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 Table of Contents  
Year : 2022  |  Volume : 8  |  Issue : 2  |  Page : 109-111

Anesthesia management of radiofrequency ablation in a pediatric patient of ebstein anomaly with wolff–Parkinson–White syndrome

Department of Anaesthesiology, B. J. Government Medical College, Pune, Maharashtra, India

Date of Submission10-Jul-2022
Date of Decision11-Jul-2022
Date of Acceptance15-Jul-2022
Date of Web Publication19-Aug-2022

Correspondence Address:
Sujit Jagannath Kshirsagar
Department of Anaesthesiology, B.J. Government Medical College, Pune, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcs.jpcs_25_22

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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

How to cite this article:
Kshirsagar SJ, Naik SV, Seth N, Bhambire P. Anesthesia management of radiofrequency ablation in a pediatric patient of ebstein anomaly with wolff–Parkinson–White syndrome. J Pract Cardiovasc Sci 2022;8:109-11

How to cite this URL:
Kshirsagar SJ, Naik SV, Seth N, Bhambire P. Anesthesia management of radiofrequency ablation in a pediatric patient of ebstein anomaly with wolff–Parkinson–White syndrome. J Pract Cardiovasc Sci [serial online] 2022 [cited 2023 Feb 3];8:109-11. Available from: https://www.j-pcs.org/text.asp?2022/8/2/109/354132

  Introduction Top

Ebstein anomaly (EA) is a rare congenital malformation with an incidence of 0.72 cases/10,000 live births.[1] It is a primary abnormality of the tricuspid valve and right ventricle. Tricuspid valve is displaced downward, causing atrialization of the right ventricle. It is associated with abnormalities of mitral valve, aortic valve, and pulmonary atresia.[2] It can also cause intracardiac shunting leading to cyanosis, pulmonary hypertension, cardiac dysrhythmias, and Wolf–Parkinson–White (WPW) syndrome.[3] WPW syndrome is a preexcitation syndrome characterized by abnormal accessory pathway (AP) between atria and ventricles. In electrophysiological study (EPS)/radiofrequency ablation (RFA) procedures, it is important to choose anesthesia agents that have no or minimal effect on the cardiac conduction system.

  Case Report Top

An 8-year-old male child, 3rd of three children, born out of nonconsanguinous marriage, was brought to the hospital by his parents for elective procedure of EPS and RFA. In past history, the patient had developed bluish discoloration of their faces, limbs, and trunk and refused feeds on days 8–9 of life. At that time, he was evaluated and was diagnosed as having EA, which was managed conservatively on medications. The patient responded well to medications and was then discharged. The patient had recurrent episodes of chest infections, for which he had taken treatment on outpatient department basis. The last episode of chest infection was 6 months ago. At present, the patient has no complaints. Four months ago, a cardiac catheterization study was performed for hemodynamic evaluation. It showed systemic venous drainage was by right superior vena cava and inferior vena cava into the right atrium. The right ventricular angiogram revealed significant apical displacement of the septum of tricuspid valve with moderate tricuspid regurgitation, large portion of the right ventricle atrialized and functional right ventricle was limited to body and outlet. The pulmonary arteries were normal in size, as were the pulmonary venous drainage. The left ventricular angiogram showed a normal-sized left ventricle, normal left ventricle systolic function, and no mitral regurgitation. Coronary arteries and aorta were normal. An electrocardiogram (ECG) showed delta waves (slurred upstroke of QRS complex – WPW syndrome) with the left anterior descending and right bundle branch block left anterior hemiblock [Figure 1]. Chest X-ray showed cardiomegaly [Figure 2]. The patient was started on tablet propranolol 10 mg, tablet amiodarone 100 mg, and tablet 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 with endotracheal (ET) intubation with controlled mechanical ventilation was planned. Preoperatively, defibrillator was kept ready. Patient's weight was recorded (18 kg).
Figure 1: ECG showing delta waves with LAHB. ECG: Electrocardiograms and LAHB: Left anterior hemiblock

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Figure 2: CXR showing cardiomegaly. CXR: Chest X-ray.

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After taking written informed consent, the patient was taken inside the catheterization laboratory. Intravenous fluid was started through intracath. ECG, noninvasive blood pressure (NIBP), SpO2, and automated external defibrillator pads were attached to patient. The patient's vitals were noted. Heart rate was 92 beats/min. Blood pressure was 106/64 mmHg. SpO2 was 99% on room air. The patient was premedicated with injection glycopyrrolate (0.004 mg/kg), injection ondansetron (0.15 mg/kg), injection midazolam (0.05 mg/kg), and injection fentanyl (1 μg/kg). The patient was preoxygenated with 100% oxygen for 3 min. Anesthesia was induced with injection of propofol 2.5 mg/kg, and the patient was relaxed with injection of atracurium 0.75 mg/kg. The patient was intubated orally with cuffed endotracheal (ET) tube No. 5. ET tube placement was confirmed with EtCo2. Anesthesia was maintained with sevoflurane with O2:N2O (50:50) and atracurium as skeletal muscle relaxant. Monitoring included ECG, pulse oximetry, end-tidal carbon dioxide, NIBP, invasive blood pressure (IBP), and urine output.

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 right femoral artery was cannulated and transduced for IBP measurement. Intraoperatively, to locate the pathway injection of isoprenaline (0.05 μg/kg/h), infusion was started and tachycardia was induced. 3D mapping of the right side of the heart was done, and AP was located. The location was right basolateral and was ablated successfully. The patient was hemodynamically stable throughout the procedure.

After the procedure, the patient was reversed with injection glycopyrrolate (0.008 mg/kg) and injection neostigmine (0.06 mg/kg) and the patient was extubated. The patient was observed in the postoperative period for saturation and ECG. Then, the patient was shifted to the intensive care unit.

  Discussion Top

EA is a congenital malformation of tricuspid valve and right ventricle. In this anomaly, there is failure of separation of fibrous and muscular attachments of the tricuspid valve to the right ventricular myocardium (failure of delamination). There is displacement of hinge points of septal and posterior (inferior) leaflets into the right ventricle toward the apex and right ventricular outflow tract which is the hallmark finding of EA. This causes decrease in size of right ventricle and the proximal portion of right ventricle gets “atrialized.” The atrialized right ventricle is thin walled and has poor contractility which causes decreased ejection fraction of the right ventricle. Furthermore, in EA, there is variable degree of regurgitation of tricuspid valve from mild-to-severe degrees due to fenestrations. Additional associated anomalies include bicuspid or atretic aortic valves, pulmonary atresia or hypoplastic pulmonary artery, subaortic stenosis, coarctation, mitral valve prolapse, accessory mitral valve tissue or muscle bands of the left ventricle, ventricular septal defects, and pulmonary stenosis.[2]

Approximately 14%–20% of EA patients will have one or more accessory conduction pathways with WPW syndrome.[4] Atrial fibrillation leading to sudden cardiac death, atrial reentrant 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 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


Conflicts of interest

There are no conflicts of interest.

  References Top

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.  Back to cited text no. 1
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.  Back to cited text no. 2
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.  Back to cited text no. 3
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.  Back to cited text no. 4
Hebe J. Ebstein's anomaly in adults. Arrhythmias: Diagnosis and therapeutic approach. Thorac Cardiovasc Surg 2000;48:214-9.  Back to cited text no. 5
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.  Back to cited text no. 6
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.  Back to cited text no. 7
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.  Back to cited text no. 8
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.  Back to cited text no. 9
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.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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