Abstract
Arrhythmogenic cardiomyopathy (ACM) is an inheritable heart muscle disease characterised pathologically by fibrofatty myocardial replacement and clinically by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). Although, in its original description, the disease was believed to predominantly involve the right ventricle, biventricular and left-dominant variants, in which the myocardial lesions affect in parallel or even mostly the left ventricle, are nowadays commonly observed. The clinical management of these patients has two main purposes: the prevention of SCD and the control of arrhythmic and heart failure (HF) events. An implantable cardioverter defibrillator (ICD) is the only proven lifesaving treatment, despite significant morbidity because of device-related complications and inappropriate shocks. Selection of patients who can benefit the most from ICD therapy is one of the most challenging issues in clinical practice. Risk stratification in ACM patients is mostly based on arrhythmic burden and ventricular dysfunction severity, although other clinical features resulting from electrocardiogram and imaging modalities such as cardiac magnetic resonance may have a role. Medical therapy is crucial for treatment of VAs and the prevention of negative ventricular remodelling. In this regard, the efficacy of novel anti-HF molecules and drugs acting on the inflammatory pathway in patients with ACM is, to date, unknown. Catheter ablation represents an effective strategy to treat ventricular tachycardia relapses and recurrent ICD shocks. The present review will address the current strategies for prevention of SCD and treatment of VAs and HF in patients with ACM.
Highlights
Among AADs, amiodarone and sotalol are the most effective drugs with a relatively low proarrhythmic risk. They are recommended in Arrhythmogenic cardiomyopathy (ACM) patients with frequent appropriate implantable cardioverter defibrillator (ICD) discharges to improve symptoms in patients with frequent premature ventricular beats (PVBs) and/or nonsustained ventricular tachycardia (VT) and as an adjunctive therapy when betablockers alone are not sufficient to control the arrhythmic burden in symptomatic patients with frequent PVBs and/or non-sustained VT [42,73]
Arrhythmogenic cardiomyopathy is characterised by progressive scarring of the ventricular myocardium and ventricular dilatation and dysfunction
Risk stratification to guide ICD implantation remains a crucial point in the management of ACM patients for primary prevention of sudden cardiac death (SCD)
Summary
Arrhythmogenic cardiomyopathy (ACM) is a genetically determined heart muscle disease characterised pathologically by fibrofatty replacement of right and left ventricular myocardium and clinically by ventricular arrhythmias (VAs) and arrhythmic sudden cardiac death (SCD) [1]. The fibrofatty tissue is usually considered the hallmark lesion of ACM, it should be more properly regarded as a marker of advanced stages of the disease [2]. 2 of 17 2 of 17 inal description, the disease was characterised by a predominant involvement of the RV (w“Aasrrchhyatrhamctoegriesnedic bryigahtpvreednotrmiciunlaanrtcianrvdoilovmemyoepnattohfyt,hAeRRVVC(“”A), rwrhityhthlemftogvenitcrirciuglhatr vdeen-tterriicourlaatriocnarodciocumryrionpgaltahtye,rAinRVthCe”h),iswtoitrhyloefttvhendtrisiceualsaer. ACM = arrhythmogenic cardiomyopathy; LGE = late gadolinium enhancement; LV = left ventricle. The disease is phenotypically classified in three variants: “right dominant”, characteriseTdhbeydtihseeapsreeidsopmhiennaonttypRiVcailnlyvocllavsesmifieendt,iwn itthhreneovLaVriaanbtns:o“rmrigahlittideso;m“ibnivanent”tr, icchualarar”cwrLomo“wtrLodhmneaVhVpiieyirrtesytnayhirtehiistthhotnonahheiimfypovvisndmynet“nooa,vohvaobtllAeopgvvhmorgydlehCreeylveohvnmtmde,MnehgyeinimAiceesemetc”onhennlCetpleaietmtyneis,Mf,rsncfpttweecotwtcid”uvogpvcioaietorfetehirhfrenhmsndbneexbtnionitnrcpcinooronittuoiorcthlmccahyetrRuayurnsRtyVlruethpslatdoVhanisreiaRoiprceetobclnVaRdaceymaRnbstaVxrthoVinryoupd[ndri7oaosreimveaio]erenpson.onmmsd”adamclsd,lTvtoaiiyhtLotwyhelmoLoiiiVnmoeitVephspisspes;ea;ie.an”tca[astnaTe7s,hc.thnnrsth]howyT,dm.hdyetm,Twhhh,a“Ab“hepeiAssilcrlhtieaLweohbsLhbfosVfaVrtetshtuhnodeeCdCodalraotnedo”mlhsd”oreLm)ep)mebtnVsscercreiopwhhroinhenttmeaoapaaenhcabbrunrrootnan“amperlstlcdtAu”coeer”tctomdrern“oe(smp(rraAronpahitloiosotalfsserysfseuolerbcteoidcthdshtetdhorreiymrbeeucembdettfsoymhyoofae;pnewmgbra“acrrfreoouiborieoppntsvefimsdhgerivrdeeceteemepdhttadndtcohnreooaeiwosietcasrnmermaadeisiortctbsciiiihianao“nueoon“reAnsmatvladsAaheneenooriryedr----tfft”-rdieifsfeorfednitffceornendtitcioonnsditthioant sshtharaet snhoanr-eisncohnem-isicchmemyoiccamrdyoiaclasrcdairarlisncgararnindgthanedprtohpeepnrsoiptyentossictyart-oreslcaatre-dreVlaAtesd[7V].AIsn[t7h].eIpnrtehseenptrerseevnietwre,vthieewt,etrhme AteCrmMAreCfMersretofetrhsetopthheenpohtyepnioctyvpaircivaanrtisanotfsaogfenaegtiecnaelltyicdaleltyerdmetineremdicnaerddicoamrdyioopmaythoypawthhyoswe hhoalslemhaarkllmleasrioknleissitohne fiisbrthofeatftiybrreopflaatcteymreepntla, cwehmicehntc,awnhloicchalcisaeninlotchaelisReVin(AthReVRCV), (LAVR(VACLV),CL)Vor(AbLotVhCv)eonrtrbicoltehs v(beinvternictlreics(ubliavrenAtCriMcu)l.ar ACM)
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