Ebstein a Anomaly: Zoyambitsa, Zizindikiro, Matendawa, Chithandizo

Mmodzi vuto osowa mtima ndi anomaly Ebstein a. Ndi matenda obadwa imene tricuspid vavu mtima asamukira ventricle bwino, ngakhale izo ziyenera kukhala pa malire pakati pa atria ndi ventricles. Ndi magazi kwambiri m'munsi otaya.

mbali anatomical

anomaly Ebstein Adzapeza chakuti M'mimbamo wa ventricle bwino amakhala ang'onoang'ono, ndi atrium bwino - kuposa mwakale. Khumudwitsidwa vavu amasiyana. Iwo akhoza molakwika kwambiri, kapena pokhala. Iwo akusokoneza chitukuko ndi onse chordal ndi akulu dongosolo, amene amayendetsa ndi zida tricuspid.

Chifukwa cha kukula yaing'ono ya ventricle bwino kwa izo limatulutsa zochepa kuchuluka kwa magazi. Ndi kuchuluka kukula kwa atrium bwino chifukwa chakuti zifika mbali ya magazi venous.

Odwala ambiri ndi anomaly Ebstein ndipo anandipeza ndi matenda ndi setifiketi foramen ovale. Chilema ina anthu ambiri chopulumutsa moyo. Kudzera septal chilema kuchokera yodzaza bwino atrium shunt amapezeka. Zoona, magazi venous mu atrium chatsalira wothira wolumikizira. Izi zimapangitsa mpweya njala ya zimakhala ndi ziwalo.

Zimayambitsa matenda

Zosakwana 1% ya anthu ndi matenda obadwa nawo a mtima matenda anomaly Ebstein a. Zifukwa chitukuko sanathe titsimikize molondola. Malinga ndi mfundo, zikuoneka chifukwa anomalies chibadwa. Komanso, mu Baibulo limodzi, chifukwa ndi chitukuko cha minofu mtima kungachititse phwando nthawi yokonzekera mimba munali lifiyamu mchere.

Limodzi ndi matenda obadwa nawo mu 50% ya milandu matenda. Nthawi zina, pali kuphwanya mungoli. Kuti timvetse chifukwa pali kuphatikiza zina vuto mtima, madokotala sizinathe anapambana.

mitundu zotheka matendawa

anomaly Ebstein ndi yovuta kobadwa nako matenda a mtima. Zimachitika anyamata ndi atsikana omwe. Disease Inde ndi madokotala ananena zawo kudzadalira mlingo wa mapindikidwe wa valavu tricuspid. Komanso kusintha zofunika pathophysiological zimene zinachitika mu atrium chabwino ndi ventricle.

Nthawi zina, chilema pakati zinthu kufalitsa yoopsa. Pali mtima kulephera, hydrops akufotokozera, ndi 27% milandu zimachitika utero imfa ya mwana. Ndi kudwala kwambiri atabadwa ana amafa pasanathe mwezi woyamba wa moyo. Malinga ndi kafukufuku, izi ndi pafupifupi 25% ya ana onse ndi anomaly Ebstein anali wapezeka. Matenda a mimba angathe kuikidwa ngakhale pa sabata la 20.

About 68% wa makanda ndi matenda kufa miyezi isanu ndi umodzi, ndipo mpaka zaka 5 - 64% ya ana. gulu Izi zikuphatikizapo ana amene ntchito ya valavu tricuspid ndi ventricle bwino ndi zogwira mtima. Iwo amafa nthawi zambiri chifukwa cholephera mwapang'onopang'ono mtima ndi arrhythmias.

Gulu la matenda

Akatswiri kudziwa magawo angapo a matenda, lotchedwa anomaly Ebstein a. Zizindikiro kudzadalira kulephera yaikulu motani mtima. The yosowa kwambiri ndi gawo asymptomatic. odwala sizingakhale ngakhale kuganiza za kudwala ndi munthu wabwinobwino. Iwo amanyamula zolimbitsa wabwino.

Mu gawo lachiwiri mawonetseredwe matenda kulitchula. anomaly Zikuoneka ana natenga yovuta mokwanira. Payokha anachira mapazi a:

- II ndi - izo amakhala ndi pakalibe arrhythmias mtima;

- II wa B - matenda pafupipafupi.

Mbali yachitatu amatchedwa nthawi decompensation chosatha. Iwo amawapeza pamene thupi sangathe kukwaniritsa ntchito ya mtima ndi aliyense servo-limagwirira.

The matenda chithunzi cha matenda

Pobadwa, ana ndi anomaly Ebstein ndi yodziwika ndi cyanosis. Cyanosis pambuyo miyezi 2-3 moyo yafupika chifukwa chakuti m'mapapo mwanga mtima kukana yafupika. Koma ana ndi zilema pang'ono septum mtima kumaonjezera ngozi ya imfa ku matenda a cyanosis ndi kupata kulephera mtima.

Ana ndi anomaly Ebstein ndi zambiri anati zizindikiro zimenezi:

- kufupika kwa mpweya ngakhale pa nthawi mpumulo;

- edema, amene Tingaone pa malekezero m'munsi;

- kutopa, makamaka noticeable pa zofuna kukhetsa thukuta;

- mtima mungoli matenda;

- cyanosis wa khungu ndi milomo.

Cyanosis limapezeka ana pamaso zizindikiro za matenda a mtima. Ana okulirapo angadandaule za ayi kobwereza wa tachycardia.

matenda a matenda

Nthawi zina, matenda anatsimikiza pa mimba kapena chakachi kubadwa mu chipatala. Pali zinthu zingapo khalidwe, zomwe dokotala anu mwina akuganiza kuti anomaly mwana Ebstein a. Matendawa zikuphatikizapo auscultation chifuwa radiography, echocardiography, EKG. Zonse mwa njira zimenezi osakaniza athe matenda zolondola.

Auscultation dokotala amve kayimbidwe kotani chosiyana zingakhale three- kapena zinayi khola. Iye drowns kunja chete mkulu systolic kudandaula za insufficiency tricuspid. Komanso, kutchulidwa ndi akuwaza wa kamvekedwe chachiwiri, sizisintha pa kupuma. Pamwamba pa nsonga yatsala sternum ayenera anamva mezodiastolichesky chete squeaky phokoso.

Pa radiography visualized kuzikonzanso mtima mthunzi, ichi ndi chifukwa kuwonjezeka kwambiri mu atrium bwino. M'mapapo mwanga mtima chitsanzo ambiri wotumbululuka. Mtima nthawi zimatengera mtundu wa dera a.

Kusintha anati mu ECG lapansi. Pa ECG amasonyeza zizindikiro kusonyeza hypertrophy wa atrium lamanja imeneyi PQ ndi anawonjezera, ndipo pali okwana kapena tsankho anatseka misewu ya mtolo bwino nthambi chipika mtengo.

Pa echocardiography kuwona ndikatsegule wa valavu tricuspid. Septal khumudwitsidwa si olondola cha pamwamba.

Machenjerero mu anomalies kobadwa nako

Pambuyo matenda ndi kudziwa mlingo wa chiwonongeko anasankha chithandizo. Ndithudi, odwala amenewo sayenela alowererepo pa opaleshoni. Nokha ndiye kuti kagulu ka odwala anomaly Ebstein ndi asymptomatic.

Chithandizo ikuchitika malingana nkhani zina. Ngati wodwala bwinobwino anapulumuka wakhanda, ntchito akhoza kulephereka mpaka nthawi imeneyi mpaka pali kutchulidwa zizindikiro za matenda a mtima.

ana onse ndi vutoli muyenera kusamala Dokotala cardiologist amene imakhazikika matenda obadwa nawo a mtima. Ndipo udindo wake ayenera kuunikira madokotala mtima.

Ngati odwala anasonyeza kuchepetsa otaya m'mapapo mwanga magazi ndipo pali zizindikiro za matenda a mtima, ndi kulowetsedwa yoikidwiratu ionotropic mankhwala prostogladinov magulu E. amasonyezanso akugwira kuwukonza acidosis kagayidwe kachakudya. Zinthu zimenezi angadwale linanena bungwe mtima ndi kuchepetsa kumanzere yamitsempha yamagazi psinjika a mbali anatambasula dzanja lamanja.

Nthawi kumene tachycardia, m'pofunika kuchita chithandizo antiarrhythmics wapadera.

Zikuonetsa kwa opaleshoni

Mu nthawi neonatal yesetsani kuchita opaleshoni. Koma m'pofunika pa nthawi imene pomwe yamitsempha yamagazi dysplasia limachulidwa ndi limodzi mwa chisokonezo akulowera antegrade magazi m'mapapo njira. Pa nthawi yomweyo pali kuwonjezeka lakuthwa wa mtima wabwino ndi psinjika munthawi yomweyo ya ventricle kumanzere.

Pakuti contraindications wachibale monga zaka 4-5. Koma, ngati n'koyenera, kupanga ntchito ndi ana. Amagwiritsidwanso osati opaleshoni ndi amene anayamba organic inasinthiratu mu ziwalo mkati.

Odwala amene anomaly Ebstein a anapeza palibe ntchito akhoza kukhala kuti avareji zaka 20. Imfa yawo ndi imfa zambiri mwadzidzidzi. Kumachitika chifukwa yamitsempha yamagazi fibrillation za mtima.

opareshoni

Kokha kupyolera opaleshoni akhoza kwathunthu kukonza zinthu. Pankhaniyi, madokotala amalangiza ngati n'kotheka kuchita izo ali wamkulu chifukwa cha kukula chochepa mtima ali wakhanda.

amachita opaleshoni yotsegula mtima, chifukwa mwayi wake yokonza ndondomeko kulambalala mouzirira. Owonjezera sutured gawo la atrium lamanja, ndi tricuspid vavu seams limatuluka malo amene ali pafupi ndi wabwinobwino. Pa nthawi imene zimenezi sizingatheke, izo chikutha n'kuikapo prosthesis a. Zimenezi zimathandiza kuiwala zoti wodwala anali anomaly Ebstein a. Odwala wamkulu akhoza kuchititsa yopanga, koma zaka ana osaposa 15 zaka wodzipangitsa vavu pulasitiki.

Imfa pa ntchito si upambana 2-5%. Mwina imfa zimadalira zinachitikira dokotala mtima, ndi kwa wachiwiri yokoka. Pafupifupi 90% ya odwala mwa chaka akhoza kubwerera ku moyo wabwinobwino.

chidwi ayenera kuperekedwa kwa odwala ngati Anesthesiologists. Ndipotu, pamafunika njira yapadera kwa odwala amene Ebstein a anomaly. Features wa mankhwala oletsa ululu ayenera kudziwika kwa dokotala. Ziyenera adzanyamula kukumbukira kuti kukakamiza odwala awa akhoza kukhala osakhazikika. Choncho, dzanzi oyang'anira odwala ake ndi pambuyo opaleshoni.

mitundu ya kupeza mano otere

Nthawi zambiri, kusankha momwe idzachitike opareshoni, n'zotheka pokhapokha ngati wodwala kale pa tebulo ntchito. Ngati n'zotheka kugwira valavu plasty, zokonda zapatsidwa kwa njirayi. Koma pali zochitika pamene prosthesis ali wofunikila odwala amene ali anomaly Ebstein a. Chotero anaitana vavu yokumba, zomwe zikuwoneka ngati mphete, zokuzira ndi pilo kupanga. Mkati ndi limagwirira angatsegule ndi kutseka otchedwa pachipata. Zimakhala makina kapena tizilombo. Chimake choyamba unapangidwa aloyi titaniyamu, ndi wachiwiri akhale wa valavu wa nkhumba kapena anthu mtima malaya minofu.

Pamene inu kukhazikitsa makina vavu wodwala ayenera nthawi zonse kumwa mankhwala, thinners magazi, koma kutsatira ntchito zikhoza yayitali. Tizilombo chipangizo wochepa cholimba.