Ebstein anomaly wani nau'i ne na cututtukan cututtukan zuciya da ke tattare da ɓarna na ci gaba da basirar tricuspid da kuma ƙananan ventricle . Dangane da mummunar wadannan haukarar, bayyanar cututtuka waɗanda waɗanda aka haifa tare da Abstein anomaly na iya bambanta da yawa.
Wasu jarirai da wannan yanayin suna rashin lafiya a lokacin haifuwa. Wasu tare da Ebstein anomaly suna rayuwa a cikin balagaguni ba tare da wata alama ba.
Duk da haka, kusan dukkanin wanda aka haifa da wannan yanayin zai bunkasa matsalolin zuciya ta jimawa ko daga baya.
Menene Ebstein Anomaly?
Mahimmanci, anasaly ta Ebstein ya haifar da rashin nasarar basirar tricuspid don bunkasa kullum cikin tayin. Takardun tarin gado ba su matsa zuwa matsayinsu na al'ada ba, a cikin jigon hagu na dama da kuma cin hanci da dama, yayin ci gaba. Maimakon haka, an cire takardun mujallu zuwa ƙasa, a cikin hannun dama na ventricle. Bugu da ƙari, waƙaƙunansu suna laminated ("makale") a kan bango na hannun dama, kuma ba haka ba ne ya buɗe kuma ya rufe da kyau.
Saboda tarkon da aka yi amfani da shi a Abstein anomaly an cire shi zuwa ƙasa, sashi na ƙwayar ventricle mai kyau wadda ke samuwa a sama da basirar tricuspid maras kyau an "ƙaddara." Wato, ɗakin da ke cikin zuciya zai ƙunshi ba kawai abin da ke cikin al'ada ba amma har ma na nama mai cin hanci.
Matsalar Cardiac
Saboda matsananciyar matsayi da kuma ɓarna na bala'in tricuspid da ke faruwa a cikin Abosh anomaly, basus din yana yawanci "ƙyale." A sakamakon haka, tricuspid regurgitation yawanci shine babban bayyanar wannan yanayin.
Bugu da ƙari, ƙaddamarwa game da ɓangaren ƙwayar ventricle mai kyau da ke sama da ƙwaƙwalwar tricuspid mai hijira yana haifar da matsalolin.
Ƙungiyar da aka yanke wa ƙwararrun ƙwararrun ƙwaƙwalwa yana damu lokacin da sauran ƙananan fitilun na dama suka yi damuwa, ba lokacin da atrium na dama ya ji tsoro ba. Wannan rikicewar rikice-rikice na muscular a cikin ɗakin shari'ar yana kara ƙaddamar da tricuspid kuma yana haifar da yanayin jinin a cikin hagu na dama don ya damu-yanayin da zai iya haifar da yatsun jini.
Mahimmanci na Ebstein anomaly yana da alaƙa da ƙaura da ɓarna da basirar tricuspid, da kuma adadin abincin mai cin hanci da ke ciki wanda aka ƙaddara a baya. Mutanen da aka haife su tare da Ebstein anomaly wadanda ke da ƙananan (ko babu) alamar cututtuka yawanci suna da matsi kaɗan.
Mutanen da aka haifa tare da Ebstein anomaly kuma suna da mummunan tasiri na samun wasu matsalolin zuciya. Wadannan sun hada da oval marayu , ƙananan ƙananan ƙetare, ɓarkewar ƙyama, ɓarkewar ductus arteriosus , gurguntaccen kwakwalwa bakwai, da kuma karin hanyoyin lantarki cikin zuciya wanda zai iya haifar da arrhythmias na zuciya . Idan daya ko fiye daga cikin wadannan matsalolin da ke faruwa a halin yanzu, akwai alamun bayyanar cututtuka da kuma sakamakon mutanen da suke dauke da Ebstein a cikin lokaci.
Dalilin
Ebstom anomaly yana faruwa a cikin kimanin 1 na 20,000 haihuwa.
Yayinda wasu maye gurbin kwayoyin sun danganta da Abstein anomaly, babu wani maye gurbin da ya zama babban dalilin wannan yanayin. An haɗu da wata ƙungiya ta tsakanin Ebstein da kuma mahaifiyar ciki mai amfani da lithium ko benzodiazepines, amma ba a tabbatar da kungiya ba. A mafi yawancin, anomaly na Ebstein ya fara faruwa.
Cutar cututtuka
Abubuwan da alamun da mutanen da suke ciki tare da Ebstein sun bambanta sosai, dangane da maƙasudin rashin ciwo da bala'i da kuma kasancewa ko rashin wasu matsaloli na zuciya.
Yaran da aka haife su tare da mummunan bala'i na valve wanda cutar ta Ebstein ta samu sau da yawa suna da wasu matsaloli na zuciya kamar yadda ya kamata, kuma yana iya zama mummunar rashin lafiya daga haihuwa.
Wadannan jariran suna da mummunan cyanosis (ƙananan yanayin oxygen), dyspnea , rauni, da edema (kumburi).
Yara da aka haifa tare da Ebstein anomaly wanda ke da matukar damuwa, amma ba wani mummunan matsalolin zuciya ba, na iya kasancewa yara masu lafiya amma zai bunkasa cikewar zuciya ta haƙiƙa ko dai a lokacin yarinya ko girma.
A gefe guda, idan tarkon da ba shi da kyau ba shi da kyau, mutumin da zai iya zama tare da Ebstein zai iya zama ba tare da bayyanar cututtuka ba.
Akwai ƙungiya mai karfi ta tsakanin Ebstein da kuma matakan lantarki a cikin zuciya. Wadannan hanyoyin da ake kira "hanyoyi masu haɗi" suna haifar da haɗin haɗin haɗi tsakanin ɗaya daga cikin atria da ɗaya daga cikin ventricles; a cikin Ebnous anomaly, sun kusan haɗa kai tsaye tare da hakikanin ventricle.
Wadannan hanyoyi masu amfani sukan haifar da wani nau'i na tachycardia supraventricular da ake kira tachycardia na reentrant (AVRT) . Wani lokaci wadannan hanyoyi masu amfani zasu iya haifar da ciwo na Wolff Parkinson , wanda zai iya haifar da AVRT kawai, amma har zuwa mafi yawan haɗari mai haɗari, ciki har da fibrillation ventricular . A sakamakon haka, wadannan hanyoyi masu dacewa zasu iya haifar da haɗarin haɗari ga mutuwa ta kwatsam.
Saboda fatalwar Ebstein yakan haifar da jini a cikin hannun dama, yaduwar jini yana samuwa a can. Idan waɗannan ƙyallen jini (wato, karyawa), za su iya tafiya ta hanyar tafiya da kuma haifar da lalacewar nama. Saboda haka anasaly din Ebstein yana haɗuwa da ƙara yawan haɓakar amintattun zuciya , da kuma (saboda kullun daga hannun dama yana iya motsawa ta hanyar motar da aka samu a cikin hagu na hagu), suna iya haifar da bugun jini.
Babban mawuyacin lalacewa daga mutuwar Ebstein sune mummunar zuciya da mutuwar mutuwa.
Sanin asali
Babban gwajin gwajin gwagwarmaya ta Ebstein shine rubutun echocardiogram -a hankali, gwajin ƙwaƙwalwar ƙwaƙwalwar jini yana bada cikakkiyar sakamako. Tare da zane-zane, ana iya ganewa da kuma mataki na ɓarnaccen ɓaɓɓuka na tricuspid wanda ya dace, kuma mafi yawan sauran cututtukan zuciya na zuciya wanda zai iya kasancewa ma za'a iya gano su.
A cikin tsofaffi da yara da suka karu na farko na kimantawa na Ebstein, ana gwada gwajin gwaji don tantance ikon su, yaduwar jini a lokacin motsa jiki, da kuma amsawar karfin zuciya da karfin jini don yin aiki. Wadannan ma'auni suna taimakawa wajen yin la'akari da mummunan yanayin yanayin zuciya, da kuma wajibi da gaggawa na magani.
Yana da mahimmanci a kimanta mutanen da suke da Ebstein anomaly don kasancewar arrhythmias na zuciya. Bugu da ƙari da zaɓuɓɓukan electrocardiograms (ECG) da kulawa na ECG , yawancin wadannan mutane ya kamata a kimanta su daga likitan kwakwalwa na asibiti idan an bincikar su, don tantance yiwuwar yin tasowa akan arrhythmias mai hatsari.
Jiyya
Bugu da ƙari, idan Ebstein anomaly yana samar da alamun bayyanar cututtuka, magani shine gyaran gyare-gyare.
An yi amfani da tiyata a cikin jariri tare da mai tsanani mai suna Ebstein wanda aka yi jinkiri har tsawon lokacin da zai yiwu, saboda mummunar haɗarin gyara wannan matsalar a jarirai. Wadannan jariran ana gudanar da su tare da goyon baya na likita a cikin kulawa mai kulawa mai tsanani, a ƙoƙari na jinkirta tiyata har sai sun sami zarafin girma. A duk lokacin da zai yiwu, tiyata yana jinkirta a kalla watanni da yawa.
A cikin tsofaffi yara da tsofaffi wanda aka gano tare da Abstein anomaly, gyaran gyare-gyaren gyare-gyare yana da karfi sosai da zarar duk wani bayyanar cututtuka ya ci gaba. Duk da haka, idan suna da matukar mahimmanci na rashin nasara na zuciya, an yi ƙoƙari don daidaita su da likita kafin a yi aiki.
Hanyoyin da aka yi amfani da su na Ebstein suna iya rikitarwa, kuma ayyukan da ake yi na musamman wanda aka yi sun bambanta daga mutum zuwa mutum, dangane da yanayin ɓarjin tricuspid, a gaban ko babu ƙarin ciwon zuciya na ciki, akan ko a'a rashin ciwo mai tsanani a halin yanzu, kuma a kan shekarun masu haƙuri.
Bugu da ƙari, manufar tiyata shine don daidaitawa (kamar yadda ya yiwu) matsayi da kuma aiki na bawul ɗin tricuspid, kuma don rage yawan abin da ke tattare da ventricle mai kyau. A cikin mawuyacin hali wannan manufa za a iya kusata ta yin amfani da fasaha don gyaran gyare-gyare na jiki kuma a sake mayar da bashin tricuspid. A cikin lokuta mafi tsanani, sauƙaƙen valve tricuspid tare da bashi na wucin gadi ya zama dole. Magunguna na Abstein anomaly kuma sun haɗa da gyaran kullun kwayoyin cutar da / ko ventricular na bakwai idan akwai, da kuma duk wasu matsalolin zuciya wanda aka gano.
Yara da tsofaffi waɗanda aka bincikar su tare da dangin Ebstein ne kawai, kuma waɗanda ba su da wata alamar wariyar launin fata, sau da yawa ba sa bukatar gyaran gyare-gyare. Duk da haka, har yanzu suna bukatar kula da hankali ga sauran rayuwar su don kowane canji a yanayin kwakwalwarsu. Bugu da ƙari kuma, duk da cewa suna da mahimmanci na kwaskwarima na Ebstein, har yanzu suna iya samun hanyoyin lantarki kuma saboda haka akwai hadarin cututtuka na zuciya, ciki har da haɗarin ƙari na mutuwa. Saboda haka dole ne a yi la'akari da hankali don bincika wannan hadarin. Idan an gano hanya mai haɗari mai haɗari mai haɗari, dole ne a yi la'akari da farfadowa don kawar da haɗin wutar lantarki.
Ƙarshen lokaci mai tsawo
Mahimmancin albarkarin Ebstein ya dogara ne akan mummunar matsala ta matsalar tarin, kuma a gaban ko babu wasu matsalolin zuciya. A cikin jarirai da wannan yanayin wanda aka haife shi da rashin lafiya, hadarin mace-mace yana da yawa-fiye da kashi 30 cikin 100 mutu kafin a iya fitar da shi daga asibitin.
Hasarin mutuwa ta farko lokacin da aka gano cutar ta Ebstein a lokacin ƙuruciya ko kuma girma ya dogara ne akan tsananin da yanayin. Duk da haka, a cikin 'yan shekarun nan, tsauraran ƙwarewar cuta, da kuma maganin ƙwaƙwalwar ƙwaƙwalwar ƙwaƙwalwar ƙwayar zuciya, sun inganta ingantaccen kwakwalwa ga mutane tare da Abstein anomaly.
Kalma Daga
Ebstein anomaly shine maganin lalacewa da kuma maganganu na basirar tricuspid. Mahimmancin wannan yanayin ya bambanta daga mutum zuwa mutum, kuma ya kasance daga kasancewa mai tsanani ga rashin lafiya. Yana da mahimmanci ga kowa da kowa tare da Ebstein anomaly, ko da waɗanda suke da siffofin da ya dace, don karɓar cikakken bincike na zuciya da kuma kulawa na tsawon lokaci. Tare da sababbin hanyoyin fasahar zamani da kulawa da hankali, halayen mutanen da ke dauke da Ebstein anomaly sun inganta sosai a cikin 'yan shekarun nan.
> Sources:
> Bonow RO, Carabello BA, Chatterjee K, et al. 2008 Tallafawa Intanit Gida a cikin Acc / Aha 2006 Jagororin Ga Gudanarwa na marasa lafiya Tare da Valvular Heart Ciwon daji: Rahoton Cibiyar Kasuwanci ta Kasuwancin Amirka / Ƙungiyar Ayyukan Ƙungiyar Amurkan Amurka ta Amincewa da Sharuɗɗan Ɗaukaka (Kwamitin Rubutun don Gyara Harkokin Gudanarwa ta 1998 don Gudanarwa na Marasa lafiya tare da Ciwon Zuciya Valvular): Ƙungiyar Ciwon Halitta Tsuntsauran Tsarin zuciya, Ƙungiyar ta Hanyoyin Cutar Kwayoyin Tsari da Harkokin Tsarin Lafiyar Jama'a, da Ƙungiyar Turare na Thoracic. Yanayi na 2008; 118: e523.
> Dearani JA, Mora BN, Nelson TJ, et al. Ebstein Anomaly Review: Menene Yanzu, Mene Ne? Gwani Rev Cardiovasc Ther 2015; 13: 1101.