Ebstein ta Anomaly: Sanadin, Alamun, ganewar asali, Jiyya

Daya daga rare zuciya lahani ne Ebstein ta anomaly. Yana da wani nakasar cuta a cikin abin da tricuspid zuciya bawul aka koma dama ventricle, ko da yake dole ne su kasance a kan iyaka tsakanin atria da ventricles. Shi ne muhimmanci ƙananan jini ya kwarara.

sifofin

Ebstein ta anomaly take kaiwa zuwa gaskiya cewa rami na dama ventricle zama karami, kuma dama atrium - fiye da kullum. Kada bawul ma bambanta. Sun iya improperly sifa ko gudun hijira. Yana disrupted da ci gaba da kuma duk chordal kuma murdede tsarin, wanda gudãnar da tricuspid na'ura.

Saboda da kananan girman da dama ventricle daga emits kasa adadin jini. Kuma da ƙara girman da dama atrium saboda gaskiyar cewa shi samun wani ɓangare na venous jini.

A da yawa marasa lafiya da Ebstein ta anomaly kuma kamu da atrial septal aibi da lamban kira foramen ovale. Wannan tabbaci lahani ga mutane da yawa ne rai-ceto. Ta hanyar septal aibi daga maƙil dama atrium shunt faruwa. Gaskiya, da venous jini a bar atrium ne gauraye da jijiya. Wannan sa oxygen yunwa na tsokoki da kuma gabobin.

A Sanadin cutar

Kasa da 1% na mutane da zuciya wadda tun ainahi cuta kamu Ebstein ta anomaly. Dalilan da ta ci gaba da ba za a iya ƙaddara sosai. Kamar yadda zato, ta bayyana saboda kwayoyin anomalies. Har ila yau, a daya version, domin irin wannan ci gaban da zuciya tsoka iya haifar da liyafar lokacin daukar ciki na shirye-shirye dauke da lithium gishiri.

Tare da wani nakasar cuta a cikin 50% na lokuta da atrial septal lahani. A wasu lokuta, a can ne take hakkin launi. Don me ya sa fahimta akwai wani hade da zuciya lahani, likitoci sun ba tukuna nasara.

Zai yiwu bambance-bambancen karatu da cutar

Ebstein ta anomaly ne mai muhimmanci nakasar zuciya cuta. Yana auku a duka biyu maza da mata daidai da juna. Cuta hanya da kuma hangen nesa zai dogara ne a kan mataki na nakasawa na tricuspid bawul. Har ila yau muhimmanci pathophysiological canje-canje da suka faru a dama atrium da ventricle.

A wasu lokuta, da aibi a material warwarewarsu da fetal wurare dabam dabam. Akwai zuciya rashin cin nasara, hydrops tasowa, da kuma a 27% na lokuta faruwa ne a utero mutuwar yaro. Tare da zurfin Pathology bayan haihuwar jariran mutu a cikin watan farko na rayuwa. Bisa kididdigar da, wannan shi ne game da 25% na dukkan yara tare da Ebstein ta anomaly aka gano. A ganewar asali da ciki za a iya shigar ma a kan 20th mako.

Game da 68% na jarirai da wannan cuta mutu kafin watanni shida, kuma har zuwa 5 shekaru - 64% na yara. Wannan kungiyar hada da jariran suke da aiki na tricuspid bawul da dama ventricle ne mai gamsarwa. Su mutu a mafi yawan lokuta saboda m zuciya da kuma gazawar arrhythmias.

A rarrabuwa na daban na cuta

Masana sun gano dama, saukarwa da cutar, da aka sani da Ebstein ta anomaly. A bayyanar cututtuka zai dogara ne kan yadda manyan zuciya rashin cin nasara. A mafi rare ne asymptomatic mataki. Irin wannan marasa lafiya na iya ba ko da zargin na Pathology da kai a rayuwa. Sun kawo kyau motsa jiki.

A cikin mataki na biyu na asibiti bayyanuwar furta. Wannan anomaly bayyana a cikin shimfiɗar jariri, da kuma daukan wuya isa. Dabam gano matakai na:

- II a - shi ne halin da rashi na cardiac arrhythmias.

- II na b - harin ne m.

The uku mataki ne ake kira lokacin da naci decompensation. An kamu lokacin da jiki yake iya rama domin ayyuka na zuciya da wani servo-inji.

The asibiti hoto da cutar

A haihuwa, yara da Ebstein ta anomaly halin da cyanosis. Cyanosis bayan 2-3 watanni na rayuwa an rage saboda gaskiyar cewa na huhu jijiyoyin bugun gini juriya da aka rage. Amma a yara tare da kadan lahani a cardiac septum kara hadarin mutuwa daga rikitarwa na cyanosis da m zuciya rashin cin nasara.

A yara da Ebstein ta anomaly ne sau da yawa lura da wadannan alamun:

- shortness na numfashi ko a sauran lokaci.

- edema, wanda za a iya gani a kan ƙananan extremities.

- gajiya, musamman m lokacin jiki exertion.

- zuciya kari cuta.

- cyanosis na fata da kuma lebe.

Cyanosis bayyana a cikin yara kafin bayyanar cututtuka na zuciya rashin cin nasara. Mazan yara na iya kai ƙarar maimaita bouts na tachycardia.

ganewar asali da cutar

A wasu lokuta, da cutar da aka ƙaddara a lokacin daukar ciki ko jim kadan bayan haihuwa a asibiti. Akwai da dama halayyar siffofin, wanda your likita yi zargin cewa wani yaro Ebstein ta anomaly. Ganewar asali hada auscultation, kirji radiography, echocardiography, EKG. Dukan wadannan hanyoyin a hade taimaka daidai ganewar asali.

Auscultation likita iya ji wani rarrabe kari, zai iya zama wani uku- ko hudu-Musulunci. Ya drowns fitar da m high systolic gunaguni na tricuspid insufficiency. Bugu da kari, ya furta ne tsagawa na biyu sautin, shi ba ya canza a lokacin numfashi. A saman bar gefen sternum ya kamata a ji mezodiastolichesky shiru squeaky amo.

A radiography visualized waye, cardiac inuwa, wannan shi ne saboda wani gagarumin karuwa a dama atrium. Na huhu jijiyoyin bugun gini juna yawanci kodadde. Zuciya sau da yawa daukan wani nau'i na Sphere.

Canje-canje lura a cikin ECG. A cikin ECG nuna alamun nuna hypertrophy na dama atrium, da PQ tazara da aka mika, kuma akwai duka ko m kawancen na dama cuta reshe block masaƙa.

A echocardiography iya ganin m bude na tricuspid bawul. Septal kada ake son zuciya zuwa saman.

A dabara a nakasar anomalies

Bayan da bincike da kuma sanin ko za a mataki na halakar da aka zaba magani. Hakika, irin wannan marasa lafiya bukatar m baki. The kawai togiya ne cewa wani karamin rukuni na marasa lafiya da Ebstein ta anomaly ne asymptomatic.

Jiyya ne da za'ayi dangane da sauran related al'amurran da suka shafi. Idan wani haƙuri kullum tsira jariri, da aiki za a iya dage har sai da irin wannan lokaci har akwai pronounced bayyanar cututtuka na zuciya rashin cin nasara.

All yara tare da wannan matsala dole duba yara likitan zuciyar wanda ya ƙware a nakasar zuciya cuta. Kuma ta halin ya kamata a kula cardiac likitoci.

Idan marasa lafiya bayyana raguwa a huhu jini ya kwarara da kuma akwai alamun zuciya rashin cin nasara, da jiko nada ionotropic kwayoyi prostogladinov kungiyoyin E. Har ila yau nuna rike da gyara na na rayuwa acidosis. Irin wannan jiyya iya kara cardiac fitarwa da kuma rage hagu na ramin zuciya matsawa na mika hannun dama gefen.

A lokuta inda akwai wani tachycardia, shi wajibi ne a gudanar da musamman magani antiarrhythmics.

Alamomi ga tiyata

A cikin neonatal lokaci kokarin ba su yi aiki. Amma ya zama dole a lokuta inda dama na ramin zuciya dysplasia ne da sunan kuma yana tare da disturbances na antegrade jini ya kwarara a cikin huhu tashar. A lokaci guda akwai wani kaifi fadada dama zuciya da kuma lokaci daya matsawa na hagu ventricle.

Domin zumunta contraindications sun hada da shekaru na 4-5 shekaru. Amma, idan ya cancanta, yin aiki da jarirai. Haka kuma an ba a yi tiyata da waɗanda suka yi sun fara gudanar da komowar canje-canje a cikin kayan ciki.

Marasa lafiya da suke Ebstein ta anomaly aka gano, babu ayyukan iya rayuwa zuwa wani talakawan na shekaru 20. Su mutuwa ne yawanci kwatsam. Yana auku saboda na ramin zuciya fibrillation na zuciya.

The aiki

Kawai ta hanyar tiyata iya gaba daya gyara halin da ake ciki. A wannan yanayin, likitoci bayar da shawarar idan zai yiwu a yi shi a wani mazan shekaru saboda ma kananan zuciya size a jarirai.

The aiki aka yi bude-zuciya tiyata, domin ta yiwuwa na shirya da aiwatar da cardiopulmonary kewaye. Rarar sutured rabo daga dama atrium, kuma tricuspid bawul seams yakan zuwa matsayi wanda yake kusa da na al'ada. A lokuta inda wannan ba zai yiwu, aka cire da kuma maye gurbinsu da wani prosthesis. Wannan ba ka damar manta game da gaskiyar cewa da haƙuri da Ebstein ta anomaly. A adult marasa lafiya na iya gudanar da prosthetics, amma yara a karkashin shekaru 15 da haihuwa da suke roba bawul.

Mace-mace a lokacin ayyukan ba ya wuce 2-5%. Yiwuwar mutuwa dogara a kan kwarewa da zuciya mai fiɗa, kuma daga mataimakin nauyi. Kusan 90% na marasa lafiya a cikin shakka daga cikin shekara iya komawa zuwa rayuwa.

Musamman hankali ya kamata a bai wa wannan marasa lafiya anesthesiologists. Bayan duk, shi bukatar na musamman m ga marasa lafiya da suke Ebstein ta anomaly. Features na maganin sa barci ya kamata a sanar da likita. Ya kamata a haifa tuna cewa matsa lamba a wadannan marasa lafiya na iya zama m. Saboda haka, anesthesiologist na sa ido da marasa lafiya da kuma bayan tiyata.

iri hakoran roba

A mafi yawan lokuta, don yanke shawarar yadda za a za'ayi da aiki, shi ne zai yiwu ne kawai a lokacin da haƙuri ne riga a kan aiki tebur. Idan yana yiwuwa a rike da bawul plasty, baicin aka bai wa wannan zaɓi. Amma akwai yanayi a lokacin da prosthesis ake bukata a cikin marasa lafiya da suke da Ebstein ta anomaly. Don haka ya kira ta wucin gadi bawul, wanda ya dubi kamar zobe, Fitted da wani roba matashin kai. Ciki shi ne wani inji cewa zai iya bude da kuma rufe wani da ake kira ƙofa. Yana iya zama inji ko nazarin halittu. A farko embodiment da aka yi da wani titanium gami, da kuma na biyu da za a iya sanya ta a bawul na alade, ko mutum cardiac nama shirts.

Lokacin da ka shigar da inji bawul haƙuri dole ne kullum sha magunguna, jini thinners, amma riko ta yi aiki da shi zai iya šauki tsawon. Halittu na'urar ne kasa m.