Rev Port Cardiol

RevPortCardiol.2012;31(4):313---316

Revista

Portuguesa

de

Cardiologia

Portuguese

Journal

of

Cardiology

CASE

REPORT

Caseous

calcification

of

the

mitral

annulus:

A

multi-modality

imaging

perspective

Sílvia

Ribeiro

,

Alberto

Salgado

,

Nuno

Salomé

,

Nuno

Bettencourt

,

Pedro

Azevedo

_,

_António

_Costeira

_,

_Adelino

_Correia

a_{CardiologyDepartment---BragaHospital,Braga,Portugal}

b_{VilaNovadeGaia/EspinhoHospitalCentre,VilaNovadeGaia,Portugal}

Received15August2011;accepted15September2011 Availableonline17March2012

KEYWORDS

Intracardiacmass; Caseouscalcification ofmitralannulus; Mitralregurgitation; Transthoracic echocardiography; Cardiaccomputed tomography; Cardiacmagnetic resonance

Abstract Mitralannuluscalcificationisacommonechocardiographicfinding,particularlyin theelderlyandinend-stagerenaldiseasepatientsunderchronicdialysis.Caseouscalcification or liquefaction necrosisofmitralannulus calcification isa rare evolutionofmitral annular calcification.Earlyrecognitionofthisentityavoidsaninvasivediagnosticapproach,sinceitis benignand,unlikeintracardiactumorsandabscesses,hasafavorableprognosis.Theauthors presentthecaseofan84-year-oldwomanwithasuspiciouslarge,echodensemassatthelevel oftheposteriormitralleafletwithassociated severemitralregurgitation.Cardiacmagnetic resonanceimagingdemonstratedahypoperfusedmasswithstrongperipheralenhancement10 minutesaftergadoliniumadministration.Multislicecomputedtomographyshowedthecalcified nature ofthe mass. A multi-modality imagingapproach confirmed thediagnosis ofcaseous calcificationoftheposteriormitralannulus.Thepatientrefusedsurgicaltreatment.

© 2011Sociedade Portuguesa de Cardiologia. Published by Elsevier España, S.L. All rights reserved.

PALAVRAS-CHAVE

Massaintracardíaca; Calcificac¸ãocaseosa doanelmitral; Insuficiênciamitral; Ecocardiograma transtorácico; Angio-TCcardíaca;

Degenerescênciacaseosadacalcificac¸ãodoanelmitral:umaperspectiva multi-imagem

Resumo Acalcificac¸ãodoanelmitraléumachadoecocardiográficocomum,particularmente emidososedoentescominsuficiênciarenalterminalemdiálise.Adegenerescêncicacaseosa, ounecroseliquefeita,dacalcificac¸ãodoanelmitraléumaformararadecalcificac¸ãodoanel mitral.Oreconhecimentoprecocedestaentidadeevitaprocedimentosdiagnósticosinvasivos namedidaemqueéumapatologiabenignae,aocontráriodetumoreseabcessocardíacos, temumbomprognóstico.Osautoresapresentamocasoclínicodeumadoentede84anosde idadecomumamassahiperecogénicaaoníveldofolhetoposteriordaválvulamitral,associada

∗_{Correspondingauthor.}

E-mailaddress:silviamartinsribeiro@gmail.com(S.Ribeiro).

0870-2551/$–seefrontmatter©2011SociedadePortuguesadeCardiologia.PublishedbyElsevierEspaña,S.L.Allrightsreserved. doi:10.1016/j.repc.2012.02.007

314 S.Ribeiroetal.

Ressonância magnéticacardíaca

ainsuficiênciamitralsevera.Aressonânciamagnéticacardíacademonstrouumamassa hipop-erfundida,comrealcetardio,apósadministrac¸ãodegadolíneo.Atomografiacomputadorizada cardíacaevidenciouanaturezacalcificadadalesão.Umaabordagemmulti-imagempermitiuo diagnósticodecalcificac¸ãocaseosadoanelmitralposterior.Adoenterecusoucirurgiacardíaca. © 2011 SociedadePortuguesa de Cardiologia. Publicado por Elsevier España, S.L.Todos os direitosreservados.

Introduction

Mitralannuluscalcificationisacommonechocardiographic finding, particularly in the elderly andin end-stage renal diseasepatientsunderchronic dialysis.1 _Caseous calcifica-tionofthe mitralannulus(CCMA) or liquefaction necrosis ofmitralannuluscalcificationis arareevolution ofmitral annularcalcification,withaprevalenceof0.6%inthis pop-ulationandof 0.06---0.07%inlargeseriesofpatientsofall ages.2,3 _{Early recognition of thisentity avoidsan invasive} diagnosticapproach,sinceitisbenignand,unlike intracar-diactumorsandabscesses,hasafavorableprognosis.2---4,8---10 Echocardiographytypicallyidentifiesthisentityasaround, calcified mass with an echolucent, liquid-like inner part. Theauthorspresentthecaseofan84-year-oldwomanwith caseouscalcificationoftheposteriormitralannulus.

Case

report

An84-year-oldwoman,withahistoryofhypertensionand dyslipidemia, was admitted to our institution because of an intracardiac mass. She described a five-month history of shortnessof breath onexertion (NYHA functional class III) and peripheral edema. Physical examination revealed a grade III/VI mitral systolic murmur. The electrocardio-gram was unremarkable. Laboratory tests results showed nosignificantalterations.Thetransthoracicechocardiogram demonstrated a large, round, heterogeneous, echodense mass at the base of the posterior leaflet (Fig. 1). Dopplerexamination documentedseveremitral regurgita-tion.The three-dimensionaltransthoracicechocardiogram revealed the mass involving the posterior mitral annu-lus, distorting it into a more triangular shape (Fig. 2). Three-dimensionaltransthoraciccolorflowimagingshowed

Figure 1 Two-dimensional echocardiogram, parasternal long-axis view (A) and apical two-chamber view (B), revealing an echodensemassatthebaseoftheposteriorleaflet.

an eccentric mitralregurgitation jet originatingfrom the posteromedial commissure (Fig. 3). The transesophageal echocardiogram confirmed the location of the mass and severemitralregurgitation(Fig.4),withtwojetsidentified, onecentralandtheothereccentric,apparentlyoriginating fromtheposteromedialcommissure.Cardiacmagnetic res-onance(CMR)imagingwasperformedtobetterevaluatethe mass.Thisshowedahypointensemassintheposteriorregion of the mitral annulus/basal segment of the inferior wall (Figs.5and6).RestperfusionCMRdemonstrated hypoper-fusionofthemasscomparedtonormalmyocardium(Fig.7). Phase-sensitiveinversion-recoveryCMRshowedlate gadolin-ium enhancement in the peripheral margin of the mass (Fig.8).Onthebasisoftheabovefindings,apresumptive diagnosis of a centrally liquefied mass containing a high-proteinorhemorrhagiccontentwithaninflammatoryand/or fibroticwallwasmade.Amultislicecomputedtomography (CT) scan of the heart was also performed, demonstrat-inga relatively homogeneous,high-densitymasswithfoci of calcification (Figs. 9and 10).Amulti-modality imaging approachconfirmedthediagnosisofcaseouscalcificationof theposteriormitralannulus.

Thepatientrefusedsurgery.Medicaltreatmentwas opti-mized.

Discussion

Caseouscalcificationofthemitralannulus 315

Figure 2 Three-dimensional transthoracic echocardiogram withavisualizationperspectivefromwithintheleftventricle. Notethemassinvolvingtheposteriormitralleaflet,distorting itsshape.

Figure 3 Three-dimensional transthoracic color flow imag-ingofmitralregurgitation,showinganeccentricjetoriginating fromtheposteromedialcommissure.

Figure4 Transesophagealtwo-chamberviewdemonstrating severemitralregurgitation.

Figure 5 Steady-state free precession cine CMR image in modified two-chamber view, showing a hypointense mass (arrow)intheposteriorregionofthemitralannulus/basal seg-mentoftheinferiorwall.

Figure 6 Steady-state free precession cine CMR image in basalshort-axisview,demonstratingthemass(arrow).

Figure7 RestperfusionCMRimaginginshort-axisview.Note thehypoperfusionofthemasscomparedtonormalmyocardium.

316 S.Ribeiroetal.

Figure9 MaximumintensitymultisliceCTprojectionshowing apartiallycalcifiedhyperintensemass(arrow)innon-contrast acquisition(four-chamberandshort-axisview).

Figure10 MultisliceCTvolume-renderingtechnique reveal-ing the extent of the mass (arrow) and its relation to the posteriormitralannulus.

contrast-enhanced inversion-recovery technique.5 _CT confirms the calcified aspect of the mass. Histological examination of the inner fluid usually reveals an amor-phous, basophilic content, composed of a putty-like admixtureof fatty acids,cholesterol, calciumand inflam-matorycells, mainly macrophages.4,6 _{Early recognition of} thisentityavoidsaninvasivediagnosticapproach, sinceit is benign and, unlike intracardiac tumors and abscesses, hasa favorableprognosis.7_{Surgeryshouldbereservedfor} cases with severe co-existent mitral valve dysfunction, originatingfromcompromisedmitralleafletcoaptation sec-ondarytomitralannulusdistortionduetoCCMA.Thereare briefreferences in the literature to possible spontaneous resolutionofthisentity.8,9 _{CCMAmayrarely beassociated} withheartrhythmdisturbancesandsystemicembolism.10

Conclusions

Caseouscalcification of themitral annulus(CCMA) or liq-uefactionnecrosisof mitralannuluscalcification is arare

evolutionofmitralannularcalcification.Although transtho-racic echocardiographycan besensitive indiagnosing this entity, the diagnosis can sometimes still be inconclusive. Multi-modality imaging with CMR or CT can lead to a definitivediagnosis,avoidingasurgicalapproach.Oncethe diagnosis is made, the patient should be treated medi-callyandbemonitoredclinicallyandechocardiographically unless there is associated severe mitral valve dysfunc-tion,inwhichcasecardiacsurgeryisthebesttherapeutic option.

Conflicts

of

interest

Theauthorshavenoconflictsofinteresttodeclare.

References

1.StoneE,CohnD,DealC.Calcificatrialmassinend-stagerenal failure.NephrolDialTransplant.1997;12:807---10.

2.HarpazD,AuerbachI,VeredZ,etal.Caseouscalcificationof themitralannulus:aneglected,unrecognizeddiagnosis.JAm SocEchocardiogr.2001;14:825---31.

3.KronzonI,WinerHE,CohenML.Sterile,caseousmitralannular abscess.JAmCollCardiol.1983;2:186---90.

4.MinardiG,ManzaraC,PulignanoG,etal.Caseouscalcification of the mitral annulus with mitral regurgitation and impair-mentoffunctional capacity:a casereport.JMedCaseRep. 2008;2:205.

5.DiBellaG, MasciPG, GanameJ,et al.Liquefactionnecrosis of mitral annulus calcification: detection and characteriza-tion with cardiac magnetic resonance imaging. Circulation. 2008;117:e292---4.

6.PomeranceA.Pathologicalandclinicalstudyofcalcificationof themitralvalvering.JClinPathol.1970;23:354---61.

7.TejaK, GibsonRS,NolanSP. Atrialextensionofmitral annu-lar calcification mimicking intracardiac tumor. Clin Cardiol. 1987;10:546---8.

8.Gramenzi S,Mazzola AA, TagliaferriB, et al. Caseous calci-fication of the mitral annulus: unusual caseof spontaneous resolution.Echocardiography.2005;22:510---3.

9.Yokoyama N, Komo K, Suzuki S, et al. Serial assessment of liquefactionnecrosisofmitralannuluscalcificationby echocar-diography and multislice computed tomography. Circulation. 2007;115:e1---2.