CLINICAL RESEARCH
Europace (2014) 16, 1327–1333 doi:10.1093/europace/euu024
Pacing and resynchronization therapy
Transvenous permanent pacemaker implantation in dextrocardia: technique, challenges, outcome, and a brief review of literature Jayaprakash Shenthar*, Maneesh K. Rai, Rohit Walia, Somasekhar Ghanta, Praveen Sreekumar, and Satish S. Reddy Electrophysiology Unit, Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bannerghatta Road, Jayanagar 9th Block, Bangalore 560069, Karnataka, India Received 2 January 2014; accepted after revision 22 January 2014; online publish-ahead-of-print 2 March 2014
Aims
----------------------------------------------------------------------------------------------------------------------------------------------------------Keywords
Permanent pacemaker implantation † Complete heart block † Congenital heart disease † Situs solitus dextrocardia † Corrected transposition of great arteries
Introduction Dextrocardia is defined as the right-sided embryologic development of the heart, with most of the cardiac mass positioned in the right hemithorax and a base-to-apex axis pointing to the right. It is a rare congenital anomaly with an incidence of 0.83 per 10 000 pregnancies.1 It may occur as situs solitus (normal visceroatrial arrangement), situs inversus (‘mirror image’ of normal visceroatrial arrangement), or situs ambiguous (visceroatrial isomerism). Major intracardiac defects are likely to be associated with .96% of patients with situs solitus dextrocardia and 23 –70% of patients with situs inversus dextrocardia and almost all patients with isomerism. The most common cardiac abnormalities being atrioventricular (AV) discordance, transposition complexes, and ventricular septal defects.1,2
The literature regarding permanent pacemaker implantation (PPI) in patients with dextrocardia is sparse and is limited to a few case reports.3 – 7 The complex anatomy and altered fluoroscopic orientation can pose considerable challenge to transvenous PPI in dextrocardia. Here, we describe a series of patients with dextrocardia who underwent PPI at our institution between Years 2006 and 2013. The challenges involved, the steps taken to overcome these challenges, long-term outcomes, and a brief review of literature are presented here.
Methods Sri Jayadeva Institute of Cardiovascular Sciences is a tertiary care, single specialty, teaching hospital dedicated to cardiovascular sciences. Between
* Corresponding author. Tel: +91 9845028386; fax: +91 8026534477, E-mail: [email protected] Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].
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Dextrocardia is a rare congenital anomaly. Pacemaker implantation in dextrocardia can be challenging because of the distorted anatomy and associated anomalies. The literature regarding implantation of pacemaker in dextrocardia is scarce. ..................................................................................................................................................................................... Methods The study involved retrospective analysis of records of patients with dextrocardia who had undergone pacemaker implantation between January 2006 and July 2013 from a single centre. Six patients with dextrocardia (five males and and results one female) underwent permanent pacemaker implantation (PPI) between January 2006 and July 2013. Of them, three had situs solitus dextrocardia and three situs inversus dextrocardia. All three patients with situs solitus dextrocardia had associated corrected transposition of great arteries. The indication for pacemaker implantation was symptomatic complete atrioventricular (AV) block in four, high-grade AV block in one, and sinus node dysfunction in one patient. A favourable outcome was noted during a mean follow-up of 3.9 years (4 months to 7 years) with one patient needing a pulse generator replacement. ..................................................................................................................................................................................... Conclusion Permanent pacemaker implantation in dextrocardia can be challenging because of the distorted anatomy. Use of a technique employing angiography to delineate chamber anatomy and relationship can assist the operator during such difficult PPIs. The medium- and long-term survival after a successful pacemaker implantation in dextrocardia is favourable.
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What’s new? † The literature regarding permanent pacemaker implantation in patients with dextrocardia and normal or abnormal situs is limited. † Transvenous pacemaker implantation is challenging because of the complex anatomy and abnormal fluoroscopic orientation. † A technique that employs angiography at the time of implantation provides valuable information regarding the anatomy of the cardiac chambers and the orientation of the interventricular septum and can assist the operator in challenging implantations. † The medium- and long-term survival after a successful pacemaker implantation in dextrocardia is favourable.
Procedure
lead was then manipulated by giving the stillete a clockwise torque and advanced to enter the pulmonary artery. The lead was then positioned in the RV apex by slow withdrawal from the pulmonary artery over a straight stilette. The final position of the lead was confirmed using angulated fluoroscopic projections left anterior oblique (LAO) 308 and right anterior oblique (RAO) 408. The lead position was considered satisfactory if the lead tip pointed towards the spine in RAO 408 view and if it pointed towards the cardiac apex in LAO 308 view (Figure 1). The RV lead was secured if a stable position and satisfactory lead parameters were achieved. A straight, bipolar, active fixation atrial lead was introduced via a separate venous puncture and positioned in the right atrium. The lead was manoeuvred into the right atrial appendage (RAA) over a pre-shaped J-shaped stillete available in the kit. The right atrial appendage position was confirmed by to-and-fro movement of the lead tip and by an anterior position of the lead in LAO view.
Dextrocardia with situs solitus (Patients 2, 4, and 5) All three patients with situs solitus dextrocardia had corrected transposition of great arteries (CTGA). In the first patient with situs solitus dextrocardia and CTGA, pacemaker implantation was done as described above. However, based on the difficulty encountered while positioning the leads in this patient, an angiographic technique was used in Patients 4 and 5 to assist the implantation. After securing two separate venous accesses, the RV lead was introduced into the right atrium and then manipulated into the RV over a curved stillette.
All patients had a temporary pacemaker inserted through the right femoral vein prior to the procedure. Patients were taken up for PPI in the fasting state. Prophylactic intravenous antibiotic was administered 1 h prior to the procedure as per institutional protocol. Implantation was performed from the right side in all the patients. After preparing and draping the proposed implantation site, 20 mL of 1% lignocaine was infiltrated and a 3 cm long incision was made one finger breadth below and parallel to the right clavicle across the deltopectoral groove such that two-third of the incision was medial and one-third lateral to the groove. Blunt dissection was continued until the deltopectoral pad of fat and the lateral border of pectoralis major was exposed. Cephalic vein was isolated and if adequate was used to insert the lead. If the cephalic vein was not of adequate calibre or would accept only a single lead, an extra thoracic axillary vein puncture was performed. Two separate venous accesses for atrial and ventricular leads were secured. Separate approaches were used to complete the rest of the implantation based on the situs and the presence or absence of associated cardiac defects.
Dextrocardia with situs inverses (mirror image) (Patients 1, 3, and 6) The procedure was performed in the anteroposterior (AP) view in all patients. Active fixation leads were used in all patients. The ventricular lead was introduced up to the right atrium where the straight stillete was exchanged for a stillete that was shaped to a large curve. The
Figure 1 Final lead position in situs inversus dextrocardia. The ventricular lead points towards the right in AP view (A), towards the spine in RAO view (B), and anteriorly in LAO view (C). The right atrial lead (positioned in the RAA) occupies a position on the left of the spine in situs inversus. (AP, anteroposterior; RAO, right anterior oblique; LAO, left anterior oblique.)
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January 2006 and July 2013, a total of 1814 patients underwent PPI. During this period, six patients with dextrocardia underwent transvenous PPI. The case records of these patients were retrospectively analysed. History, physical examination, 12-lead electrocardiogram, chest X-ray, detailed echocardiography, haematological investigations, associated conditions and procedures, indication for pacing, the procedure details including the fluoroscopic cine runs of the procedures, mode of pacing, type and model of pacemaker and leads, post-procedure follow-up were available for all the patients.
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Through the second access that was meant for the atrial lead, a 6F-valved sheath (AVANTIw+, Cordis Corporation) with a side port was inserted and its distal tip was positioned in the superior vena cava. Through the side port, 20 mL of non-ionic intravenous contrast was rapidly injected by hand and cine films were acquired in the AP, LAO 308, and RAO 408 views to define the position and orientation of the septum and lateral walls of the ventricle and also that of the RAA. Since both patients had associated CTGA and normal situs, the septum was found to be parallel to the spine in AP view, while the RAA was found in its usual position on the right side of the spine. The acquired films served as roadmaps to facilitate lead positioning (Figure 2). The 6F sheath was later exchanged over guidewire for a 7F peel away introducer and the atrial lead was positioned in the RAA over a curved stillete using the previously acquired cine films as roadmaps.
Between January 2006 and July 2013, there were six patients with dextrocardia who underwent a PPI. Of these, there were five males and one female. The mean age at presentation was 48.5 years (32– 62 years). Indication for pacemaker implantation was symptomatic complete AV block in four, high-grade AV block in one, and sinus node dysfunction in one patient. There were three patients with situs inversus (mirror image) and three with situs solitus dextrocardia. Three patients with situs solitus dextrocardia had associated corrected transposition of great vessels and one patient with situs inversus (Patient 6) had associated interrupted inferior vena cava with azygous continuation. All patients had normal systemic ventricular function on echocardiography. Associated intracardiac defects in the form of ventricular septal defects or outflow obstruction were absent in all the patients. The clinical details of the six patients are represented in Table 1. The course of a temporary pacemaker indicated the atrial situs in all but one patient. Patient 6 with situs inversus dextrocardia had interrupted inferior vena cava with left azygous connection to the superior vena cava. Because of the venous anomaly there was difficulty in positioning the temporary lead in the RV. A 110 cm length balloon tipped temporary pacemaker lead (C.R Bard Inc.) could be manipulated finally into the RV (Figure 3). During PPI for Patients 2 and 4, acute angulation between the subclavian vein and the superior vena cava prevented the guidewire from entering the right atrium. A short guidewire was exchanged over the dilator for a 135 cm length, radifocus, 0.35′′ guidewire (Terumo Corporation), and a Judkins 3.5 right coronary catheter was used to manipulate the guidewire into the right atrium. This was then exchanged for a longer peel away introducer to complete the procedure. In Patients 4 and 5 with situs solitus dextrocardia, angiography was used to define the anatomy. An angiogram performed at the time of the procedure guided the lead placement in these two patients. The implant parameters and follow-up data of the six patients are presented in Table 2. A dual-chamber pacemaker was implanted in five patients. A single-chamber pacemaker was implanted for Patient 2 because of financial reasons. Active fixation leads were used in all patients. There were no complications during any of the procedures. During a mean follow-up of 3.9 years (range: 0.4 –7 years), there were no complications. Patient 3 has undergone a pulse generator replacement at the end of 6 years.
Discussion Dextrocardia is a rare congenital anomaly with an incidence of 0.83 per 10 000 pregnancies.1 It may occur as situs solitus (normal visceroatrial arrangement), situs inversus (‘mirror image’ of normal visceroatrial arrangement), or situs ambiguous (visceroatrial isomerism). The first mention of AV block in dextrocardia was in 1929 by Yater.8 However, a detailed description of a asymptomatic 20-year-old patient with dextrocardia and complete AV block was given by Leys in 1943.9 In view of the young age of the patients in the above reports, it is quite likely that the cause of complete AV block was congenital. Complete AV block can occur as a congenital anomaly or after surgical repair of intracardiac defects. However,
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Figure 2 Angiography guided implantation in dextrocardia and situs solitus: (A, B) Subclavian venous angiography in AP and LAO views to demonstrate the septum and lateral walls of the ventricle. The permanent pacemaker lead appears to be mid-septal in position in both the views. The temporary pacemaker lead is also seen. (C) In RAO view, the lead is seen pointing away from the septum and towards the lateral wall. (D, E) Venous angiography in RAO view demonstrating the RAA (arrow in D) and the morphological left ventricle (E). Note the normal position of RAA in situs solitus. Also note that the morphological left ventricle is smooth and devoid of trabeculations. The permanent lead has been repositioned and screwed to the apex. (AP, anteroposterior; RAO, right anterior oblique; LAO, left anterior oblique; RAA, right atrial appendage.)
Results
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Table 1 Clinical characteristics of the patients Patient no
Age
Sex
Indication for PPI
Situs
Associated intracardiac anomaly/procedure
1
52
M
CHB
Inversus
–
2 3
32 57
M F
CHB High-grade AV block
Solitus Inversus
CTGA –
4
39
M
CHB
Solitus
CTGA
5 6
62 49
M M
CHB Sinus node dysfunction
Solitus Inversus
CTGA Post-CABG status, Interrupted IVC with left azygous vein
...............................................................................................................................................................................
M, male; F, female; CHB, complete heart block; IVC, inferior vena cava; CABG, coronary artery bypass surgery; CTGA, corrected transposition of great arteries.
dextrocardia patients without associated intracardiac major anomalies can survive to adult hood and may present with degenerative conduction system disease including AV blocks or sinus node dysfunction. The literature regarding PPI in patients with dextrocardia is sparse and is limited to a few case reports.3 – 7,10 The first report of pacemaker implantation in situs inversus dextrocardia and sinus node dysfunction was in 1976 by Goyal et al. 3 An angiographic study performed prior to the implantation of a pacemaker in the patient
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Figure 3 Anomalous course of the inferior vena cava. (A, B) AP and LAO views in Patient 6 with interrupted IVC and azygous continuation. A balloon-tipped temporary pacing lead is seen to course through the IVC, azygous vein, and the SVC into the right ventricle. (IVC, inferior vena cava; SVC, superior vena cava.)
revealed venous anomaly in the form of double superior vena cava and absent inferior vena cava. In fact, the report suggests an angiographic study in patients with dextrocardia prior to pacemaker implantation. Subsequently, all the cases reported so far have been in patients with dextrocardia with situs inversus, i.e., ‘mirror image’ dextrocardia. To the best of our knowledge, there appears to be no reports of pacemaker implantation in patients with situs solitus dextrocardia including those who have undergone implantation of cardiac resynchronization therapy. The details of the case reports are summarized in Table 3. Since dextrocardia with situs inversus is a mirror image of the normal, the use of a flipped image and the use of opposite angulated views, i.e. RAO in the place of LAO and vice versa, have been suggested to facilitate implantation.11 In our series, all patients with situs inversus dextrocardia underwent successful implantation in the AP view without having the need to flip the images. Moreover, the technique of flipping of image may be considered only in mirror image dextrocardia, since the anatomy is the mirror image of normal. However, this technique may not be useful in situs solitus dextrocardia where associated anomalies like corrected transposition of great vessels may be present and where the anatomy is not mirror image of the normal. In dextrocardia with normal situs, both atria along with the appendages and connecting great veins retain their normal relationship, while the ventricles are rotated to the right (Figure 4). Such malformations may be associated with cardiac defects in the form of ventricular inversion. In such a situation, fluoroscopic orientation can become difficult and positioning the lead challenging. To circumvent this problem and to guide the implantation of the leads, a technique of performing a venous angiogram via the second access was used as described. Cine angiograms serve as roadmaps to facilitate lead positioning in the atria and the ventricle. In addition to serving as roadmaps, the cine runs also reveal important anatomical information regarding the orientation of the septum, morphology of the venous chambers (whether trabeculated or smooth), and the presence of venous anomaly if any. In the two patients with situs solitus, dextrocardia and CTGA, in whom angiography was performed, the septum was found to be parallel to the spine in AP view while the RAA was found in its usual position on the right side of the spine. The acquired cine runs were used as roadmaps to facilitate lead positioning in these patients. The difficulty posed by altered fluoroscopic orientation and benefit of angiography guided implantation was clearly demonstrated in Patient 5 where initial RV lead placement using fluoroscopy alone resulted in lateral wall pacing. Angiography confirmed the lateral wall
Characteristics
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Patient 6
Pulse generator Mode
Medtronic Relia DDD
Medtronic Relia VVI
St Jude’s Integrity DDDR
Medtronic Relia DR DDD
Medtronic Relia DDD
Medtronic Relia DDD
Cephalic Axillary
– Cephalic
Axillary Axillary
Axillary Axillary
Cephalic Axillary
Axillary Axillary
.............................................................................................................................................................................................................................................
Access RA lead RV lead RA lead
52 cm, bipolar, active
–
52 cm, bipolar, active
52 cm, bipolar, active
52 cm, bipolar, active
52 cm, bipolar, active
RV lead Position
58 cm, bipolar, active
58 cm, bipolar, active
58 cm, bipolar, active
58 cm, bipolar, active
58 cm, bipolar, active
58 cm, bipolar, active
RA lead RV lead Threshold
Appendage
–
Appendage
Appendage
Appendage
Appendage
Apex
Apex
Apex
Apex
Apex
Apex
RA lead
0.6 V/0.4 ms
–
0.5 V/0.4 ms
0.7 V/0.4 ms
0.8 V/0.4 ms
0.3 V/0.4 ms
RV lead Sensitivity
0.6 V/0.4 ms
0.4 V/0.4 ms
0.7 V/0.4 ms
0.6 V/0.4 ms
0.9 V/0.4 ms
0.4 V/0.4 ms
2.5/12
– /9
2.7/14
3.2/11
3.0/15
2.5/10
890
700
450
820
910
770
680
990
610
760
Nil 7
Nil 6
Nil 6
Nil 4
Nil 0.4
Nil 0.2
P/R (mV) Impedance (V) RA RV Peri-procedural complications Follow-up (years)
780
Permanent pacemaker implantation in dextrocardia
Table 2 Implant data and outcome
RA, right atrium, RV, right ventricle.
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Table 3 Reports on pacing therapy in dextrocardia Author
Year
Age (years)
Indication
Situs
Device
Associated anomalies
Goyal et al. 3
1976
57
SND
Inversus
VVI
Double SVC, interrupted IVC
Badui et al. 4 Brito et al. 5
1995 2001
41 39
SND AVB
Inversus Inversus
DDD DDD
– –
Subbiah et al. 6
2007
65
AVB
Inversus
DDD
CTGA, post-systemic AV valve replacement
Fang et al. 7 Scott et al. 12
2009 2009
46 66
SND CHB
Inversus Inversus
DDD CRTD
Post-ASD surgery LV dysfunction
Zartner et al. 13
2009
18
CHB
Inversus
CRTD
D-TGA, VSD with PS post-surgical closure of VSD, and mustard operation
...............................................................................................................................................................................
SVC, superior vena cava; IVC, inferior vena cava; CTGA, corrected transposition of great arteries; AV, atrioventricular; ASD, atrial septal defect; LV, left ventricle; D-TGA, D-transposition of great arteries; PS, pulmonary stenosis; VSD, ventricular septal defect.
Figure 4 Lead position in situs solitus dextrocardia. Demonstrates the final lead position in AP view (A), RAO view (B), and LAO view (C ) in situs solitus and dextrocardia. Note, unlike in situs inversus dextrocardia, the atrial lead (positioned in the RAA) occupies its normal position on the right of the spine. (AP, anteroposterior; RAO, right anterior oblique; LAO, left anterior oblique.)
position of the lead and aided lead repositioning. Thus, the altered orientation and the morphology of the venous ventricle can easily be demonstrated by angiography that can be performed by a single operator with no added equipment necessary. Interestingly, the first case report suggests that an angiogram be used to guide the pacemaker implantation.3 Though this could have been due to the lack of detailed echocardiographic information available during the period, we feel that the angiogram performed using a second access can provide valuable information that may help the operator during a challenging implantation. The implant was performed from the right side in all cases irrespective of the situs. This was an operator preference. Though
Conclusion Dextrocardia is a rare congenital anomaly. Patients with dextrocardia and no major intracardiac lesions may survive to adulthood and present with degenerative conduction system disease. Transvenous PPI in dextrocardia can be challenging because of the distorted anatomy. Angiography can assist the operator in a challenging implantation. The medium- and long-term survival after a successful pacemaker implantation is favourable.
Conflict of interest: none declared.
References 1. Bohun CM, Potts JE, Casey BM, Sandor GGS. A population-based study of cardiac malformations and outcomes associated with dextrocardia. Am J Cardiol 2007;100: 305 –309. 2. Garg N, Agarwal BL, Modi N, Radhakrishnan S, Sinha N. Dextrocardia: an analysis of cardiac structures in 125 patients. Int J Cardiol 2003;88:143–155; discussion 155 –6. 3. Goyal SL. Sick sinus syndrome requiring permanent pacemaker implantation in a patient with mirror-image dextrocardia. CHEST J 1976;69:558. 4. Badui E, Lepe L, Solorio S, Sanchez H, Enciso R, Garcia P. Heart block in dextrocardia with situs inversus: a case report. Angiology 1995;46:537 –540.
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passive fixation leads could have been used, active fixation lead was preferred as it offered the advantage of flexibility of choosing an optimal pacing site in the setting of abnormal anatomy. Successful PPI with acceptable lead parameters could be achieved in all patients with no periprocedural complications. Stable lead thresholds were noted even on long-term follow-up that ranged up to 7 years. Follow-up echocardiograms did not reveal a deterioration of systemic ventricular function and one patient has even undergone a pulse replacement. In this series of pacemaker implants in patients with dextrocardia, an attempt has been made to describe the challenges encountered while performing the procedure. Techniques used to overcome these challenges have been described. To our knowledge, this is the largest series of implantation of pacemaker in patients with dextrocardia that has been performed in both situs solitus and situs inversus. There were no patients with isomerism in this series. Also, the challenges in implanting a transvenous pacemaker in patients with dextrocardia and situs solitus and corrected transposition have been described which has not been described in the literature so far.
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5. Brito MR, Miranda CE, Barros VC, Castro LR, Borges MH. Heart block in dextrocardia and situs inversus. Ann Noninvasive Electrocardiol 2001;6:369 –372. 6. Subbiah RN, Gula LJ, Yee R, Skanes AC, Klein GJ, Krahn AD. Images in cardiovascular medicine. Pacemaker implantation in a patient with dextrocardia, corrected transposition, and situs inversus. Circulation 2007;115:e607 –e609. 7. Fang Y, Jiang L-C, Chen M. Successful pacemaker implantation in a patient with dextrocardia situs inversus totalis. Europace 2009;11:1568 –1569. 8. Yater WM. Congenital heart block. Review of the literature: report of a case with incomplete heterotaxy; the electrocardiogram in dextrocardia. Arch Pediatr Adolesc Med 1929;38:112.
9. Leys D. Congenital heart block with dextrocardia. Br Heart J 1943;5:8 –10. 10. Pang BJ, Grigg LE, Mond HG. Where are the leads? Pacemaker implantation in dextrocardia. Europace 2013;15:569. 11. Mond HG, Karpawich PP. Pacing Options in the Adult Patient with Congenital Heart Disease. NJ: John Wiley and Sons; 2008. p 152. 12. Scott P, Roberts PR. Cardiac resynchronization therapy upgrade in a patient with dextrocardia and situs inversus. Europace 2009;11:1562 –1563. 13. Zartner P, Wiebe W, Volkmer M, Thomas D, Schneider M. Transvenous cardiac resynchronization therapy in complex congenital heart diseases: dextrocardia with transposition of great arteries after Mustard operation. Europace 2009;11:530–532.
IMAGES IN ELECTROPHYSIOLOGY
doi:10.1093/europace/euu206
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Thermal reconnection of chronically isolated pulmonary veins Shinsuke Miyazaki*, Hiroaki Nakamura, and Yoshito Iesaka Cardiology Division, Cardiovascular Center, Tsuchiura Kyodo Hospital, 11-7 Manabeshin-machi, Tsuchiura, Ibaraki 300-0053, Japan
* Corresponding author. Tel: +81 29 823 3111; fax: +81 29 826 2411. E-mail address: [email protected]
Conflict of interest: none declared.
Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].
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A 57-year-old man underwent second ablation procedure for recurrent atrial fibrillation with rapid ventricular response (110 b.p.m.) appeared at rest, 4 years after pulmonary vein (PV) isolation. Despite no PV reconnection at baseline, adenosine injection revealed transient reconnection of right ipsilateral PVs (Panel A, asterisk). Radiofrequency energy was applied at the dormant conduction site anterior to the right PVs (Panel B). Despite no reconnection before the application, transient reconnection was reproduced for 10 s during the radiofrequency application (Panel C, asterisk). Then, disappearance of dormant conduction was confirmed by repeated adenosine test. Although the mechanism is unclear, thermal effects on ion channel function may have facilitated transient reconnection.
Europace (2014) 16, 1327–1333 doi:10.1093/europace/euu024
Pacing and resynchronization therapy
Transvenous permanent pacemaker implantation in dextrocardia: technique, challenges, outcome, and a brief review of literature Jayaprakash Shenthar*, Maneesh K. Rai, Rohit Walia, Somasekhar Ghanta, Praveen Sreekumar, and Satish S. Reddy Electrophysiology Unit, Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bannerghatta Road, Jayanagar 9th Block, Bangalore 560069, Karnataka, India Received 2 January 2014; accepted after revision 22 January 2014; online publish-ahead-of-print 2 March 2014
Aims
----------------------------------------------------------------------------------------------------------------------------------------------------------Keywords
Permanent pacemaker implantation † Complete heart block † Congenital heart disease † Situs solitus dextrocardia † Corrected transposition of great arteries
Introduction Dextrocardia is defined as the right-sided embryologic development of the heart, with most of the cardiac mass positioned in the right hemithorax and a base-to-apex axis pointing to the right. It is a rare congenital anomaly with an incidence of 0.83 per 10 000 pregnancies.1 It may occur as situs solitus (normal visceroatrial arrangement), situs inversus (‘mirror image’ of normal visceroatrial arrangement), or situs ambiguous (visceroatrial isomerism). Major intracardiac defects are likely to be associated with .96% of patients with situs solitus dextrocardia and 23 –70% of patients with situs inversus dextrocardia and almost all patients with isomerism. The most common cardiac abnormalities being atrioventricular (AV) discordance, transposition complexes, and ventricular septal defects.1,2
The literature regarding permanent pacemaker implantation (PPI) in patients with dextrocardia is sparse and is limited to a few case reports.3 – 7 The complex anatomy and altered fluoroscopic orientation can pose considerable challenge to transvenous PPI in dextrocardia. Here, we describe a series of patients with dextrocardia who underwent PPI at our institution between Years 2006 and 2013. The challenges involved, the steps taken to overcome these challenges, long-term outcomes, and a brief review of literature are presented here.
Methods Sri Jayadeva Institute of Cardiovascular Sciences is a tertiary care, single specialty, teaching hospital dedicated to cardiovascular sciences. Between
* Corresponding author. Tel: +91 9845028386; fax: +91 8026534477, E-mail: [email protected] Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].
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Dextrocardia is a rare congenital anomaly. Pacemaker implantation in dextrocardia can be challenging because of the distorted anatomy and associated anomalies. The literature regarding implantation of pacemaker in dextrocardia is scarce. ..................................................................................................................................................................................... Methods The study involved retrospective analysis of records of patients with dextrocardia who had undergone pacemaker implantation between January 2006 and July 2013 from a single centre. Six patients with dextrocardia (five males and and results one female) underwent permanent pacemaker implantation (PPI) between January 2006 and July 2013. Of them, three had situs solitus dextrocardia and three situs inversus dextrocardia. All three patients with situs solitus dextrocardia had associated corrected transposition of great arteries. The indication for pacemaker implantation was symptomatic complete atrioventricular (AV) block in four, high-grade AV block in one, and sinus node dysfunction in one patient. A favourable outcome was noted during a mean follow-up of 3.9 years (4 months to 7 years) with one patient needing a pulse generator replacement. ..................................................................................................................................................................................... Conclusion Permanent pacemaker implantation in dextrocardia can be challenging because of the distorted anatomy. Use of a technique employing angiography to delineate chamber anatomy and relationship can assist the operator during such difficult PPIs. The medium- and long-term survival after a successful pacemaker implantation in dextrocardia is favourable.
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What’s new? † The literature regarding permanent pacemaker implantation in patients with dextrocardia and normal or abnormal situs is limited. † Transvenous pacemaker implantation is challenging because of the complex anatomy and abnormal fluoroscopic orientation. † A technique that employs angiography at the time of implantation provides valuable information regarding the anatomy of the cardiac chambers and the orientation of the interventricular septum and can assist the operator in challenging implantations. † The medium- and long-term survival after a successful pacemaker implantation in dextrocardia is favourable.
Procedure
lead was then manipulated by giving the stillete a clockwise torque and advanced to enter the pulmonary artery. The lead was then positioned in the RV apex by slow withdrawal from the pulmonary artery over a straight stilette. The final position of the lead was confirmed using angulated fluoroscopic projections left anterior oblique (LAO) 308 and right anterior oblique (RAO) 408. The lead position was considered satisfactory if the lead tip pointed towards the spine in RAO 408 view and if it pointed towards the cardiac apex in LAO 308 view (Figure 1). The RV lead was secured if a stable position and satisfactory lead parameters were achieved. A straight, bipolar, active fixation atrial lead was introduced via a separate venous puncture and positioned in the right atrium. The lead was manoeuvred into the right atrial appendage (RAA) over a pre-shaped J-shaped stillete available in the kit. The right atrial appendage position was confirmed by to-and-fro movement of the lead tip and by an anterior position of the lead in LAO view.
Dextrocardia with situs solitus (Patients 2, 4, and 5) All three patients with situs solitus dextrocardia had corrected transposition of great arteries (CTGA). In the first patient with situs solitus dextrocardia and CTGA, pacemaker implantation was done as described above. However, based on the difficulty encountered while positioning the leads in this patient, an angiographic technique was used in Patients 4 and 5 to assist the implantation. After securing two separate venous accesses, the RV lead was introduced into the right atrium and then manipulated into the RV over a curved stillette.
All patients had a temporary pacemaker inserted through the right femoral vein prior to the procedure. Patients were taken up for PPI in the fasting state. Prophylactic intravenous antibiotic was administered 1 h prior to the procedure as per institutional protocol. Implantation was performed from the right side in all the patients. After preparing and draping the proposed implantation site, 20 mL of 1% lignocaine was infiltrated and a 3 cm long incision was made one finger breadth below and parallel to the right clavicle across the deltopectoral groove such that two-third of the incision was medial and one-third lateral to the groove. Blunt dissection was continued until the deltopectoral pad of fat and the lateral border of pectoralis major was exposed. Cephalic vein was isolated and if adequate was used to insert the lead. If the cephalic vein was not of adequate calibre or would accept only a single lead, an extra thoracic axillary vein puncture was performed. Two separate venous accesses for atrial and ventricular leads were secured. Separate approaches were used to complete the rest of the implantation based on the situs and the presence or absence of associated cardiac defects.
Dextrocardia with situs inverses (mirror image) (Patients 1, 3, and 6) The procedure was performed in the anteroposterior (AP) view in all patients. Active fixation leads were used in all patients. The ventricular lead was introduced up to the right atrium where the straight stillete was exchanged for a stillete that was shaped to a large curve. The
Figure 1 Final lead position in situs inversus dextrocardia. The ventricular lead points towards the right in AP view (A), towards the spine in RAO view (B), and anteriorly in LAO view (C). The right atrial lead (positioned in the RAA) occupies a position on the left of the spine in situs inversus. (AP, anteroposterior; RAO, right anterior oblique; LAO, left anterior oblique.)
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January 2006 and July 2013, a total of 1814 patients underwent PPI. During this period, six patients with dextrocardia underwent transvenous PPI. The case records of these patients were retrospectively analysed. History, physical examination, 12-lead electrocardiogram, chest X-ray, detailed echocardiography, haematological investigations, associated conditions and procedures, indication for pacing, the procedure details including the fluoroscopic cine runs of the procedures, mode of pacing, type and model of pacemaker and leads, post-procedure follow-up were available for all the patients.
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Through the second access that was meant for the atrial lead, a 6F-valved sheath (AVANTIw+, Cordis Corporation) with a side port was inserted and its distal tip was positioned in the superior vena cava. Through the side port, 20 mL of non-ionic intravenous contrast was rapidly injected by hand and cine films were acquired in the AP, LAO 308, and RAO 408 views to define the position and orientation of the septum and lateral walls of the ventricle and also that of the RAA. Since both patients had associated CTGA and normal situs, the septum was found to be parallel to the spine in AP view, while the RAA was found in its usual position on the right side of the spine. The acquired films served as roadmaps to facilitate lead positioning (Figure 2). The 6F sheath was later exchanged over guidewire for a 7F peel away introducer and the atrial lead was positioned in the RAA over a curved stillete using the previously acquired cine films as roadmaps.
Between January 2006 and July 2013, there were six patients with dextrocardia who underwent a PPI. Of these, there were five males and one female. The mean age at presentation was 48.5 years (32– 62 years). Indication for pacemaker implantation was symptomatic complete AV block in four, high-grade AV block in one, and sinus node dysfunction in one patient. There were three patients with situs inversus (mirror image) and three with situs solitus dextrocardia. Three patients with situs solitus dextrocardia had associated corrected transposition of great vessels and one patient with situs inversus (Patient 6) had associated interrupted inferior vena cava with azygous continuation. All patients had normal systemic ventricular function on echocardiography. Associated intracardiac defects in the form of ventricular septal defects or outflow obstruction were absent in all the patients. The clinical details of the six patients are represented in Table 1. The course of a temporary pacemaker indicated the atrial situs in all but one patient. Patient 6 with situs inversus dextrocardia had interrupted inferior vena cava with left azygous connection to the superior vena cava. Because of the venous anomaly there was difficulty in positioning the temporary lead in the RV. A 110 cm length balloon tipped temporary pacemaker lead (C.R Bard Inc.) could be manipulated finally into the RV (Figure 3). During PPI for Patients 2 and 4, acute angulation between the subclavian vein and the superior vena cava prevented the guidewire from entering the right atrium. A short guidewire was exchanged over the dilator for a 135 cm length, radifocus, 0.35′′ guidewire (Terumo Corporation), and a Judkins 3.5 right coronary catheter was used to manipulate the guidewire into the right atrium. This was then exchanged for a longer peel away introducer to complete the procedure. In Patients 4 and 5 with situs solitus dextrocardia, angiography was used to define the anatomy. An angiogram performed at the time of the procedure guided the lead placement in these two patients. The implant parameters and follow-up data of the six patients are presented in Table 2. A dual-chamber pacemaker was implanted in five patients. A single-chamber pacemaker was implanted for Patient 2 because of financial reasons. Active fixation leads were used in all patients. There were no complications during any of the procedures. During a mean follow-up of 3.9 years (range: 0.4 –7 years), there were no complications. Patient 3 has undergone a pulse generator replacement at the end of 6 years.
Discussion Dextrocardia is a rare congenital anomaly with an incidence of 0.83 per 10 000 pregnancies.1 It may occur as situs solitus (normal visceroatrial arrangement), situs inversus (‘mirror image’ of normal visceroatrial arrangement), or situs ambiguous (visceroatrial isomerism). The first mention of AV block in dextrocardia was in 1929 by Yater.8 However, a detailed description of a asymptomatic 20-year-old patient with dextrocardia and complete AV block was given by Leys in 1943.9 In view of the young age of the patients in the above reports, it is quite likely that the cause of complete AV block was congenital. Complete AV block can occur as a congenital anomaly or after surgical repair of intracardiac defects. However,
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Figure 2 Angiography guided implantation in dextrocardia and situs solitus: (A, B) Subclavian venous angiography in AP and LAO views to demonstrate the septum and lateral walls of the ventricle. The permanent pacemaker lead appears to be mid-septal in position in both the views. The temporary pacemaker lead is also seen. (C) In RAO view, the lead is seen pointing away from the septum and towards the lateral wall. (D, E) Venous angiography in RAO view demonstrating the RAA (arrow in D) and the morphological left ventricle (E). Note the normal position of RAA in situs solitus. Also note that the morphological left ventricle is smooth and devoid of trabeculations. The permanent lead has been repositioned and screwed to the apex. (AP, anteroposterior; RAO, right anterior oblique; LAO, left anterior oblique; RAA, right atrial appendage.)
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Table 1 Clinical characteristics of the patients Patient no
Age
Sex
Indication for PPI
Situs
Associated intracardiac anomaly/procedure
1
52
M
CHB
Inversus
–
2 3
32 57
M F
CHB High-grade AV block
Solitus Inversus
CTGA –
4
39
M
CHB
Solitus
CTGA
5 6
62 49
M M
CHB Sinus node dysfunction
Solitus Inversus
CTGA Post-CABG status, Interrupted IVC with left azygous vein
...............................................................................................................................................................................
M, male; F, female; CHB, complete heart block; IVC, inferior vena cava; CABG, coronary artery bypass surgery; CTGA, corrected transposition of great arteries.
dextrocardia patients without associated intracardiac major anomalies can survive to adult hood and may present with degenerative conduction system disease including AV blocks or sinus node dysfunction. The literature regarding PPI in patients with dextrocardia is sparse and is limited to a few case reports.3 – 7,10 The first report of pacemaker implantation in situs inversus dextrocardia and sinus node dysfunction was in 1976 by Goyal et al. 3 An angiographic study performed prior to the implantation of a pacemaker in the patient
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Figure 3 Anomalous course of the inferior vena cava. (A, B) AP and LAO views in Patient 6 with interrupted IVC and azygous continuation. A balloon-tipped temporary pacing lead is seen to course through the IVC, azygous vein, and the SVC into the right ventricle. (IVC, inferior vena cava; SVC, superior vena cava.)
revealed venous anomaly in the form of double superior vena cava and absent inferior vena cava. In fact, the report suggests an angiographic study in patients with dextrocardia prior to pacemaker implantation. Subsequently, all the cases reported so far have been in patients with dextrocardia with situs inversus, i.e., ‘mirror image’ dextrocardia. To the best of our knowledge, there appears to be no reports of pacemaker implantation in patients with situs solitus dextrocardia including those who have undergone implantation of cardiac resynchronization therapy. The details of the case reports are summarized in Table 3. Since dextrocardia with situs inversus is a mirror image of the normal, the use of a flipped image and the use of opposite angulated views, i.e. RAO in the place of LAO and vice versa, have been suggested to facilitate implantation.11 In our series, all patients with situs inversus dextrocardia underwent successful implantation in the AP view without having the need to flip the images. Moreover, the technique of flipping of image may be considered only in mirror image dextrocardia, since the anatomy is the mirror image of normal. However, this technique may not be useful in situs solitus dextrocardia where associated anomalies like corrected transposition of great vessels may be present and where the anatomy is not mirror image of the normal. In dextrocardia with normal situs, both atria along with the appendages and connecting great veins retain their normal relationship, while the ventricles are rotated to the right (Figure 4). Such malformations may be associated with cardiac defects in the form of ventricular inversion. In such a situation, fluoroscopic orientation can become difficult and positioning the lead challenging. To circumvent this problem and to guide the implantation of the leads, a technique of performing a venous angiogram via the second access was used as described. Cine angiograms serve as roadmaps to facilitate lead positioning in the atria and the ventricle. In addition to serving as roadmaps, the cine runs also reveal important anatomical information regarding the orientation of the septum, morphology of the venous chambers (whether trabeculated or smooth), and the presence of venous anomaly if any. In the two patients with situs solitus, dextrocardia and CTGA, in whom angiography was performed, the septum was found to be parallel to the spine in AP view while the RAA was found in its usual position on the right side of the spine. The acquired cine runs were used as roadmaps to facilitate lead positioning in these patients. The difficulty posed by altered fluoroscopic orientation and benefit of angiography guided implantation was clearly demonstrated in Patient 5 where initial RV lead placement using fluoroscopy alone resulted in lateral wall pacing. Angiography confirmed the lateral wall
Characteristics
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Patient 6
Pulse generator Mode
Medtronic Relia DDD
Medtronic Relia VVI
St Jude’s Integrity DDDR
Medtronic Relia DR DDD
Medtronic Relia DDD
Medtronic Relia DDD
Cephalic Axillary
– Cephalic
Axillary Axillary
Axillary Axillary
Cephalic Axillary
Axillary Axillary
.............................................................................................................................................................................................................................................
Access RA lead RV lead RA lead
52 cm, bipolar, active
–
52 cm, bipolar, active
52 cm, bipolar, active
52 cm, bipolar, active
52 cm, bipolar, active
RV lead Position
58 cm, bipolar, active
58 cm, bipolar, active
58 cm, bipolar, active
58 cm, bipolar, active
58 cm, bipolar, active
58 cm, bipolar, active
RA lead RV lead Threshold
Appendage
–
Appendage
Appendage
Appendage
Appendage
Apex
Apex
Apex
Apex
Apex
Apex
RA lead
0.6 V/0.4 ms
–
0.5 V/0.4 ms
0.7 V/0.4 ms
0.8 V/0.4 ms
0.3 V/0.4 ms
RV lead Sensitivity
0.6 V/0.4 ms
0.4 V/0.4 ms
0.7 V/0.4 ms
0.6 V/0.4 ms
0.9 V/0.4 ms
0.4 V/0.4 ms
2.5/12
– /9
2.7/14
3.2/11
3.0/15
2.5/10
890
700
450
820
910
770
680
990
610
760
Nil 7
Nil 6
Nil 6
Nil 4
Nil 0.4
Nil 0.2
P/R (mV) Impedance (V) RA RV Peri-procedural complications Follow-up (years)
780
Permanent pacemaker implantation in dextrocardia
Table 2 Implant data and outcome
RA, right atrium, RV, right ventricle.
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Table 3 Reports on pacing therapy in dextrocardia Author
Year
Age (years)
Indication
Situs
Device
Associated anomalies
Goyal et al. 3
1976
57
SND
Inversus
VVI
Double SVC, interrupted IVC
Badui et al. 4 Brito et al. 5
1995 2001
41 39
SND AVB
Inversus Inversus
DDD DDD
– –
Subbiah et al. 6
2007
65
AVB
Inversus
DDD
CTGA, post-systemic AV valve replacement
Fang et al. 7 Scott et al. 12
2009 2009
46 66
SND CHB
Inversus Inversus
DDD CRTD
Post-ASD surgery LV dysfunction
Zartner et al. 13
2009
18
CHB
Inversus
CRTD
D-TGA, VSD with PS post-surgical closure of VSD, and mustard operation
...............................................................................................................................................................................
SVC, superior vena cava; IVC, inferior vena cava; CTGA, corrected transposition of great arteries; AV, atrioventricular; ASD, atrial septal defect; LV, left ventricle; D-TGA, D-transposition of great arteries; PS, pulmonary stenosis; VSD, ventricular septal defect.
Figure 4 Lead position in situs solitus dextrocardia. Demonstrates the final lead position in AP view (A), RAO view (B), and LAO view (C ) in situs solitus and dextrocardia. Note, unlike in situs inversus dextrocardia, the atrial lead (positioned in the RAA) occupies its normal position on the right of the spine. (AP, anteroposterior; RAO, right anterior oblique; LAO, left anterior oblique.)
position of the lead and aided lead repositioning. Thus, the altered orientation and the morphology of the venous ventricle can easily be demonstrated by angiography that can be performed by a single operator with no added equipment necessary. Interestingly, the first case report suggests that an angiogram be used to guide the pacemaker implantation.3 Though this could have been due to the lack of detailed echocardiographic information available during the period, we feel that the angiogram performed using a second access can provide valuable information that may help the operator during a challenging implantation. The implant was performed from the right side in all cases irrespective of the situs. This was an operator preference. Though
Conclusion Dextrocardia is a rare congenital anomaly. Patients with dextrocardia and no major intracardiac lesions may survive to adulthood and present with degenerative conduction system disease. Transvenous PPI in dextrocardia can be challenging because of the distorted anatomy. Angiography can assist the operator in a challenging implantation. The medium- and long-term survival after a successful pacemaker implantation is favourable.
Conflict of interest: none declared.
References 1. Bohun CM, Potts JE, Casey BM, Sandor GGS. A population-based study of cardiac malformations and outcomes associated with dextrocardia. Am J Cardiol 2007;100: 305 –309. 2. Garg N, Agarwal BL, Modi N, Radhakrishnan S, Sinha N. Dextrocardia: an analysis of cardiac structures in 125 patients. Int J Cardiol 2003;88:143–155; discussion 155 –6. 3. Goyal SL. Sick sinus syndrome requiring permanent pacemaker implantation in a patient with mirror-image dextrocardia. CHEST J 1976;69:558. 4. Badui E, Lepe L, Solorio S, Sanchez H, Enciso R, Garcia P. Heart block in dextrocardia with situs inversus: a case report. Angiology 1995;46:537 –540.
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passive fixation leads could have been used, active fixation lead was preferred as it offered the advantage of flexibility of choosing an optimal pacing site in the setting of abnormal anatomy. Successful PPI with acceptable lead parameters could be achieved in all patients with no periprocedural complications. Stable lead thresholds were noted even on long-term follow-up that ranged up to 7 years. Follow-up echocardiograms did not reveal a deterioration of systemic ventricular function and one patient has even undergone a pulse replacement. In this series of pacemaker implants in patients with dextrocardia, an attempt has been made to describe the challenges encountered while performing the procedure. Techniques used to overcome these challenges have been described. To our knowledge, this is the largest series of implantation of pacemaker in patients with dextrocardia that has been performed in both situs solitus and situs inversus. There were no patients with isomerism in this series. Also, the challenges in implanting a transvenous pacemaker in patients with dextrocardia and situs solitus and corrected transposition have been described which has not been described in the literature so far.
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5. Brito MR, Miranda CE, Barros VC, Castro LR, Borges MH. Heart block in dextrocardia and situs inversus. Ann Noninvasive Electrocardiol 2001;6:369 –372. 6. Subbiah RN, Gula LJ, Yee R, Skanes AC, Klein GJ, Krahn AD. Images in cardiovascular medicine. Pacemaker implantation in a patient with dextrocardia, corrected transposition, and situs inversus. Circulation 2007;115:e607 –e609. 7. Fang Y, Jiang L-C, Chen M. Successful pacemaker implantation in a patient with dextrocardia situs inversus totalis. Europace 2009;11:1568 –1569. 8. Yater WM. Congenital heart block. Review of the literature: report of a case with incomplete heterotaxy; the electrocardiogram in dextrocardia. Arch Pediatr Adolesc Med 1929;38:112.
9. Leys D. Congenital heart block with dextrocardia. Br Heart J 1943;5:8 –10. 10. Pang BJ, Grigg LE, Mond HG. Where are the leads? Pacemaker implantation in dextrocardia. Europace 2013;15:569. 11. Mond HG, Karpawich PP. Pacing Options in the Adult Patient with Congenital Heart Disease. NJ: John Wiley and Sons; 2008. p 152. 12. Scott P, Roberts PR. Cardiac resynchronization therapy upgrade in a patient with dextrocardia and situs inversus. Europace 2009;11:1562 –1563. 13. Zartner P, Wiebe W, Volkmer M, Thomas D, Schneider M. Transvenous cardiac resynchronization therapy in complex congenital heart diseases: dextrocardia with transposition of great arteries after Mustard operation. Europace 2009;11:530–532.
IMAGES IN ELECTROPHYSIOLOGY
doi:10.1093/europace/euu206
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Thermal reconnection of chronically isolated pulmonary veins Shinsuke Miyazaki*, Hiroaki Nakamura, and Yoshito Iesaka Cardiology Division, Cardiovascular Center, Tsuchiura Kyodo Hospital, 11-7 Manabeshin-machi, Tsuchiura, Ibaraki 300-0053, Japan
* Corresponding author. Tel: +81 29 823 3111; fax: +81 29 826 2411. E-mail address: [email protected]
Conflict of interest: none declared.
Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].
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A 57-year-old man underwent second ablation procedure for recurrent atrial fibrillation with rapid ventricular response (110 b.p.m.) appeared at rest, 4 years after pulmonary vein (PV) isolation. Despite no PV reconnection at baseline, adenosine injection revealed transient reconnection of right ipsilateral PVs (Panel A, asterisk). Radiofrequency energy was applied at the dormant conduction site anterior to the right PVs (Panel B). Despite no reconnection before the application, transient reconnection was reproduced for 10 s during the radiofrequency application (Panel C, asterisk). Then, disappearance of dormant conduction was confirmed by repeated adenosine test. Although the mechanism is unclear, thermal effects on ion channel function may have facilitated transient reconnection.
