Ebstein sika Anomaly: Izimbangela, Izimpawu, Ihlahlubo, Ukwelashwa

Omunye ezingavamile inhliziyo yayo yayinezinkinga ezicini kuyinto anomaly Ebstein sika. Kuyinto yesifo azalwa naso lapho tricuspid inhliziyo umphini idluliswa ku-ventricle yangakwesokudla, nakuba kumelwe abe umngcele phakathi Atria kanye ventricles. Kuyinto aphansi ukugeleza kwegazi.

izici yokwakheka

anomaly Ebstein sika kuholela yokuthi ngalo ingaphakathi of i-ventricle yangakwesokudla iba ezincane, kanye ATRIUM kwesokudla - ezingaphezu kuka kuyinsakavukela. valve Flap nazo ziyehluka. Zingase engafanele kwakhiwa noma bashiya amakhaya. It kwaphazamisa ukuthuthukiswa futhi zonke uhlelo chordal futhi kwemisipha, okuyinto ushayela apharathasi tricuspid.

Ngenxa yobukhulu encane i-ventricle yangakwesokudla kusukela lukhipha imali engaphansi kwegazi. Futhi ubukhulu okwandisiwe ATRIUM ilungelo ngenxa yokuthi ukuthi uthola ingxenye igazi emithambo yegazi.

In iziguli eziningi nge anomaly Ebstein futhi kwatholakala ukuthi nginesifo atrial septal ukwephuka kuthi foramen patent ovale. Lokhu kungapheleli eseduze abaningi ekuphileni-bonge. Ngokusebenzisa isici septal kusukela ophithizelayo kwesokudla ATRIUM shunt kwenzeka. Yiqiniso, igazi emithambo yegazi in the ATRIUM kwesokunxele kuyinto exutshwe ekhipha igazi enhliziyweni. Lokhu kubangela i-oksijini yokulamba kwezicubu eziyishumi nemitshingo.

Izimbangela zesifo

Ngaphansi kuka 1% abantu yesifo senhliziyo azalwa naso kutholakala ukuthi uphethwe yini anomaly Ebstein sika. Izizathu ukuthuthukiswa yayo ayihlonzekanga ngokunembile. Ngokusho ezicatshangwayo, kubonakala ngenxa anomalies zofuzo. Futhi, omunye inguqulo, sokuchuma of imisipha yenhliziyo ezinjalo zingaholela ekuphathekeni reception ngesikhathi sokukhulelwa amalungiselelo equkethe lithium usawoti.

Ehamba yesifo azalwa naso e-50% amacala isici atrial septal. Kwezinye izimo, kukhona ukwephulwa isigqi. Ukuze uqonde ukuthi kungani kukhona inhlanganisela ethile sesimweni inhliziyo, odokotela baye okwamanje kuphumelele.

ezahlukeneyo olungenzeka sifo

anomaly Ebstein kuba ezibucayi yesifo senhliziyo azalwa naso. Senzeka kokubili abafana namantombazane ngokufanayo. Izifo Yiqiniso futhi Izibikezelo kuzoncika degree of ukusonteka of valve tricuspid. Futhi izinguquko ezibalulekile pathophysiological ukuthi senzeka ATRIUM kwesokudla futhi ventricle.

Kwezinye izimo, isici e ukwephulwa of kwegazi fetus. Kukhona ukumelwa inhliziyo, hydrops eba, futhi u-27% amacala zenzeka utero ukufa kwengane. Nge zokugula ezijulile esezele kufa izinsana luphakathi lwenyanga yokuqala yokuphila. Ngokwezibalo, lokhu kumayelana 25% azo zonke izingane anomaly Ebstein sika itholiwe. Imiphumela yaloku kuhlola okungenhla yokukhulelwa ingafakwa ngisho isonto lama-20.

Mayelana 68% izinsana nge lesi sifo afe ngaphambi kwezinyanga eziyisithupha, futhi kuze iminyaka 5 - 64% izingane. Leli qembu kuhlanganisa izingane abanale umsebenzi valve tricuspid futhi i-ventricle yangakwesokudla kuyinto egculisayo. Bayafa ezimweni eziningi ngenxa lesichubekako inhliziyo ukwehluleka kanye arrhythmias.

Ukwahlukaniswa izinhlobo zezifo

Ochwepheshe ukukhomba izigaba eziningana lesi sifo, esaziwa ngokuthi anomaly Ebstein sika. Izimpawu kuzoncika senhliziyo nendlela enkulu. I ezingavamile kakhulu esiteji asymptomatic. Iziguli ezinjalo kungenzeka ngisho usola zokugula futhi waphila ukuphila okuvamile. Bathwala umsebenzi omuhle.

In esigabeni sesibili ukubonakaliswa emitholampilo libizwa. Lokhu anomaly livela ebuntwaneni futhi kuthatha kanzima ngokwanele. Eceleni walulama izinyathelo:

- II a - it libhekene ukungabi khona arrhythmias inhliziyo;

- II b - ukuhlaselwa ziyinsakavukela.

Isigaba sesithathu ubizwa ngokuthi inkathi decompensation eziphikelelayo. It sitholakale lapho umzimba ayikwazi isinxephezelo okwenzeka enhliziyweni yinoma yimuphi Servo-mshini.

Lwasemtholampilo isithombe lesi sifo

Ekuzalweni kwakhe, izingane anomaly Ebstein sika libhekene cyanosis. Cyanosis ngemuva kwezinyanga 2-3 zokuphila kuyehla ngenxa yokuthi ukuthi yamaphaphu ukumelana semithambo sinciphile. Kodwa ezinganeni zinokukhubazeka okuncane septum inhliziyo kwandisa ingozi yokufa emirarweni ka cyanosis futhi lesichubekako yokumelwa yinhliziyo.

Ngo izingane anomaly Ebstein uvame babona lezi izimpawu:

- iphika ngisho at isikhathi siphumule;

- edema, okuyinto kungabonwa emachosheni aphansi;

- ukukhathala, kwaphawuleka ngokukhethekile phakathi ukuzikhandla ngokomzimba;

- inhliziyo isigqi disorder;

- cyanosis kwesikhumba futhi izindebe.

Cyanosis livela izingane ngaphambi izimpawu yinhliziyo. Izingane ezindadlana kungase esekhala neziqubu yansukuzonke ye tachycardia.

sitholakele isifo

Kwezinye izimo, lesi sifo kunqunywa ngesikhathi bekhulelwe noma ngokushesha ngemva kokuzalwa esibhedlela. Kukhona izici eziningana sici, esingekho kuwo udokotela wakho Bangase basole ukuthi anomaly ingane Ebstein sika. Sifo kuhlanganisa auscultation, esifubeni esimweni, echocardiography, EKG. Zonke yalezi zindlela ekuhlanganisweni unike amandla ukusazi ngokunembile isifo esikuphethe.

udokotela Auscultation ungase uzwe isigqi ehlukile, kungaba amathathu- noma amane-emhlanjini. Yena drowns ukuthi ethule okusezingeni eliphezulu systolic nokububula ka esingamahlalakhona tricuspid. Ngaphezu kwalokho, wakhipha isinqumo sokuthi kuyinto wokuqhekeka ithoni yesibili, akashintshi ngesikhathi ukuphefumula. Phezulu onqenqemeni kwesokunxele sternum kumele wezwa mezodiastolichesky ethule umsindo Squeaky.

On esimweni ibukeke ethuthukisiwe isithunzi inhliziyo, lokhu kungenxa nokwanda okuphawulekayo le ATRIUM kwesokudla. Yamaphaphu iphethini semithambo ngokuvamile okhanyayo. Inhliziyo kuvame ngendlela uyindilinga.

Izinguquko nasemkhakheni ECG. On the ECG enezimpawu ekuboniseni hypertrophy we ATRIUM sokunene, PQ isikhawu sandisiwe, futhi kukhona ukuvinjelwa Imininingwane noma olungaphelele besigaxa kwesokudla block legatsha uvumelanise.

On echocardiography ungabona nokuvulwa kakhulu valve tricuspid. ichopho Septal is nganhlanye ebe phezulu.

Amaqhinga e anomalies azalwa naso

Ngemva Ihlahlubo ukunquma degree of ukubhujiswa kuyakhethwa ukwelashwa. Yiqiniso, iziguli ezinjalo kudingeka kokungenela kuhlinzwa. Okuwukuphela kutsi licembu lelincane kweziguli anomaly Ebstein sika asymptomatic.

Ukwelashwa Kwenziwa kuye kwamanye nezindaba ezihambisana nayo. Uma isiguli ngokuvamile ngasinda ewusana, ukusebenza kungenziwa selihlehliselwa isikhathi esinjalo kuze kukhona izimpawu evelele ephethwe yisifo senhliziyo.

Zonke izingane nale nkinga kumele ubuke wenhliziyo ezingane owelapha ngokukhethekile yesifo senhliziyo azalwa naso. Futhi isimo salo kufanele ziqashwe odokotela abahlinzayo inhliziyo.

Uma iziguli uzwakalise nokuncipha yamaphaphu ukugeleza kwegazi futhi kukhona izimpawu yinhliziyo, lo ukumnika esimisiwe ionotropic izidakamizwa prostogladinov amaqembu E. kuboniswe Futhi ubambe ukulungiswa lactic umzimba. Ukuphathwa kanjalo kungandisa okukhipha zenhliziyo ukunciphisa kwesokunxele ventricular kokucindezelwa we lelula isandla ngasohlangothini lwesokunene.

Ezimweni lapho kukhona tachycardia, kubalulekile lokufeza ukwelashwa antiarrhythmics ekhethekile.

Izinkomba ukuhlinzwa

Ngo kwesikhathi ezisanda kubelethwa uzame ukwenza msebenzi. Kodwa kubalulekile ezimweni lapho ilungelo dysplasia ventricular uphinyiswa futhi kuhambisana thuthuva ka antegrade ukugeleza kwegazi esiteshini wamaphaphu. Ngesikhathi esifanayo kukhona ukunwetshwa abukhali inhliziyo kwesokudla kanye kokucindezelwa ngesikhathi esisodwa-ventricle engakwesobunxele.

Ukuze Izimo isihlobo zihlanganisa kweminyaka eyi-4-5. Kodwa, uma kudingeka, kwenze ukusebenza nezinsana zabo. Liphinde hhayi bahlinza futhi labo abaye baqala izinguquko organic engelapheki ku izitho zangaphakathi.

Iziguli anomaly Ebstein sika kwatholakala, akukho imisebenzi ongayenza ukuze okuyisilinganiso seminyaka engu-20. Ukufa kwabo ngokuvamile okungazelelwe. Kwenzeka ngenxa ventricular fibrillation zenhliziyo.

ukusebenza

Kuphela ngokusebenzisa ukuhlinzwa ungalungisa ngokuphelele isimo. Kulokhu, odokotela batusa uma kungenzeka ukukwenza esemncane amadala ngenxa yobukhulu ezincane kakhulu inhliziyo isakhasa.

Ukusebenza wenziwa Ukuhlinzwa okukhulu kwenhliziyo, ngoba amathuba ayo yokuhlela inqubo dlula cardiopulmonary. ingxenye okweqile sutured we ATRIUM kwesokudla, futhi tricuspid umphini ezivuzayo ikhuphukela isikhundla okuyinto eduze evamile. Ezimweni lapho loku kungenteki, it is lasuswa futhi kwafakwa nge sokwenziwa. Lokhu kukuvumela ukuba bayakhohlwa yokuthi isiguli kwadingeka anomaly Ebstein sika. In iziguli omdala angase aqhube emkhakheni wezitho zokwenziwa, kodwa iminyaka izingane ezingaphansi kweminyaka engu-15 ubudala ukwakheka plastic valve.

Nokufa phakathi nokuhlinzwa ingeqi 2-5%. Amathuba okufa incike isipiliyoni udokotela ohlinza inhliziyo, futhi kusukela okuphambene adonsela phansi. Cishe ama-90% eziguli ngokuhamba konyaka ungabuyela ukuphila okuvamile.

Ngokukhethekile kufanele inikwe enjalo iziguli nababulala izinzwa. Phela, kudinga indlela ekhethekile iziguli anomaly Ebstein sika. Izici ngokusungula indlela yokubulala izinzwa kufanele ezaziwa udokotela. Kumele kukhunjulwe ukuthi bakufake ingcindezi yokuthi kulezi iziguli kungenzeka engazinzile. Ngakho-ke, lo obulala izinzwa uqaphe neziguli zakhe ngemva kokuhlinzwa.

izinhlobo amazinyo okufakelwa

Ezimweni eziningi, ukunquma ukuthi oyokwenziwa ukusebenza, kungenzeka kuphela uma isiguli isivele etafuleni yokusebenza. Uma kungenzeka ukuba ubambe plasty valve, kubonelelwa kakhulu ama-le ndlela. Kodwa kunezimo lapho sokwenziwa iyadingeka iziguli anomaly Ebstein sika. Ngakho ngokuthi valve yokufakelwa, okuyinto ifana indandatho, efakwe ngumuntu zokwenziwa umcamelo. Ngaphakathi it is a indlela ezingaba uqale futhi uphethe okuthiwa kwesango. Kungaba mechanical noma ezingamagciwane. Samuntu sokuqala senziwa we ingxubevange titanium, kanti eyesibili singenziwa ka valve lengulube noma womuntu inhliziyo izicubu amahembe.

Lapho ufaka valve nesineke mechanical kumele njalo baphuze imithi, igazi thinners, kodwa ukunamathela usebenze kungaba isikhathi eside. kudivayisi eziphilayo lingaphansi okuhlala.