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Case Report

Anesthesia Management of Radiofrequency Ablation

in a Pediatric Patient of Ebstein Anomaly with
Wolff–Parkinson–White Syndrome
Sujit Jagannath Kshirsagar, Sanyogita Vijay Naik, Neharica Seth, Pradnya Bhambire
Department of Anaesthesiology, B. J. Government Medical College, Pune, Maharashtra, India

Abstract
Ebstein anomaly (EA) is a congenital malformation with an incidence of 0.72/10,000 populations. It is characterized by abnormal displacement
of tricuspid valve, atrialization of proximal portion of the right ventricle, right atrial enlargement, and tricuspid valve regurgitation. Twenty
percent of patients with EA are associated with Wolff–Parkinson–White syndrome (WPW syndrome). WPW syndrome is a preexcitation
syndrome characterized by abnormal accessory pathway between atria and ventricles which can cause perioperative life‑threatening
arrhythmias such as atrial fibrillation and paroxysmal supraventricular tachycardia. Here, we describe the successful anesthesia management
of electrophysiological study with three‑dimensional mapping and radiofrequency ablation procedure in an 8‑year‑old child of EA with
WPW syndrome under general anesthesia.

Keywords: Ebstein anomaly, radiofrequency ablation, Wolf–Parkinson–White syndrome

Introduction history, the patient had developed bluish discoloration of their

faces, limbs, and trunk and refused feeds on days 8–9 of life.
Ebstein anomaly (EA) is a rare congenital malformation
At that time, he was evaluated and was diagnosed as having
with an incidence of 0.72 cases/10,000 live births. [1] It
EA, which was managed conservatively on medications. The
is a primary abnormality of the tricuspid valve and right
patient responded well to medications and was then discharged.
ventricle. Tricuspid valve is displaced downward, causing
The patient had recurrent episodes of chest infections, for
atrialization of the right ventricle. It is associated with
which he had taken treatment on outpatient department basis.
abnormalities of mitral valve, aortic valve, and pulmonary
The last episode of chest infection was 6 months ago. At
atresia.[2] It can also cause intracardiac shunting leading to
present, the patient has no complaints. Four months ago, a
cyanosis, pulmonary hypertension, cardiac dysrhythmias,
cardiac catheterization study was performed for hemodynamic
and Wolf–Parkinson–White (WPW) syndrome. [3] WPW
evaluation. It showed systemic venous drainage was by right
syndrome is a preexcitation syndrome characterized by
superior vena cava and inferior vena cava into the right atrium.
abnormal accessory pathway (AP) between atria and
The right ventricular angiogram revealed significant apical
ventricles. In electrophysiological study (EPS)/radiofrequency
ablation (RFA) procedures, it is important to choose anesthesia
Address for correspondence: Dr. Sujit Jagannath Kshirsagar,
agents that have no or minimal effect on the cardiac conduction Department of Anaesthesiology, B.J. Government Medical College, Pune,
system. Maharashtra, India.
E‑mail: bjsujit@gmail.com

Case Report Submitted: 10‑Jul‑2022 Revised: 11-Jul-2022

An 8‑year‑old male child, 3rd of three children, born out of Accepted: 15‑Jul‑2022 Published: 19-Aug-2022

nonconsanguinous marriage, was brought to the hospital by

This is an open access journal, and articles are distributed under the terms of the Creative
his parents for elective procedure of EPS and RFA. In past Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to
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How to cite this article: Kshirsagar SJ, Naik SV, Seth N, Bhambire P.
DOI: Anesthesia management of radiofrequency ablation in a pediatric patient of
10.4103/jpcs.jpcs_25_22 Ebstein anomaly with Wolff–Parkinson–White syndrome. J Pract Cardiovasc
Sci 2022;8:109-11.

© 2022 Journal of the Practice of Cardiovascular Sciences | Published by Wolters Kluwer - Medknow 109
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Kshirsagar, et al.: RFA in WPW syndrome with Ebstein anomaly

displacement of the septum of tricuspid valve with moderate right femoral artery was cannulated and transduced for IBP
tricuspid regurgitation, large portion of the right ventricle measurement. Intraoperatively, to locate the pathway injection
atrialized and functional right ventricle was limited to body of isoprenaline (0.05 µg/kg/h), infusion was started and
and outlet. The pulmonary arteries were normal in size, as were tachycardia was induced. 3D mapping of the right side of the
the pulmonary venous drainage. The left ventricular angiogram heart was done, and AP was located. The location was right
showed a normal‑sized left ventricle, normal left ventricle basolateral and was ablated successfully. The patient was
systolic function, and no mitral regurgitation. Coronary hemodynamically stable throughout the procedure.
arteries and aorta were normal. An electrocardiogram (ECG)
After the procedure, the patient was reversed with
showed delta waves (slurred upstroke of QRS complex – WPW
injection glycopyrrolate (0.008 mg/kg) and injection
syndrome) with the left anterior descending and right bundle
neostigmine (0.06 mg/kg) and the patient was extubated. The
branch block left anterior hemiblock [Figure 1]. Chest X‑ray
patient was observed in the postoperative period for saturation
showed cardiomegaly [Figure 2]. The patient was started on
and ECG. Then, the patient was shifted to the intensive care
tablet propranolol 10 mg, tablet amiodarone 100 mg, and tablet
unit.
ecospirin 50 mg orally OD. On auscultation, early systolic
murmur was present. Hence, the patient was posted for EPS with
three‑dimensional mapping and RFA of AP. General anesthesia Discussion
with endotracheal (ET) intubation with controlled mechanical EA is a congenital malformation of tricuspid valve and right
ventilation was planned. Preoperatively, defibrillator was kept ventricle. In this anomaly, there is failure of separation of
ready. Patient’s weight was recorded (18 kg). fibrous and muscular attachments of the tricuspid valve to
the right ventricular myocardium (failure of delamination).
After taking written informed consent, the patient was
There is displacement of hinge points of septal and
taken inside the catheterization laboratory. Intravenous
posterior (inferior) leaflets into the right ventricle toward the
fluid was started through intracath. ECG, noninvasive blood
apex and right ventricular outflow tract which is the hallmark
pressure (NIBP), SpO2, and automated external defibrillator
finding of EA. This causes decrease in size of right ventricle
pads were attached to patient. The patient’s vitals were noted.
and the proximal portion of right ventricle gets “atrialized.”
Heart rate was 92 beats/min. Blood pressure was 106/64 mmHg.
The atrialized right ventricle is thin walled and has poor
SpO2 was 99% on room air. The patient was premedicated
contractility which causes decreased ejection fraction of the
with injection glycopyrrolate (0.004 mg/kg), injection
right ventricle. Furthermore, in EA, there is variable degree
ondansetron (0.15 mg/kg), injection midazolam (0.05 mg/kg),
of regurgitation of tricuspid valve from mild‑to‑severe
and injection fentanyl (1 µg/kg). The patient was preoxygenated
degrees due to fenestrations. Additional associated anomalies
with 100% oxygen for 3 min. Anesthesia was induced with
include bicuspid or atretic aortic valves, pulmonary atresia or
injection of propofol 2.5 mg/kg, and the patient was relaxed
hypoplastic pulmonary artery, subaortic stenosis, coarctation,
with injection of atracurium 0.75 mg/kg. The patient was
mitral valve prolapse, accessory mitral valve tissue or muscle
intubated orally with cuffed endotracheal (ET) tube No. 5.
bands of the left ventricle, ventricular septal defects, and
ET tube placement was confirmed with EtCo2. Anesthesia
pulmonary stenosis.[2]
was maintained with sevoflurane with O2:N2O (50:50) and
atracurium as skeletal muscle relaxant. Monitoring included Approximately 14%–20% of EA patients will have one or more
ECG, pulse oximetry, end‑tidal carbon dioxide, NIBP, invasive accessory conduction pathways with WPW syndrome.[4] Atrial
blood pressure (IBP), and urine output. fibrillation leading to sudden cardiac death, atrial reentrant
The right femoral vein was cannulated for insertion of
mapping electrodes (decapolar and quadripolar) and left
femoral vein was cannulated for insertion of ablator. The

Figure 1: ECG showing delta waves with LAHB. ECG: Electrocardiograms

and LAHB: Left anterior hemiblock. Figure 2: CXR showing cardiomegaly. CXR: Chest X‑ray.

110 Journal of the Practice of Cardiovascular Sciences ¦ Volume 8 ¦ Issue 2 ¦ May-August 2022
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Kshirsagar, et al.: RFA in WPW syndrome with Ebstein anomaly
tachycardia, atrial fibrillation, atrial flutter, ectopic atrial
tachycardia, and ventricular tachyarrhythmias may occur.[5]
WPW syndrome is a preexcitation syndrome that occurs due
to an AP connecting atria and ventricles, allowing electrical
activity to bypass the atrioventricular node and activate
His‑Purkinje system. This AP when conducts impulse in
antegrade direction; the ECG shows a delta wave and the
WPW pattern on ECG.
In EPS with ablative procedures, the mainstay of anesthesia
management is that anesthetics should not interfere with
cardiac electrophysiology.[6] In general anesthetics, propofol
is considered safe as it does not impede the generation of
diagnostic supraventricular tachycardias, also it does not affect
the refractory periods of accessory pathways in WPW patients.[6]
In children, isoflurane prolongs the atrial, ventricular, and AP
anterograde effective refractory period.[7] Similarly, enflurane,
halothane, and desflurane also prolong the refractory period
and slow the conduction in APs, interfering EPS.[8] Sevoflurane
has only moderate effect on cardiac electrophysiology, and in
children, it causes only moderate prolongation of AP antegrade
effective refractory period.[9] Fentanyl is found safe even at very
high doses.[10] Among neuromuscular blockers, atracurium and
cisatracurium are better choices.
In conclusion, the successful management of RFA in EA with
WPW syndrome consists of adequate preparation of Operation
Theatre (OT) and thorough knowledge of pathophysiology
of disease. Multidisciplinary approach with prior discussion
with cardiologist about the exact procedure helps in proper
management of case.
Ethics clearance
Appropriate consent was taken from the relatives of the patient
before making the case report. As this is a case report the
university's guideline does not require an ethical committee
and/or Institutional Review Board (IRB) clearance for its
scientific publication.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form, the patient’s guardian has
Journal of the Practice of Cardiovascular Sciences ¦ Volume 8 ¦ Issue 2 ¦ May-August 2022
given consent for images and other clinical information to be
reported in the journal. The patient’s guardian understands
that name and initials will not be published and due efforts
will be made to conceal identity, but anonymity cannot be
guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
1. Lupo PJ, Langlois PH, Mitchell LE. Epidemiology of Ebstein anomaly:
Prevalence and patterns in Texas, 1999‑2005. Am J Med Genet A
2011;155A: 1007‑14.
2. Attenhofer Jost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA,
Danielson GK. Ebstein’s anomaly‑Review of a multifaceted congenital
cardiac condition. Swiss Med Wkly 2005;135:269‑81.
3. Misa VS, Pan PH. Evidence‑based case report for analgesic and
anesthetic management of a parturient with Ebstein’s Anomaly
and Wolff‑Parkinson‑White syndrome. Int J Obstet Anesth
2007;16:77‑81.
4. Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC Jr.
Operative treatment of Ebstein’s anomaly. J Thorac Cardiovasc Surg
1992;104:1195‑202.
5. Hebe J. Ebstein’s anomaly in adults. Arrhythmias: Diagnosis and
therapeutic approach. Thorac Cardiovasc Surg 2000;48:214‑9.
6. Moore JP, Kannankeril PJ, Fish FA. Isoproterenol administration
during general anesthesia for the evaluation of children with ventricular
preexcitation. Circ Arrhythm Electrophysiol 2011;4:73‑8.
7. Chang RK, Stevenson WG, Wetzel GT, Shannon K, Baum VC,
Klitzner TS. Effects of isoflurane on electrophysiological measurements
in children with the Wolff‑Parkinson‑White syndrome. Pacing Clin
Electrophysiol 1996;19:1082‑8.
8. Sharpe MD, Dobkowski WB, Murkin JM, Klein G, Guiraudon G,
Yee R. The electrophysiologic effects of volatile anesthetics
and sufentanil on the normal atrioventricular conduction system
and accessory pathways in Wolff‑Parkinson‑White syndrome.
Anesthesiology 1994;80:63‑70.
9. Pérez ER, Bartolomé FB, Carretero PS, Fernández CS, Mateos EJ,
Tarlovsky LG. Electrophysiological effects of sevoflurane in comparison
with propofol in children with Wolff‑Parkinson‑White syndrome. Rev
Esp Anestesiol Reanim 2008;55:26‑31.
10. Celermajer DS, Cullen S, Deanfield JE, Sullivan ID. Congenitally
corrected transposition and Ebstein’s anomaly of the systemic
atrioventricular valve: Association with aortic arch obstruction. J Am
Coll Cardiol 1991;18:1056‑8.
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