李广平

• 1:天津医科大学第二医院心脏科天津市心血管病离子与分子机能重点实验室天津心脏病学研究所天津医科大学第二医院心血管病中心

摘要(Abstract)：

心脏淀粉样变（CA）是淀粉样蛋白在心肌及心脏传导系统沉积所致的浸润性心肌病，主要包括转甲状腺素蛋白型CA和免疫球蛋白轻链型CA。CA除可导致心功能受损和心力衰竭外，还常伴有窦房结功能障碍、房室传导阻滞、心房颤动和室性心律失常。由于不同CA亚型的传导系统受累、室性心律失常风险及死亡机制存在差异，其心脏植入式电子装置治疗也具有一定特殊性。现有研究提示，不同心脏植入式电子装置在CA患者中的适用人群和潜在获益有所不同，临床应用时需结合CA分型、传导系统受累程度、心功能及预期生存期进行个体化评估。随着转甲状腺素蛋白型CA疾病修饰治疗的发展，CA患者的自然病程正在发生改变，心脏植入式电子装置的最佳介入时机和适宜人群仍有待进一步研究。本文重点介绍CA相关传导系统疾病和室性心律失常的电生理特点，以及永久心脏起搏器、心脏再同步化治疗和植入式心律转复除颤器的临床应用。

关键词(KeyWords)： 心脏淀粉样变;心律失常;心脏植入式电子装置;临床处理

Abstract:

Keywords:

基金项目(Foundation): 国家自然科学基金项目（82270336）;; 天津市临床医学重点学科（专科）建设项目支持（TJYXZDXK-3-006B）;; 天津市卫生健康科技项目（ZC20125）

作者(Author): 李广平

参考文献(References)：

• [1]Giancaterino S,Urey MA,Darden D,et al.Management of arrhythmias in cardiac amyloidosis[J].JACC Clin Electrophysiol,2020,6(4):351-361.DOI:10.1016/j.jacep.2020.01.004.
• [2]John RM,Stern DL.Use of implantable electronic devices in patients with cardiac amyloidosis[J].Can J Cardiol,2020,36(3):408-415.DOI:10.1016/j.cjca.2019.12.002.
• [3]Cheung CC,Roston TM,Andrade JG,et al.Arrhythmias in cardiac amyloidosis:challenges in risk stratification and treatment[J].Can J Cardiol,2020,36(3):416-423.DOI:10.1016/j.cjca.2019.11.039.
• [4]Knight DS,Zumbo G,Barcella W,et al.Cardiac structural and functional consequences of amyloid deposition by cardiac magnetic resonance and echocardiography and their prognostic roles[J].JACC Cardiovasc Imaging,2019,12(5):823-833.DOI:10.1016/j.jcmg.2018.02.016.
• [5]Fontana M,??orovi??A,Scully P,et al.Myocardial amyloidosis:the exemplar interstitial disease[J].JACC Cardiovasc Imaging,2019,12(11 Pt 2):2345-2356.DOI:10.1016/j.jcmg.2019.06.023.
• [6]Minamisawa M,Claggett B,Adams D,et al.Association of patisiran,an RNA interference therapeutic,with regional left ventricular myocardial strain in hereditary transthyretin amyloidosis:the APOLLO study[J].JAMA Cardiol,2019,4(5):466-472.DOI:10.1001/jamacardio.2019.0849.
• [7]Gillmore JD,Judge DP,Cappelli F,et al.Efficacy and safety of acoramidis in transthyretin amyloid cardiomyopathy[J].N Engl JMed,2024,390(2):132-142.DOI:10.1056/NEJMoa2305434.
• [8]Fontana M,Berk JL,Gillmore JD,et al.Vutrisiran in patients with transthyretin amyloidosis with cardiomyopathy[J].N Engl JMed,2025,392(1):33-44.DOI:10.1056/NEJMoa2409134.
• [9]Bukhari S,Khan SZ,Ghoweba M,et al.Arrhythmias and device therapies in cardiac amyloidosis[J].J Clin Med,2024,13(5):1300.DOI:10.3390/jcm13051300.
• [10]Palladini G,Malamani G,CòF,et al.Holter monitoring in AL amyloidosis:prognostic implications[J].Pacing Clin Electrophysiol,2001,24(8 Pt 1):1228-1233.DOI:10.1046/j.1460-9592.2001.01228.x.
• [11]杨兴霞,黄霞霞,刘婷,等.植入式心电监测仪在心血管病诊疗中的研究进展[J].中国介入心脏病学杂志,2025,33(6):353-357.DOI:10.3969/j.issn.1004-8812.2025.06.009.
• [12]Thakkar S,Patel HP,Chowdhury M,et al.Impact of arrhythmias on hospitalizations in patients with cardiac amyloidosis[J].Am JCardiol,2021,143:125-130.DOI:10.1016/j.amjcard.2020.12.024.
• [13]Ridolfi RL,Bulkley BH,Hutchins GM.The conduction system in cardiac amyloidosis.Clinical and pathologic features of 23 patients[J].Am J Med,1977,62(5):677-686.DOI:10.1016/0002-9343(77)90870-1.
• [14]Boldrini M,Salinaro F,Mussinelli R,et al.Prevalence and prognostic value of conduction disturbances at the time of diagnosis of cardiac AL amyloidosis[J].Ann Noninvasive Electrocardiol,2013,18(4):327-335.DOI:10.1111/anec.12032.
• [15]Orini M,Graham AJ,Martinez-Naharro A,et al.Noninvasive mapping of the electrophysiological substrate in cardiac amyloidosis and its relationship to structural abnormalities[J].J Am Heart Assoc,2019,8(18):e012097.DOI:10.1161/JAHA.119.012097.
• [16]Alreshq R,Tugal D,Siddiqi O,et al.Conduction abnormalities and role of cardiac pacing in cardiac amyloidosis:a systematic review[J].Pacing Clin Electrophysiol,2021,44(12):2092-2099.DOI:10.1111/pace.14375.
• [17]Reisinger J,Dubrey SW,Lavalley M,et al.Electrophysiologic abnormalities in AL(primary)amyloidosis with cardiac involvement[J].J Am Coll Cardiol,1997,30(4):1046-1051.DOI:10.1016/s0735-1097(97)00267-2.
• [18]Barbhaiya CR,Kumar S,Baldinger SH,et al.Electrophysiologic assessment of conduction abnormalities and atrial arrhythmias associated with amyloid cardiomyopathy[J].Heart Rhythm,2016,13(2):383-390.DOI:10.1016/j.hrthm.2015.09.016.
• [19]Givens RC,Russo C,Green P,et al.Comparison of cardiacamyloidosis due to wild-type and V122l transthyretin in older adultsreferred to an academic medical center[J].Aging Health,2013,9(2):229-235.DOI:10.2217/ahe.13.10.
• [20]Algalarrondo V,Dinanian S,Juin C,et al.Prophylactic pacemaker implantation in familial amyloid polyneuropathy[J].Heart Rhythm,2012,9(7):1069-1075.DOI:10.1016/j.hrthm.2012.02.033.
• [21]Sayed RH,Rogers D,Khan F,et al.A study of implanted cardiac rhythm recorders in advanced cardiac AL amyloidosis[J].Eur Heart J,2015,36(18):1098-1105.DOI:10.1093/eurheartj/ehu506.
• [22]Eriksson P,Karp K,Bjerle P,et al.Disturbances of cardiac rhythm and conduction in familial amyloidosis with polyneuropathy[J].Br Heart J,1984,51(6):658-662.DOI:10.1136/hrt.51.6.658.
• [23]Kusumoto FM,Schoenfeld MH,Barrett C,et al.2018 ACC/AHA/HRS guideline on the evaluation and management of patients with bradycardia and cardiac conduction delay:executive summary:a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines,and the Heart Rhythm Society[J].J Am Coll Cardiol,2019,74(7):932-987.DOI:10.1016/j.jacc.2018.10.043.
• [24]López-Sainz??,de Haro-Del Moral FJ,Dominguez F,et al.Prevalence of cardiac amyloidosis among elderly patients with systolic heart failure or conduction disorders[J].Amyloid,2019,26(3):156-163.DOI:10.1080/13506129.2019.1625322.
• [25]Donnellan E,Wazni OM,Saliba WI,et al.Prevalence,incidence,and impact on mortality of conduction system disease in transthyretin cardiac amyloidosis[J].Am J Cardiol,2020,128:140-146.DOI:10.1016/j.amjcard.2020.05.021.
• [26]晋宇阳,王建华,徐健,等.心脏植入式电子装置电极导线与三尖瓣反流的相关性研究[J].中国介入心脏病学杂志,2025,33(3):141-149.DOI:10.3969/j.issn.1004-8812.2025.03.004.
• [27]Fichtner S,Estner HL,N??bauer M,et al.Percutaneous extraction of a leadless Micra pacemaker after dislocation:a case report[J].Eur Heart J Case Rep,2019,3(3):ytz113.DOI:10.1093/ehjcr/ytz113.
• [28]Matsuzoe H,Tanaka H,Sato S,et al.Severe functional tricuspid regurgitation due to wild-type transthyretin amyloid cardiomyopathy without left ventricular hypertrophy:a case report[J].Eur Heart J Case Rep,2022,6(12):ytac462.DOI:10.1093/ehjcr/ytac462.
• [29]Fischer K,Lellouche N,Damy T,et al.Cardiovascular outcomes after cardiac resynchronization therapy in cardiac amyloidosis[J].ESCHeart Fail,2022,9(1):740-750.DOI:10.1002/ehf2.13663.
• [30]Hartnett J,Jaber W,Maurer M,et al.Electrophysiological manifestations of cardiac amyloidosis:JACC:Cardiooncology state-of-the-art review[J].JACC Cardio Oncol,2021,3(4):506-515.DOI:10.1016/j.jaccao.2021.07.010.
• [31]Aimo A,Nesti M,Vergaro G,et al.Cardiac resynchronization therapy,remodeling,and outcome in patients with amyloid transthyretin cardiomyopathy[J].J Am Heart Assoc,2025,14(22):e043049.DOI:10.1161/JAHA.125.043049.
• [32]Pham-Trung C,Veloza-Urrea D,Segura-Domínguez M,et al.Feasibility and safety of left bundle branch area pacing in cardiac amyloidosis.A single center experience[J].Pacing Clin Electrophysiol,2024,47(1):149-155.DOI:10.1111/pace.14894.
• [33]Sudo K,Uematsu M,Nakamura K,et al.Impact of left bundle branch area pacing-optimized cardiac resynchronized therapy for transthyretin cardiac amyloidosis[J].Eur Heart J Cardiovasc Imaging,2024,25(5):e156.DOI:10.1093/ehjci/jead331.
• [34]Ahmed T,Lodhi SH,Lak H,et al.Left bundle branch pacing in transthyretin cardiac amyloidosis and alternating bundle branch block[J].JACC Case Rep,2020,2(7):1004-1008.DOI:10.1016/j.jaccas.2020.04.040.
• [35]Nath RK,Shrivastava A.A case report-facing blues in cardiac amyloidosis:no more a zebra[J].Eur Heart J Case Rep,2022,6(2):ytac081.DOI:10.1093/ehjcr/ytac081.
• [36]Glikson M,Burri H,Abdin A,et al.European Society of Cardiology(ESC)clinical consensus statement on indications for conduction system pacing,with special contribution of the European Heart Rhythm Association of the ESC and endorsed by the Asia Pacific Heart Rhythm Society,the Canadian Heart Rhythm Society,the Heart Rhythm Society,and the Latin American Heart Rhythm Society[J].Europace,2025,27(4):euaf 050.DOI:10.1093/europace/euaf050.
• [37]Herrera Bethencourt M,Kristen AV,Algalarrondo V,et al.Left bundle branch area pacing in transthyretin cardiac amyloidosis:a narrative review[J].J Clin Med,2026,15(1):305.DOI:10.3390/jcm15010305.
• [38]Dubrey SW,Cha K,Anderson J,et al.The clinical features of immunoglobulin light-chain(AL)amyloidosis with heart involvement[J].QJM,1998,91(2):141-157.DOI:10.1093/qjmed/91.2.141.
• [39]Kristen AV,Dengler TJ,Hegenbart U,et al.Prophylactic implantation of cardioverter-defibrillator in patients with severe cardiac amyloidosis and high risk for sudden cardiac death[J].Heart Rhythm,2008,5(2):235-240.DOI:10.1016/j.hrthm.2007.10.016.
• [40]Zeppenfeld K,Tfelt-Hansen J,de Riva M,et al.2022 ESCGuidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death[J].Eur Heart J,2022,43(40):3997-4126.DOI:10.1093/eurheartj/ehac262.
• [41]Kittleson MM,Maurer MS,Ambardekar AV,et al.Cardiac amyloidosis:evolving diagnosis and management:a scientific statement from the American Heart Association[J].Circulation,2020,142 (1):e7-e22.DOI:10.1161/CIR.0000000000000792.
• [42]Varr BC,Zarafshar S,Coakley T,et al.Implantable cardioverterdefibrillator placement in patients with cardiac amyloidosis[J].Heart Rhythm,2014,11(1):158-162.DOI:10.1016/j.hrthm.2013.10.026.
• [43]Hamon D,Algalarrondo V,Gandjbakhch E,et al.Outcome and inci-dence of appropriate implantable cardioverterdefibrillator therapy inpatients with cardiac amyloidosis[J].Int JCardiol,2016,222:562-568.DOI:10.1016/j.ijcard.2016.07.254.
• [44]Rezk T,Lachmann HJ,Fontana M,et al.Cardiorenal ALamyloidosis:risk stratification and outcomes based upon cardiac and renal biomarkers[J].Br J Haematol,2019,186(3):460-470.DOI:10.1111/bjh.15955.
• [45]Wei HQ,Wang J,Liang Y,et al.Prophylactic implantation of cardioverter‐defibrillator in patients with advanced light-chain amyloidosis-a pilot study[J].J Arrhythm,2025,41(2):e70068.DOI:10.1002/joa3.70068.
• [46]Towbin JA,Mc Kenna WJ,Abrams DJ,et al.2019 HRS expert consensus statement on evaluation,risk stratification,and management of arrhythmogenic cardiomyopathy[J].Heart Rhythm,2019,16(11):e301-e372.DOI:10.1016/j.hrthm.2019.05.007.