Zusammenfassung
Die kardiale Amyloidose ist eine infiltrative Kardiomyopathie, die durch die extrazelluläre Ablagerung von Amyloidfibrillen im Myokard verursacht wird. Obwohl Patienten mit kardialer Amyloidose hauptsächlich Symptome von Herzinsuffizienz zeigen, sind Arrhythmien und Erkrankungen des Reizleitungssystems häufige Krankheitsmanifestationen. Vorhofflimmern (VHF) wird bei bis zu 80 % der Patienten zum Zeitpunkt der Diagnosestellung beobachtet. Typischerweise haben die Patienten aufgrund gleichzeitig auftretender Erkrankungen des Reizleitungssystems eine normale Herzfrequenz. Das thrombembolische Risiko ist bei Patienten mit kardialer Amyloidose besonders hoch, linksatriale Thromben wurden sogar ohne Vorhandensein von VHF beobachtet. AV-Knoten- und infrahisäre Erkrankungen sind hochprävalent, und permanente Schrittmacher werden häufig benötigt. Die Verwendung implantierbarer Defibrillatoren in dieser Population ist umstritten. Diese Übersicht fasst die veröffentlichten Daten und therapeutischen Strategien im Zusammenhang mit Arrhythmien und Erkrankungen des Reizleitungssystems zusammen, mit dem Ziel, den Lesern bei der Entscheidungsfindung in teilweise komplexen klinischen Situationen zu unterstützen.
Abstract
Cardiac amyloidosis is an infiltrative cardiomyopathy characterized by the extracellular deposition of amyloid fibrils within the myocardium. Beyond heart failure, patients with cardiac amyloidosis commonly present with arrhythmias and conduction system disorders. Atrial fibrillation is observed in up to 80% of patients at the time of diagnosis, with patients typically maintaining normal heart rates due to concurrent atrioventricular nodal disease. The thromboembolic risk is particularly high in patients with cardiac amyloidosis, and left atrial thrombi have been observed even in the absence of atrial fibrillation. Conduction system diseases are also highly prevalent, often necessitating permanent pacemaker implantation. The use of implantable defibrillators in this population remains controversial. This overview of published data and therapeutic strategies related to arrhythmias and conduction system disorders aims to assist readers in decision-making in complex clinical scenarios.
Literatur
Ruberg FL, Grogan M, Hanna M, Kelly JW, Maurer MS (2019) Transthyretin amyloid cardiomyopathy: JACC state-of-the-art review. J Am Coll Cardiol 73:2872–2891
González-López E, Gallego-Delgado M, Guzzo-Merello G et al (2015) Wild-type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction. Eur Heart J 36:2585–2594
Treibel TA, Fontana M, Gilbertson JA et al (2016) Occult transthyretin cardiac amyloid in severe calcific aortic stenosis: prevalence and prognosis in patients undergoing surgical aortic valve replacement. Circ Cardiovasc Imaging. https://doi.org/10.1161/circimaging.116.005066
Goette A, Kalman JM, Aguinaga L et al (2016) EHRA/HRS/APHRS/SOLAECE expert consensus on atrial cardiomyopathies: definition, characterization, and clinical implication. Europace 18:1455–1490
Pinney JH, Smith CJ, Taube JB et al (2013) Systemic amyloidosis in England: an epidemiological study. Br J Haematol 161:525–532
Donnellan E, Wazni OM, Hanna M et al (2020) Atrial fibrillation in transthyretin cardiac amyloidosis: predictors, prevalence, and efficacy of rhythm control strategies. JACC Clin Electrophysiol 6:1118–1127
Papathanasiou M, Jakstaite AM, Oubari S et al (2022) Clinical features and predictors of atrial fibrillation in patients with light-chain or transthyretin cardiac amyloidosis. ESC Heart Fail 9:1740–1748
Feng D, Edwards WD, Oh JK et al (2007) Intracardiac thrombosis and embolism in patients with cardiac amyloidosis. Circulation 116:2420–2426
Feng D, Syed IS, Martinez M et al (2009) Intracardiac thrombosis and anticoagulation therapy in cardiac amyloidosis. Circulation 119:2490–2497
Donnellan E, Elshazly MB, Vakamudi S et al (2019) No association between CHADS-VAsc score and left atrial appendage thrombus in patients with transthyretin amyloidosis. JACC Clin Electrophysiol 5:1473–1474
Garcia-Pavia P, Rapezzi C, Adler Y et al (2021) Diagnosis and treatment of cardiac amyloidosis. A position statement of the European society of cardiology working group on myocardial and pericardial diseases. Eur J Heart Fail 23:512–526
Mitrani LR, De Los Santos J, Driggin E et al (2021) Anticoagulation with warfarin compared to novel oral anticoagulants for atrial fibrillation in adults with transthyretin cardiac amyloidosis: comparison of thromboembolic events and major bleeding. Amyloid 28:30–34
Rubinow A, Skinner M, Cohen AS (1981) Digoxin sensitivity in amyloid cardiomyopathy. Circulation 63:1285–1288
El-Am EA, Dispenzieri A, Melduni RM et al (2019) Direct current cardioversion of atrial arrhythmias in adults with cardiac amyloidosis. J Am Coll Cardiol 73:589–597
Loungani RS, Rehorn MR, Geurink KR et al (2020) Outcomes following cardioversion for patients with cardiac amyloidosis and atrial fibrillation or atrial flutter. Am Heart J 222:26–29
Donnellan E, Wazni O, Kanj M et al (2020) Atrial fibrillation ablation in patients with transthyretin cardiac amyloidosis. Europace 22:259–264
Tan NY, Mohsin Y, Hodge DO et al (2016) Catheter ablation for atrial arrhythmias in patients with cardiac amyloidosis. J Cardiovasc Electrophysiol 27:1167–1173
Donnellan E, Wazni OM, Saliba WI et al (2020) Prevalence, incidence, and impact on mortality of conduction system disease in transthyretin cardiac amyloidosis. Am J Cardiol 128:140–146
Rapezzi C, Merlini G, Quarta CC et al (2009) Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation 120:1203–1212
Saturi G, De Frutos F, Sguazzotti M et al (2023) Predictors and outcomes of pacemaker implantation in patients with cardiac amyloidosis. Heart 110:40–48
Rehorn MR, Loungani RS, Black-Maier E et al (2020) Cardiac implantable electronic devices: a window into the evolution of conduction disease in cardiac amyloidosis. JACC Clin Electrophysiol 6:1144–1154
Sayed RH, Rogers D, Khan F et al (2015) A study of implanted cardiac rhythm recorders in advanced cardiac AL amyloidosis. Eur Heart J 36:1098–1105
Palladini G, Malamani G, Cò C et al (2001) Holter monitoring in AL amyloidosis: prognostic implications. Pacing Clinical Electrophis 24:1228–1233
Goldsmith YB, Liu J, Chou J, Hoffman J, Comenzo RL, Steingart RM (2009) Frequencies and types of arrhythmias in patients with systemic light-chain amyloidosis with cardiac involvement undergoing stem cell transplantation on telemetry monitoring. Am J Cardiol 104:990–994
Hörnsten R, Wiklund U, Olofsson B‑O, Jensen SM, Suhr OB (2004) Liver transplantation does not prevent the development of life-threatening arrhythmia in familial amyloidotic polyneuropathy, Portuguese-type (ATTR val30met) patients. Transplantation. https://doi.org/10.1097/01.tp.0000133517.20972.27
Varr BC, Zarafshar S, Coakley T et al (2014) Implantable cardioverter-defibrillator placement in patients with cardiac amyloidosis. Heart Rhythm 11:158–162
Li R, Yang ZG, Wen LY et al (2016) Regional myocardial microvascular dysfunction in cardiac amyloid light-chain amyloidosis: assessment with 3T cardiovascular magnetic resonance. J Cardiovasc Magn Reson 18:16
Alkindi S, Almasoud A, Younes A et al (2015) Increased risk of heart block in patients with cardiac amyloidosis on amiodarone. J Cardiac Fail 21:S125
Zeppenfeld K, Tfelt-Hansen J, de Riva M et al (2022) 2022 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: developed by the task force for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death of the European society of cardiology (ESC) endorsed by the association for European paediatric and congenital cardiology (AEPC). Eur Heart J 43:3997–4126
Brown MT, Yalamanchili S, Evans ST et al (2022) Ventricular arrhythmia burden and implantable cardioverter-defibrillator outcomes in transthyretin cardiac amyloidosis. Pacing Clin Electrophysiol 45:443–451
Donnellan E, Wazni OM, Hanna M, Saliba W, Jaber W, Kanj M (2020) Primary prevention implantable cardioverter-defibrillators in transthyretin cardiac amyloidosis. Pacing Clin Electrophysiol 43:1401–1403
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Interessenkonflikt
M. Papathanasiou, L.S. Schlender, V.L. Johnson und R. Wakili geben an, dass kein Interessenkonflikt besteht.
Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.
Additional information
Hinweis des Verlags
Der Verlag bleibt in Hinblick auf geografische Zuordnungen und Gebietsbezeichnungen in veröffentlichten Karten und Institutsadressen neutral.
QR-Code scannen & Beitrag online lesen
Supplementary Information
Video. Transösophageale Echokardiographie mit Nachweis einer linksatrialen Thrombose im Kontext einer kardialen Leichtketten-Amyloidose ohne Nachweis von Vorhofflimmern.
Rights and permissions
About this article
Cite this article
Papathanasiou, M., Schlender, L.S., Johnson, V.L. et al. Arrhythmien bei Amyloidose. Herzschr Elektrophys (2024). https://doi.org/10.1007/s00399-024-01016-y
Received:
Accepted:
Published:
DOI: https://doi.org/10.1007/s00399-024-01016-y