac Surg 2007;83:1183-1185.[Abstract/Free Full Text]
  3. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's anomaly Circulation 2007;115:277-285.[Free Full Text]
  4. Boston US, Dearani JA, O'Leary PW, Driscoll DJ, Danielson GK. Tricuspid valve repair for Ebstein's anomaly in young children: a 30-year experience Ann Thorac Surg 2006;81:690-695discussion 695–6.[Abstract/Free Full Text]
  5. Oechslin EN, Harrison DA, Connelly MS, Webb GD, Siu SC. Mode of death in adults with congenital heart disease Am J Cardiol 2000;86:1111-1116.[Medline]
  6. Bardy GH, Lee KL, Mark DB, et al. Amiodarone or an implantable cardioverter-defibrillator for congestive heart failure N Engl J Med 2005;352:225-237.[Medline]
  7. Molina JE, Benditt DG. An epicardial subxiphoid implantable defibrillator lead: superior effectiveness after failure of standard implants Pacing Clin Electrophysiol 2004;27:1500-1506.[Medline]
  8. Stephenson EA, Batra AS, Knilans TK, et al. A multicenter experience with novel implantable cardioverter defibrillator configurations in the pediatric and congenital heart disease population J Cardiovasc Electrophysiol 2006;17:41-46.[Medline]



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This Article
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Right arrow Congenital - cyanotic
Right arrow Electrophysiology - arrhythmias


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