MRI在系统性淀粉样变性心脏受累的患者中的预后的评估.pdf
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1、BRIEF OBSERVATIONPrognosis Assessment of Cardiac Involvement in SystemicAL Amyloidosis by Magnetic Resonance ImagingArsene Mekinian,MD,aChristophe Lions,MD,bXavier Leleu,MD,cAlain Duhamel,MPHD,dNicolas Lamblin,MD,eValerie Coiteux,MD,cPascal De Groote,MD,ePierre-Yves Hatron,MD,PhD,aThierry Facon,MD,P
2、hD,cJean-Paul Beregi,MD,PhD,bEric Hachulla,MD,PhD,aDavid Launay,MD,PhDafor the Lille Amyloidosis Study GroupaService de Mdecine Interne,Hpital Claude Huriez,Centre Hospitalier Rgional Universitaire(CHRU),Lille,France;bService deRadiologie,Hpital Cardiologique,CHRU,Lille,France;cService des Maladies
3、du Sang,Hpital Claude Huriez,CHRU,Lille,France;dCentre detudes et de recherch en informatique mdicale(CERIM),Laboratoire de Statistiques Mdicales,Facult deMdecine,Lille,France;andeService de Cardiologie,Hpital Cardiologique,CHRU,Lille,France.ABSTRACTBACKGROUND:Cardiac involvement is one of the most
4、important prognostic factors in systemic ALamyloidosis.The aim of our study was to assess the role of cardiovascular magnetic resonance(CMR)imaging in prognosis evaluation in AL amyloidosis.METHODS:We retrospectively analyzed 29 consecutive patients with AL amyloidosis who had undergoneCMR.Clinical,
5、laboratory,echocardiographic,and CMR characteristics were compared between CMR-positive(ie,with CMR signs of cardiac localization of AL amyloidosis)and CMR-negative patients.Univariate and multivariate analyses were performed to assess the prognostic value of positive CMR incomparison with other pro
6、gnostic factors.RESULTS:CMR was positive in 11 patients(38%).The overall survival rates for CMR-positive patients were28%,14%,and 14%versus 84%,77%,and 45%at 1,2,and 5 years,respectively,for CMR-negative patients(P?.002).Late gadolinium enhancement patterns,biventricular hypertrophy,and pericardial
7、effusion on CMRwere more frequent in nonsurvivors.Congestive heart failure,abnormal echocardiography,Eastern CooperativeOncology Group grade?1,brain natriuretic peptide,and left ventricular ejection fraction?55%also wereassociated with a decreased survival.The presence of congestive heart failure wa
8、s the only significant variableassociated with survival on multivariate analysis.CONCLUSION:We found that the presence of a positive CMR in AL amyloidosis was associated with asignificantly increased risk of death,in particular of cardiac origin,but was not independent of clinicalcongestive heart fa
9、ilure.2010 Elsevier Inc.All rights reserved.The American Journal of Medicine(2010)123,864-868KEYWORDS:AL amyloidosis;Cardiovascular magnetic resonance imaging;Prognosis;SurvivalThe heart is affected in near 50%of cases in AL amy-loidosis and is a major determinant of prognosis.1Somestudies have trie
10、d to establish prognosis tools,especiallybased on cardiac biomarkers like troponin or natriureticpeptides in AL amyloidosis.2Recently,cardiovascularmagnetic resonance imaging(CMR)with late gadoliniumenhancement sequences was shown to be useful for thediagnosis of cardiac involvement in AL amyloidosi
11、s.3-7However,data about the prognostic value of an abnormalCMR are scarce,8,9and a comparison of CMR with otherprognostic tools such as echocardiography or biomarkersis lacking.2The aims of this study were to assess the prognosticvalue of CMR in AL amyloidosis and to compare theprognostic performanc
12、e of CMR with that of other knownprognostic factors.Funding:None.Conflict of Interest:The authors have no potential conflicts of interestto declare.Authorship:All authors had access to the data and a role in writing themanuscript.Requests for reprints should be addressed to David Launay,MD,PhD,Servi
13、ce de Mdecine Interne,Hpital Claude Huriez,CHRU,Lille,France.E-mail address:d-launaychru-lille.fr0002-9343/$-see front matter 2010 Elsevier Inc.All rights reserved.doi:10.1016/j.amjmed.2010.03.022PATIENTS AND METHODSPatientsWe retrospectively studied 29 patients with AL amyloidosis whohadundergoneCM
14、Rbetween2003and2008andfollowedbytheAmyloidosis Study Group of LilleHospital,France.In all cases,the diagnosis ofamyloidosis was ascertained bya tissue biopsy(fat aspirationn?4,accessory salivary glandn?7,rectum n?5,bone mar-row n?3,kidney n?7,heartn?1,or muscle n?2).Thetype of amyloidosis,the presen
15、ceof monoclonal light chain protein,and the organ involvement weredeterminedasrecommended.10AL cardiac involvement in echo-cardiography was defined as leftventricular(LV)mean wall thick-ness?12 mm,in the absence of hypertension or otherpotential causes of left ventricular hypertrophy.10CMR was perfo
16、rmed on a 1.5 Tesla MR scan(Intera,Philips Medical Systems,Best,The Netherlands).After asecond dose of 0.1 mmol/kg meglumine gadoterate,thedelayed contrast enhancement sequence was performed inthree cardiac axes(short axis,long axis,4 chambers)usinginversion recovery gradient echo with a Look-Locker
17、 se-quence.CMR was considered as positive,that is,revealingcardiac amyloidosis,when it was impossible to obtain anormal myocardial signal on a late gadolinium enhancementLook-Locker sequence with inversion time?300 ms.11Clinical,CMR,echocardiographic,and laboratory datawere collected at diagnosis,be
18、fore any treatment.Clinicaland laboratory data also were collected after a completecourse of treatment.Survival was considered from the date of the diagnosis ofamyloidosis until July 2008(cut-off date).Statistical AnalysisSurvival was analyzed using the variables that could affectsurvival in amyloid
19、osis:age,age?65 years,Eastern Coop-erative Oncology Group grade(ECOG)?1,congestiveheart failure,type of amyloidosis,weight loss at diagnosis,number of involved organs,time between diagnosis andtreatment,CMR,echocardiography,LV ejection fraction?55%(in echocardiography),LV mean wall thickness.Theseru
20、m?2 microglobulin,albumin,creatinine,troponin,brain natriuretic peptide(BNP),serum free light chain(sFLC)levels,and the bone marrow plasma cell percentagewere analyzed.Univariate survival analysis(Cox proportional hazardregression models or Kaplan Meier)and a multivariateanalysis by Cox regression m
21、odel with stepwise selectionwere carried out to identify significant death predictorswithin variables linked to survival(P?.05)and CMR(P?.5).Statistical analysis was performed using SAS soft-ware(SAS Institute Inc.,Cary,NC).A value of P?.05 wasconsidered significant.RESULTSBaseline CharacteristicsTh
22、e baseline characteristics of the29 AL amyloidosis patients aresummarized in Table 1.Mean du-ration of follow-up was 32?5months.On echocardiography,LV mean wall thickness?12 mmwas present in 15 cases(54%).Nopatients with LV mean wall thick-ness?12 mm had a restrictive di-astolic pattern or diffuse“g
23、ranularsparkling.”Specific treatment wasmelphalan-dexamethasone in 21cases,bortezomib-dexamethasonein 4 cases,lenalidomide-dexa-methasone in 2 cases,and high-dose melphalan plus stemcell transplantation in 2 cases.Cardiovascular Magnetic ResonanceCMR was positive in 11 cases(38%).CMR characteristics
24、 ofpatients are summarized in Table 2.There was no difference inbaseline characteristics or laboratory findings between CMR-CLINICAL SIGNIFICANCEPositive cardiovascular magnetic reso-nance is associated with a significantlyincreased risk of death,especially ofcardiac origin,in AL amyloidosis.In the
25、presence of congestive heart fail-ure,cardiovascular magnetic resonancedoes not have to be performed,as it doesnot modify prognosis determination.Table 1Patients Baseline CharacteristicsCharacteristics(n?29)Age(years)63?11Sex(M/F)19/10ECOG 2/3/4,n(%)8(28)/3(10)/1(3)Involved organs(n)Heart14Kidney13L
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