学科分类
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1 个结果
  • 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

  • 标签: cardiac SARCOID CARDIOMYOPATHY SARCOIDOSIS heart failure