A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly

<b>Background</b>: Ebstein anomaly (EA) is a rare congenital heart disease characterized by the apical displacement of the tricuspid leaflets, creating an enlarged functional right atrium. Supraventricular arrhythmias (SVA) are common, and catheter ablation remains challenging. SVA is co...

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Main Authors: Sonia Alicja Nartowicz, Aleksandra Ciepłucha, Michał Waśniewski, Izabela Miechowicz, Justyna Rajewska-Tabor, Agnieszka Bartczak-Rutkowska, Maciej Lesiak, Małgorzata Pyda, Olga Trojnarska
Format: Article
Language:English
Published: MDPI AG 2024-04-01
Series:Journal of Clinical Medicine
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Online Access:https://www.mdpi.com/2077-0383/13/7/2039
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author Sonia Alicja Nartowicz
Aleksandra Ciepłucha
Michał Waśniewski
Izabela Miechowicz
Justyna Rajewska-Tabor
Agnieszka Bartczak-Rutkowska
Maciej Lesiak
Małgorzata Pyda
Olga Trojnarska
author_facet Sonia Alicja Nartowicz
Aleksandra Ciepłucha
Michał Waśniewski
Izabela Miechowicz
Justyna Rajewska-Tabor
Agnieszka Bartczak-Rutkowska
Maciej Lesiak
Małgorzata Pyda
Olga Trojnarska
author_sort Sonia Alicja Nartowicz
collection DOAJ
description <b>Background</b>: Ebstein anomaly (EA) is a rare congenital heart disease characterized by the apical displacement of the tricuspid leaflets, creating an enlarged functional right atrium. Supraventricular arrhythmias (SVA) are common, and catheter ablation remains challenging. SVA is considered a risk factor for sudden cardiac death in this population. Still, there are very few real-life data on the impact of SVA treated invasively or conservatively on a patient’s prognosis. We aimed to analyze the incidence of SVA in adults with EA, evaluate the effectiveness of catheter ablation, and analyze the impact of SVA and catheter ablation on survival in this population. <b>Methods and results</b>: 71 pts (median age 53 years; range 24–84 years) with EA were evaluated retrospectively from 1988 to 2020. Forty patients (56.3%) had SVA, and eighteen of them (45.0%) required at least one catheter ablation (35 procedures in total). Indications for ablation were mostly intra-atrial reentrant tachycardia (IART) and atrioventricular reentrant tachycardia (AVRT) (14 pts [77.8% and 9 pts [50.0%], respectively. IART and AVRT coexisted in nine pts. One patient suffered from persistent atrial fibrillation. Procedural effectiveness was reported in 28 (80%) cases; over a longer follow-up (mean 12.6 ± 5.4 years), only eight (44.4%) patients were completely free from SVA after the first ablation. In total, 10 patients (14%) died due to cardiovascular events. There was no difference in survival between patients with or without SVA (<i>p</i> = 0.9) and between ablated and non-ablated EA individuals (<i>p</i> = 0.89). <b>Conclusions</b>: Supraventricular arrhythmia is frequent in adults with Ebstein anomaly. Patients often require more than one catheter ablation but eventually become free from arrhythmias. The imaging parameters assessed by echocardiography or cardiac magnetic resonance do not seem to be associated with ablation outcomes. The impact of supraventricular arrhythmia itself or treatment with radiofrequency ablation is questionable and should be thoroughly investigated in this population.
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spelling doaj.art-63ef322a93964f74ba5271748f5848fb2024-04-12T13:21:22ZengMDPI AGJournal of Clinical Medicine2077-03832024-04-01137203910.3390/jcm13072039A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein AnomalySonia Alicja Nartowicz0Aleksandra Ciepłucha1Michał Waśniewski2Izabela Miechowicz3Justyna Rajewska-Tabor4Agnieszka Bartczak-Rutkowska5Maciej Lesiak6Małgorzata Pyda7Olga Trojnarska81st Department of Cardiology, Poznan University of Medical Sciences, 60-806 Poznań, Poland1st Department of Cardiology, Poznan University of Medical Sciences, 60-806 Poznań, Poland1st Department of Cardiology, Poznan University of Medical Sciences, 60-806 Poznań, Poland1st Department of Cardiology, Poznan University of Medical Sciences, 60-806 Poznań, Poland1st Department of Cardiology, Poznan University of Medical Sciences, 60-806 Poznań, Poland1st Department of Cardiology, Poznan University of Medical Sciences, 60-806 Poznań, Poland1st Department of Cardiology, Poznan University of Medical Sciences, 60-806 Poznań, Poland1st Department of Cardiology, Poznan University of Medical Sciences, 60-806 Poznań, Poland1st Department of Cardiology, Poznan University of Medical Sciences, 60-806 Poznań, Poland<b>Background</b>: Ebstein anomaly (EA) is a rare congenital heart disease characterized by the apical displacement of the tricuspid leaflets, creating an enlarged functional right atrium. Supraventricular arrhythmias (SVA) are common, and catheter ablation remains challenging. SVA is considered a risk factor for sudden cardiac death in this population. Still, there are very few real-life data on the impact of SVA treated invasively or conservatively on a patient’s prognosis. We aimed to analyze the incidence of SVA in adults with EA, evaluate the effectiveness of catheter ablation, and analyze the impact of SVA and catheter ablation on survival in this population. <b>Methods and results</b>: 71 pts (median age 53 years; range 24–84 years) with EA were evaluated retrospectively from 1988 to 2020. Forty patients (56.3%) had SVA, and eighteen of them (45.0%) required at least one catheter ablation (35 procedures in total). Indications for ablation were mostly intra-atrial reentrant tachycardia (IART) and atrioventricular reentrant tachycardia (AVRT) (14 pts [77.8% and 9 pts [50.0%], respectively. IART and AVRT coexisted in nine pts. One patient suffered from persistent atrial fibrillation. Procedural effectiveness was reported in 28 (80%) cases; over a longer follow-up (mean 12.6 ± 5.4 years), only eight (44.4%) patients were completely free from SVA after the first ablation. In total, 10 patients (14%) died due to cardiovascular events. There was no difference in survival between patients with or without SVA (<i>p</i> = 0.9) and between ablated and non-ablated EA individuals (<i>p</i> = 0.89). <b>Conclusions</b>: Supraventricular arrhythmia is frequent in adults with Ebstein anomaly. Patients often require more than one catheter ablation but eventually become free from arrhythmias. The imaging parameters assessed by echocardiography or cardiac magnetic resonance do not seem to be associated with ablation outcomes. The impact of supraventricular arrhythmia itself or treatment with radiofrequency ablation is questionable and should be thoroughly investigated in this population.https://www.mdpi.com/2077-0383/13/7/2039Ebstein anomalycatheter ablationsupraventricular arrhythmiasadult congenital heart defectoutcome
spellingShingle Sonia Alicja Nartowicz
Aleksandra Ciepłucha
Michał Waśniewski
Izabela Miechowicz
Justyna Rajewska-Tabor
Agnieszka Bartczak-Rutkowska
Maciej Lesiak
Małgorzata Pyda
Olga Trojnarska
A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly
Journal of Clinical Medicine
Ebstein anomaly
catheter ablation
supraventricular arrhythmias
adult congenital heart defect
outcome
title A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly
title_full A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly
title_fullStr A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly
title_full_unstemmed A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly
title_short A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly
title_sort twenty year follow up of adults with ebstein anomaly with special focus on supraventricular arrhythmias supraventricular arrhythmias and effectiveness of catheter ablation in 20 year follow up of adults with ebstein anomaly
topic Ebstein anomaly
catheter ablation
supraventricular arrhythmias
adult congenital heart defect
outcome
url https://www.mdpi.com/2077-0383/13/7/2039
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