CaseReport
Atrial standstill in suspected isolated cardiac sarcoidosis
Tae-HunKim (MD)a,Hyungseop Kim(MD)a,*, Hyoung-SeobPark(MD)a, SeongwookHan(MD)a,Nam-Hee Park(MD)b
aDivisionofCardiology,DepartmentofInternalMedicine,KeimyungUniversityDongsanMedicalCenter,Daegu,RepublicofKorea
bDepartmentofChestSurgery,KeimyungUniversityDongsanMedicalCenter,Daegu,RepublicofKorea
Introduction
The chronicinflammationcausedbycardiac sarcoidosis(CS) could impair the cardiac conduction system which is usually associatedwithtachyarrhythmiaorconductionblock.Withatrial standstill,we recommenda comprehensiveeffort toinvestigate the potential etiology including CS, particularly in the case of enlargedatriumandventriculardysfunction.
Casereport
A54-year-oldmalewasadmittedforevaluationofdizziness.
Hisvitalsigns,bodytemperature,andserologicaltestswerewithin normallimitsexceptforahighN-terminalpro-B-typenatriuretic peptidelevel(27,550pg/mL).Theelectrocardiogramrevealedthe absenceofP-waveandventricularescaperhythmwith36beats perminute(Fig.1A).
A chest X-ray showed cardiomegaly, and echocardiography revealed biatrial enlargement, decreased left ventricular (LV) contractility(ejectionfraction;38%),andmyocardialthinningof theinterventricularbasalseptum.ColorDopplerimagingrevealed severemitralandtricuspidregurgitationduetoannulardilation andLVdysfunction.Holtermonitoringrevealedthetotalabsence
of P-wave, ventricular escape beats, and ventricular pause (maximal duration: 6.72s), as well as sustained ventricular tachycardia(VT).
Cardiac magnetic resonance(CMR) imaging wasperformed witha1.5Tscanner(Avanto,Siemens,Erlangen,Germany)with 6-channel phased array cardiac coil. Atrial late gadolinium enhancement (LGE)images were acquired about 15min after gadoliniuminjectionusing3Dnavigatorandelectrocardiograph- ically gated inversion-recovery gradient-echo sequence: voxel size=1.25mm1.25mm2.5mm, slice size=2mm, inver- siontime=300ms,repetitiontime=5.4ms,echotime=2.3ms, flipangle=208.CMR revealedepicardialdelayedenhancement ofgadoliniuminthebasalinferiorandinferoposteriorwallatthe mid-ventricularlevel(Fig.2).Mid-walllineardelayedenhance- ment was also observed at the thinned interventricularbasal septumintheapical4-chamberviewandatthemid-ventricular septum in the parasternal short-axis view. Both atriashowed LGE in the apical 2-/4-chamber view. Cardiac perfusion and positronemissiontomography(PET)studiesindicateddecreased perfusion with 13N-ammonia in the inferoposterior segment despiteanincreaseduptakeof18F-fluorodeoxyglucose.However, PET did not show any evidence of systemic sarcoidosis and inflammatoryresponse.Anelectrophysiologicalstudy(EPS)was performed to evaluate the electrical status of the atrium considering synchronized permanent pacemakerimplantation.
However, the atrium was not captured with the maximum outputofcurrent,andtherewerenoelectricalactivitiesinthe atria(Fig.1B).
JournalofCardiologyCases14(2016)136–138
ARTICLE INFO
Articlehistory:
Received2May2016
Receivedinrevisedform15June2016 Accepted27June2016
Keywords:
Isolatedcardiacsarcoidosis Atrialstandstill
Arrhythmia
ABSTRACT
Most of the abnormalcardiac conduction system findings are atrial tachyarrhythmias in cardiac sarcoidosis.However,atrialstandstillasasick-sinussyndromecouldbecomplicatedinthecaseof diffuseatrial fibrosis.Herein, we presentaninterestingandvaluablecase ofatrial standstillwith suspectedisolatedcardiacsarcoidosis.
<Learningobjective:Thechronicinflammationcausedbyisolatedcardiacsarcoidosiscouldimpairthe conductionsystem.Withatrialstandstill,werecommendacomprehensiveeffortto investigatethe potential etiology including cardiac sarcoidosis, particularly in the case of enlarged atrium and ventriculardysfunction.>
ß2016JapaneseCollegeofCardiology.PublishedbyElsevierLtd.Allrightsreserved.
* Correspondingauthorat:56Dalseong-Ro,Jung-Gu,Daegu700-712,Republicof Korea.Fax:+82532507034.
E-mailaddress:[email protected](H.Kim).
ContentslistsavailableatScienceDirect
Journal of Cardiology Cases
j our na l h ome p a ge : w ww . e l se v i e r. co m/ l oc a te / j c ca se
http://dx.doi.org/10.1016/j.jccase.2016.06.010
1878-5409/ß2016JapaneseCollegeofCardiology.PublishedbyElsevierLtd.Allrightsreserved.
Surgical biopsies of both the atria and myocardium were performedduringsurgicalcorrectionofboth valvularregurgita- tions.Severefibrosiswasnotedinbothatria,andinflammatory cellswereinfiltratedin theLV myocardiumwithoutgranuloma compatible for sarcoidosis (Fig. 3). After correction of valvular regurgitation,asingle-chamberimplantablecardioverterdefibril- lator(ICD)wasimplantedduetodocumentedsustainedVT.The patientwasdischargedandmanagedwithbeta-blocker,angioten- sin-convertingenzymeinhibitor,andsteroid.However,hehasnot showna remarkableimprovement in LV systolic functioneven thoughhisclinicalsymptomsimproved.
Discussion
TheprevalenceofCSmayvaryfromapproximately5%to50%of casesofsystemicsarcoidosis,anditischaracterizedbymyocardial inflammation,impairmentoftheconductionsystem,arrhythmias,
and decreasedventricularfunction [1].Regardingatrialinvolve- ment,atrialfibrillation(AF)isthemostcommonarrhythmiathatis associatedwithinflammationand/orfibrosisoftheatria[2].
Inparticular,isolatedCSisrare,anddifficulttodiagnosewithout highsuspicion.Becauseoflimitationofbiopsy,twothirdsofthe suspected CS patientscould not beidentified and didnot have appropriate medical concern despite typical cardiac features [3]. Recently, it has beensuggested that isolated CScould exist withoutpathologicfindingsofgranulomaandclinicaldiagnosisof isolated CS is characterized as follows [4]: clinical symptoms suggestiveofheartdisease,arrhythmiaorconductiondisturbances, LV systolic dysfunction, absence of coronary artery disease, abnormalcardiacimagingofCMR,orradionuclidescansuchasPET.
Atrialstandstill,whichisnotacommondisorder,ischaracter- izedbytheabsenceofatrialelectricalimpulseproduction,andthe diagnosisusuallyrequiresanEPSforexclusionofsimilardisorders suchasfineAF[5].Amongthesuggestedetiologicalmechanisms, Fig.1. Thepatient’selectrocardiographyshowsthetotalabsenceofP-wavesandventricularescapebeats(bradycardiaof36bpm)(A).Anactionpotentialoriginating
fromtherightatriumwasnotobserved,andatrialstimulationwithhighoutputtriggerfailedtoproduceandmaintainrightatrialactivation(B).
Fig.2.
Cardiacmagneticresonanceimagingandpositronemissiontomographyforcardiacsarcoidosis.(AandB)Delayedenhancementofgadoliniumisobservedin boththeleftandrightatria(arrow-head)ineachapical2-chamberview.(C)Thosefindingsarealsoseenintheapical4-chamberviewwiththebasalseptum thinnedandenhanced(arrow-head).(D)Strongepicardialenhancementisnotedatthebasalinferoposteriorwall(arrow),alongwithinterventricularseptum (arrow-head).(E)Cardiacperfusionwith13N-ammoniawasdecreasedatthebasalinferoposteriorwall.(F)Anincreaseduptakeof18F-fluorodeoxyglucosewas observedinthesamelesion.
T.-H.Kimetal./JournalofCardiologyCases14(2016)136–138 137
chronicinflammationorinfiltrationmaybeinvolved,anditmaybe accompanied by resultant fibrosis of both atria. Although CS frequentlyinvolvesthe LVmyocardium includingtheinterven- tricularbasalseptum,itisnotsurprisingthatCSmaycauseatrial standstill in the long term because it is a systemic and inflammatorydisorder.
OncetheconductionsystemisaffectedbyCS,atrialarrhyth- miasoccurmorefrequentlythanventriculararrhythmias[6].The incidencewasreportedtoreach20–30%:atrialtachycardiassuch asAFarecommon,whereasbradycardiahasbeenrarelyreported [6].Itis unclearwhether inflammatory involvement orfibrosis caused by chronic pressure loading to the atrium would be associated with atrial standstill. However, the fibrosis occurs predominantlyatthelatestageofCS:bothdecreasedperfusion andinflammationareobserved.
Therehasnotbeenclearconsensusabouttreatmentofisolated CS,but valvular disorders suchas severe regurgitation needed surgical correction considering patient’s symptoms, chronic volume overloading, and ventricular dysfunction. Mitral or tricuspidregurgitationwassecondarytoannulardilationandLV dysfunction,notto primaryvalvular disordersuch asprolapse.
Huge atrial enlargement per se hasbeen related withchronic volume overloading status, and diffuse LGE could indicate myocardialfibrosis,irreversibleLVdysfunction,andunfavorable prognosisevenwithantiarrhythmicandsteroidtherapy[4,7,8].
It is necessary to unravel the causal relationship between inflammatory sarcoidosisand fibrotic changes in the atriumor lesioninvolvement.However,thisiscomplicatedbyalongdelay betweenCSactivityanditsdiagnosis.Althoughthereisalimitation ofsimilarthicknessbetweenCMRimageresolutionandatrialwall, we determined to obtain both atrial specimens surgically to overcomethoselimitations.Consideringatrialfibrosis,CMRwould providean incremental value in identifying atrial fibrosis over ventricularfibrosis.
Additionally, we did not perform electroanatomical bipolar voltagemappingwhichseemstohavegoodcorrelationwithatrial orventricularscarorfibrosisdistributiondetectedonCMR-LGE [9,10].However,therearelimiteddataabouttranslatingthistothe atrialfibrosisandthus,clearevidencemayberequiredwithregard totheirrelationship[10].
Conclusion
This case underlines the requirement of a comprehensive evaluationtodetecttheetiologyofatrialstandstill,particularlyin cases of biatrial enlargement and ventricular dysfunction. The diagnosticconfirmationcouldbebasedoncardiovascularimaging ortissuebiopsy.
Conflictofinterest
Therearenoconflictsofinterest.
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Fig.3. Basedonsurgicalbiopsy,massivefibrosisisobservedintheleftatrium(A).Theinflammatorycellsinfiltratedtherightatrium(B)andleftventricles(C).
T.-H.Kimetal./JournalofCardiologyCases14(2016)136–138 138
