Highlights
- •Ventricular arrhythmias are more frequently observed in light chain than transthyretin cardiac amyloidosis (CA)
- •Multimodality imaging can potentially identify CA patients at increased risk of ventricular arrhythmias
- •The role of implantable cardioverter-defibrillator in CA is controversial
Summary
Cardiac amyloidosis is an underdiagnosed disease that is caused by myocardial deposition
of misfolded light chain (AL) or transthyretin (ATTR) amyloid fibrils, leading to
restrictive cardiomyopathy and eventually death if untreated. Ventricular arrhythmias
are common in cardiac amyloidosis, and the prevalence is higher in AL than ATTR. There
are multiple suspected pathogenic mechanisms for ventricular arrhythmia including
activation of inflammatory cascade from direct amyloid deposition, and electro-mechanical
as well as autonomic dysfunction due to systemic amyloid deposition. Cardiac amyloidosis
is associated with an increased risk of sudden cardiac death, and the risk is higher
in AL than ATTR. Finally, the role of implantable cardioverter-defibrillators in cardiac
amyloidosis is controversial, and while successful termination of life-threatening
ventricular arrhythmias has been reported in few studies, there has been no evidence
of improvement in outcomes when used for primary prevention in patients with cardiac
amyloidosis.
Graphical abstract
Graphical Abstract
Keywords
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References
- Atrial fibrillation, thromboembolic risk, and anticoagulation in cardiac amyloidosis: a review.J Card Fail. 2023; 29 (PMID: 36122817): 76-86https://doi.org/10.1016/j.cardfail.2022.08.008
- Recent advances in the management of AL amyloidosis.Br J Haematol. 2016; 172 (PMID: 26491974): 170-186https://doi.org/10.1111/bjh.13805
- Amyloidogenic light chains induce cardiomyocyte contractile dysfunction and apoptosis via a non-canonical p38alpha MAPK pathway.Proc Natl Acad Sci U S A. 2010; 107 (PMID: 20150510): 4188-4193https://doi.org/10.1073/pnas.0912263107
- Molecular imaging of cardiac amyloidosis.J Nucl Med. 2020; 61 (PMID: 32482792): 965-970https://doi.org/10.2967/jnumed.120.245381
- Efficient 1-hour technetium-99 m pyrophosphate imaging protocol for the diagnosis of transthyretin cardiac amyloidosis.Circ Cardiovasc Imaging. 2020; 13 (PMID: 32063053)e010249https://doi.org/10.1161/CIRCIMAGING.119.010249
- (99m)Tc-DPD uptake reflects amyloid fibril composition in hereditary transthyretin amyloidosis.Ups J Med Sci. 2016; 121 (PMID: 26849806): 17-24https://doi.org/10.3109/03009734.2015.1122687
- Transthyretin V122I in African Americans with congestive heart failure.J Am Coll Cardiol. 2006; 47 (PMID: 16631014): 1724-1725https://doi.org/10.1016/j.jacc.2006.01.042
- Baseline ECG features and arrhythmic profile in transthyretin versus light chain cardiac amyloidosis.Circ Heart Fail. 2020; 13 (PMID: 20150510)e006619https://doi.org/10.1161/CIRCHEARTFAILURE.119.006619
- Prevalence of atrial fibrillation and thromboembolic risk in wild-type transthyretin amyloid cardiomyopathy.Circulation. 2021; 143 (PMID: 33779268): 1335-1337https://doi.org/10.1161/CIRCULATIONAHA.120.052136
- Risks of ventricular tachyarrhythmia and mortality in patients with amyloidosis - a long-term cohort study.Acta Cardiol Sin. 2022; 38 (PMID: 26849806): 464-474https://doi.org/10.6515/ACS.202207_38(4).20220221A
- The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement.QJM. 1998; 91 (PMID: 36122817): 141-157https://doi.org/10.1093/qjmed/91.2.141
- 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. 2009; 104 (PMID: 19766769): 990-994https://doi.org/10.1016/j.amjcard.2009.05.040
- Liver transplantation does not prevent the development of life-threatening arrhythmia in familial amyloidotic polyneuropathy, Portuguese-type (ATTR Val30Met) patients.Transplantation. 2004; 78 (PMID: 15257048): 112-116https://doi.org/10.1097/01.tp.0000133517.20972.27
- A study of implanted cardiac rhythm recorders in advanced cardiac AL amyloidosis.Eur Heart J. 2015; 36 (PMID: 25549725): 1098-1105https://doi.org/10.1093/eurheartj/ehu506
- Electrocardiographic findings in primary systemic amyloidosis and biopsy-proven cardiac involvement.Am J Cardiol. 2005; 95 (PMID: 16631014): 535-537https://doi.org/10.1016/j.amjcard.2004.10.028
- Prophylactic implantation of cardioverter-defibrillator in patients with severe cardiac amyloidosis and high risk for sudden cardiac death.Heart Rhythm. 2008; 5 (PMID: 18242546): 235-240https://doi.org/10.1016/j.hrthm.2007.10.016
- Implantable cardioverter-defibrillator placement in patients with cardiac amyloidosis.Heart Rhythm. 2014; 11 (PMID: 24121001): 158-162https://doi.org/10.1016/j.hrthm.2013.10.026
- Outcome and incidence of appropriate implantable cardioverter-defibrillator therapy in patients with cardiac amyloidosis.Int J Cardiol. 2016; 222 (PMID: 27513652): 562-568https://doi.org/10.1016/j.ijcard.2016.07.254
- Holter monitoring in AL amyloidosis: prognostic implications.Pacing Clin Electrophysiol. 2001; 24 (PMID: 11523608): 1228-1233https://doi.org/10.1046/j.1460-9592.2001.01228.x
- Maternal stroke: a call for action.Circulation. 2021; 143 (PMID: 11523608): 727-738https://doi.org/10.1161/CIRCULATIONAHA.120.051460
- Late gadolinium enhancement in cardiac amyloidosis: attributable both to interstitial amyloid deposition and subendocardial fibrosis caused by ischemia.Heart Vessels. 2016; 31 (PMID: 25794983): 990-995https://doi.org/10.1007/s00380-015-0658-0
- Regional cardiac sympathetic denervation in patients with ventricular tachycardia in the absence of coronary artery disease.J. Am. Coll. Cardiol. 1993; 22 (PMID: 8227790): 1344-1353https://doi.org/10.1016/0735-1097(93)90541-8
- Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements.J Clin Oncol. 2012; 30 (PMID: 22331953): 989-995https://doi.org/10.1200/JCO.2011.38.5724
- A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis.Blood. 2013; 121 (PMID: 27513652): 3420-3427https://doi.org/10.1182/blood-2012-12-473066
- Development and validation of a survival staging system incorporating BNP in patients with light chain amyloidosis.Blood. 2019; 133 (PMID: 19766769): 215-223https://doi.org/10.1182/blood-2018-06-858951
- Natural history of wild-type transthyretin cardiac amyloidosis and risk stratification using a novel staging system.J Am Coll Cardiol. 2016; 68 (PMID: 27585505): 1014-1020https://doi.org/10.1016/j.jacc.2016.06.033
- A new staging system for cardiac transthyretin amyloidosis.Eur Heart J. 2018; 39 (PMID: 29048471): 2799-2806https://doi.org/10.1093/eurheartj/ehx589
- Prognostic utility of echocardiographic atrial and ventricular strain imaging in patients with cardiac amyloidosis.JACC Cardiovasc Imaging. 2021; 14 (PMID: 33744146): 1508-1519https://doi.org/10.1016/j.jcmg.2021.01.016
- Prognostic significance of strain Doppler imaging in light-chain amyloidosis.JACC Cardiovasc Imaging. 2010; 3 (PMID: 32482792): 333-342https://doi.org/10.1016/j.jcmg.2009.11.013
- State of the art: quantitative cardiac MRI in cardiac amyloidosis.J Magn Reson Imaging. 2022; 56 (PMID: 35770942): 1287-1301https://doi.org/10.1002/jmri.28314
- Prognostic value of late gadolinium enhancement cardiovascular magnetic resonance in cardiac amyloidosis.Circulation. 2015; 132 (PMID: 26362631): 1570-1579https://doi.org/10.1161/CIRCULATIONAHA.115.016567
- Noninvasive mapping of the electrophysiological substrate in cardiac amyloidosis and its relationship to structural abnormalities.J Am Heart Assoc. 2019; 8 ([PMID: 31496332 doi:)e012097
- Signal-averaged electrocardiography in patients with AL (primary) amyloidosis.Am Heart J. 1997; 134 (PMID: 9424057): 994-1001https://doi.org/10.1016/s0002-8703(97)70017-6
- Electrophysiologic abnormalities in AL (primary) amyloidosis with cardiac involvement.J Am Coll Cardiol. 1997; 30 (PMID: 9316537): 1046-1051https://doi.org/10.1016/s0735-1097(97)00267-2
- Electrophysiologic assessment of conduction abnormalities and atrial arrhythmias associated with amyloid cardiomyopathy.Heart Rhythm. 2016; 13 (PMID: 26400855): 383-390https://doi.org/10.1016/j.hrthm.2015.09.016
- Epidemiology, mechanisms, and management of atrial fibrillation in cardiac amyloidosis.Curr Probl Cardiol. 2022; (PMID: 36584731)101571https://doi.org/10.1016/j.cpcardiol.2022.101571
- Catheter ablation of ventricular tachycardia/fibrillation in a patient with right ventricular amyloidosis with initial manifestations mimicking arrhythmogenic right ventricular dysplasia/cardiomyopathy.Korean Circ J. 2017; 2017 (PMID: 28382086): 282-285https://doi.org/10.4070/kcj.2016.0328
- Implantable cardioverter defibrillators in patients with cardiac amyloidosis.J Cardiovasc Electrophysiol. 2013; 24 (PMID: 23489983): 793-798https://doi.org/10.1111/jce.12123
- Survival following implantable cardioverter-defibrillator implantation in patients with amyloid cardiomyopathy.J Am Heart Assoc. 2020; 9 (PMID: 22331953)e016038https://doi.org/10.1161/JAHA.120.016038
- Primary prevention implantable cardioverter-defibrillators in transthyretin cardiac amyloidosis.Pacing Clin Electrophysiol. 2020; 43 (PMID: 32725816): 1401-1403https://doi.org/10.1111/pace.14023
- Ventricular arrhythmia burden and implantable cardioverter-defibrillator outcomes in transthyretin cardiac amyloidosis.Pacing Clin Electrophysiol. 2022; 45 (PMID: 36122817): 443-451https://doi.org/10.1111/pace.14458
- 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: 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: Association for European Paediatric and Congenital Cardiology (AEPC).Eur Heart J. 2015; 36 (PMID: 26320108): 2793-2867https://doi.org/10.1093/eurheartj/ehv316
- 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.Heart Rhythm. 2018; 15 (PMID: 29097319): e73-e189https://doi.org/10.1016/j.hrthm.2017.10.036
Article info
Publication history
Published online: March 15, 2023
Accepted:
February 28,
2023
Received in revised form:
February 27,
2023
Received:
December 16,
2022
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2023 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
