雜交技術(shù)在肌部室間隔缺損治療中的應(yīng)

雜交技術(shù)在肌部室間隔缺損治療中的應(yīng)用安琪石應(yīng)康四川大學(xué)華西醫(yī)院胸心血管外科.研究背景外科直視手術(shù)修補(bǔ)肌部室間隔缺（MVSD）存在：顯露差、右室切開率高，殘余分流的發(fā)生率高對(duì)于年齡小、體重輕，且缺損較大，需早期干預(yù)的患兒，經(jīng)皮介入封堵受外周血管大小等條件限制經(jīng)皮介入封堵肌部室間隔缺（MVSD）具有微創(chuàng)的優(yōu)勢(shì)。也存在建立軌道復(fù)雜、成功率低等弊端.研究背景經(jīng)心室穿刺封堵室缺（perventriculardeviceclosure）可能是除傳統(tǒng)手術(shù)和經(jīng)皮介入以外，治療MVSD的第三種選擇，使部分病人仍然有機(jī)會(huì)得到相對(duì)的微創(chuàng)手術(shù)。.資料與方法2007年5月-2009年7月，12例罹患單發(fā)或多發(fā)MVSD的先天性心臟病患者，1例心臟刀刺傷導(dǎo)致MVSD，均行經(jīng)心室穿刺封堵室缺年齡:6月-22歲，平均4.61±6.8歲其中5例＜1歲，1~3歲3例.資料與方法——肌部VSD體重：平均15.8±16.1kg（5-60kg）。單發(fā)肌部VSD7例，其中，外傷性肌部室缺1例；多發(fā)肌部VSD6例（兩處缺損5例，4處缺損1例）。缺損直徑2-10mm，平均5.4±2.7mm。.資料與方法——肌部VSDNo.Sex(M/F)Age(years)Weight(kg)Diameter(mm)/LocationAssociatedCHDsMVSD1MVSD2MVSD3MVSD41F8/1287/middle——————PAPVC,ASD(posterior,12mm),PVSD(12mm),TR(moderate)

2M3125/middle————————3M6/125.58/middle————————4F6/1253/middle——————PVSD(8mm)5M10/1288/middle————————6F143212/middle————————7M22607/apical——————.No.Sex(M/F)Age(years)Weight(kg)Diameter(mm)/LocationAssociatedCHDsMVSD1MVSD2MVSD3MVSD48F9/12610/anterior6/apical————ASD(ostiumsecundum,6mm)

9F19/129.56/apical4/apical————PDA(10mm×8mm),PVSD(15mm)10M1127.54/posterior2/posterior————PVSD(25mm),TR(mild)

11M4/1276/posterior3/apicalPVSD

(15mm)12M4173/middle3/middle——————13F9/1263.5/anterior3.5/anterior3/middle3/posteriorASD(ostiumsecundum,12mm)

資料與方法——肌部VSD.資料與方法——封堵技術(shù)氣管插管后常規(guī)放入TEE，對(duì)室缺的大小、位置、與主動(dòng)脈瓣的關(guān)系再次予以評(píng)估，再次檢查各瓣膜的開閉情況多發(fā)MVSD根據(jù)其大小、相互的位置關(guān)系，確定封堵的先后順序、封堵方式、及封堵器的型號(hào)單純肌部MVSD干備體外循環(huán)，合并其他需CPB下矯治畸形的MVSD同時(shí)準(zhǔn)備體外循環(huán).資料與方法——封堵技術(shù)切口：1）單純MVSD，行劍突下3-4cm小切口，鋸開下分胸骨，切開心包并懸吊，顯露右心室游離壁。2）如MVSD合并其他需同期矯治畸形，行常規(guī)胸骨正中切口

...資料與方法——封堵技術(shù)穿刺點(diǎn)選擇：開胸后TEE再次確認(rèn)VSD位置，外科醫(yī)生以手指輕壓右心室表面，配合TEE上手指圖象確定穿刺點(diǎn)及角度。....資料與方法——封堵技術(shù)穿刺及軌道建立1）所選穿刺點(diǎn)帶墊片“U”字縫合一針、20G穿刺針穿刺，右心室導(dǎo)入引導(dǎo)鋼絲。在TEE引導(dǎo)下將引導(dǎo)鋼絲穿過VSD進(jìn)入左心室腔建立軌道。2）沿鋼絲置入8F動(dòng)脈鞘至左心室腔，退出鋼絲及內(nèi)鞘，并將安裝好封堵器的裝載器接于鞘管尾部，輸送封堵器并分別打開左右傘。.資料與方法——封堵器肌部封堵器使用PDA封堵器（心尖肌部缺損）..資料與方法——隨訪出院前及出院后6個(gè)月時(shí)接受TTE和心電圖檢查，記錄有無殘余分流、新出現(xiàn)的二尖瓣及三尖瓣反流、心律失常.結(jié)果.結(jié)果——MVSD單發(fā)VSD使用單枚封堵器7例，同期膜部VSD封堵1例多發(fā)VSD因相距較近，使用單枚封堵器3例。其中一例存在殘余分流，同期直視修復(fù)使用2枚VSD封堵器3例、3枚VSD封堵器1例，其中2例同時(shí)施行了ASD封堵MVSD封堵后，CPB下矯治合并畸形4例.病例1:MVSD×2+ASD.MVSD×2+ASD第一枚封堵器釋放.MVSD×2+ASD第二根引導(dǎo)鋼絲（視頻）.MVSD×2+ASD第一枚傘釋放后的第二支鞘管.MVSD×2+ASD兩枚VSD、一枚ASD封堵器釋放.病例二:心尖部MVSD×2+PVSD一例合并PVSD的患兒，其心尖部兩肌部缺損直徑分別為6mm和4mm，相距5mm術(shù)中于非CPB下使用8、6mmPDA封堵器成功關(guān)閉兩個(gè)缺損。體外循環(huán)下修補(bǔ)大的PVSD.結(jié)果——MVSD×2兩枚PDA封堵器.病例3：單枚封堵器封堵2個(gè)MVSD該技術(shù)在兩個(gè)相鄰的肌部室缺中使用...病例4：MVSD×4+ASD9月，6kg，重度PH4個(gè)MVSD、合并ASD使用3枚MVSD封堵器、1枚ASD封堵器，全部封堵成功4個(gè)MVSD中，有3個(gè)缺損彼此相鄰.病例4：MVSD×4+ASD需要預(yù)先建立兩個(gè)軌道第一個(gè)鞘管進(jìn)入并封堵室缺第二個(gè)鞘管進(jìn)入并封堵室缺封堵第三個(gè)室缺.圖中見2枚MVSD傘和ASD傘.圖中見3個(gè)肌部傘.手術(shù)后X光片見3枚肌部VSD傘和ASD傘..討論經(jīng)皮介入封堵存在的不足１）受病人年齡和體重的限制２）對(duì)外周血管潛在的損傷３）室缺靠室隔前份或心尖時(shí)，經(jīng)皮封堵失敗的可能性大４）存在射線照射對(duì)嬰幼兒的影響.討論心室穿刺封堵術(shù)的優(yōu)勢(shì)有:1）操作靈活、準(zhǔn)確放傘2）封堵器收放容易便于選傘3）實(shí)時(shí)評(píng)估療效，減少殘余漏；合理選傘.討論4）合并其他畸形時(shí)，如封堵成功，可明顯縮短體外循環(huán)時(shí)間；單純MVSD可避免體外循環(huán)5）避免心室切開，保護(hù)右室功能，減少心律失常的發(fā)生.討論□超聲心動(dòng)圖的重要性１）術(shù)前超聲檢查；２）TEE在穿刺點(diǎn)選擇中的引導(dǎo)作用；３）TEE在軌道建立過程中的引導(dǎo)作用；４）超聲在評(píng)估中的作用.超聲醫(yī)生-外科醫(yī)生的配合保證手術(shù)的成功超聲醫(yī)生對(duì)外科的理解外科醫(yī)生對(duì)圖像的理解兩者的結(jié)合：經(jīng)超聲醫(yī)生的手得到圖像經(jīng)外科醫(yī)生的手實(shí)施操作。.結(jié)論經(jīng)心室穿刺封堵MVSD是一種安全、有效和微創(chuàng)的治療手段，其短期效果滿意經(jīng)心室穿刺與經(jīng)皮介入封堵肌部室缺相比，基本不受患兒年齡及缺損位置限制，且選傘靈活其遠(yuǎn)期效果，如心律失常、心室功能的遠(yuǎn)期影響尚有待進(jìn)一步觀察...謝謝.英文版.TheapplicationofhybridtechniqueformuscularventricularseptaldefectsYing-kangShi,QiAnDepartmentofThoracicandCardiovascularSurgeryWestChinaHospital,SichuanUniversity.BackgroundSurgicalrepairofMVSD:poorexposure,chanceofventriculotomy,andhighincidenceofresidualshunt;Transcatheterclosure:limitedbyvesselconditioninyoungchildrenneedingearlyintervention;Transcatheterclosure:minimallyinvasive,buthardtoestablishthepathwayandthesuccessrateislower..BackgroundPerventriculardeviceclosure(PDC)maybethethirdchoiceandprobablyprovidespartofthepatientsachancetogetminimallyinvasivetreatmentcomparedtoon-pumpsurgery.Patients&MethodsMay2007toJuly2009,thirteenpatientswithsingleormultipleMVSDs,includingatraumaticone(Knife)receivedPDC;Age:6M-22Y,average4.61±6.8Y5cases＜1Y,3caseswithin1Yto3Y.Patients&MethodsWeight:15.8±16.1kg(range5.0-60kg);SevensingleMVSDs(includingthetraumaticone)andsixmultipleMVSDs(fivewithtwodefectsandonewithfour);Diameterofdefect:5.2±2.7mm(range2-12mm)..Patients&MethodsNo.Sex(M/F)Age(years)Weight(kg)Diameter(mm)/LocationAssociatedCHDsMVSD1MVSD2MVSD3MVSD41F8/1287/middle——————PAPVC,ASD(posterior,12mm),PVSD(12mm),TR(moderate)

2M3125/middle————————3M6/125.58/middle————————4F6/1253/middle——————PVSD(8mm)5M10/1288/middle————————6F143212/middle————————7M22607/apical——————.No.Sex(M/F)Age(years)Weight(kg)Diameter(mm)/LocationAssociatedCHDsMVSD1MVSD2MVSD3MVSD48F9/12610/anterior6/apical————ASD(ostiumsecundum,6mm)

9F19/129.56/apical4/apical————PDA(10mm×8mm),PVSD(15mm)10M1127.54/posterior2/posterior————PVSD(25mm),TR(mild)

11M4/1276/posterior3/apicalPVSD

(15mm)12M4173/middle3/middle——————13F9/1263.5/anterior3.5/anterior3/middle3/posteriorASD(ostiumsecundum,12mm)

Patients&Methods.Technique--generalconsiderationTEEafterintubation,evaluatethedefect(diameter,location,relationshipwithAV)andcheckthevalveagain;Decidethedevicesize,processandsequenceofclosureformultipleMVSDs;CPBreadyforassociatedCHDs,andjuststandbywithoutprimeforisolatedMVSD..Technique--incisionSub-xiphoid3-4cmincisionandpartialsternotomyforisolatedMVSD;ConventionalmediansternotomyifwithassociatedCHDs.

...Technique—puncturelocationUndercontinuousTEEmonitor,theRVfreewallwasgentlydepressedwiththesurgeon’indexfinger.ThisdepressionoftheRVfreewallcouldbeclearlyvisualizedbyTEE,anditsspacialrelationshiptothedefectwasdetermined.....Technique—pathwayestablishmentapurse-stringsutureatthelocation,puncturewitha20gaugeneedle,guidewirewasintroducedintotheLVthroughthedefectunderTEEAdeliversheathwasadvancedoverthewireintotheLV,thenthedevicewasdeliveredthroughthesheath..Technique--occluderMuscularoccluderPDAoccluder(forapicaldefect)..Follow-upTTEandECGatdischargeand6monthsafterdischarge;Anyresidualshunt,arrhythmiaornewvalveproblemwouldberecorded..Results.ResultsSevendevicesforsevensingleMVSDs(aPVSDPDCsimultaneously);Threesingledevicesforthreepairsofnearbydefects(oneresidualshunt,conventionalrepairedlater);Threecasesusingtwodevices,andonecasewiththreedevices(twoASDPDCsimultaneously)Fourcasesreceivedon-pumpsurgeryfortheirassociatedCHDs..Case1:MVSD×2+ASD.MVSD×2+ASDReleaseofthefirstdevice.MVSD×2+ASDThesecondguidewire(video).MVSD×2+ASDThesecondsheathafterthereleaseofthefirstdevice.MVSD×2+ASDTwoMVSDdevicesandoneASDdevice(video).Case2:twoapicalMVSDs+PVSDTwoapicalMVSDs(6mmand4mm),5mmapart;ClosedbytwoPDAoccluders(8mmand6mm)withoutCPB.ThebigPVSDreceivedon-pumprepaire.MVSD×2TwoPDAoccluders.Case3:singledevicefortwoMVSDs

Thistechniqueisapplicablefortwonearbydefects...Case4:MVSD×4+ASD9Mgirl,6kg,severePHMVSD×4plusoneASDAlldefectswereclosedsuccessfullywiththreeMVSDdevicesandoneASDdevice;Threeoutofthefourmusculardefectswereclosedtoeachother..Case4:MVSD×4+ASDTwopathwaysshouldbeestablishedinadvanceThefirstsheathinandMVSDclosedThesecondsheathinandMVSDclosedThethirdMVSDbeclosed.TwoMVSDdevicesandoneASDdevice.ThreeMVSDdevices.ThreeMVSDdevicesandoneASDdevicewereseenonthepost-surgX-ray..DiscussionDisadvantagesoftranscatheterclosureLimitedbypatientageandweight;Potentialvesseldamage;HardtoestablishapathwayinapicaloranteriorMVSDsUndesirableradiation(esp.forsmallbabies).DiscussionAdvantagesofP