Management of arrhythmogenic right ventricular cardiomyopathy
© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ..
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease characterised by fibrofatty replacement of the ventricular myocardium due to specific mutations, leading to ventricular arrhythmias and sudden cardiac death. Treating this condition can be challenging due to progressive fibrosis, phenotypic variations and small patient cohorts limiting the feasibility of conducting meaningful clinical trials. Although widely used, the evidence base for anti-arrhythmic drugs is limited. Beta-blockers are theoretically sound, yet their efficacy in reducing arrhythmic risk is not robust. Additionally, the impact of sotalol and amiodarone is inconsistent with studies reporting contradictory results. Emerging evidence suggests that combining flecainide and bisoprolol may be efficacious.Radiofrequency ablation has shown some potential in disrupting ventricular tachycardia circuits, with combined endo and epicardial ablation yielding better results which could be considered at the index procedure. In addition, stereotactic radiotherapy may be a future option that can decrease arrhythmias beyond simple scar formation by altering levels of Nav1.5 channels, Connexin 43 and Wnt signalling, potentially modifying myocardial fibrosis.Future therapies, such as adenoviruses and GSk3b modulation, are still in early-stage research. While implantable cardioverter-defibrillator implantation is a key intervention for reducing arrhythmic death, the risks of inappropriate shocks and device complications must be carefully considered.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2024 |
|---|---|
| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:110 |
|---|---|
| Enthalten in: |
Heart (British Cardiac Society) - 110(2024), 3 vom: 10. Jan., Seite 156-162 |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Al-Aidarous, Sayed [VerfasserIn] |
|---|
| Links: |
|---|
| Themen: |
A6D97U294I |
|---|
| Anmerkungen: |
Date Completed 12.01.2024 Date Revised 12.01.2024 published: Electronic Citation Status MEDLINE |
|---|
| doi: |
10.1136/heartjnl-2023-322612 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM359345123 |
|---|
| LEADER | 01000caa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM359345123 | ||
| 003 | DE-627 | ||
| 005 | 20240114234210.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 231226s2024 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1136/heartjnl-2023-322612 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1257.xml |
| 035 | |a (DE-627)NLM359345123 | ||
| 035 | |a (NLM)37433658 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Al-Aidarous, Sayed |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Management of arrhythmogenic right ventricular cardiomyopathy |
| 264 | 1 | |c 2024 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 12.01.2024 | ||
| 500 | |a Date Revised 12.01.2024 | ||
| 500 | |a published: Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ. | ||
| 520 | |a Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease characterised by fibrofatty replacement of the ventricular myocardium due to specific mutations, leading to ventricular arrhythmias and sudden cardiac death. Treating this condition can be challenging due to progressive fibrosis, phenotypic variations and small patient cohorts limiting the feasibility of conducting meaningful clinical trials. Although widely used, the evidence base for anti-arrhythmic drugs is limited. Beta-blockers are theoretically sound, yet their efficacy in reducing arrhythmic risk is not robust. Additionally, the impact of sotalol and amiodarone is inconsistent with studies reporting contradictory results. Emerging evidence suggests that combining flecainide and bisoprolol may be efficacious.Radiofrequency ablation has shown some potential in disrupting ventricular tachycardia circuits, with combined endo and epicardial ablation yielding better results which could be considered at the index procedure. In addition, stereotactic radiotherapy may be a future option that can decrease arrhythmias beyond simple scar formation by altering levels of Nav1.5 channels, Connexin 43 and Wnt signalling, potentially modifying myocardial fibrosis.Future therapies, such as adenoviruses and GSk3b modulation, are still in early-stage research. While implantable cardioverter-defibrillator implantation is a key intervention for reducing arrhythmic death, the risks of inappropriate shocks and device complications must be carefully considered | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Review | |
| 650 | 4 | |a Arrhythmias, Cardiac | |
| 650 | 4 | |a Arrhythmogenic Right Ventricular Dysplasia | |
| 650 | 4 | |a Catheter Ablation | |
| 650 | 4 | |a Defibrillators, Implantable | |
| 650 | 4 | |a Tachycardia, Ventricular | |
| 650 | 7 | |a Anti-Arrhythmia Agents |2 NLM | |
| 650 | 7 | |a Sotalol |2 NLM | |
| 650 | 7 | |a A6D97U294I |2 NLM | |
| 700 | 1 | |a Protonotarios, Alexandros |e verfasserin |4 aut | |
| 700 | 1 | |a Elliott, Perry M |e verfasserin |4 aut | |
| 700 | 1 | |a Lambiase, Pier D |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Heart (British Cardiac Society) |d 1996 |g 110(2024), 3 vom: 10. Jan., Seite 156-162 |w (DE-627)NLM085989274 |x 1468-201X |7 nnns |
| 773 | 1 | 8 | |g volume:110 |g year:2024 |g number:3 |g day:10 |g month:01 |g pages:156-162 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1136/heartjnl-2023-322612 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 110 |j 2024 |e 3 |b 10 |c 01 |h 156-162 | ||