I-Brugada syndrome iyinjwayelo engavamile, okungafani nefa le- electrician system engabangela ukufiphaza kwe-ventricular nokufa okungazelelwe kubantu abasha abanobuhle. Ngokungafani nezinye izimo eziningi ezibangelwa ukufa ngokuzumayo kubantu abasha, ama- arrhythmias akhiqizwa yi-Brugada syndrome ngokuvamile avame ngesikhathi sokulala, hhayi ngesikhathi sokuzivocavoca.
Isikhathi
Abantu abaningi abatholwe nge-Brugada syndrome bancane kubantu abadala asebekhulile, abaneminyaka yobudala obuphakathi ngesikhathi sokuxilongwa yi-41. I-Brugada syndrome ibonakala kaningi emadodeni kunabesifazane-kwezinye izifundo ukukhula kwamadoda izikhathi ezingu-9 ngaphezulu kunabesifazane. E-United States, i-Brugada syndrome kucatshangwa ukuthi iyenzeka cishe kubantu abayi-10 000. Kodwa-ke, ivame kakhulu-mhlawumbe ephakeme kunomunye ku-100-kubantu abavela emndenini we-Southeast Asia. Ukuphela kwesimo senhliziyo kuphela yombane kagesi; izinhliziyo zabantu abane-Brugada syndrome zijwayelekile.
Izimpawu
Inkinga ebhubhisa kakhulu i-Brugada syndrome ukufa okungazelelwe ngesikhathi sokulala. Kodwa-ke, abantu abane-Brugada syndrome bangase babe neziqephu zesikhumba esiphansi , isizungu , noma i- syncope (ukulahlekelwa kokuqonda) ngaphambi komcimbi obulalayo. Uma lezi ziqephu ezingezona ezibulalayo zibayisa kudokotela, kungenziwa ukwelashwa futhi ukwelashwa kuqaliswe ukuvimbela ukufa okungazelelwe okulandelayo.
I-Brugada syndrome ibonwe njengesizathu sokwemangalisa "ngokungazelelwe okuchazwe ngokungazelelwe kokubulawa kwesifo se-syndrome," noma i-SUNDS. I-SUNDS yaqale yachazwa emashumini ambalwa eminyaka edlule njengesimo esithinta abesilisa abancane eNingizimu-mpumalanga ye-Asia. Kuye kwaqaphela ukuthi la madoda ase-Asia ayenayo i-Brugada syndrome, evelele kakhulu kulowo mkhakha wezwe kunezinye izindawo eziningi.
Izimbangela
I-Brugada syndrome ibonakala ingenxa yezinto ezihlukile noma ezingaphezu kwezinto eziphilayo ezithinta amangqamuzana enhliziyo, ikakhulukazi, ezakhiweni ezilawula isiteshi sodium. Ifa njengefayela elizimele , kodwa akuwona wonke umuntu onesifo esingavamile noma izakhi zofuzo ezithintekayo ngendlela efanayo.
Isignali kagesi elawula isigqi senhliziyo ikhiqizwa iziteshi ezingxenyeni zamangqamuzana ezinhliziyo, ezivumela izinhlayiya ezikhokhwayo (ezibizwa ngokuthi i-ions) ukugeleza emuva naphambili ngaphesheya kwebhubhane. Ukugeleza kwama-ions ngokusebenzisa lezi ziteshi kuveza isignali kagesi kagesi. Enye yeziteshi ezibaluleke kakhulu isiteshi se-sodium, esivumela i-sodium ukungena kwamangqamuzana enhliziyo. E-Brugada syndrome, isiteshi se-sodium sivinjiwe kancane, ukuze isignali kagesi eyenziwe yinhliziyo ishintshwe. Lokhu kuguqulwa kubangela ukungazinzi kagesi ukuthi, ngaphansi kwezimo ezithile, kungabangela ukufiphaza kwe-ventricular.
Ngaphezu kwalokho, abantu abane-Brugada syndrome bangaba nesimo se- dysautonomia- ukungalingani phakathi kwezwi elizwelayo nokuzwela . Kucatshangelwa ukuthi ukunyuka okuvamile kwezwi elibuhlungu elibhekene ne-parasympathetic elenzeka ngesikhathi sokulala kungase linyanyiswe kubantu abane-Brugada syndrome, nokuthi le ithoni ephawulekayo ye-parasympathtic ingabangela iziteshi ezingavamile ukuba zizinzile, futhi zikhiqize ukufa okungazelelwe.
Ezinye izici ezingabangela ukubulawa kwesifo esibulalayo kubantu abane-Brugada syndrome kubandakanya umkhuhlane, ukusebenzisa i-cocaine, nokusetshenziswa kwemithi ehlukahlukene, ikakhulukazi izidakamizwa ezithile zokucindezeleka.
Ukuxilongwa
Ukungajwayelekile kwamandla kagesi okubangelwa yi-Brugada syndrome kungenza umdwebo we- ECG , iphethini ebizwa ngokuthi iphethini yeBrugada. Leli phethini linomkhiqizo wegatsha lesigxobo sokubamba iqhaza, elihambisane nokuphakama kwezingxenye ze-ST ku-V1 naku-V2.
Akuwona wonke umuntu onesifo se-Brugada one-"typical" ye-Brugada iphethini ku-ECG yawo, nakuba kungenzeka ukuthi izoba nezinye izinguquko ezicashile zokusikisela.
Ngakho-ke, uma i-Brugada syndrome isolwa (ngoba, isibonelo, i-syncope yenzekile noma ilungu lomndeni liye lafa ngokuzumayo ebuthongweni babo), noma yikuphi ukungajwayelekile kwe-ECG kufanele kuhanjiswe kuchwepheshe we-electrophysiology, ukuhlola ukuthi i-pattern "ye-atypical" yaseBrugada ingase ibe okwamanje.
Uma i-ECG yomuntu ibonisa iphethini yeBrugada, futhi uma ibuye ibe neziqephu zokuzizwa okungenalutho noma i-syncope, isinde ekuboshiwe inhliziyo , noma inomlando womndeni wokufa okungazelelwe ngaphansi kweminyaka engu-45, ingozi yokufa okungazelelwe liphezulu. Kodwa-ke, uma iphethini leBrugada likhona futhi akukho neyodwa yalezi ezinye izimo eziyingozi, isengozi sokufa okungazelelwe sibheke kakhulu.
Abantu abane-Brugada syndrome abanengozi enkulu yokufa ngokungazelelwe kufanele baphathwe kabi. Kodwa kulabo abanesibonelo seBrudada ku-ECG yabo kodwa akukho nezinye izici ezingozini, ukukhetha ukuthi ukufutheka kangakanani kungabi njengokucacile.
Ukuhlolwa kwe-Electrophysiologic kuye kwasetshenziswa ukusiza ngalesi sinqumo esinzima sokwelashwa, ngokucacisa ingozi yomuntu ngokufa okungazelelwe. Ikhono lokuhlolwa kwe-electrophysiologic ukuhlola ngokunembile ukuthi ubungozi buphansi kakhulu kunokuphelela. Noma kunjalo, imiphakathi emikhulu yezobuchwepheshe okwamanje isekela ukwenza lokhu kuhlolwa kubantu abanephethini yeBrugada kuma-ECG abo ngaphandle kwezizathu ezengeziwe zokufaka ingozi.
Ukuvivinya izifo kungasiza ekuqinisekiseni ukuthi i-Brugada syndrome ixilongwa, kodwa ngokuvamile akusizi ekuhloleni ingozi yesiguli sokufa okungazelelwe. Ngaphezu kwalokho, ukuhlolwa kofuzo e-Brugada syndrome kuyinkimbinkimbi kakhulu, futhi kaningi akunikezi izimpendulo ezicacile. Ngakho-ke ochwepheshe abaningi abacebisi ukuhlolwa kwezibalo zofuzo kubantu abanobujamo.
Ngenxa yokuthi i-Brugada syndrome iyinkinga yesifo sofuzo ngokuvamile ezuzwa njengefa, izincomo zamanje zibiza ukuhlola zonke izihlobo zokuqala zesilinganiso saso sonke esitholakalayo sinesimo. Ukuhlolwa kufanele kuhlolwe ngokuhlola i-ECG, nokuthatha umlando wezokwelapha ngokucophelela ukufuna iziqephu ze-syncope noma ubukhulu obukhulu bokukhanya.
Ukwelapha
Indlela kuphela eqinisekisiwe yokuvimbela ukufa okungazelelwe ku-Brugada syndrome efaka i- defibrillator engasetshenziswa . Ngokuvamile, izidakamizwa ezithathelwanayo kufanele zigwenywe. Ngenxa yokuthi lezi zidakamizwa zisebenza eziteshini zamangqamuzana ezinhliziyo zomzimba, azigcini nje ukwehluleka ukunciphisa ingozi ye-fibrication ye-ventricular e-Brugada syndrome, kodwa futhi ingase ibe nokwandisa leyo ngozi.
Kungakhathaliseki ukuthi umuntu ophethe i-Brugada syndrome kufanele athole i-defibrillator engakwazi ukugxila kuncike ekutheni ingozi yabo yokufa okungazelelwe iyagwetshwa njengephezulu noma iphansi. Uma ingozi iphezulu (ngokusekelwe ezimpawu noma ukuhlolwa kwe-electrophysiologic), kufanele kuphakanyiswe i-defibrillator. Kodwa ama-defibrillator angenawo amafutha ayabiza futhi aphethe izinkinga zawo , ngakho-ke uma ingozi yokufa okungazelelwe ihlulelwe njengento ephansi, lawa mafoni ayitholakali okwamanje.
Izincomo Zokuzivocavoca
Noma nini lapho umuntu osemusha ethola ukuthi unesifo senhliziyo esingaveza ukufa okungazelelwe, umbuzo wokuthi ngabe uphephile yini ukwenza umsebenzi wokuzivocavoca kufanele ucelwe. Lokhu kungenxa yokuthi ama-arrhythmi amaningi enza ukufa okungazelelwe kubantu abasha cishe kungenzeka ngesikhathi sokuzama.
E-Brugada syndrome, ngokuphambene, ama-arrhythmias abulalayo angenzeka kakhulu ngesikhathi sokulala kunesikhathi sokuzivocavoca. Noma kunjalo, kucatshangwa (okungekho ubufakazi obuncane noma obungenayo inhloso) ukuthi ukuzikhandla okukhulu kungabangela ingozi ephakeme kunaleyo evamile kubantu abakulesi simo. Ngenxa yalesi sizathu i-Brugada syndrome ifakwe emihlahlandleleni ehlelekile eyenziwe ngamaphaneli ochwepheshe abhekisele kuzo izincomo zohlelo lokusebenza kumathamathi abasha abanezimo zenhliziyo.
Ekuqaleni, imihlahlandlela mayelana nokuzivocavoca ne-Brugada syndrome yayinqande kakhulu. Inhlangano ye-Bethesda ye-36 ye-36 ye-Eligibility Izincomo Zabadlali Abanokuncintisana Nezinkinga Zezinhliziyo Eziphakanyisiwe zanconywa ukuthi abantu abane-Brugada syndrome bagweme ukuzivocavoca kakhulu.
Noma kunjalo, lo mkhawulo ophelele uye wabonakala sengathi unzima kakhulu. Ngenxa yokuthi ama-arrhythmias abonakala nge-Brugada syndrome ngokuvamile akenzeki ngesikhathi sokuvivinya umzimba, lezi zincomo zanikezwa ngo-2015 ngaphansi kweziqondiso ezintsha ezivela e-American Heart Association nase-American College of Cardiology.
Ngokusho kwezincomo zamuva zakwa-2015, uma abadlali abasha abane-Brugada syndrome bengenayo izimpawu ezihlobene nokuzivocavoca, kunengqondo ukuba bahlanganyele emidlalweni yokuncintisana uma:
- Bona, odokotela babo, nabazali noma ababheki baqonda izingozi ezingase zihileleke, futhi bavumelene ukuthatha izinyathelo ezidingekayo zokuqapha.
- I- defibrillator yangaphandle yokuzenzakalela (AED) ingxenye evamile yemishini yabo yezemidlalo.
- Izikhulu zeqembu ziyakwazi futhi zizimisele ukusebenzisa i-AED futhi zenze i-CPR uma kudingeka.
Isifingqo
I-Brugada syndrome yisimo esingavamile sezakhi zofuzo esibangela ukufa okungazelelwe, ngokuvamile ngesikhathi sokulala, ngokunye abantu abasha abanobungane. Ubuqili ukuhlolisisa lesi simo ngaphambi kokuba kwenzeke umcimbi ongenakuguquka. Lokhu kudinga odokotela ukuba baqaphele-ikakhulukazi kunoma ubani oye waba ne-syncope noma iziqephu ezingabonakali zesikhumba esiphansi-kokuthola okucashile kwe-ECG okubonwayo ku-Brugada syndrome.
Abantu abaxilongwa ukuthi bane-Brugada syndrome bangalinda njalo ukugwema imiphumela ebulalayo ngokuphathwa ngendlela efanele, futhi bangalindela ukuba baphile impilo evamile.
> Imithombo:
> Brugada P, Brugada J. I-Bundle Branch Block Block, Isigaba se-ST esiqhubekayo Ukuphakama nokufa okungazelelwe komzimba: I-Distinct Clinical and Electrocardiographic Syndrome. Umbiko Wezithombe Eziningi. J Am Coll Cardiol 1992; 20: 1391.
> Maron BJ, Zipes DP, Kovacs RJ, et al. Izincomo Zokufaneleka Nokunciphisa Izintambo Zabadlali Abanokuncintisana Nezinkinga Ze-Cardiovascular. Ukujikeleza kuka-2015; I-DOI: 10.1161 / CIR.0000000000000236.
> Priori SG, Wilde AA, Horie M, et al. I-HRS / i-EHRA / i-APHRS isazi Isitatimende sokubambisana mayelana nokuHlola nokuPhathwa kweziguli ezine-Syndromes eziPhambili eziHlanganisiwe. I-Document evunyelwe yi-HRS, i-EHRA, ne-APHRS ngoMeyi 2013 kanye ne-ACCF, AHA, PACES, ne-AEPC ngoJuni 2013. Isikhathi senhliziyo 2013 ; 10: 1932.
> Zipes, DP, Ackerman, MJ, Estes NA, 3rd, et al. Umsebenzi Weqhaza 7: I-Arrhythmias. J Am Coll Cardiol 2005; 45: 1354.