Ebstein amadziwika ndi mtundu wa matenda a mtima obadwa nawo omwe amadziwika ndi chitukuko chosawonongeka cha valve ya tricuspid ndi ventricle yoyenera . Malingana ndi kuopsa kwa zovutazi, zizindikiro za omwe anabadwa ndi Ebstein zimakhala zosiyana kwambiri.
Ana ena omwe ali ndi matendawa akudwala kwambiri pa kubadwa. Ena omwe ali ndi Ebstein amatha kukhala achikulire popanda zizindikiro.
Komabe, pafupifupi aliyense wobadwa ndi matendawa adzakhala ndi vuto la mtima nthawi ndi nthawi.
Kodi Ebstein Anomaly N'chiyani?
Kwenikweni, Ebstein akusowa chifukwa cha kulephera kwa viveti yotchedwa tricuspid valve kuti ikule bwino mu fetus. Makapu a valveti amatha kusunthira kumalo awo oyenera, pampando wa atrium woyenera ndi ventricle yoyenera, panthawi ya chitukuko. Mmalo mwake, timapepala timathamangitsidwa pansi, mkati mwa ventricle yoyenera. Kuwonjezera apo, timapepala timene timapangidwira kawirikawiri ("amamatira") ku khoma la ventricle yoyenera, motero musatsegule ndi kutseka moyenera.
Chifukwa chakuti valveti ya Ebstein imakhala yotsika pansi, gawo la ventricle yoyenera yomwe ili pamwamba pa valavu yosadziwika ndi "atrialized." Ndiko, chipinda chapachiwalo cha mtima sichingakhale ndi minofu yachibadwa yokhayokha komanso gawo za minofu yoyenera ya ventricular.
Matenda a mtima
Chifukwa cha malo osalongosoka ndi kupotoza kwa vivevu yotchedwa tricuspid valve yomwe imapezeka mu Ebstein, vutoli ndilo "lotha." Chifukwa chaichi, kubwezeretsa mwatsatanetsatane ndizowonetseredwa kwakukulu kwa chikhalidwe ichi.
Kuwonjezera pamenepo, kuyang'ana kwa gawo la ventricle yoyenera yomwe ili pamwamba pa mavenda othamangitsidwa amatha kuchotsa mavuto.
Gawo labwino la ventricle limagunda pamene zonsezi zimagunda, osati pamene amawomba. Mchitidwe wokhudzana ndi mimba mkati mwa chipinda chowongolera kuntchito umaphatikizapo kubwezeretsa mofulumizitsa ndikupanganso chizolowezi cha mwazi mkati mwa malo oyenera kuti awonongeke-chikhalidwe chomwe chingayambitse magazi.
Kuopsa kwake kwa Ebstein kumakhudzana ndi kuchuluka kwa kusuntha ndi kusokonekera kwa viveti, ndi kuchuluka kwa minofu yolondola ya ventricular yomwe imayambitsidwa. Anthu obadwa ndi Ebstein omwe ali ndi zizindikiro zochepa (kapena ayi) amakhala ndi malo ochepa kwambiri othawa kwawo.
Anthu obadwa ndi Ebstein omwe ali ndi vutoli amakhalanso ndi mavuto akuluakulu a mtima. Izi zimaphatikizapo patent foramen ovale , kupweteka kwapadera kwa septal , kupopera kwa pulmonary outflow, patent ductus arteriosus , ventricular septal defect, ndi njira zowonjezera zamagetsi mu mtima zomwe zingapangitse mtima wamaganizo . Ngati chimodzi kapena zingapo za mavuto ena obwera chifukwa chogonana alipo, zizindikiro ndi zotsatira za anthu omwe ali ndi Ebstein ndizovuta kwambiri.
Zimayambitsa
Ku Ebstein kumakhala kovuta pakati pa amodzi oposa 20,000 obadwa.
Ngakhale kuti kusintha kwa majeremusi kunayanjanitsidwa ndi Ebstein kuipa, palibe kusintha kwapadera komwe kumayesedwa kuti ndilo vuto lalikulu la chikhalidwe ichi. Chiyanjano pakati pa Ebstein ndi amayi omwe ali ndi pakati omwe akugwiritsa ntchito lithiamu kapena benzodiazepine amadziwika, koma palibe mgwirizano wotsutsa. Kawirikawiri, Ebstein akuoneka kuti akuchitika mosavuta.
Zizindikiro
Zizindikiro za anthu omwe ali ndi Ebstein zimasiyana mosiyanasiyana, malingana ndi kukula kwake kwa vagupid valve zosadziwika komanso kukhalapo kapena kupezeka kwa mavuto ena okhudzidwa mtima.
Ana obadwa ndi vuto lopweteka kwambiri la valve chifukwa cha Ebstein vutoli nthawi zambiri amakhala ndi mavuto ena a mtima, ndipo akhoza kukhala odwala kwambiri kuyambira kubadwa.
Ana amenewa nthawi zambiri amakhala ndi cyanosis (otsika magazi), dyspnea , kufooka, ndi edema (kutupa).
Ana obadwa ndi Ebstein omwe ali ndi vuto lalikulu, koma palibe vuto lalikulu la mtima, akhoza kukhala ndi thanzi labwino koma nthawi zambiri amakhala ndi ubongo wamtima ngakhale ali mwana kapena akuluakulu.
Komabe, ngati kutsegula kwa vivekesi kumakhala kofatsa, munthu amene ali ndi Ebstein amatha kukhalabe wopanda zizindikiro pamoyo wawo wonse.
Pali mgwirizano wolimba pakati pa Ebstein ndi zovuta zamagetsi mumtima. Zomwe zimatchedwa "njira zowonjezeramo" zimapanga kugwirizana kosagwirizana kwa magetsi pakati pa imodzi ya atria ndi imodzi mwa zinyama; mu Ebstein osaly, pafupifupi nthawi zonse amagwirizanitsa malo abwino ndi ventricle yoyenera.
Njirazi zimayambitsa mtundu wa tachycardia wotchedwa atrioventricular reentrant tachycardia (AVRT) . Nthawi zina njira zofananazi zingayambitse matenda a Wolff Parkinson White , omwe amatha kuwongolera osati ku AVRT, komanso ku arrhythmias zoopsa kwambiri, kuphatikizapo ventricular fibrillation . Zotsatira zake, njirazi zowonjezera zikhoza kuonjezera chiopsezo cha imfa yadzidzidzi.
Chifukwa Ebstein amachititsa kuti thupi likhale lopweteka kwambiri m'magazi ofunika, magazi amatha kukhala pamenepo. Ngati magaziwa atsekedwa (kutanthauza kuti, achoke), amatha kuyendayenda ndikuyambitsa minofu. Choncho Ebstein amavomerezedwa ndi kuwonjezeka kwa ziwalo za pulmonary , ndipo (chifukwa kuchoka pa right atrium akhoza kudutsa mwa patent foramen ovale kumanzere atrium), angayambitsenso stroke.
Zomwe zimayambitsa imfa kuchokera kwa Ebstein zimakhudza mtima ndi imfa mwadzidzidzi.
Kudziwa
Chiyeso chofunika kwambiri pozindikira kuti Ebstein ndi wovuta kwambiri, ndiye kuti echocardiogram , yomwe imayesedwa bwino, imakhala ndi zotsatira zabwino kwambiri. Pogwiritsa ntchito mawu ofotokoza zapamwamba, kukhalapo ndi mlingo wa vivekedwe tomwe timapanga tricuspid kungayesedwe molondola, ndipo zina zambiri zofooka za mtima zomwe zingakhalepo zingatheke kudziwika.
Kwa akuluakulu ndi ana akuluakulu omwe akulandira kafukufuku wawo wa Ebstein, zoyezetsa zochitika zimayesedwa kuti awonetse mphamvu zawo zochita zolimbitsa thupi, magazi ochita masewera olimbitsa thupi, komanso kuyankhidwa kwa mtima wawo komanso kuthamanga kwa magazi. Miyeso imeneyi imathandiza pozindikira kuopsa kwa chikhalidwe chawo cha mtima, komanso kufunika kochiritsira mwamsanga.
Ndikofunikanso kufufuza anthu omwe ali ndi Ebstein omwe amadziwika kuti alipo mtima. Kuphatikiza pa ma electrocardiogram (ECG) pachaka ndi kuwunika kwa ECG , ambiri mwa anthuwa ayenera kuyesedwa ndi electrophysiologist ya mtima kamodzi atapezeka, kuti aone ngati ali ndi mwayi wokhala ndi zida zowopsya.
Chithandizo
Kawirikawiri, ngati Ebstein akuwonetsa kuti akuwonetsa zizindikiro zazikulu, mankhwalawa ndi kukonzanso opaleshoni.
Kuchita opaleshoni kwa ana omwe amabadwa ndi Ebstein osokonezeka nthawi zambiri amachedwa nthawi yaitali, chifukwa cha ngozi yaikulu yokonzera vutoli kwa makanda. Ana awa nthawi zambiri amatha kukhala ndi thandizo lachipatala mwamphamvu mu chipangizo cholera kwambiri, poyesera kuchedwa opaleshoni mpaka atakhala ndi mwayi wokula. Nthawi zonse, opaleshoni imachedwa kwa miyezi yambiri.
Kwa ana achikulire ndi achikulire omwe atulukira kale ali ndi Ebstein osavuta, kukonza opaleshoni kumalimbikitsidwa mwamsanga pamene zizindikiro zilipo. Komabe, ngati ali ndi vuto lalikulu la mtima, amayesayesa kuti azilimbitsa ndi chithandizo chamankhwala musanagwiritse ntchito.
Njira zopaleshoni zomwe amagwiritsira ntchito Ebstein zikhoza kukhala zovuta kwambiri, ndipo njira zothandizira opaleshoni zomwe zimagwiritsidwa ntchito zimapangidwa kuchokera kwa munthu wina, malinga ndi momwe zimakhalira pamagetsi, pamakhalapo kapena kupezeka kwa zolakwika zina zapachiyambi, kaya kapena ayi Kulephera kwa mtima kulipo, komanso pa msinkhu wa wodwalayo.
Kawirikawiri, cholinga cha opaleshoni ndichokhazikika (monga momwe zingathere) malo ndi ntchito ya valve tricuspid, ndi kuchepetsa kuyang'ana kwa ventricle yoyenera. M'madera ovuta kwambiri, cholingachi chingayambe kugwiritsidwa ntchito pogwiritsa ntchito njira zopangira opaleshoni ndikugwiritsanso ntchito valveti. Pa milandu yowopsya, kuthamanga kwa valve pogwiritsa ntchito valve yopangira ndi kofunikira. Kuchita opaleshoni kwa Ebstein kumaphatikizapo kukonzanso zofooka zaperepala ndi / kapena zowonjezereka zapadera ngati zilipo, ndi mavuto ena onse omwe amabwera.
Ana ndi akulu omwe amapezeka kuti ali ndi ubweya wochepa chabe wa Ebstein, ndipo omwe alibe zizindikiro, nthawi zambiri samafunikanso kukonza opaleshoni. Komabe, iwo amafunikanso kuwunika mosamala kwa moyo wawo wonse chifukwa cha kusintha kulikonse mu chikhalidwe chawo cha mtima. Komanso, ngakhale kuti Ebstein ali ndi "wofatsa", akhoza kukhala ndi njira zamagetsi ndipo nthawi yomweyo amatha kukhala ndi matenda a mtima, kuphatikizapo ngozi yowonongeka. Choncho kufufuza mosamalitsa kuyenera kuchitidwa kuti muwone zotsatira za zoopsazi. Ngati njira yowopsa yowonjezeramo ikudziwika, njira yothetsera vutoli iyenera kuganiziridwa mozama kuti iwononge kugwirizana kosagwirizana kwa magetsi.
Zotsatira Zakale
Zozizwitsa za Ebstein zonyansa zimadalira kukula kwa vuto la valve, ndi kupezeka kapena kupezeka kwa mavuto ena okhudzidwa mtima. Kwa makanda omwe ali ndi vutoli omwe amabadwa odwala kwambiri, chiopsezo cha kufa ndipamwamba -posa 30 peresenti amafa asanatuluke kuchipatala.
Kuopsa kwa imfa yoyambirira pamene Ebstein akupezeka kuti akupezeka mu ubwana wake kapena munthu wamkulu zimadalira kukula kwake kwa chikhalidwe. Komabe, m'zaka makumi angapo zapitazi, chithandizo chogwiritsira ntchito opaleshoni, komanso chithandizo chowopsa cha mtima wamakono, chasintha kwambiri chiwerengero cha anthu omwe ali ndi Ebstein omwe alibe.
Mawu Ochokera
Ebstein amavomereza ndi matenda omwe amachititsa kuti thupi likhale lopweteka. Kufunika kwa chikhalidwe ichi kumasiyanasiyana kuchokera kwa munthu ndi munthu, ndipo kumakhala kovuta kwambiri kufika pofatsa. Ndikofunika kuti aliyense amene ali ndi Ebstein akuwonongeke, ngakhale omwe ali ndi mawonekedwe ofatsa kwambiri, kuti alandire kuunika kwathunthu ndi kuyang'anitsitsa moyo wonse. Ndi njira zamakono zopangira opaleshoni komanso kusamalira mosamala, momwe anthu omwe ali ndi Ebstein amachitira zinthu zakale zapitazi.
> Zotsatira:
> Bonow RO, Carabello BA, Chatterjee K, et al. Kukonzekera kwa 2008 kunaphatikizidwira kulowa m'chaka cha 2006. Malangizo Othandizira Otsogolera Odwala Ndi Matenda a Mtima wa Valvular: Lipoti la American College of Cardiology / American Heart Association Ntchito Yogwiritsa Ntchito Malangizo Otsogolera (Komiti Yolemba Kukonzekera Malangizo a 1998 a Ulamuliro Odwala Amene Ali Ndi Matenda a Mtima wa Valvular): Ovomerezedwa ndi Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, ndi Society of Thoracic Surgeons. Kuyendayenda kwa 2008; 118: e523.
> Dearani JA, Mora BN, Nelson TJ, et al. Kubwereza kwa Ebstein Anomaly: Kodi tsopano, Kodi Chotsatira Ndi Chiyani? Katswiri Rev Cardiovasc Ther 2015; 13: 1101.