Ebstein inonyanya kuratidza chirwere chepfungwa chinokonzera chirwere chepfungwa chinoratidzirwa nekusimudzirwa kwechirwere chetricuspid uye ventricle yakarurama . Zvichienderana nekuoma kwezvinhu izvi zvisina kukodzera, zviratidzo zvinowanikwa nevaya vakaberekwa neEbstein anomaly zvinogona kusiyanisa zvakanyanya.
Vamwe vana vane chirwere ichi vanorwara zvakanyanya pakuberekwa. Vamwe vane Ebstein vanorarama vanorarama kuva vanhu vakuru pasina zviratidzo zvachose.
Zvisinei, anenge munhu wose akaberekwa ane chirwere ichi achakurisa matambudziko emwoyo nokukurumidza kana kuti gare gare.
Chii chinonzi Ebstein Anomaly?
Chaizvoizvo, Ebstein anomaly inokonzerwa nokukundikana kwevhavhavha yegricus kuti ikure zvakakwana mumucheche. Mashizha emagetsi eti tricuspid haafaniri kupinda munzvimbo yavo yakakwana, pamakakatanwa eatrium chaiyo uye ventricle yakarurama, munguva yekuvandudza. Pane kudaro, mapepa acho anoputika pasi, mukati me ventricle yakarurama. Uyezve, mapepa acho pachawo anowanzoputirwa ("akabatanidzwa") kumadziro ehurukuro yakarurama, uye naizvozvo usasvinura nekuvhara zvakakodzera.
Nemhaka yekuti valve inoputika muEbstein inonyangadza inodzingwa pasi, chikamu chebhururo yakarurama iyo iri pamusoro pechigadzirwa chisina kujairika chinonzi "valrified valve" chiri "atrialized." Izvi zvinoreva kuti imba yepamusoro yemoyo inenge iine zvisikwa zvepanyama asiwo chikamu yezvinodiwa ventricular tissue.
Cardiac Matambudziko
Nemhaka yekusava nemafungiro uye kusvibiswa kwevhirivha inonzi tricuspid iyo inowanikwa muEbstein anomaly, irovhavha rinowanzo "rakanyangadza." Somugumisiro, kunyanya kushanduka kunowanzoita kukuratidzwa kukuru kwechiitiko ichi.
Mukuwedzera, kuwedzerwa kwemugove we ventricle yakarurama iyo iri pamusoro pemharavha yakasununguka inokonzerawo zvinetso.
Chikamu cheatrialized chekodzero yakarurama inorova apo zororo rose rakarurama rinorova, uye kwete apo inoramu yakanaka inorova. Izvi zvinopesana nemisimba mukati memukamuri yeperiyano zvinopesana nekutengesa kwemaitiro uye kunounzawo tsika yeropa mukati mekodzero yakakodzera kuti iparadze-chiitiko chinogona kubudisa ropa.
Kuoma kweEbstein kunetseka kwakabatana nehuwandu hwekuparadzanisa uye kusagadzikana kwevhidhiyo inonzi tricuspid, uye chiyero chakanaka chechipiri chinonzi ventricular chinowanzosvibiswa. Vanhu vakaberekerwa neEbstein anomaly vane zvishomanana (kana kwete) zviratidzo zvinowanzova nediki shovl displacement.
Vanhu vakaberekerwa neEbstein vanonyanyawo vane mikana yakawanda yekuva nemamwe matambudziko emwoyo. Izvi zvinosanganisira patent foramen ovale , kupera septal defect , pulmonary outflow kudzivisa, patent ductus arteriosus , ventricular septal defect, uye dzimwe nzira dzemagetsi mumoyo unogona kubudisa mwoyo arrhythmias . Kana imwe kana akawanda emamwe matambudziko epabonde aripo, zviratidzo nemigumisiro yevanhu vane Ebstein vanonyanya kunowedzera kuipa.
Zvinokonzera
Ebstein inoratidzika inowanikwa munenge 1 kubva ku20 000 kuberekwa patsva.
Nepo mamwe mazamu echinyakare akabatanidzwa neEbstein anomaly, hapana kuchinja kwakasiyana kunofungidzirwa kuti ndicho chikonzero chikuru chechiitiko ichi. Kushamwaridzana pakati peEbstein anomaly uye kushandiswa kwaamai vane pamuviri we lithiamu kana benzodiazepines zvakataurwa, asi hapana hurumende yekushungurudza yakaratidza. Pamusoro pezvo, Ebstein anomaly inoita seinoitika pasina.
Zviratidzo
Zviratidzo zvinowanikwa nevanhu vane Ebstein vanoratidzika zvakasiyana-siyana, zvichienderana nehupamhi hwechirwere chechivharo chisina kunaka uye pakuvapo kana kusavapo kwemamwe matambudziko emwoyo.
Vana vakaberekwa vane tsvina yakaoma inokonzerwa neEvstein anomaly vanowanzove nemamwe matambudziko emwoyo pamwe chete, uye vanogona kurwara zvikuru kubva pakuberekwa.
Ivana ava vanowanzova ne- cyanosis yakawanda (yakadzika mukati memhepo yakisiya), dyspnea , utera, uye edema (kuzvimba).
Vana vakaberekerwa neEbstein anomaly vane maitiro makuru ekudzoka zvakare, asi hapana zvimwe zvakakomba zvepfungwa dzemwoyo, vanogona kunge vane vana vakagwinya asi kazhinji vanowedzera kukanganisa kwemwoyo kusina kana paudiki kana kuti munhu mukuru.
Kune rumwe rutivi, kana tricuspid valve kusagadzikana kune unyoro chete, munhu ane Ebstein anomaly anogona kuramba asina zviratidzo zvehupenyu hwavo hwose.
Kune sangano rakasimba pakati peEbstein anomaly uye anomalous magetsi nzira mumoyo. Izvo zvinonzi "nzira dzinoshandiswa nadzo" dzinogadzira kusagadzikana kwemasvomhu emagetsi pakati peimwe yeatria uye imwe yevhirikiri; muEbstein vanaly, ivo vanowanzotanidza hutano hwakanaka ne ventricle yakarurama.
Nzira idzi dzinowanzoita kuti chikwata chepamusoro che tachycardia chinonzi atrioventricular reentrant reachrant tachycardia (AVRT) . Dzimwe nguva nzira idzi dzakafanana dzinogona kukonzera Wolff Parkinson White syndrome , iyo inogona kutungamirira kwete AVRT chete, asiwo kune magythmiyasi akaipisisa, kusanganisira ventricular fibrillation . Somugumisiro, nzira idzi dzekushandisa dzinogona kuwedzera ngozi yekufa kamwe kamwe.
Nemhaka yokuti Ebstein anomaly anowanzokonzera kusvibiswa kweropa ropa mukati mekutenderera kwakakodzera, ropa rinoputika rinowanzoita ipapo. Kana izvi zvipfeko zveropa zvichivhara (kureva kuti, bvisa), vanogona kufamba kuburikidza nekuparadzirwa uye kukonzera kukanganisa kwechivi. Saka Ebstein inoratidzirwa inobatanidza nekuwedzera kwezvinyorwa zveplummum embolus , uye (nokuti inoputika kubva pa right atrium inogona kufamba kuburikidza nepa patent foramen ovale kuruboshwe rwetrium), zvinogona kukonzera chirwere.
Zvinonyanya kukonzera rufu kubva kuna Ebstein zvakashata ndezvekushaya moyo uye kufa kamwe kamwe.
Kuziva
Chiyero chinokosha pakuongorora Ebstein chinonyanya kushandiswa ndiyo echocardiogram -usu, test echo transesophageal echo inopa mhinduro yakarurama kwazvo. Nekodhidhirodhiyo, kuvapo uye chiyero chechirwere chinonzi tricuspid chisina kuwanikwa chinogona kunyatsoongororwa, uye dzimwe dzakawanda dzepenital heart defect dzinogona kuvapo dzinogonawo kuonekwa.
Kuvanhu vakuru nevana vakuru vari kuwana kugadziriswa kwavo kwekutanga kweEbstein kunonyanya, kuongororwa kwekuenzanisa kunowanzoitwa kuti vaongorore basa ravo rekuita basa, ropa rekuisa muviri mukati mekurovedza muviri, uye kupindura kwemaitiro emwoyo yavo nekudzvinyirirwa kweropa kuti vashandise. Izvi zviyero zvinobatsira mukutonga kukanganisa kwese kwechimiro chepfungwa yavo, uye kudikanwa uye kukurumidza kwekurapwa kwekuvhiya.
Zvakakoshawo kuongorora vanhu vane Ebstein vane hanya nokuda kwekuvapo kwechimiro arrhythmias. Kuwedzera kune electrocardiograms gore negore (ECG) uye kuvhara kwekuchengetedza kweEECG , vazhinji vevanhu ava vanofanira kuongororwa ne-electrophysiologist yemwoyo pavanenge vaongororwa, kuti vaongorore mukana wavo wekuita zvirongwa zvingangova nengozi.
Kurapa
Muzvinyorwa, kana Ebstein anomaly iri kubudisa zviratidzo zvakakura, kurapwa uku kugadzirwa.
Kuvhima muzvitsva zvakanyanya neEbstein anomaly yakawandisa kazhinji kunonoka kwenguva yakareba sezvinobvira, nekuda kwekukuvara kwekugadzirisa dambudziko iri mumucheche. Vana ava vanowanzogadziriswa nehutsinye hutsinye mune zvekuchengetedzwa kwechiremba, muchiedza kunonoka kuvhiyiwa kusvikira vane mukana wokukura. Pose pazvinenge zvichibvira, kuvhiyiwa kunonoka kwemwedzi inoverengeka.
Muvana vakwegura uye vakuru vatsva vaongororwa neEbstein anomaly, kugadziriswa kwekugadzira kwakagadzirirwa zvakasimba nokukurumidza apo chero zviratidzo zvinokura. Zvisinei, kana vane chiremera chikuru chekushaya simba kwemoyo, kuedza kunogadziriswa kugadzikana navo nerapi yezvechiremba vasati vashandisa.
Kuvhiya kunoshandiswa kweEbstein kunonyanya kunogona kuoma zvakaoma, uye kupindira kwakananga kunoshandiswa kunoitwa kunosiyana kubva kumunhu kusvika kune mumwe munhu, zvichienderana nehurumende yevricuspid valve, pakuvapo kana kusavapo kwekuwedzera kwepfungwa dzisina kunaka, kana mwoyo wakaremara kukanganisa uripo, uye pazera remurwere.
Nenzira yakawanda, chinangwa chekuvhiya ndechekusimudzira (zvakanyanya sezvinobvira) nzvimbo uye basa re tricuspid valve, uye kuderedza kushandiswa kwe ventricle yakarurama. Mumamiriro ezvinhu akaoma iyi chinangwa chinogona kushandiswa kushandiswa maitiro ekugadzira chirwere uye kugadzirisa vhidhiyo inonzi tricuspid valve. Mune mamiriro akaoma, tricuspid valve kushandiswa nevhavha yekugadzirira inokosha. Kuvhiya kweEbstein kunokonzerawo kunogadzirisa kugadziriswa kwepanyama uye / kana kuputika kwemafial septal kana iripo, uye chero ipi zvimwe dambudziko rekuzvarwa kwemoyo rinowanikwa.
Vana nevakuru vanoongororwa vane vane bhedha chete Ebstein anomaly, uye avo vasina zviratidzo, kazhinji havadi kugadziriswa pakushanda zvachose. Zvisinei, ivo vachiri kungwarira kutarisa kwehupenyu hwavo hwose nokuda kwechinangwa chipi nechipi mumamiriro avo emwoyo. Uyezve, pasinei ne "vanyoro" veEbstein, vanogona kunge vane nzira yekushandisa magetsi uye saka ingozi yemaridythmias yemwoyo, kusanganisira kuwedzera kungozi yekufa kamwe kamwe. Saka kuongorora kwakanyatsofanirwa kunofanira kuitwa kuti uongorore dambudziko iri. Kana nzira inogona kunge ine ngozi inowanika, inorapwa inofanira kuve yakanyatsotaridzirwa kubvisa kushandiswa kwemagetsi kusina kujairika.
Mhedziso Yenguva refu
Kufananidzira kweEbstein kunonyanya kunetseka nehutambudziro hwechinetso chevhavhavhavha, uye pakuvapo kana kusavapo kwemamwe matambudziko emwoyo. Mucheche ane chirwere ichi ane chirwere chinorwara, njodzi yekufa yakareba-inopfuura 30 muzana inofa vasati vasunungurwa kubva kuchipatara.
Ngozi yekufa kwekutanga apo Ebstein anomaly anowanikwa muhutano kana hukuru hunoenderanawo nekuoma kwemamiriro acho. Zvisinei, mumakumi emakore achangopfuura, kurapwa kwechisimba zvekuvhiya, uye kurapwa kweprophylactic yevamwe chimiro chechirwere chemwoyo, kwakanyatsogadzirisa kufungidzira kwevanhu vane Ebstein anomaly.
Shoko Rinobva
Ebstein inonyanya kufanana nehutano husina kunaka uye kuipa kwevhidhiyo inonzi tricuspid valve. Zvinokosha zvechiitiko ichi zvakasiyana kubva pamunhu kusvika kune munhu, uye zvinowanzobva zvakanyanya kuoma kune zvinyoro. Zvakakosha kune ani zvake ane Ebstein anomaly, kunyange avo vane mafomu chaiwo, kuti vagamuchire kuongorora kwemoyo wose uye kuongorora nguva dzose. Nemazuva ano ekugadzira nzira uye kutarisira kwakanyatsogadzirwa, kufungidzira kwevanhu vane Ebstein anomaly kwakavandudzwa zvikuru mumakumi emakore apfuura.
> Sources:
> Bonow RO, Carabello BA, Chatterjee K, et al. 2008 Inotarisa Kuvandudzwa Kunopindirwa MuAka / Aha 2006 Mirayiridzo Yekutungamira Kwevarwere NeValvular Heart Disease: Mharidzo yeAmerican College yeCardiology / American Heart Association Task Force pamusoro pekushandisa mazano (Komiti yekunyora kuongorora mazano e 1998 ekutungamirira Varwere Vane Valvular Heart Disease): vanobvumirwa neSosaiti yeCrodiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, uye Society of Thoracic Surgeons. Circulation 2008; 118: e523.
> Dearani JA, Mora BN, Nelson TJ, et al. Ongororo yeEbstein Anomaly: Ndezvipi zvino, Chii Chinotevera? Nyanzvi Rev Cardiovasc Ther 2015; 13: 1101.