I-Ebstein anomaly luhlobo lwesifo senhliziyo esiswini esibonakaliswe ngophuhliso olungavumelekanga lwe-valve ye-tricuspid kunye ne -ventricle efanelekileyo . Ngokuxhomekeke kubukhulu bezinto eziqhelekileyo, iimpawu ezibangelwa ngabo bazalwa nge-Ebstein anomaly ziyahlukahluka kakhulu.
Ezinye iintsana ezile meko zigula kakhulu xa zizalwa. Abanye nge-Ebstein anomaly bahlala bekhulile ngaphandle kwempawu.
Nangona kunjalo, phantse wonke umntu ozalwe ngolu hlobo uya kuba neengxaki zengqondo kungekudala okanye kamva.
Yintoni uEbstein Anomaly?
Ngokubalulekileyo, i-Ebstein anomaly ibangelwa ukungaphumeleli kwevaji ye-tricuspid ukukhula ngokuqhelekileyo kumntwana. Amaphetshana e-valve ye-tricuspid ayifaki kwisimo sayo esifanelekileyo, ekuhlanganiseni kwe-atrium elungileyo kunye ne-ventricle efanelekileyo, ngexesha lophuhliso. Kunoko, amaqabunga ayaxoshwa phantsi, ngaphakathi kwiphepha elifanelekileyo. Ukongeza, amaqabunga ngokwabo ahlala ahlanjululwa ("anamathele") eludongeni lwe-ventricle efanelekileyo, kwaye ke ungavulwa kwaye uvale ngokufanelekileyo.
Ngenxa yokuba i-valve ye-tricuspid e-Ebstein i-anomaly ihlaselwa phantsi, isahlulo se-ventricle esilungileyo esiphezu kwe-valve engavumelekanga i-tricuspid "i-atrialized". kweetyhubhu ezifanelekileyo.
Iingxaki zeCardia
Ngenxa yesimo esingavumelekanga kunye nokuphazamiseka kwintsimbi ye-tricuspid eyenzeka kwi-Ebstein anomaly, i-valve isoloko "ihlaziyekile." Ngenxa yoko, ukulungiswa kwintsimbi ngokuqhelekileyo kukubonakaliswa okubonakalayo kwesi simo.
Ukongezelela, ukuphunyezwa kwesahlulo se-ventricle elungileyo esiphezulu kwinqwelo ye-tricuspid evaliweyo nayo ibangela iingxaki.
Inxalenye ye-ventricle ye-ventricle ibetha xa lonke i-ventricle ibetha, kwaye kungekhona xa i-atrium ibetha. Isenzo esicatshungulwayo se-muscular ngaphakathi kwegumbi le-atrial ligxininisa ukuphindaphindiwe kwezinto ezinobungozi kwaye kudala ukutyekela kwegazi ngaphakathi kwinqanaba elungileyo ukunyuka-imeko enokuvelisa i-blood clots.
Ubunzima be-Ebstein anomaly luhambelana nomlinganiselo we-displacement kunye ne-malformation ye-valve ye-tricuspid, kunye nenani leethambo ezivakalayo ezinokuthi zenzeke. Abantu abazalelwe nge-Ebstein anomaly abanemibonakalo embalwa (okanye ayikho) imvama yokufuduka kwe-valvular.
Abantu abazalwa no-Ebstein anomaly banesimo esiphezulu sokuba nezinye iingxaki zentliziyo yokuzalwa. Ezi ziquka i- patent foramen ovale , i-defectary septal defect , i-pulmonary outflow block, i- patent ductus arteriosus , i-defectary septal defect, kunye neendlela ezongezelelweyo zamandla entliziyweni ezinokuvelisa i- arrhythmias ye-cardiac . Ukuba enye okanye ngaphezulu kwezi ngxaki zentlungu zikhona, iimpawu kunye neziphumo zabantu abane-Ebstein anomaly zidla kakhulu.
Izizathu
I-Ebstein i-anomaly ivela kwi-1 ye-20 000 yokuzalwa.
Nangona iinguqu ezithile zentsholongwane ziye zahambelana no-Ebstein anomaly, akukho tshintsho oluthile lucingelwa ukuba luyimbangela enkulu yale meko. Ubudlelwane phakathi kwe-Ebstein anomaly kunye nokusetyenziswa kukaMama okhulelweyo kwe-lithium okanye i-benzodiazepines kuye kwaxelwa, kodwa akukho mbutho oye waboniswa. Ngokona nxalenye, i-Ebstein anomaly ibonakala ivela ngokungahleliwe.
Iimpawu
Iimpawu ezibangelwa ngabantu abane-Ebstein anomaly ziyahlukahluka kakhulu, kuxhomekeke kumgangatho we-valve engavamile kunye nokuba khona okanye ukungabikho kwezinye iingxaki zentliziyo yokuzalwa.
Abantwana abazalwa benesifo esichukumisayo se-valve ezibangelwa yi-Ebstein anomaly banesinye iinjongo zentliziyo yokuzalwa, kwaye banokugula kakhulu ngenxa yokuzalwa.
Ezi zintsana zihlala zine- cyanosis enamandla (amanqanaba e-oxygen ephantsi), i- dyspnea , ubuthathaka, kunye ne-edema (ukukhukhuma).
Abantwana abazalelwe nge-Ebstein anomaly abanesifo esibalulekileyo sokuvuselelwa, kodwa akukho nenye ingxaki ezinzulu zesifo senhliziyo, bangabantwana abasempilweni kodwa bahlala behlakulela ukungaphumeleli kwintliziyo kwintlungu okanye ebuntwini.
Ngakolunye uhlangothi, ukuba i-valve ye-tricuspid i-dysfunction ilula, umntu onobungozi be-Ebstein unokuhlala ngaphandle kwempawu zabo ubomi bonke.
Kukho ubudlelwane obuqilileyo phakathi kwe-Ebstein anomaly kunye neendlela ezingenamonakalo zombane entliziyweni. Ezi zibizwa ngokuba "iindlela zokufikelela" zenza uxhulumano olungavamile phakathi komnye we-atria kunye nenye yee-ventricle; Ebstein anomaly, bahlala bexhuma i-atrium efanelekileyo ne-ventricle efanelekileyo.
Ezi ndlela zokufumana iinkqubo zivame ukudala uhlobo lwe -tachycardia ephezulu ebizwa ngokuba yi- atrioventricular reentrant tachycardia (AVRT) . Ngamanye amaxesha iindlela ezifanayo zokufumana iinkcukacha zingabangela i- Wolff Parkinson White syndrome , engakhokelela kuphela kwi-AVRT, kodwa nakwii-arrhythmias eziyingozi kakhulu, kubandakanywa i- fibrillation ye-ventricular . Ngenxa yoko, ezi ndlela zokufikelela zingenza ubungozi obungakumbi bokufa ngokukhawuleza.
Ngenxa yokuba i-Ebstein i-anomaly ivelisa ukuhamba kwegazi ephazamisayo ngaphakathi kwe-atrium elungileyo, iingubo zegazi zivame ukwenza apho. Ukuba la macala egazi adibanisa (oko kukuthi, aphule), anokuhamba ngokujikeleza kwaye enze umonakalo weethambo. Ngoko i-Ebstein anomaly ihambelana nokunyuka kwenyameko ye- pulmonary embolus , kwaye (ngenxa yokuba iphuma kwi-right atrium ingadlulela kwi-patent foramen ovale kwi-atrium ekhohlo), inokubangela ukuba unxephezelo.
Izizathu ezibangela ukufa ngenxa ye-Ebstein anomaly yintliziyo yokuhluleka nokufa ngokukhawuleza.
Ukuxilongwa
Uvavanyo oluphambili ekufumaneni i-Ebstein anomaly yi- echocardiogram- ngokuqhelekileyo, uvavanyo lwe-echo e-transesophageal linika iziphumo ezichanekileyo. Nge-echocardiogram, ubukho kunye neqondo le-valve ye-tricuspid engafanelekiyo ingahlolwa ngokuchanekileyo, kwaye ezinye ezininzi iziphoso zesifo sokuzalwa komzimba ezinokuthi zibekho nazo ziyafumaneka.
Kubantu abadala kunye nabantwana abakhulileyo abafumana ukuvavanya kwabo okokuqala ku-Ebstein, abavanyo, ukuvavanywa rhoqo kuvame ukuhlola amandla abo okusebenzisa, i-oxygenation ngexesha lokuzivocavoca, kunye nokuphendula kwezinga lentliziyo kunye nokuxinzelelo lwegazi ukusetyenziswa. Le milinganiselo inceda ekugwebeni ubunzima beemeko zabo zomzimba, kunye nokuyimfuneko yokuphuculwa kwonyango.
Kwakhona kubalulekile ukuvavanya abantu abano-Ebstein abangabonakaliyo phambi kobuchopho be-cardiac arrhythmias. Ukongeza kwi- electrocardiograms yonyaka (ECG) kunye nokubeka iliso kwi-ECG yokujonga , abaninzi bala bantu kufuneka bahlolwe yi-electrophysiologist ye-cardiac emva kokuba bafumaniswe, ukuvavanya amathuba abo okuphuhlisa izicwangciso eziyingozi.
Unyango
Ngokuqhelekileyo, ukuba i-Ebstein anomaly ivelisa iimpawu ezibalulekileyo, unyango lukulungiswa kocwangco.
Ukuhlinzwa kwiintsana ezisandul 'u-Ebstein anomaly ngokuqhelekileyo kubambezeleka ngokukhawuleza kunokwenzeka, ngenxa yengozi enkulu yokulungisa le ngxaki kwiintsana. Ezi ntsana zihlala zilawulwa ngenkxaso yonyango enamandla kwiyunithi yonyango enzulu, kumzamo wokulibazisa ukuhlinzwa kuze kube yilapho banethuba lokukhula. Xa kunokwenzeka, utyando lulibaziseka ubuncinane kwiinyanga eziliqela.
Kubantwana abadala kunye nabantu abadala abasanda kuhlolisiswa nge-Ebstein anomaly, ukulungiswa kocwangco kunconywa ngokukhawuleza ukuba kukho naziphi na iimpawu eziphuhlisayo. Nangona kunjalo, ukuba banesidanga esikhulu sokwehluleka kwentliziyo, iinzame zenzelwa ukuzinzisa ngeyeza lonyango ngaphambi kokusebenza.
Iinkqubo zokugonywa ezisetyenziselwa i-Ebstein anomaly zinokuba nzima kakhulu, kwaye ukungenelelo oluthile olwenziweyo luyahlukahluka kumntu kumntu, kuxhomekeke kwimeko yesigufi se-tricuspid, phambi okanye ukungabikho kweziphene zentliziyo eseleyo, nokuba kungenjalo ukuhluleka kwentliziyo ekhoyo, kunye neminyaka yesigulane.
Ngokuqhelekileyo, injongo yokuhlinzwa iyakuqhelanisa (ngokusemandleni akho) isikhundla kunye nomsebenzi we-valve ye-tricuspid, kunye nokunciphisa i-ventricle efanelekileyo. Kwiimeko ezinzima le njongo ingasetyenziselwa ukusebenzisa ubuchule ukulungiswa ngokugqithiseleyo nokubeka i-valve ye-tricuspid. Kwiimeko ezinzima kakhulu, ukutshintshwa kwe-valvpid valve kunye ne-valve yokufakelwa kuyimfuneko. Uphando lwe-Ebstein anomaly luquka ukulungiswa kweempazamo zeemvelo kunye / okanye i-ventricular defal if ikhona, kunye nayiphi na enye yeengxaki zentliziyo ezithobelayo ezifunyaniswayo.
Abantwana kunye nabantu abadala abafunyaniswa ukuba bane-Ebstein anomaly kuphela, kwaye abangenayo impawu, kaninzi abadingi ukulungiswa kokuhlinzwa. Nangona kunjalo, basadingeka ukubeka iliso ngokubhekiselele kubo bonke ubomi babo nangayiphi na inguqu kwiimeko zabo zenhliziyo. Ngapha koko, nangona "bumnene" be-Ebstein anomaly, basenokuba neendlela zokufumana umbane kunye nokubeka ingozi ye-cardiac arrhythmias, kubandakanywa ingozi yokufa ngokukhawuleza. Ngoko kufuneka kuhlolwe ngenyameko ukujonga lo mngcipheko. Ukuba kukho i-pathway eyingozi enokuthi ichongiwe, ukuxilongwa kweyeza kufuneka kuthathelwe ingqalelo ngokugqithiseleyo ukulahla ukuxhumeka kombane okungavamile.
Isiphumo sexesha elide
Ukugxekwa kwe-Ebstein anomaly kuxhomekeke kubukrakra beengxaki ze-valve ze-valve, kunye nokuba khona okanye ukungabikho kwezinye iingxaki zentliziyo yokuzalwa. Kwiintsana ezile meko ezizalwa zigula kakhulu, umngcipheko wokufa uphezulu-ngaphezu kweepesenti ezingama-30 ziyafa ngaphambi kokuba zikhutshwe esibhedlele.
Umngcipheko wokufa kwangaphambili xa u-Ebstein anomaly efunyaniswa kwi-childhood okanye umntu omdala ngokuxhomekeke kwimeko enzima. Nangona kunjalo, kwiminyaka emva nje, ulawulo lokugonywa ngogonyamelo, kunye nokunyangwa kweprophylactic ye-arrhythmias ye-cardiac, kuphucule kakhulu ukugxekwa kwabantu abane-Ebstein anomaly.
ILizwi
I-Ebstein anomaly yintsholongwane yokuzalwa kunye nokuxhatshazwa kwe-valve ye-tricuspid. Ukubaluleka kwalo mqathango kuyahlukahluka kumntu ukuya kumntu, kwaye ububanzi bube bukhulu kakhulu kunokuba bubuhlungu. Kubalulekile ukuba nabani na u-Ebstein u-anomaly, kwanabo abaneefom zezona zinto zincinci, ukufumana ukuhlolwa kwenhliziyo epheleleyo kunye nokubeka iliso. Ngeendlela zobugcisa zanamhlanje kunye nokuphathwa ngokucophelela, ukuxela abantu abano-Ebstein anomaly kuye kwaphucula kakhulu kwiminyaka emva nje.
> Imithombo:
> Bonow RO, uCarabello BA, Chatterjee K, et al. Ukuhlaziywa okugxininisiweyo ka-2008 kubandakanywa kwi-Acc / Aha 2006 Izikhokelo zoLawulo lwezilwelisi ezinezifo zentliziyo ye-Valvular: Ingxelo ye-American College of Cardiology / i-American Heart Association Umsebenzi weeKhokelo zoLwazi (iKomiti yokuBhala ukuHlola izikhokelo zoLawulo lwe-1998 Izigulane nge-Valvular Heart Disease): zivunywe nguMbutho wee-Cardiovascular Anesthesiologists, uMbutho wee-Cardiovascular Angiography kunye neNongenelo, kunye noMbutho weeNgcali zeTriracic. Uhambo luka-2008; 118: e523.
> Dearani JA, uMora BN, uNelson TJ, et al. Uhlolo lwe-Ebstein Anomaly: Yintoni ngoku, Yintoni elandelayo? Ingcali Rev Cardiovasc Ther 2015; 13: 1101.