I-Ebstein i-anomaly yilona uhlobo lwesifo senhliziyo esibangelwa yi-valve ye-tricuspid ne -ventricle efanele . Ngokuya ngobukhulu bezinto ezingavamile, izimpawu ezitholwa yibo abazalwa nge-Ebstein anomaly zingahlukahluka kakhulu.
Ezinye izinsana ezinalesi sifo zigula kakhulu lapho zizalwa. Abanye abane-Ebstein anomaly bahlala ebuntwini ngaphandle kwempawu.
Kodwa-ke, cishe wonke umuntu ozalwa nalesi simo uzokuthuthukisa izinkinga zenhliziyo ngokushesha noma kamuva.
Uyini u-Ebstein Anomaly?
Ngokuyinhloko, i-Ebstein i-anomaly ibangelwa ukwehluleka kwe-valve ye-tricuspid ukuthuthukisa ngendlela evamile ebusweni. Amapheshana we-valve ye-tricuspid ayifinyeleli endaweni yawo evamile, ekuhlanganiseni kwe-atrium efanele kanye ne-ventricle efanele, ngesikhathi sokuthuthukiswa. Esikhundleni salokho, amapheshana aphelelwa phansi, ngaphakathi kwe-ventricle efanele. Ngaphezu kwalokho, amaqabunga ngokwawo avame ukubhujiswa ("anamathela") odongeni lwe-ventricle efanele, ngakho-ke ungavuli futhi uvale ngendlela efanele.
Ngenxa yokuthi i-valve ye-tricuspid e-Ebstein ehluleka ukuhlala phansi, isabelo se-ventricle esifanele esitholakala ngaphezu kwe-valve engavamile "i-atrialized." Yilapho, ikamelo lokubulala i-heart eliphakathi kwenhliziyo ngeke liqukathe izicubu ezingavamile kuphela kodwa futhi liyingxenye wezicubu ezifanele ze-ventricular.
Izinkinga zenhliziyo
Ngenxa yokuma okungavamile nokuphazamiseka kwe-valve ye-tricuspid eyenzeka ku-Ebstein anomaly, i-valve ivame "ukuphazamiseka." Ngenxa yalokho, ukuphindaphindiwe ngokujwayelekile kuwukubonakaliswa okuyinhloko kwalesi simo.
Ngaphezu kwalokho, ukutholakala kwesigaba se-ventricle efanele esingaphansi kwe-valve ye-tricuspid ibuyele ebangela izinkinga.
Ingxenye ye-ventricle ye-atrialized ishaya uma lonke i-ventricle elungile lishaya, hhayi lapho i-atrium ibamba khona. Isenzo se-muscular discordant ngaphakathi ekamelweni le-atrial siphuthumisa ukuphindaphindiwe kwe-tricuspid futhi kudala ukuthambekela kwegazi ngaphakathi kwe-atrium elungile ukuzulazula-isimo esingaveza ama-blood clots.
Ubukhulu be-Ebstein anomaly buhlobene nezinga lokuthutha kanye nokuhlukunyezwa kwe-valve ye-tricuspid, kanye nenani lezicubu ezihamba kahle ze-ventricular ezithintekayo. Abantu abazalelwa nge-Ebstein anomaly abanemibandela embalwa (noma cha) ngokuvamile banokuhamba okuncane kakhulu kwe-valvular.
Abantu abazalelwa nge-Ebstein abahlukumezekile banesimo esiphezulu sokuba nezinye izinkinga zenhliziyo yokuzalwa. Lokhu kufaka i- patent foramen ovale , i-septoral defect , i-pulmonary outflow obstruction, i- patent ductus arteriosus , i-ventricular septal defect, neminye indlela kagesi enhliziyweni engakwazi ukukhiqiza ama- arrhythmias enhliziyo . Uma enye noma ngaphezulu yalezi ezinye izinkinga zesifo sofuba zikhona, izimpawu nemiphumela yabantu abane-Ebstein anomaly ngokuvamile zibi kakhulu.
Izimbangela
I-Ebstein i-anomaly ivela ekuzalweni okuphilayo okungu-1 kwangu-20 000.
Nakuba ezinye izakhi zofuzo ezithinta izakhi ziye zahlotshaniswa no-Ebstein anomaly, akukho ukuguqulwa okunye okucatshangwa ukuthi kuyimbangela enkulu yalesi simo. Ukuhlangana phakathi kuka-Ebstein anomaly kanye nokusetshenziswa kukaMama okukhulelwe kwe-lithium noma i-benzodiazepines kuye kwabikwa, kodwa akukho buhlobo obunjalo obuye lwafakazelwa. Ngokuyinhloko, i-Ebstein anomaly ibonakala ivele ngezikhathi ezithile.
Izimpawu
Izimpawu ezitholwa abantu abano-Ebstein abahlukumezekayo ziyahlukahluka kakhulu, kuye kokubili ngesilinganiso se-valve ye-tricuspid engavamile kanye nokuba khona noma ukungabikho kwamanye izinkinga zenhliziyo yokuzalwa.
Izingane ezizalwa nge-severe tricuspid valve ukungasebenzi kahle okubangelwa yi-Ebstein anomaly ngokuvamile kunezinye izinkinga zenhliziyo yokuzalwa, futhi kungenzeka ukuthi zigula kakhulu kusukela ekuzalweni.
Lezi zinsuku zivame ukuba ne- cyanosis enamandla (amazinga aphansi e-oxygen), i- dyspnea , ubuthakathaka, ne-edema (ukuvuvukala).
Izingane ezizalwa nge-Ebstein anomaly ezinezinkinga ezibalulekile zokubuyela emuva, kodwa azikho ezinye izinkinga zenhliziyo ezingathí sina, zingase zibe izingane ezinempilo kodwa zivame ukuhlakulela ukuhluleka kwenhliziyo noma ngesikhathi sokukhula komuntu.
Ngakolunye uhlangothi, uma ukungasebenzi komzimba kwe-valve kuyinto emnene, umuntu obhekene ne-Ebstein angakwazi ukuhlala ngaphandle kokubonisa izimpilo zawo zonke.
Kukhona ubudlelwane obuqinile phakathi kwe-Ebstein okungahambi kahle nemigwaqo engasebenzi kagesi enhliziyweni. Lezi okuthiwa "izindlela zokufinyelela" zakha ukuxhumana okungavamile kagesi phakathi kweyodwa kwe-atria kanye neyodwa yezingqimba; E-Ebstein anomaly, cishe cishe baxhuma i-atrium efanele ne-ventricle efanele.
Lezi zindlela zokufinyelela zivame ukudala uhlobo lwe -tachycardia ephakeme ebizwa ngokuthi i- atrioventricular reentrant tachycardia (AVRT) . Ngezinye izikhathi lezi zindlela ezifanayo zokufinyelela zingabangela i- Wolff Parkinson White syndrome , engabangele kuphela ku-AVRT, kodwa nakwe-arrhythmias eyingozi kakhulu, kufaka phakathi i -fibrillation ye-ventricular . Ngenxa yalokho, lezi zindlela zokufinyelela zingadala ingozi yokufa okungazelelwe.
Ngenxa yokuthi u-Ebstein ovame ukhiqiza ukugeleza kwegazi okulukhuni ngaphakathi kwe-atrium efanele, ama-clots egazi ayenzeka ukwakha lapho. Uma lawa maqabunga egazi ebopha (okungukuthi, aphule), angahamba ngokusebenzisa ukusabalalisa futhi abangele ukulimala kwezicubu. Ngakho-ke i-Ebstein anomaly ihlotshaniswa nokwanda kwe- pulmonary embolus , futhi (ngoba ivala i-atrium elungile ingadlulisa i-patent foramen ovale engxenyeni yesokunxele), ingabangela nesifo.
Izimbangela ezinkulu zokufa ezivela e-Ebstein anomaly yizinkinga zenhliziyo nokufa okungazelelwe.
Ukuxilongwa
Isivivinyo esiyinhloko ekuhloleni i-Ebstein anomaly yi- echocardiogram , ngokujwayelekile, ukuhlolwa kwe-echo yokuguqula umzimba kunika imiphumela enembile kakhulu. Nge-echocardiogram, ukutholakala kanye nesilinganiso se-valve ye-tricuspid okungavamile kungacutshungulwa ngokunembile, kanti nezinye izinkinga ezingenayo yokuzalwa kwengqondo ezingase zikhona zingabuye zitholakale.
Kubantu abadala kanye nezingane ezindala ezithola ukuhlolwa kwabo kokuqala kwe-Ebstein, ukuhlolwa kokuvivinya umzimba kuvame ukuqhutshwa ukuhlola amandla abo okuzivocavoca, i-blood oxygenation ngesikhathi sokuzivocavoca, nokuphendula kwenhliziyo yabo nokucindezela kwegazi ukuze basebenzise umzimba. Lezi zilinganiso ziwusizo ekugwebeni ubukhulu besimo sabo senhliziyo, kanye nesidingo nokuphuthuma kokwelashwa okuhlinzekwayo.
Kubalulekile ukuhlola abantu abane-Ebstein abanomqondo wokuba khona kwe-arrhythmias yenhliziyo. Ngaphandle kwe- electrocardiograms yaminyaka yonke (ECG) nokuqapha kwe-ECG yokuthungatha , iningi lalaba bantu kufanele lihlolwe yi-electrophysiologist yenhliziyo lapho kutholakala ukuthi, ukuhlola amathuba abo okuthuthukisa ama-arrhythmias angaba yingozi.
Ukwelapha
Ngokuvamile, uma i-Ebstein anomaly ikhiqiza izimpawu ezibalulekile, ukwelashwa ukulungiswa kokuhlinzwa.
Ukwelashwa kwezinsana ezinama-Ebstein anomaly kakhulu kubambezeleka isikhathi eside ngangokunokwenzeka, ngenxa yengozi enkulu yokulungisa le nkinga ezinganeni. Lezi zingane zivame ukuphathwa ngokuxhaswa ngezokwelapha ezinyunithi zokunakekelwa okukhulu, emzamweni wokulibala ukuhlinzwa kuze kube yilapho zithola ithuba lokukhula. Uma kunokwenzeka, ukuhlinzekwa kubambezeleka okungenani izinyanga eziningana.
Kubantwana abadala kanye nabantu abadala abasanda kutholakala nge-Ebstein anomaly, ukulungiswa kokuhlinzwa kunconywa kakhulu uma nje noma yiziphi izimpawu zakha. Kodwa-ke, uma benesilinganiso esibalulekile sokuhluleka kwenhliziyo, kwenziwa umzamo wokuzinzisa ngemithi yokwelapha ngaphambi kokusebenza.
Izinqubo zokuhlinza ezisebenziselwa i-Ebstein anomaly zingaba nzima kakhulu, kanti ukuhlinzekwa okukhethekile okuhlinzekwa okwenziwe kuyahlukahluka komunye nomuntu, kuye ngokuthi isimo se-valve ye-tricuspid, lapho kukhona khona noma ukungabi khona kweziphambeko zenhliziyo ezengeziwe, uma kungenjalo ukuhluleka okukhulu kwenhliziyo kukhona, futhi eminyakeni yobugulane.
Ngokuvamile, umgomo wokuhlinza ukujwayela (ngokusemandleni) isimo kanye nomsebenzi we-valve ye-tricuspid, nokunciphisa ukusebenza kwe-ventricle efanele. Ezimweni ezibucayi lo mgomo ungasondelwa usebenzisa amasu okulungisa nokuhlinzekela i-valve ye-tricuspid. Ezimweni ezimbi kakhulu, ukuguqulwa kwe-valve i-tricuspid nge-valve yokufakelwa kuyadingeka. Ukwelashwa kwe-Ebstein okuphazamiseka nakho kuhlanganisa ukulungiswa kweziphene ze-septal kanye / noma ezisezintanjeni uma zikhona, kanye nanoma yiziphi ezinye izinkinga zenhliziyo ezibangelwa ukufa.
Izingane kanye nabantu abadala abatholwa ukuthi bane-Ebstein anomaly kuphela, futhi abangenayo izimpawu, ngokuvamile abadingi ukukhanda okuphelele. Kodwa-ke, basadingeka ukuqapha ngokucophelela konke ukuphila kwabo nganoma yisiphi ushintsho esimweni sabo senhliziyo. Ngaphezu kwalokho, naphezu "kobumnene" babo abakwa-Ebstein, bangase babe nemikhakha yokuthola amandla kagesi futhi ngaleyo ndlela babe nengozi ye-arrhythmias yenhliziyo, kuhlanganise nengozi eyengeziwe yokufa okungazelelwe. Ngakho kufanele kuhlolwe ngokucophelela ukuhlola le ngozi. Uma kutholakala indlela yokuthola izinto ezinobungozi, ukwelashwa kwe-ablation kufanele kucatshangelwe ngokuqinile ukuqeda ukuxhumana okungavamile kagesi.
Umphumela Wesikhathi Eside
Ukuchazwa kwe-Ebstein anomaly kuncike ebucayi benkinga ye-valve i-tricuspid, kanye nokuba khona noma ukungabikho kwamanye izinkinga zenhliziyo yokuzalwa. Ezinganeni ezinalezi zifo ezizalwa zigula kakhulu, ingozi yokufa iphezulu-engamaphesenti angu-30 afe ngaphambi kokuba ikhululwe esibhedlela.
Ingozi yokufa ekuqaleni lapho i-Ebstein anomaly itholakale ekufikeni komntwana noma esekhulile nayo incike ebukhulu besimo. Kodwa-ke, emashumini eminyaka amuva nje, ukuphathwa kwezidakamizwa ezinonya, kanye nokwelashwa kwe-prophylactic ye-arrhythmias engaba yimizimba, kuthuthukise kakhulu ukuchazwa kwabantu abane-Ebstein anomaly.
Izwi elivela
I-Ebstein i-anomaly yi-malformation ebusweni futhi ihlukumeza i-valve ye-tricuspid. Ukubaluleka kwalesi simo kuhlukahluka komuntu kumuntu, futhi kuyahluka kusukela ekubeni nzima kakhulu kunomusa. Kubalulekile kunoma ngubani onobungozi be-Ebstein, ngisho nalabo abanamafomu amancane, ukuthola ukuhlolwa kwenhliziyo egcwele nokuqapha konke okuqhubekayo. Ngezinqubo zesimanje zokuhlinzeka nokuphathwa ngokucophelela, ukuchazwa kwabantu abane-Ebstein anomaly kuye kwanda kakhulu eminyakeni emashumini amuva nje.
> Imithombo:
> Bonow RO, uCarabello BA, Chatterjee K, et al. Ukubuyekezwa okugxilwe ngo-2008 okuhlanganisiwe ku-Acc / Aha 2006 Izikhombandlela Zokuphathwa Kwabagulayo Ngezifo Zezinhliziyo Ze-Valvular: Umbiko We-American College of Cardiology / American Heart Association Umsebenzi Wezinkombandlela Zemihlahlandlela Yokuzikhandla (IKomidi Yokubhala Ukubukeza Iziqondiso Zika-1998 Zokuphathwa Kwezifo Iziguli ezine-Valvular Heart Disease): zamukelwa yiNhlangano Yezinhliziyo Zezinhliziyo Ze-Anesthesiologists, i-Society for Cardiovascular Angiography and Interventions, kanye neNhlangano Yezifo Ezihlinzeka Ngama-Thoracic. Ukujikeleza ngo-2008; 118: e523.
> Dearani JA, Mora BN, uNelson TJ, et al. Ukubuyekeza kuka-Ebstein Anomaly: Yini manje, Yini Okulandelayo? Umhloli Rev Cardiovasc Ther 2015; 13: 1101.