Distal His bundle pacing in a patient with surgically corrected complex Ebstein anomaly and symptomatic second-degree atrioventricular block: a case report

Ivelin Koev; G Andre Ng; Aidan P Bolger; Mokhtar Ibrahim

doi:10.1093/ehjcr/ytad531

Distal His bundle pacing in a patient with surgically corrected complex Ebstein anomaly and symptomatic second-degree atrioventricular block: a case report

Eur Heart J Case Rep. 2023 Oct 31;7(12):ytad531. doi: 10.1093/ehjcr/ytad531. eCollection 2023 Dec.

Authors

Ivelin Koev^{1

2}, G Andre Ng^{1

2

3}, Aidan P Bolger^{1

3

4}, Mokhtar Ibrahim²

Affiliations

¹ Department of Cardiovascular Sciences, University of Leicester, University Rd, Leicester LE1 7RH, UK.
² Department of Cardiology, Glenfield Hospital, University Hospitals of Leicester NHS Trust, Groby Rd, Leicester LE3 9QP, UK.
³ National Institute for Health Research Leicester Biomedical Research Centre, Leicester, UK.
⁴ East Midlands Congenital Heart Centre, Glenfield Hospital, University Hospitals of Leicester NHS Trust, Leicester, UK.

Abstract

Background: Ebstein's anomaly occurs when there is an apical displacement of the tricuspid valve with septal and posterior valve leaflets tethering. This condition often occurs in association with other congenital, structural, or conduction system diseases, including intracardiac shunts, valvular lesions, arrhythmias, accessory conduction pathways, and first-degree atrioventricular (AV) block. We present for the first time a case of a patient with Ebstein's anomaly who presented with second-degree Mobitz II AV block and was successfully treated with conduction system pacing (CSP) due to her young age and the likelihood of a long-term high percentage of pacing.

Case summary: We present a case of a 42-year-old lady with a background of complex congenital heart disease, including severe pulmonary stenosis, Ebstein anomaly, and atrial septal defect (ASD). She required complex surgical intervention, including tricuspid valve (TV) repair and subsequently replacement, ASD closure, and pulmonary balloon valvuloplasty. She presented to our hospital with symptomatic second-degree Mobitz II AV block (dizziness, shortness of breath, and exercise intolerance) and right bundle branch block (RBBB) on her baseline ECG. Her echocardiogram showed dilated right ventricle (RV) and left ventricle (LV) with low normal LV systolic function. Due to her young age and the likelihood of a long-term high percentage of RV pacing, we opted for CSP after a detailed discussion and patient consent. The distal HIS position is the preferred pacing strategy at our centre. We could not cross the TV with the standard Medtronic C315 HIS catheter, so we had to use the deflectable C304 HIS catheter. Mapping and pacing of the distal HIS bundle were achieved by Medtronic Selectsecure 3830, 69 cm lead. HIS bundle pacing led to the correction of both second-degree Mobitz II AV block and pre-existing RBBB. The implantation was uneventful, and the patient was discharged home the next day without any acute complications.

Discussion: Distal HIS pacing is feasible in patients with surgically treated complex Ebstein anomaly and heart block. This approach can normalize the QRS complex with a high probability of preserving or improving LV function.

Keywords: 2nd-degree Mobitz II AV block; Case report; Conduction system pacing; Ebstein’s anomaly; His pacing; Right bundle branch block.

Publication types

Case Reports