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Electrophysiology and surgery intertwined in complex treatment of Ebstein’s anomaly in childhood

Open AccessPublished:September 30, 2022DOI:https://doi.org/10.1016/j.hrcr.2022.09.014
Electrophysiology and surgery intertwined in complex treatment of Ebstein’s anomaly in childhood
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      Keywords

      Introduction

      Ebstein’s anomaly, a rare and highly variable congenital heart defect,
      • Lupo P.J.
      • Langlois P.H.
      • Mitchell L.E.
      Epidemiology of Ebstein anomaly: prevalence and patterns in Texas, 1999-2005.
      Am J Med Genet Part A. 2011; 155: 1007-1014
      still presents a treatment challenge. The currently used cone repair of the tricuspid valve has carried favorable results in suitable patients.
      • Holst K.A.
      • Dearani J.A.
      • Said S.
      • et al.
      Improving results of surgery for Ebstein anomaly: where are we after 235 cone repairs?.
      Ann Thorac Surg. 2018; 105: 160-168
      Arrhythmogenic substrates including accessory pathways
      • Hebe J.
      Ebstein’s anomaly in adults. Arrhythmias: diagnosis and therapeutic approach.
      Thorac Cardiovasc Surg. 2000; 48: 214-219
      ,
      • Watson H.
      Natural history of Ebstein’s anomaly of tricuspid valve in childhood and adolescence. An international cooperative study of 505 cases.
      Br Heart J. 1974; 36: 417-427
      and right bundle branch block
      • Kastor J.A.
      • Goldreyer B.N.
      • Josephson M.E.
      • et al.
      Electrophysiologic characteristics of Ebstein’s anomaly of the tricuspid valve.
      Circulation. 1975; 52: 987-995
      associated with electromechanical ventricular dyssynchrony present additional therapeutic targets. We present a patient with Ebstein’s anomaly of tricuspid valve and Wolff-Parkinson-White syndrome in whom joint electrophysiological and surgical interventions were used to address all major disease components. This report is unique in highlighting a combination of different treatment modalities for managing severe Ebstein’s anomaly, including catheter pathway mapping and ablation, surgery with cryoablation, and right ventricular cardiac resynchronization therapy (RV-CRT), with optimal results.
      Key Teaching Points
      • •
        Ebstein’s anomaly is a rare and highly variable congenital heart defect presenting with different degrees of severity. The currently used cone repair of the tricuspid valve has carried favorable results in suitable patients.
      • •
        Accessory atrioventricular pathways causing atrioventricular reentrant tachycardia are frequent and present an additional therapeutic target.
      • •
        Owing to difficulties with pathway mapping and ablation typical for the Ebstein’s anomaly and high recurrence rate, surgical cryodestruction associated with tricuspid valve repair may be an appealing option if catheter ablation fails.
      • •
        Manifest right-sided accessory pathway ablation may demask right bundle branch block with subsequent right ventricular electromechanical discoordination amenable to right ventricular cardiac resynchronization therapy.

      Case report

      A 4.5-year-old girl with Ebstein’s anomaly of the tricuspid valve and Wolff-Parkinson-White syndrome has suffered from recurrent poorly tolerated paroxysms of orthodromic atrioventricular reentrant tachycardia despite treatment with sotalol. Echocardiography showed a type C Ebstein’s anomaly of the tricuspid valve with third-degree tricuspid regurgitation and no shunting on the atrial level (Supplemental Video 1). Her resting electrocardiogram revealed a sinus and low right atrial rhythm with ventricular preexcitation compatible with a right posteroseptal-to-posterior manifest accessory pathway (Figure 1A ). During electrophysiological study using the EnSite Precision™ 3D cardiac mapping system, an orthodromic atrioventricular reentrant tachycardia with right bundle branch block QRS morphology was easily inducible by single atrial extrastimuli (Figure 1B). Initially, successful cryoablation of a right posteroseptal manifest accessory pathway was performed, resulting in a change of the preexcitation pattern and separation of local atrial and ventricular electrograms (Figure 1C–1E). Another right posterior manifest pathway could not be ablated successfully (Figure 1F and 1G). The distance of approximately 10 mm between both mapping sites (Figure 1G) is in favor of the presence of 2 distinct pathways rather than 1 broad pathway, although this could not be definitely proven. Cryoenergy was used to limit the risk of right coronary artery damage reported during radiofrequency catheter ablation in Ebstein’s anomaly
      • Bertram H.
      • Bökenkamp R.
      • Peuster M.
      • Hausdorf G.
      • Paul T.
      Coronary artery stenosis after radiofrequency catheter ablation of accessory atrioventricular pathways in children with Ebstein’s malformation.
      Circulation. 2001; 103: 538-543
      owing to the presence of thin ventricular wall at the anatomical tricuspid annulus.
      Figure thumbnail gr1ad
      Figure 1Electrophysiological study. A: Twelve-lead electrocardiogram (ECG) prior to ablation. B: Orthodromic atrioventricular reentrant tachycardia with right bundle branch block QRS morphology and QRS complex duration of 120 ms. C: Local electrograms at successful ablation site of the right posteroseptal accessory pathway from the cryo-catheter (FRZ dist and uni) with ventricular signal coinciding with delta wave onset. The strictly negative ventricular signal from the ablation catheter tip (FRZ uni) is in favor of correct placement at the ventricular insertion site of the posteroseptal pathway. D: Local electrograms after posteroseptal pathway ablation (FRZ dist) with clear division of the atrial and ventricular electrogram at the ablation site. E: Twelve-lead ECG showing different QRS morphology after posteroseptal pathway ablation with a delta wave evident especially in leads I and V4. F: Local electrograms at the site of the second unsuccessfully ablated right posterior pathway (FRZ dist) with ventricular signal clearly preceding delta wave onset. G: Three-dimensional electroanatomical map of the earliest ventricular activation on the tricuspid annulus (delineated by the black line) during sinus rhythm. First manifest pathway could be successfully ablated in the right posteroseptal region (green arrow). The second manifest pathway could be mapped to the posterior tricuspid annulus (blue arrow) and could not be ablated. The distance between the 2 mapping sites was estimated to be 10 mm (white line) and is in favor of 2 distinct pathways rather than 1 broad pathway, although this cannot be definitely proven. FRZ dist indicates distal bipolar signal from the ablation catheter; FRZ uni, unipolar signal from the ablation catheter tip; CS, signals from the coronary sinus catheter from proximal to distal. Vertical lines depict delta wave onset.
      Figure thumbnail gr1eg
      Figure 1Electrophysiological study. A: Twelve-lead electrocardiogram (ECG) prior to ablation. B: Orthodromic atrioventricular reentrant tachycardia with right bundle branch block QRS morphology and QRS complex duration of 120 ms. C: Local electrograms at successful ablation site of the right posteroseptal accessory pathway from the cryo-catheter (FRZ dist and uni) with ventricular signal coinciding with delta wave onset. The strictly negative ventricular signal from the ablation catheter tip (FRZ uni) is in favor of correct placement at the ventricular insertion site of the posteroseptal pathway. D: Local electrograms after posteroseptal pathway ablation (FRZ dist) with clear division of the atrial and ventricular electrogram at the ablation site. E: Twelve-lead ECG showing different QRS morphology after posteroseptal pathway ablation with a delta wave evident especially in leads I and V4. F: Local electrograms at the site of the second unsuccessfully ablated right posterior pathway (FRZ dist) with ventricular signal clearly preceding delta wave onset. G: Three-dimensional electroanatomical map of the earliest ventricular activation on the tricuspid annulus (delineated by the black line) during sinus rhythm. First manifest pathway could be successfully ablated in the right posteroseptal region (green arrow). The second manifest pathway could be mapped to the posterior tricuspid annulus (blue arrow) and could not be ablated. The distance between the 2 mapping sites was estimated to be 10 mm (white line) and is in favor of 2 distinct pathways rather than 1 broad pathway, although this cannot be definitely proven. FRZ dist indicates distal bipolar signal from the ablation catheter; FRZ uni, unipolar signal from the ablation catheter tip; CS, signals from the coronary sinus catheter from proximal to distal. Vertical lines depict delta wave onset.
      On the subsequent day, surgical cryoablation of the remaining accessory pathway followed by the cone repair of tricuspid valve was performed. No further intraoperative mapping was performed. A cryolesion at the anatomical tricuspid annulus extending from the lower rim of the coronary sinus ostium to the inferolateral annulus was applied to cover both mapped pathways. Bidirectional block was proven by atrial and ventricular pacing using the Medtronic CareLink 2090 Programmer/Analyzer connected to the temporary atrial and ventricular pacing wires placed on the right atrium and ventricle. Antegrade and retrograde block was seen during adenosine administration. Transesophageal echocardiography showed favorable tricuspid valve function along with severe RV mechanical dyssynchrony attributed to the right bundle branch block demasked by pathway interruption
      • Iturralde P.
      • Nava S.
      • Sálica G.
      • et al.
      Electrocardiographic characteristics of patients with ebstein’s anomaly before and after ablation of an accessory atrioventricular pathway.
      J Cardiovasc Electrophysiol. 2006; 17: 1332-1336
      ,
      • He B.J.
      • Merriman A.F.
      • Cakulev I.
      • Stambler B.S.
      • Srivastava D.
      • Scheinman M.M.
      Ebstein’s anomaly: review of arrhythmia types and morphogenesis of the anomaly.
      JACC Clin Electrophysiol. 2021; 7: 1198-1206
      (Supplemental Video 2). Acute right heart failure prevented discontinuation of cardiopulmonary bypass. Permanent RV-CRT was implemented by implanting a dual-chamber pacemaker with an epicardial right atrial and RV free wall lead, respectively, programmed to DDD mode with AV delay optimized to achieve complete fusion of the paced and spontaneous ventricular activation and narrow pseudo-normalized QRS complex without signs of right bundle branch block (Figure 2A ).
      • Janoušek J.
      • Kovanda J.
      • Ložek M.
      • et al.
      Cardiac resynchronization therapy for treatment of chronic subpulmonary right ventricular dysfunction in congenital heart disease.
      Circ Arrhythm Electrophysiol. 2019; 12: 7157
      This led to major acute improvement of hemodynamics, enabling smooth discontinuation of cardiopulmonary bypass. Coronary artery injury or spasm potentially associated with cryoablation thus seemed unlikely to be the cause of acute postrepair RV dysfunction and coronary angiography was not performed. The patient was extubated 24 hours after surgery and discharged 7 days later.
      Figure thumbnail gr2ab
      Figure 2A: Twelve-lead electrocardiogram after right ventricular cardiac resynchronization therapy (RV-CRT) showing narrow QRS complex (=70 ms) with absence of right bundle branch block. B: Longitudinal strain imaging of the right ventricle during baseline rhythm with right bundle branch block. Significant RV mechanical dyssynchrony is evident with early septal contraction (green arrow) accompanied by RV free wall pre-stretch (yellow arrow) followed by late free wall contraction (red arrow) and septal rebound stretch (orange arrow). Peak basal RV free wall contraction occurs 57 ms after pulmonary valve closure (PVC; green line), thus not contributing to RV ejection. RV septal-to–free wall mechanical delay is 97 ms. C: During RV-CRT RV dyssynchrony is abolished with synchronous contraction of the septum and RV free wall. Peak basal RV free contraction occurs almost simultaneously with pulmonary valve closure. RV contraction efficiency is restored.
      Figure thumbnail gr2c
      Figure 2A: Twelve-lead electrocardiogram after right ventricular cardiac resynchronization therapy (RV-CRT) showing narrow QRS complex (=70 ms) with absence of right bundle branch block. B: Longitudinal strain imaging of the right ventricle during baseline rhythm with right bundle branch block. Significant RV mechanical dyssynchrony is evident with early septal contraction (green arrow) accompanied by RV free wall pre-stretch (yellow arrow) followed by late free wall contraction (red arrow) and septal rebound stretch (orange arrow). Peak basal RV free wall contraction occurs 57 ms after pulmonary valve closure (PVC; green line), thus not contributing to RV ejection. RV septal-to–free wall mechanical delay is 97 ms. C: During RV-CRT RV dyssynchrony is abolished with synchronous contraction of the septum and RV free wall. Peak basal RV free contraction occurs almost simultaneously with pulmonary valve closure. RV contraction efficiency is restored.
      Postoperative transthoracic echocardiography showed excellent surgical result with synchronous RV contraction and trivial tricuspid valve regurgitation (Supplemental Video 3). Immediate resynchronization effect was tested on the 14th postoperative day by switching RV-CRT on and off, showing narrow QRS complex and lasting acute improvement of multiple hemodynamic parameters, including RV contraction synchrony and efficiency, as reflected by decreased RV systolic stretch fraction
      • Janoušek J.
      • Kovanda J.
      • Ložek M.
      • et al.
      Pulmonary right ventricular resynchronization in congenital heart disease: acute improvement in right ventricular mechanics and contraction efficiency.
      Circ Cardiovasc Imaging. 2017; 10: 6424
      (Table 1, Figure 2B and 2C).
      Table 1Improvement of hemodynamic parameters during acute right ventricular cardiac resynchronization therapy testing
      RV-CRT offRV-CRT on
      RV filling time [ms] rowhead272305
      RV maximum +dP/dt [mm Hg/s] rowhead233449
      PA velocity time integral [cm] rowhead15.116.7
      RV septal-to–free wall mechanical delay [ms] rowhead9713
      Pulm valve closure to peak RV free wall contraction [ms] rowhead5720
      RV systolic stretch fraction rowhead0.370.07
      PA = pulmonary artery; Pulm = pulmonary; RV = right ventricular; RV-CRT = right ventricular cardiac resynchronization therapy.
      During further follow-up of 6 months, the girl was asymptomatic, with New York Heart Association class 1 functional status, no supraventricular tachycardia recurrences, and good RV function on continuing RV-CRT.

      Conclusion

      Selected patients with Ebstein’s anomaly of the tricuspid valve will benefit from a complex therapy approach including treatment of the arrhythmogenic substrates and surgical tricuspid valve plasty. Owing to difficulties with pathway mapping and ablation typical for the Ebstein’s anomaly and high recurrence rate,
      • Marcondes L.
      • Sanders S.P.
      • del Nido P.J.
      • Walsh E.P.
      Examination of pathologic features of the right atrioventricular groove in hearts with Ebstein anomaly and correlation with arrhythmias.
      Heart Rhythm. 2020; 17: 1092-1098
      , 
      • Guo X.G.
      • Liu X.
      • Zhou G.B.
      • Ma J.
      • Ouyang F.
      • Zhang S.
      Frequency of fractionated ventricular activation and atrial/ventricular electrogram amplitude ratio at successful ablation target of accessory pathways in patients with Ebstein’s anomaly.
      J Cardiovasc Electrophysiol. 2015; 26: 404-411
      , 
      • Ergul Y.
      • Koca S.
      • Akdeniz C.
      • Tuzcu V.
      Electroanatomic mapping-guided catheter ablation of supraventricular tachycardia in children with Ebstein’s anomaly.
      Pediatr Cardiol. 2018; 39: 1445-1452
      , 
      • El-Assaad I.
      • DeWitt E.S.
      • Mah D.Y.
      • et al.
      Accessory pathway ablation in Ebstein anomaly: a challenging substrate.
      Heart Rhythm. 2021; 18: 1844-1851
      surgical cryodestruction associated with tricuspid valve repair may be an appealing option if catheter ablation fails. Ablation of manifest right-sided accessory pathway may demask right bundle branch block with subsequent RV electromechanical dyssynchrony amenable to RV-CRT—a so far only anecdotally reported treatment option in Ebstein’s anomaly.
      • Janoušek J.
      • Kovanda J.
      • Ložek M.
      • et al.
      Cardiac resynchronization therapy for treatment of chronic subpulmonary right ventricular dysfunction in congenital heart disease.
      Circ Arrhythm Electrophysiol. 2019; 12: 7157
      A complex electrophysiological and surgical therapy approach enables to address all major facets of the disease.

      AppendixSupplementary data

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        Supplemental material

        Echocardiographic images.

        Supplemental Video 1. Before catheter ablation and surgery. Apical 4-chamber view showing severe Ebstein malformation of the tricuspid valve. Right ventricle is on the left and left ventricle on the right of the picture.

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        Supplemental Video 2

        Intraoperative transesophageal echocardiography after cone repair and right posterior accessory pathway ablation showing RV mechanical discoordination. Right ventricle is on the bottom right side, right atrium on the top left side.

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        Supplemental Video 3

        Apical 4-chamber view 12 days after cone repair and right ventricular cardiac resynchronization therapy displaying the repaired tricuspid valve and synchronous right ventricular contraction. Right ventricle is on the left and left ventricle on the right of the picture.

      References

        • Lupo P.J.
        • Langlois P.H.
        • Mitchell L.E.
        Epidemiology of Ebstein anomaly: prevalence and patterns in Texas, 1999-2005.
        Am J Med Genet Part A. 2011; 155: 1007-1014
        View in Article
        • Holst K.A.
        • Dearani J.A.
        • Said S.
        • et al.
        Improving results of surgery for Ebstein anomaly: where are we after 235 cone repairs?.
        Ann Thorac Surg. 2018; 105: 160-168
        View in Article
        • Hebe J.
        Ebstein’s anomaly in adults. Arrhythmias: diagnosis and therapeutic approach.
        Thorac Cardiovasc Surg. 2000; 48: 214-219
        View in Article
        • Watson H.
        Natural history of Ebstein’s anomaly of tricuspid valve in childhood and adolescence. An international cooperative study of 505 cases.
        Br Heart J. 1974; 36: 417-427
        View in Article
        • Kastor J.A.
        • Goldreyer B.N.
        • Josephson M.E.
        • et al.
        Electrophysiologic characteristics of Ebstein’s anomaly of the tricuspid valve.
        Circulation. 1975; 52: 987-995
        View in Article
        • Bertram H.
        • Bökenkamp R.
        • Peuster M.
        • Hausdorf G.
        • Paul T.
        Coronary artery stenosis after radiofrequency catheter ablation of accessory atrioventricular pathways in children with Ebstein’s malformation.
        Circulation. 2001; 103: 538-543
        View in Article
        • Iturralde P.
        • Nava S.
        • Sálica G.
        • et al.
        Electrocardiographic characteristics of patients with ebstein’s anomaly before and after ablation of an accessory atrioventricular pathway.
        J Cardiovasc Electrophysiol. 2006; 17: 1332-1336
        View in Article
        • He B.J.
        • Merriman A.F.
        • Cakulev I.
        • Stambler B.S.
        • Srivastava D.
        • Scheinman M.M.
        Ebstein’s anomaly: review of arrhythmia types and morphogenesis of the anomaly.
        JACC Clin Electrophysiol. 2021; 7: 1198-1206
        View in Article
        • Janoušek J.
        • Kovanda J.
        • Ložek M.
        • et al.
        Cardiac resynchronization therapy for treatment of chronic subpulmonary right ventricular dysfunction in congenital heart disease.
        Circ Arrhythm Electrophysiol. 2019; 12: 7157
        View in Article
        • Janoušek J.
        • Kovanda J.
        • Ložek M.
        • et al.
        Pulmonary right ventricular resynchronization in congenital heart disease: acute improvement in right ventricular mechanics and contraction efficiency.
        Circ Cardiovasc Imaging. 2017; 10: 6424
        View in Article
        • Marcondes L.
        • Sanders S.P.
        • del Nido P.J.
        • Walsh E.P.
        Examination of pathologic features of the right atrioventricular groove in hearts with Ebstein anomaly and correlation with arrhythmias.
        Heart Rhythm. 2020; 17: 1092-1098
        View in Article
        • Guo X.G.
        • Liu X.
        • Zhou G.B.
        • Ma J.
        • Ouyang F.
        • Zhang S.
        Frequency of fractionated ventricular activation and atrial/ventricular electrogram amplitude ratio at successful ablation target of accessory pathways in patients with Ebstein’s anomaly.
        J Cardiovasc Electrophysiol. 2015; 26: 404-411
        View in Article
        • Ergul Y.
        • Koca S.
        • Akdeniz C.
        • Tuzcu V.
        Electroanatomic mapping-guided catheter ablation of supraventricular tachycardia in children with Ebstein’s anomaly.
        Pediatr Cardiol. 2018; 39: 1445-1452
        View in Article
        • El-Assaad I.
        • DeWitt E.S.
        • Mah D.Y.
        • et al.
        Accessory pathway ablation in Ebstein anomaly: a challenging substrate.
        Heart Rhythm. 2021; 18: 1844-1851
        View in Article

      Article Info

      Publication History

      Published online: September 30, 2022

      Publication stage

      In Press Journal Pre-Proof

      Footnotes

      Funding Sources: Václav Chaloupecký Jr, MD; Roman Gebauer, MD; Jan Kovanda, MD; Karel Koubský, MD, PhD; and Jan Janoušek, MD, PhD were supported by the Ministry of Health, Czech Republic – conceptual development of research organization, Motol University Hospital, Prague, Czech Republic 00064203. Disclosures: The authors have no conflicts to disclose.

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      DOI: https://doi.org/10.1016/j.hrcr.2022.09.014

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