心脏肿瘤-心脏粘液瘤

1、 /14心脏肿瘤心脏粘液瘤心脏肿瘤心脏粘液瘤中文：心脏粘液瘤是最常见的心脏原发肿瘤，70%为良性肿瘤，30%为恶性肿瘤，多数有瘤蒂，可发生于心脏各房、室腔，最常见于左心房。病因和形态学：粘液瘤的的发病原因不清楚，一般认为肿瘤起源于心内膜下的多潜能的间质细胞，该细胞在形态学上与房间隔卵圆窝处胚胎残留细胞相似，可分化为内皮细胞、平滑肌细胞、成纤维细胞、骨及骨髓组织，产生无定形酸性粘多糖，构成粘液瘤的组织成分。极少数粘液瘤可发生恶变，成为粘液肉瘤。瘤体多为圆形或卵圆形，有深浅不一的切迹或分叶状，或呈葡萄状或息肉状。外观多呈半透明胶冻状，表面光滑，常有散在大小不一的紫色、暗红和鲜红的出血区，偶有钙化。

2、瘤体大小可自1cm到15cm，多数在5cm6cm。质软而脆，容易破碎，脱落后可引起周围动脉栓塞或脑血管栓塞。粘液瘤可发生于各个心腔，最常见于左心房，约占75%。多数肿瘤有瘤蒂与心房壁相连，90%的左房粘液瘤附着于心房间隔卵圆窝处。瘤体可随心脏的收缩、舒张而活动。绝大多数为单发肿瘤，但也可为多发，常有家族遗传倾向。临床表现：粘液瘤的临床表现主要有：心脏血流动力学紊乱、动脉栓塞和全身表现三个部分。血流动力学紊乱。心脏粘液瘤体积增大至相当程度，尤其是蒂长有活动度者，可阻塞二尖瓣口，引起血流障碍，产生瓣膜狭窄的症状和体征。如心悸、气短、端坐呼吸、晕厥、咯血等。如果这种梗阻为间歇性发作，患者可出现短暂的

3、昏厥，且常与体位的变化有关，部分患者可能会发生猝死。少数患者由于肿瘤梗阻或瓣叶损伤，而出现二尖瓣或三尖瓣口关闭不全。肿瘤也可阻塞肺静脉和腔静脉开口，导致肺静脉和腔静脉引流障碍。动脉栓塞的原因可以是肿瘤的碎片，也可能是整个肿瘤随血流漂动，引起体动脉栓塞，息肉状和葡萄状的粘液瘤较易脱落导致栓塞。50%的体循环栓塞发生在脑血管，引起昏迷、偏瘫、失语等症状。体循环栓塞还可引起急腹症，肢体疼痛、坏死等。大约30%的心脏粘液瘤患者可出现全身反应，表现为发热，消瘦，食欲不振，乏力，关节或肌肉疼痛，杵状指（趾），贫血，血沉增快，C反应蛋白阳性，g球蛋白升高，肌酐激酶及转氨酶升高等。瘤体较大的左房粘液瘤更易出现

4、全身反应，这可能与粘液瘤的出血、变性、坏死，而引发的自身免疫反应有关，一般药物治疗无效。手术摘除肿瘤后，症状通常可立即缓解或消失。临床诊断：左房粘液瘤的临床症状酷似二尖瓣狭窄，大多数患者有心悸、气短、端坐呼吸、咯血等症状，发作时间较短，呈阵发性，常伴晕厥，病程进展较快，常出现心衰，药物治疗欠满意。栓塞主要有脑血管意外及四肢末梢冷、痛等症状。偶有因冠状动脉栓塞出现心绞痛或因肺动脉栓塞出现呼吸困难等症状，也可因腹腔干动脉栓塞出现急腹症。体征不恒定，可随体位变动而变化。左房粘液瘤听诊常有二尖瓣舒张期或双期杂音，个别只有收缩期杂音。实验室检查一般正常。心电图检查无特异性，可有心房纤颤、束支传导阻滞和异

5、常p波。X线胸片无特异性，可以有心影扩大、肺瘀血及肺动脉高压表现。超声心动图是最简便可靠的诊断方法，诊出率可达98%。二维超声可显示肿瘤的大小、部位及活动情况;多普勒超声可显示肿瘤引起瓣膜狭窄和关闭不全的程度。鉴别诊断：左房粘液瘤需要与左房血栓和左房其他肿瘤相鉴别。左房血栓的形成需要特定病因，例如心房纤颤或二尖瓣狭窄等，粘液瘤不需要上述因素。血栓多固定在左心耳和左心房后壁，而且没有活动性或者活动性极差;而粘液瘤则固定于房间隔，活动性很好，可经二尖瓣口进入左心室。左心房其他肿瘤多位于心肌内或位于瓣缘，基本没有活动性;粘液瘤则位于心腔内，活动性良好。右房粘液瘤需要与心脏转移瘤和过度增生的肌小梁相鉴

6、别。转移肿瘤患者多较消瘦，肿瘤多起自下腔静脉，成条索状，与心房壁甚至心包相粘连;而粘液瘤患者状态良好，肿瘤多起自房间隔或右心房外侧壁，呈球形或分叶状，活动性良好，偶伴有肺栓塞。个别过度增生的肌小梁会被误判为心房肿瘤，通过心脏CT扫描可以排除。手术指征：左房粘液瘤一经诊断出来原则上就应立即手术治疗。特别是有栓塞和晕厥病史的患者，应急诊手术治疗。手术方法：左房粘液瘤摘除术多经正中切口，在体外循环下切开右心房完成。从右心房面在卵圆窝中点先做12cm纵切口，检查瘤蒂的附着部位，然后沿瘤蒂周围5mm切除瘤蒂，慢慢取出肿瘤，用涤纶布修补房间隔缺损。术后处理：一般的处理原则与体外循环心内直视手术相同。左房粘

7、液瘤的病理生理改变非常近似二尖瓣狭窄患者，早期处理原则是切忌补充容量过渡，引起急性左心衰竭及肺水肿。特别是术前已经出现左心衰的患者，术后应严格限制液体的输入量和输入速度，可适量应用白蛋白，同时应用少量多巴胺与多巴酚丁胺，提高心肌的收缩力和心排血量。主要并发症1.栓塞：主要发生原因为探查时过渡搬动和挤压心脏，或切除时强行提拉瘤蒂发生瘤体破碎。因此，切除肿瘤后应用盐水冲洗心腔，避免肿瘤碎片隐藏在心房、室的肌小梁内。急性心力衰竭：主要发生原因是对该病的病理生理认识不足，术后早期补充容量过多、过快，或对心功能较差的患者，未及时应用血管活性药物等。术后心律失常：心房较大患者，手术后会出现房性心律失常;个

8、别患者可以发生传导阻滞，可能与手术损伤有关。心脏手术损伤：当肿瘤的蒂部位于左房顶、二尖瓣前叶附近、或传导束周围时，切除肿瘤时要十分小心，切除范围不能过大、过深，否则会损伤邻居结构。可以通过缩小切除范围和深度避免损伤，然后通过电烧灼，精细清除可能残留的肿瘤组织。预后：绝大多数患者预后良好，心功能可以恢复到NYHAIII级。文献报道手术死亡率在5%-10%之间，患者死亡多与粘液瘤手术本身无关，主要与手术时机、患者年龄和患者的心脏合并病变有关。在染色体正常的患者中，肿瘤复发的几率仅为1%-3%，可能与肿瘤种植、肿瘤切除不全和肿瘤再生有关;不过，家族性粘液瘤的复发比例却很高，可以达到30%-75%，平

9、均复发时间为30个月。英文：Cardiactumor,cardiacmyxomaCardiacmyxomasarethemostcommonprimarycardiactumors,70%benigntumors,30%malignanttumors,mostofBeattie,canoccurintheheartoftheatrial,ventricularcavity,mostcommonlyintheleftatrium.Etiologyandmorphology:Myxomaoftheetiologyisnotclear,isgenerallybelievedthatthetumoro

10、riginatedinthesubendocardialpluripotentialmesenchymalcells,thecellsinmorphologyandtheatrialseptumovalfossaresidualembryossimilarcells,candifferentiateintoendothelialcells,smoothmusclecells,fibroblasts,boneandbonemarrowtissue,producedamorphousacidmucopolysaccharidespolysaccharide,constituteamyxomatis

11、suecomponents.Ahandfulofmyxomacanbecomemalignant,mucussarcoma.Thetumorisroundoroval,withshadesofanotchorlobulated,oragrape-likeorpolypoidal.Theappearanceofmultipletranslucentjellyshape,smoothsurface,oftenwithscatteredindifferentsizesofpurple,redandredbleedingarea,withoccasionalcalcification.Tumorsiz

12、efrom1cmto15cm,mostlyin5cm6cm.Softandbrittle,easilybroken,aftersheddingcancauseperipheralarterialembolismorvascularembolism.Myxomacanoccurinvariousheartcavity,mostcommonlyintheleftatrium,accountingforabout75%of.ThemajorityofBeattietumorandatrialwallconnected,90%leftatrialmyxomaattachedtotheatrialsep

13、talfossaovalis.Thetumorwiththecontractionoftheheart,bloodandactivities.Forthevastmajorityofsingletumor,butalsocanprovidemultiple,oftenfamilialandgenetictendency.Clinicalmanifestation.Myxomaofthemainclinicalmanifestations:cardiachemodynamicdisorders,arterialembolizationandsystemicmanifestationsofthre

14、eparts.Hemodynamicdisorders.Cardiacmyxomavolumeincreasedtoaconsiderabledegree,especiallythepediclelengthofactivitydegree,canblockthemitralorifice,causesofhemodynamicdisorders,producevalvularstenosissymptomsandsigns.Suchaspalpitations,shortnessofbreath,orthopnea,syncope,hemoptysis.Iftheobstructionisi

15、ntermittentepisodes,patientsmaypresentwithtransientsyncope,andisoftenassociatedwithposturalchangesrelatedtopart,canoccurinpatientswithsuddendeath.Aminorityofpatientsduetotumorobstructionorleafletinjury,andthemitralortricuspidregurgitationthreemouth.Thetumorcanalsobeobstructionofthepulmonaryveinsands

16、uperiorvenacavaopening,leadingtopulmonaryveinandvenacavadrainageobstacle.Causesforarterialembolismcanbeatumorfragments,possiblytheentiretumorwithbloodflowcausedbyfloating,arteryembolization,polypoidandgrape-likemyxomaiseasytofalloffleadtoembolism.50%ofthesystemicembolismoccurredinthecerebralvasculat

17、ure,leadingtocoma,hemiplegia,aphasiaandothersymptoms.Systemicembolismcanalsocauseacuteabdomen,painofthelimbs,necrosis.Approximately30%ofpatientswithcardiacmyxomacanoccurinsystemicreactions,manifestedasfever,weightloss,lossofappetite,fatigue,jointormusclepain,clubbingfinger(toe),anemia,calcium,Creact

18、iveprotein,Gglobulinelevatedcreatininekinase,andtransaminaseelevation.Thetumourislargeleftatrialmyxomapronetosystemicreactions,whichmaybeassociatedwithmyxomaofthebleeding,degenerationandnecrosis,andcauseditsownimmuneresponse,generaldrugtreatmentisineffective.Operationaftertumorextirpation,symptomsus

19、uallycanbeimmediatelyrelievedordisappeared.Clinicaldiagnosis:Leftatrialmyxomaclinicalsymptomsmimickingmitralstenosis,themajorityofpatientswithpalpitations,shortnessofbreath,orthopnea,hemoptysisandothersymptoms,onsettimeshorter,paroxysmal,oftenwithsyncope,progressisrapidder,oftenoccursinheartfailure,

20、drugtreatmentlesssatisfactory.Embolizationofcerebralvascularaccidentandmainextremitiescold,painandothersymptoms.Sometimesduetotheonsetofanginapectoriscoronaryarteryembolizationorcausedbypulmonaryembolismappearanceofsymptomssuchasdyspnea,butalsobecauseofceliacarteryembolismsufferedfromacuteabdomen.Sy

21、mptomsarenotconstant,withthepositionchangeandchange.Leftatrialmyxomaauscultationoftenmitraldiastolicortwophasenoise,individualonlysystolicmurmur.Laboratoryexaminationofnormal.Electrocardiogramexaminationnospecificity,canhaveatrialfibrillation,bundlebranchblockandabnormalPwave.ChestX-rayisnonspecific

22、,canhaveinfluenceexpanded,pulmonarycongestionandmanifestationofpulmonaryhypertension.Ultrasonicheartbeatgraphisthemostsimpleandreliablemethodsofdiagnosis,diagnosticrateofupto98%.Twodimensionalechocardiographyshowedtumorsize,locationandactivities;Dopplerultrasoundcanshowthetumorcausedbyvalvularstenos

23、isandregurgitationdegree.Differentialdiagnosis:Leftatrialmyxomaandthrombusinleftatriumandleftatrialtumordifferentialdiagnosis.Leftatrialthrombusformationrequiresspecificetiologies,suchasatrialfibrillationormitralstenosis,myxomaisnotrequiredfortheabovefactors.Thrombosisoftheleftatrialappendageandfixe

24、dontheposteriorwalloftheleftatrium,andnoactivityoractivityrange;andthemyxomaisfixedintheatrialseptum,theactivityisverygood,butaftermitralvalveintotheleftventricle.Leftatrialtumorlocatedinthemyocardiumorlocatedonthevalveedge,noactivity;myxomaislocatedinthecavityoftheheart,theactivityisgood.Rightatria

25、lmyxomawithcardiacmetastasistumorandhyperplasiaofthemuscleoflittleLiangXiangdifferential.Metastatictumorinapatientwithmultiplerelativelythin,tumorsfromtheinferiorvenacava,afunicular,andatrialwallandpericardium.;andmyxomapatientsingoodcondition,thetumorfromtheatrialseptumorrightatriaofthelateralwall,

26、sphericaloroval,activity,occasionallyaccompaniedbypulmonaryembolism.IndividualhyperplasiaofJiXiaoliangwouldbemistakenforatrialtumor,cardiacCTscanscanbeexcludedby.Operationindication:Leftatrialmyxomafromdiagnosisprincipleshouldbeimmediatelyoperationtreatment.Especiallywithembolizationandhistoryofsync

27、opepatients,shouldbetreatedbyemergencyoperation.Operationmethod:Leftatrialmyxomaextirpationbymediansternotomy,cardiopulmonarybypassincisioninrightatriumcomplete.Fromtherightatriuminthefossaovalismidpointdofirst12cmlongitudinalincision,checkthepedicleattachmentsite,andthenalongthepedunculatedtumorres

28、ectionofpedunculatedtumoraround5mm,slowlyremovethetumor,withpolyesterfabricrepairofatrialseptaldefect.Postoperativetreatment:Generaltreatmentprinciplesandopen-heartoperationthesame.Leftatrialmyxomapathophysiologicalchangesareverysimilarinpatientswithmitralstenosis,earlytreatmentprincipleisnotaddedca

29、pacitytransition,causedbyacuteleftheartfailureandpulmonaryedema.Especiallybeforesurgeryhasemergedofleftheartfailurepatients,afterstrictrestrictionoffluidinputandinputspeed,amountofalbumin,andapplyasmallamountofdopamineanddobutamine,enhancemyocardialcontractilityandcardiacoutput.Themaincomplications1

30、:themaincauseofembolismforexplorationwhenthetransitiontomoveandsqueezetheheart,orresectionwhenforcedpullingpedunculatedtumoroccurredtumorshattering.Therefore,resectionofthetumorafterapplicationofsalineflushingchamberoftheheart,preventtumorfragmentshiddenintheatrium,ventricularmuscletrabeculaeinside.Acuteheartfailure:2majorcausesisthelackofunderstandingthepathophysiologyofthedisease,earlypostoperativesupplementaryvolumetoomuch,toosoon,oronthecardiacfunctionofpatientswithpoor,nottimelyapplicationofvasoactivedrugs.3postoperativearrhythmias:Atriallarge