Ebstein kaThixo Anomaly: Oonobangela, Iimpawu, diagnosis, Treatment

Enye yeziphene intliziyo enqabileyo yi anomaly Ebstein kaThixo. Sisifo naso apho ivelufa intliziyo tricuspid isusiwe kulo ventricle ekunene, nangona kufuneka ibe phezu umda phakathi imithambo kunye ventricles. Kuyinto flow kwegazi asezantsi kakhulu.

features yokwakheka

anomaly Ebstein kaThixo kukhokelela kukuba em- le ventricle ekunene kuba ezincinane, kwaye intendelezo ekunene - ngaphezu eqhelekileyo. ivalve caba zahlukile. Basenokuba ngendlela engafanelekanga kuphuhliswa okanye amakhaya. It miseka uphuhliso yonke inkqubo chordal kwemisipha, esilawula izixhobo tricuspid.

Ngenxa yobungakanani encinane ventricle ekunene kulo likhupha imali ngaphantsi wegazi. Kwaye ubukhulu eyandisiweyo intendelezo ekunene ngenxa yokuba ukuba ufumana inxalenye yegazi lwemithambo.

Xa izigulane ezininzi kunye anomaly Ebstein kwaye kwafunyaniswa nasiphako septal atrial kunye lomenzi foramen ovale. Esi siphene wammela abaninzi ubomi ukugcina. Ngenxa isiphako septal ukusuka ephithizelayo ekunene intendelezo shunt kwenzeka. Liyinyaniso elokuba, igazi lwemithambo kwi intendelezo lasekhohlo ngu ixutywe umfutho. Oku kubangela oksijini yindlala izicwili kunye namaziko.

Ezibangela isifo

Ngaphantsi kwe-1% yabantu abanesifo sentliziyo azalwa kwafunyaniswa anomaly Ebstein kaThixo. Izizathu uphuhliso yayo ayikwazanga zingenakuqinisekiswa ngokuchanekileyo. Ngokutsho kuqikelelo, kubonakala ngenxa okungahambi yemfuza. Kwakhona, in version mnye, zizezedini phuhliso lwe imisipha yenhliziyo leswikulu yokwamkela ngexesha amalungiselelo ukukhulelwa equlethe Lithium ityuwa.

Ehamba yesifo azalwa ngowe-50% yamatyala isiphako atrial septal. Kwezinye iimeko, kukho lunyhasho kwesingqisho. Ukuze siqonde isizathu sokuba kukho indibanisela ezithile iziphene intliziyo, oogqirha baye okwangoku baphumelele.

ezahlukeneyo kwesi sifo

anomaly Ebstein bukaThixo kubaluleke kakhulu sisifo sentliziyo naso. Oku kwenzeka kuzo zombini amakhwenkwe kunye namantombazana ngokufanayo. Ikhosi Isifo abane iya kuxhomekeka iqondo deformation ye ivalve tricuspid. Kwakhona utshintsho ebalulekileyo pathophysiological esenzeka intendelezo okulungileyo ventricle.

Kwezinye iimeko, isiphako kukophula eziphathekayo kwegazi Palsy. Kukho ukusilela intliziyo, hydrops iphuhlisa, yaye ngowe-27% yamatyala zenzeka Esibelekweni kufa umntwana. Nge ngezifo enzulu emva kokuzalwa leentsana ezisweleka kuseyinyanga yokuqala yobomi. Ngokutsho manani, oku malunga ne-25% yabo bonke abantwana anomaly Ebstein kaThixo kubonakele. Lokufunyaniswa lokukhulelwa ingafakwa nokuba kwiveki yama-20.

Malunga ne-68% zeentsana kunye esi sifo sife phambi kweenyanga ezintandathu, kwaye ukuya kwiminyaka-5 - 64% yabantwana. Eli qela libandakanya iintsana abo umsebenzi ivalve tricuspid kwaye ventricle elungile eyanelisayo. Ufe ke kwiimeko ezininzi ngenxa ukusilela inhliziyo okuqhubekayo kunye arrhythmias.

Ulwahlulo lweendidi izifo

Iingcali bachonga izigaba ezininzi zesi sifo, eyaziwa ngokuba anomaly Ebstein kaThixo. Iimpawu ziya kuxhomekeka ukusilela intliziyo ngayo inkulu. Eyona kunqabile linqanam sibizwa. izigulane enjalo baze basole leengxaki kwaye usenokuphila ubomi obuqhelekileyo. Bathwala umthambo elungileyo.

Kule yesibini Isigaba nokubonakaliswa lwezonyango libizwe. Le anomaly ibonakala ebuntwaneni kwaye ithatha kunzima ngokwaneleyo. Ngokwahlukeneyo athole amanyathelo of:

- II - oko luphawulwa kukungabikho arrhythmias intliziyo;

- II b - kuhlasela rhoqo.

Isigaba sesithathu ibizwa ngokuba ixesha decompensation ucingisise. Oku kubonakala xa umzimba akakwazi ukuba bavale umsebenzi lwentliziyo ngawo nawuphi na servo-nendlela.

Lo mfanekiso kwezonyango sifo

Ekuzalweni, abantwana anomaly Ebstein xa esibonakala cyanosis. Cyanosis emva kweenyanga 2-3 zobomi kuyehla ngenxa yokuba uxhathiso pulmonary nemithambo lifinyele. Kodwa abantwana abaneziphene okuncinci ekudibaneni kwentliziyo kwandisa amathuba okuba abulawe iingxaki cyanosis kunye nokusilela inhliziyo okuqhubekayo.

Xa abantwana anomaly Ebstein kaThixo sivame le miqondiso:

- uphelelwa ngumphefumlo nokuba kwithuba lokuphumla;

- nokudumba, leyo kungabonwa eziphelweni asezantsi;

- ukudinwa, buqapheleke ingakumbi ngexesha amandla omzimba;

- intliziyo rhythm disorder;

- cyanosis esikhumba kunye nemilebe.

Cyanosis ivela abantwana phambi kokuba iimpawu ukuphanza kwentliziyo. abantwana abadala ezikhala badla nezolo of tachycardia.

lokufunyaniswa kwesifo

Kwezinye iimeko, esi sifo uzimisele ngexesha ekhulelwe okanye kamsinya emva kokuzalwa esibhedlele. Kukho izinto ezininzi iimpawu, apho ugqirha wakho Basenokukrokrela ukuba anomaly umntwana Ebstein yakhe. Uxilongo lubandakanya auscultation, isifuba ray, echocardiography, EKG. Zonke ezi ndlela zidityaniswe ukwenzela ukuxilongwa.

ugqirha Auscultation ungeva isingqisho olwahluke, kunokuba ibe likriwa-emine emithathu okanye. Yena ugqiba eliphezulu cwaka bambombozela iSystolic of insufficiency tricuspid. Ukongeza, sho lo kukwahlukana ithoni yesibini, akukho kutshintsha ngexesha ukuphefumla. Phezulu weli cala ekhohlo kwentamo kufuneka weva ingxolo mezodiastolichesky cwaka squeaky.

On ray libonwa emthunzini yokwenza imithambo, oku kubangelwe kukunyuka kakhulu intendelezo ekunene. Pulmonary ipatheni semithambo uqobo luthuthu. Heart kaninzi kwenzeka ngohlobo lokuba sphere.

Utshintsho iqatshelwe ECG. Ngomhla ECG ubonisa iimpawu ezibonisa hypertrophy ye intendelezo ekunene, lekhefu PQ sandisiwe, kwaye kukho kwafa iyonke okanye olungaphelelanga inyanda ekunene block yesebe umqadi.

On echocardiography ungabona ukuvulwa ngokubanzi ivalve tricuspid. caba Septal zityekele phezulu.

Le amacebo okungahambi bekhubazekile

Emva kokuba ukhubazwa nokugqiba iqondo intshabalalo kukhethiwe unyango. Kakade ke, izigulana ezo kufuneka ungenelelo yotyando. Le ngaphandle kuphela kukuba kukho iqela elincinane kwizigulane kunye anomaly Ebstein likaThixo nazimpawu.

Unyango ikhutshwa phandle kuxhomekeke neminye imiba enxulumene noku. Ukuba isigulane ngesiqhelo wasinda kwasebusaneni, umsebenzi singamiswa okanye kude kube lelo xesha kude kukho iimpawu iqapheleka ukuphanza kwentliziyo.

Bonke abantwana nale ngxaki kufuneka ukubukela cardiologist ezingabantwana osebenza kakhulu yesifo sentliziyo naso. Kwanesimo sayo kufuneka esweni oogqirha inhliziyo.

Ukuba izigulane ukuncipha igazi yemiphunga kwaye kukho iimpawu ukusilela kwentliziyo, amachiza ionotropic ukumnika elimisiweyo prostogladinov amaqela E. Kwakhona eboniswe ebambe uqeqesho lactic metabolic. unyango olunjalo ukwandisa imveliso ezesifuba ukunciphisa ngasekhohlo ventricular ucinezelo ye kwicala lasekunene kwandiswa.

Kwiimeko apho kukho tachycardia, kuyimfuneko ukwenza antiarrhythmics ezizodwa unyango na.

Ubungqina obubonakalayo buxela ukuba ukuhlinza

Xa ixesha lweentsana zama ukuyenza loo msebenzi. Kodwa kuyimfuneko kwiimeko apho dysplasia ekunene ventricular libizwa kwaye lukhatshwa ukuphazamiseka kwendlela igazi flow antegrade kwisiteshi lung na. Ngelo xesha kukho ukwanda kabukhali intliziyo ekunene kwaye ucinezelo ngaxeshanye le ventricle ekhohlo.

Kuba Izimo isihlobo ziquka yobudala engama-4-5. Kodwa ke, ukuba kuyimfuneko, yenza ukusebenza kunye kubelekwa. Kwakhona akukho ukwenza utyando kunye nabo baqalise utshintsho ayibuyiseki umva komgquba nezibilini.

Abaguli anomaly Ebstein ukuba uyaziwa, akukho imisebenzi ungaphila kwi-avareji engama-20. ukufa kwabo kudla ngequbuliso. Oku kwenzeka ngenxa ventricular fibrillation kwentliziyo.

Lomsebenzi

Kuphela ngenxa utyando Ungalungisa ngokupheleleyo meko. Kulo mzekelo, oogqirha bacebisa ukuba kunokwenzeka oko xa bebudala omdala ngenxa yokuba ubukhulu intliziyo encinane kakhulu ebuntwaneni.

Olu tyando lwenziwa uqhaqho open-intliziyo, kuba amathuba kwayo kuququzelela inkqubo ecaleni cardiopulmonary. Kwemfuneko isabelo sutured ye intendelezo ekunene, kunye beentanda vana tricuspid lisingisa kwindawo ekufuphi kwisiqhelo. Kwiimeko apho oku kunokwenzeka, luthe lwancothulwa kwaye endaweni kunye sokwenziwa. Oku kukuvumela ukuba umlibale malunga yokuba isigulane waba anomaly Ebstein kaThixo. Xa izigulane abadala ukuze aqhube busebenzisa, kodwa iminyaka abantwana abangaphantsi 15 akwenza endala zeplastiki vana.

Mortality ngexesha lomsebenzi ayidluli 2-5%. Amathuba ukufa ixhomekeke kumava ugqirha wentliziyo, ukusuka vice womxhuzulane. Phantse 90% yezigulane apha ekuhambeni konyaka nga ukubuyela ubomi obuqhelekileyo.

ingqalelo ekhethekileyo kufuneka inikwe izigulane ezinjalo Iintlungu. Ngapha koko, oku kufuna indlela ekhethekileyo abaguli anomaly Ebstein kaThixo. Iimpawu zomzimba kufuneka yaziwe kwagqirha. Kufuneka Makukhunjulwe ukuba xi kula izigulane singazinzi. Ngoko ke, lo anesthesiologist ijonga izigulane bakhe emva kotyando.

iintlobo ayabiza

Kwiimeko ezininzi, ukugqiba ukuba iza kwenziwa njani loo msebenzi, ukuba kunokwenzeka kuphela xa isigulane sele phezu kwetafile yokusebenza. Ukuba kunokwenzeka ukubamba plasty vana, kukhethwa olukhetho. Kodwa ke kukho iimeko xa kufunwa kwizigulane abo anomaly Ebstein i-ngengqondo. Ngoko wabiza umba ngumntu, elijongeka umsesane, ifakelwe yi komqamelo zokwenziwa. Ngaphakathi kuyo yindlela ukuba ukuvula uvale i ebizwa ngokuba yi-yesango. Kunokuba ngomatshini okanye eziphilayo. Lwalo lokuqala senziwe intsila titanium, kwaye eyesibini kufuneka senziwe isivingco yehagu okanye iihempe yabantu inyama intliziyo.

Xa ufakela isivingco nomonde ngomatshini kufuneka rhoqo ukusela amayeza, ithinasi igazi, kodwa ukubambelela ukuyisebenzisa singahlala ixesha elide. isixhobo biological ingaphantsi ixesha elide.