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Atrioventricular reentrant tachycardia in a child with tricuspid atresia

A case report of catheter ablation

Wang, Yefeng, MDa; Liu, Qiming, MD, PHDb; Deng, Xicheng, MD, PHDc; Xiao, Yunbin, MD, PHDa; Chen, Zhi, MDa,*

Section Editor(s): NA.,

doi: 10.1097/MD.0000000000014320
Research Article: Clinical Case Report
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Rationale: Atrioventricular reentrant tachycardia (AVRT) is the most common supraventricular tachycardia occurring in children. However, in complex congenital heart disease patients with a different heart anatomy and conduction system morphology, accessory pathway modification may be particularly challenging because of distortion of typical anatomic landmarks.

Patient concerns: A 10-year-old boy with tricuspid atresia and history of bidirectional Glenn operation had recurrent chest distress and palpitation for 3 months. He had multiple hospitalizations for narrow-QRS tachycardia with poor hemodynamic tolerance, despite the use of adenosine and amiodarone.

Diagnoses: AVRT. Tricuspid atresia with secundum atrial septal defect, large ventricular septal defect, and right ventricular outflow tract stenosis.

Interventions: Cardiac catheterization, electrophysiological examination, and ablation.

Outcomes: The child has not had a recurrent AVRT during 6 months of follow-up and is waiting for Fontan operation.

Lessons: Since there is an increased risk of accessory pathways in patients with tricuspid atresia, all these patients should be checked before the Fontan operation to exclude congenital accessory pathways.

aDepartment of Cardiology, Hunan Children's Hospital

bDepartment of Cardiology, Second Xiangya Hospital, Central South University

cDepartment of Cardiothoracic Surgery, Hunan Children's Hospital, Changsha, Hunan, China.

Correspondence: Zhi Chen, Department of Cardiology, Hunan Children's Hospital, Changsha 410007, Hunan, China (e-mail: eyxxgchenzhi@163.com).

Abbreviations: AV = atrial-ventricular, AVRT = atrioventricular reentrant tachycardia, LV = left ventricular.

The authors have no conflicts of interest to disclose.

This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc/4.0

Received October 5, 2018

Received in revised form January 2, 2019

Accepted January 9, 2019

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1 Introduction

Atrioventricular reentrant tachycardia (AVRT) is the most common supraventricular tachycardia occurring in children. Catheter ablation is considered the definitive therapy of choice in the majority of patients.[1] However, in complex congenital heart disease patients with a different heart anatomy and conduction system morphology, accessory pathway modification may be particularly challenging because of distortion of typical anatomic landmarks.[2,3] Here, we report a case of AVRT in a child with tricuspid atresia and history of bidirectional Glenn operation, and describe the procedure of catheterization and ablation.

The ethics committee of Hunan Children's Hospital approved this study as a case report for retrospective analysis. The ethical approval number was HCHLL-2018-68. Informed written consent was obtained from the patient for publication of this case report and accompanying images. We anonymized all information before analysis.

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2 Case report

A 10-year-old boy with complex congenital heart disease presented with hemodynamically poorly tolerated refractory paroxysmal supraventricular tachycardia. The heart presented a single, leftsided ventricle due to severe tricuspid hypoplasia, associated with a large secundum atrial septal defect and ventricular septal defect with right ventricular outflow tract stenosis. The patient had already undergone a bidirectional Glenn procedure at age 3.

Over the last 3 months, he had multiple hospitalizations for narrow-QRS tachycardia with poor hemodynamic tolerance, despite the use of adenosine and amiodarone. When arrhythmia-free, he remained functionally stable in New York Heart Association functional class II–III/IV. Physical examination revealed cyanosis and clubbing, with a baseline oxygen saturation of 85%. Electrocardiogram showed normal sinus rhythm at 90 beats/min, no preexcitation, biatrial enlargement. The patient was pending the Fontan operation, and a preliminary electrophysiological study was requested.

The patient was brought to the cardiac electrophysiology laboratory in the fasting and unsedated state. Routine monitoring equipment including electrocardiogram and defibrillation pads was placed. With the child under intubation and general anesthesia, access was obtained in both femoral veins and the right femoral artery. Angiocardiography showed a secundum atrial septal defect and large ventricular septal defect with right ventricular outflow tract stenosis (Fig. 1). The connection between superior vena cava and right pulmonary artery was unobstructed. The pressure and oxygen saturation values of different chambers were taken during cardiac catheterization.

Figure 1

Figure 1

A 6-French 4-polar electrode catheter and a 4-mm ablation catheter were introduced through the right femoral vein into the left ventricle and right interatrial septum. Ventricular extrastimulus testing from the left ventricular (LV) demonstrated nondecremental ventricular-atrial conduction with retrograde refractory period of 280 ms. Atrial extrastimulus testing showed decremental atrial-ventricular (AV) conduction and induced a supraventricular tachycardia with a cycle length of 390 ms (Fig. 2A). A His-bundle electrogram was recorded from right interatrial septum, superior to the coronary sinus ostium with the ablation catheter. The tachycardia was pace-terminable, with anterograde atrioventricular block.

Figure 2

Figure 2

Electroanatomic mapping of right atria was performed when the patient was in tachycardia. The site of earliest atrial activation was localized in the coronary sinus, between electrode 1-2 and 3-4 (Figs. 2B, 3). In sinus rhythm, radiofrequency ablation was attempted at this point and a temperature controlled ablation system with a maximum temperature of 60°C and power output of 30 W for 120 seconds. In the process of ablation, junctional acceleration did not occur. Postablation, the tachycardia cannot be induced by atrial extrastimulus testing and overdrive pacing. Ventricular extrastimulus testing from the LV demonstrated decremental ventricular-atrial conduction with retrograde refractory period of 400 ms. These findings suggested that the most likely diagnosis was posteroseptal accessory pathway-mediated tachycardia. The child has not had a recurrent event during 6 months of follow-up and is waiting for Fontan operation.

Figure 3

Figure 3

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3 Discussion

Children with complex congenital heart disease are at risk of developing arrhythmias as a result of cardiac defects, the hemodynamic stress imposed by such malformations, or postoperative sequelae following cardiac surgery.[3] Some congenital heart malformations are associated with an abnormal architecture of the conduction system. Apart from arrhythmias caused by anatomic malformations and/or scar-related arrhythmias due to surgical corrections, all known forms of supraventricular tachycardia can occur in patients with tricuspid atresia.[4] Recurrent tachycardia can cause cardiac insufficiency and increase the risk of sudden death.[5] Therefore, a detailed electrophysiological examination is necessary to determine the mechanism of arrhythmia and risk of ablation. We completed cardiac catheterization, pulmonary artery pressure assessment, and electrophysiological examination at the same time, which can provide an effective support for Fontan operation.

As in previous studies, congenital accessory pathways in patients with tricuspid atresia are mostly on the right side,[6,7] which is the same in our case. This confirms the theory of Misaki et al[7] that the accessory pathway is on the side of the congenitally malformed atrioventricular valve, although this theory is based mainly on patients with Ebstein malformation and only on a very few number of other congenital atrioventricular valve defects. Children with tricuspid atresia usually need staged surgical correction. Arrhythmia may occur before and after surgical treatment, especially after Fontan operation. Due to the presence of atrial baffle and changes of atrioventricular connection, it is very difficult to treat the arrhythmia and even lose opportunities for ablation.[8]

Since there is an increased risk of accessory pathways in patients with tricuspid atresia,[2,9] all these patients should be checked before the Fontan operation to exclude congenital accessory pathways. If an accessory pathway is detected, it should be localized and ablated either in the same session or during surgical intervention, even if the patient is asymptomatic.[6] The application of three-dimensional ablation system has greatly improved the success rate and safety of ablation in children with complex congenital heart disease.[10] Radiofrequency ablation is possible, even in infants with a similar success rate compared with that seen in older children.

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Author contributions

Conceptualization: Yefeng Wang, Zhi Chen.

Data curation: Yefeng Wang, Yunbin Xiao.

Investigation: Yefeng Wang.

Methodology: Xicheng Deng.

Supervision: Qiming Liu, Zhi Chen.

Writing – original draft: Yefeng Wang.

Writing – review & editing: Xicheng Deng, Zhi Chen.

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References

[1]. Philip Saul J, Kanter RJ, Abrams D, et al. PACES/HRS expert consensus statement on the use of catheter ablation in children and patients with congenital heart disease: developed in partnership with the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American Academy of Pediatrics (AAP), the American Heart Association (AHA), and the Association for European Pediatric and Congenital Cardiology (AEPC). Heart Rhythm 2016;13:e251–89.
[2]. Brugada J, Blom N, Sarquella-Brugada G, et al. Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement. Europace 2013;15:1337–82.
[3]. Khairy P, Seslar SP, Triedman JK, et al. Ablation of atrioventricular nodal reentrant tachycardia in tricuspid atresia. J Cardiovasc Electrophysiol 2004;15:719–22.
[4]. Arana-Rueda E, Pedrote A, Sánchez-Brotons JA, et al. Ablation of atrioventricular nodal reentrant tachycardia in a patient with tricuspid atresia guided by electroanatomic mapping. Pacing Clin Electrophysiol 2012;35:e293–5.
[5]. Weipert J, Noebauer C, Schreiber C, et al. Occurrence and management of atrial arrhythmia after long-term Fontan circulation. J Thorac Cardiovasc Surg 2004;127:457–64.
[6]. Hager A, Zrenner B, Brodherr-Heberlein S, et al. Congenital and surgically acquired Wolff-Parkinson-White syndrome in patients with tricuspid atresia. J Thorac Cardiovasc Surg 2005;130:48–53.
[7]. Misaki T, Watanabe G, Iwa T, et al. Surgical treatment of atrioventricular atresia combined with Wolff-Parkinson-White syndrome. Chest 1995;107:669–73.
[8]. Schwagten B, Cuypers J, Szili-Torok T. The magnetic navigation system allows avoidance of puncturing a baffle during ablation of a postincisional macroreentrant tachycardia. Cardiol Young 2009;19:216–9.
[9]. Peinado R, Gnoatto M, Merino JL, et al. Catheter ablation of multiple, surgically created, atrioventricular connections following Fontan-Björk procedure. Europace 2007;9:848–50.
[10]. Ernst S. Catheter ablation: general principles and advances. Card Electrophysiol Clin 2017;9:311–7.
Keywords:

Atrioventricular reentrant tachycardia; catheter ablation; children; tricuspid atresia

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