Ebstein ba da daɗewa ba

Rashin lafiyar Ebstein cuta ce mai saurin lalacewa wanda ɓangarorin ɓangaren tricuspid bawul ya zama al'ada. Bawul din tricuspid ya raba ɗakin ƙananan ƙananan zuciya (ƙyamar dama) daga ɗakin zuciyar zuciya ta sama dama (atrium na dama). A cikin ɓacin rai na Ebstein, sanya bawul ɗin tricuspid da yadda yake aiki don raba ɗakunan biyu ba al'ada bane.

Yanayin na haihuwa ne, wanda ke nufin ya kasance lokacin haihuwa.

Bawul din tricuspid yawanci ana yinsa ne daga sassa uku, ana kiransa da takardu ko flaps. An buɗe takaddun don bawa jini damar motsawa daga atrium na dama (ɗakin sama) zuwa gefen hagu na dama (ɗakin ƙasa) yayin da zuciya ta saki jiki. Suna rufewa don hana jini motsa daga dama zuwa dama atrium yayin da zuciya ke bugawa.

A cikin mutanen da ke da ɓacin rai na Ebstein, ana sanya ƙasidun a cikin zurfin dama na dama maimakon madaidaicin matsayi. Takardun bayanan galibi sun fi na al'ada girma. Launin galibi yakan sa bawul din yayi aiki mara kyau, kuma jini na iya tafiya ba daidai ba. Maimakon ya fita zuwa huhun, jinin yana komawa cikin dama. Ajiyar jini yana iya haifar da faɗaɗa zuciya da haɓakar ruwa a jiki. Hakanan za'a iya samun ƙarancin bawul din da ke kaiwa ga huhu (huhun huhu).

A cikin lamura da yawa, mutane suna da rami a bangon da ke raba ɗakunan sama biyu na sama (nakasar atrial septal defection) kuma jinin da ke kwarara a cikin wannan ramin na iya haifar da jinin oxygen-zuwa ga jiki. Wannan na iya haifar da cutar cyanosis, shuɗi mai launin shuɗi wanda fata mai ƙarancin oxygen ke haifarwa.

Harshen Ebstein yana faruwa yayin da jariri yayi girma a cikin mahaifar. Ba a san takamaiman dalilin ba. Amfani da wasu ƙwayoyi (kamar lithium ko benzodiazepines) yayin ɗaukar ciki na iya taka rawa. Yanayin ba safai ba. An fi samun hakan a cikin fararen fata.

Rashin haɗari na iya zama ƙarami ko ƙwarai da gaske. Sabili da haka, alamun cutar na iya zama daga mai sauƙi zuwa mai tsananin gaske. Kwayar cututtukan na iya bunkasa ba da daɗewa ba bayan haihuwa, kuma na iya haɗawa da leɓɓu masu launin shuɗi da ƙusoshi saboda ƙarancin iskar oxygen. A cikin yanayi mai tsanani, jaririn yana nuna rashin lafiya sosai kuma yana da matsalar numfashi. A cikin yanayi mai sauƙi, mutumin da abin ya shafa na iya kasancewa mai rashin bayyanar cutar shekaru da yawa, wani lokacin har abada.

Kwayar cututtuka a cikin manyan yara na iya haɗawa da:

• Tari
• Rashin girma
• Gajiya
• Saurin numfashi
• Rashin numfashi
• Saurin bugun zuciya

Yaran da aka haifa suna da mummunar malalewa ta cikin bawul din tricuspid zasu sami ƙarancin oxygen a cikin jininsu da kuma faɗaɗa zuciya mai mahimmanci. Mai ba da sabis na kiwon lafiya na iya jin sautunan zuciya marasa kyau, kamar gunaguni, yayin sauraron kirji tare da stethoscope.

Gwaje-gwajen da zasu iya taimakawa wajen gano wannan yanayin sun hada da:

• Kirjin x-ray
• Hanyoyin fuska ta maganadisu (MRI) na zuciya
• Mitar aikin lantarki na zuciya (ECG)
• Duban dan tayi na zuciya (echocardiogram)

Jiyya ya dogara da tsananin lahani da takamaiman alamun. Kulawa na likita na iya haɗawa da:

• Magunguna don taimakawa tare da gazawar zuciya, kamar su diuretics.
• Oxygen da sauran taimakon numfashi.
• Yin aikin tiyata don gyara bawul
• Sauyawa bawul din tricuspid. Ana iya buƙatar wannan don yara waɗanda ke ci gaba da damuwa ko waɗanda ke da rikitarwa mafi tsanani.

Gabaɗaya, alamun farko sun fara, mafi tsananin cutar.

Wasu mutane na iya samun ko dai babu alamun bayyanar ko alamomi masu sauƙi. Wasu na iya yin rauni a kan lokaci, canza launin shuɗi (cyanosis), gazawar zuciya, toshewar zuciya, ko kuma haɗarin zuciya mai haɗari.

Zuɓɓugar ruwa mai ƙarfi na iya haifar da kumburin zuciya da hanta, da kuma gazawar zuciya.

Sauran rikitarwa na iya haɗawa da:

• Heartwayar zuciya mara kyau (arrhythmias), gami da saurin saurin rashin ƙarfi (tachyarrhythmias) da jinkirin jinkirin rashin ƙarfi (bradyarrhythmias da toshe zuciya)
• Jinin jini daga zuciya zuwa wasu sassan jiki
• Abswayar kwakwalwa

Kira mai ba ku sabis idan yaronku ya fara bayyanar cututtuka na wannan yanayin. Samu likita nan da nan idan matsalar numfashi ta auku.

Babu wata sananniyar rigakafin, banda yin magana da mai ba ka sabis kafin a sami ciki idan kana shan magunguna waɗanda ake zaton suna da alaƙa da haɓaka wannan cuta. Kuna iya hana wasu matsalolin cutar. Misali, shan maganin rigakafi kafin tiyatar hakori na iya taimakawa rigakafin endocarditis.

Halin Ebstein; Rashin kuskuren Ebstein; Ciwon zuciya na haihuwa - Ebstein; Zuciyar haihuwar haihuwa - Ebstein; Ciwon zuciya na Cyanotic - Ebstein

• Halin Ebstein

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Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Raunukan cututtukan zuciya na Cyanotic: raunuka da ke haɗuwa da ragin jini na huhu. A cikin: Kliegman RM, St.Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Littafin koyar da ilimin yara. 21st ed. Philadelphia, PA: Elsevier; 2020: babi na 457.

Stout KK, Daniels CJ, Aboulhosn JA, et al. Dokar 2018 AHA / ACC don kula da manya da cututtukan zuciya na ciki: rahoto na Kwalejin Kwalejin Zuciya ta Amurka / Heartungiyar Heartungiyar Heartungiyar Zuciya ta Amurka a kan Sharuɗɗan Ayyukan Clinical. Kewaya. 2019; 139: e698-e800. PMID: 30121239 pubmed.ncbi.nlm.nih.gov/30121239/.

Webb GD, Smallhorn JF, Therrien J, Redington AN. Cutar cututtukan ciki a cikin baligi da haƙuri na yara. A cikin: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald na Ciwon Zuciya: Littafin rubutu na Magungunan zuciya da jijiyoyin jini. 11th ed. Philadelphia, PA: Elsevier; 2019: babi na 75.