简介:Sarcoidosisisarareconditionofgranulomatousinfiltrationofmanytissuesofthebody,includingtheheart.Cardiacsarcoidosishasbeenchallengingtostudy,asitisoftenasymptomatic,althoughtheinitialpresentationcanbesuddencardiacdeath.Theincidenceandprevalencerateshavebeendifficulttoestablishandnoexpertagreeduponguidelinesfordiagnosisandmanagementofcardiacsarcoidosisexist,andclinicalmanifestationsarevaried.Thepathophysiologyofgranulomaformationinthemyocardiumaswellasothertissuesisgovernedbyimmuneresponsetosomeenvironmentalantigen.Geneticsisalsothoughttoplayarole,althoughgenealterationshavenotbeenextensivelystudied,andnospecificsetofgeneticmutationshasbeenidentifiedtoaidinidentificationofindividualsatriskofdevelopingdisease.Epigeneticfactorslikelyplayasignificantroleinmodulationofgeneexpressionwithrespecttoimmuneresponse.Thereisnostandardizedscreeningtoolfortheidentificationofcardiacsarcoidosis.Thepresenceofsystemicsarcoidosisandnew-onsetthird-degreeheartblockorventriculararrhythmiaswarrantsfurtherinvestigationforcardiacsarcoidosis.MRIandPETareusefulinhelpingtoidentifycardiacsarcoidosisbutarenotstand-alonetests.Endomyocardialbiopsyisthegoldstandardbuthasalowyieldowingtothepatchynatureofgranulomaformationinthemyocardium.Therapyshouldbeinstitutedearlyandinvolvesimmunosuppressivetherapywithpredominantuseofcorticosteroids.Arrhythmias,eitherventricularorhigh-gradeheartblocks,aremanagedwithdevicetherapy.Clinicalpresentationmaywarrantuseofantiarrhythmicagentsand/orcatheterablation.Survivalanddiseaseprognosisaredependentonearlydiagnosisandtreatment.Thisreviewdetailsthecurrentunderstandingofcardiacsarcoidosisandhighlightsdiagnosticstrategiesandtreatmentwiththeaimofguidingthecliniciantoearlyidentificationofpatientsandimplementationofappropriatemanagementinthisraredi