I-Brugada syndrome ayinto engaqhelekanga, inzala engafanelekanga yenkqubo yamandla kagesi engakhokelela ekufakeni i-fibrillation ye-ventricular kunye nokufa ngokukhawuleza kubantu abaselula abasempilweni. Ngokwahlukileyo kwezinye iimeko ezibangelwa ukufa ngokukhawuleza kubantu abancinci, ama- arrhythmias aveliswa yi-Brugada syndrome ngokuqhelekileyo ayenzeka ngexesha lokulala, kungekhona ngexesha lokuzivocavoca.
Isiganeko
Uninzi lwabantu abaxilongwa nge-Brugada syndrome bancinci kubantu abadala abaphakathi, abaneminyaka engama-age ubudala ngexesha lokuxilongwa yi-41. I-Brugada syndrome ibonakala ngokuphindaphindiweyo kumadoda kunabesifazane-kwezinye iimeko ukuxhaphaka kwamadoda amaxesha athoba ngaphezulu kunabesifazane. EUnited States, i-Brugada syndrome kucingelwa ukuba ivele malunga nabantu abayi-10 000. Nangona kunjalo, ixhaphake-mhlawumbi iphakamileyo njengenye ye-100-kubantu abavela kumzantsi we-mpuma ye-Asia. Ukuphela kwesimo senhliziyo esisodwa ngumbane; iintliziyo zabantu abane-Brugada syndrome ziqhelanisekile.
Iimpawu
Ingxaki eyingozi kakhulu ebangelwa yiBrugada syndrome kukufa ngokukhawuleza ngexesha lokulala. Nangona kunjalo, abantu abane-syndrome yeBrugada banokufumana iziqulatho zokukhanya , utywala , okanye i- syncope (ukulahlekelwa kwengqondo) ngaphambi kwesiganeko esibulalayo. Ukuba ezi ziqhelo ezingabhubhi zibazisa ngogqirha, ukuxilongwa kungenziwa kwaye unyango lubekwe ukukhusela ngokukhawuleza ukufa.
I-Brugada syndrome ibonwe njengesizathu sokungacacisanga "ngokungazelelwe okuchazwe ngokukhawuleza kweso sifo sokufa," okanye i-SUNDS. I-SUNDS zachazwa okokuqala kwiminyaka emininzi edlulileyo njengemeko echaphazela abantu abesilisa abaseMzantsi-mpuma Asia. Kuye kwaqaphela ukuba la madoda aseAsia ase-Brugada, ayeninzi kakhulu kuloo ndawo yehlabathi kunezona ndawo ezininzi.
Izizathu
I-Brugada syndrome ibonakala ngenxa yento enye okanye engafanelekanga yemfuyo echaphazela iiseli zenhliziyo, ngokukodwa, kwiijethi ezilawula ishanedi yesiteshi. Ilifa njengeli- autosomal traital trait , kodwa akusiyo wonke umntu onomdla ongaqhelekanga okanye izakhi zofuzo ezichaphazelekayo ngendlela efanayo.
Isibonakaliso sombane esilawula isigqi senhliziyo senziwa yimigudu kwiimbilini zeseli zomzimba, ezivumela iintlobo zeengqungquthela (ezibizwa ngokuba yi-ions) ziza kuhamba zijikeleze ngaphaya kwebhubhane. Ukugqithisa kweeon ngokusebenzisa ezi ziteshi kuvelisa uphawu lombane wenhliziyo. Enye yezona ndlela zibaluleke kakhulu isiteshini sodium, evumela i-sodium ukungena kwiiseli zengqondo. Kwi-syndrome yaseBrugada, umzila we-sodium uvinjiwe ngenxalenye, ukwenzela ukuba isiganeko sombane esenziwe yintliziyo sitshintshwe. Olu tshintsho lubangela ukungazinzi kombane, ngezinye iimeko, kunokuvelisa i-fibrillation ye-ventricular.
Ukongezelela, abantu abane-syndrome yeBrugada banakho uhlobo lwe- dysautonomia- ukungalingani phakathi kwethoni ebonisa uvelwano kunye ne-parasympathetic . Kucetyiswa ukuba ukunyuka okuqhelekileyo kwitheyiphu ye-parasympathetic eyenzeka ngexesha lokulala kunokuxakishwa kubantu abane-syndrome ye-Brugada, kwaye ukuba le ntoni eqhelekileyo ye-parasympathtic ingabangela ukuba iziteshi ezingavumelekanga zibe zizinzile, kwaye zivelise ukufa kwangokuzelelwe.
Ezinye izinto ezinokubangela i-arrhythmia ebulalayo kubantu abane-syndrome ye-Brugada kubandakanya umkhuhlane, ukusebenzisa i-cocaine, kunye nokusetyenziswa kwamachiza ahlukeneyo, ngakumbi izidakamizwa ezithile ezixinzelelekayo.
Ukuxilongwa
Ukungaqhelekanga kombane okubangelwa yi-Brugada syndrome kunokuvelisa uhlobo lwesimo kwi- ECG , iphethini ebizwa ngokuba ngumzekelo waseBrugada. Le pateni inesibonda sesigxina se-pseudo- right , ehamba kunye neendawo eziphakamileyo ze-ST zigaba kwi-V1 naku-V2.
Akunabo bonke abane-syndrome ye-Brugada inomzekelo "oqhelekileyo" weBrugada kwi-ECG yabo, nangona kunjalo banokuba nezinye iinguqu ezicetyisayo zokutshintsha.
Ngoko, ukuba i-Brugada syndrome ikhunjulwa (ngokuba, umzekelo, i-syncope yenzekile okanye ilungu losapho lifile ngokukhawuleza ekulala kwabo), nayiphi na i-ECG engafanelekanga ifanele ibhekiswe kwi-electrophysiology ingcali, ukuvavanya ukuba iprotected "inypical" yaseBrugada ingaba zikhoyo.
Ukuba i-ECG yomntu ibonisa iphetheni yeBrugada, kwaye ukuba nayo ibe neengqamlezo zesimo sezulu esingazichaziyo okanye isivumelwane, sisinde ekubanjweni kwintliziyo , okanye inokholo losapho lokufa ngokukhawuleza ngaphantsi kweminyaka engama-45, umngcipheko wokufa ngokukhawuleza. phezulu. Nangona kunjalo, ukuba iphepheni yeBrugada ikhoyo kwaye akukho nenye yale mingcipheko eye yenzeke, ingozi yokufa ngokukhawuleza ibonakala iphantsi.
Abantu abane-Brugada syndrome abanomngcipheko omkhulu wokufa ngokukhawuleza kufuneka baphathwe kakubi. Kodwa kulabo abanomzekelo weBrudada kwi-ECG yabo kodwa akukho mnye umngcipheko, ukugqiba ukuba ubugwenxa bube bunjani.
Uvavanyo lwe-Electrophysiologic lusetyenziswe ukunceda kule sigqibo esinzima kunonyango, ngokucacisa umngcipheko womntu wokufa ngokukhawuleza. Ukwazi ukuhlolwa kwe-electrophysiologic ukuvavanya ngokuchanekileyo loo mngcipheko ungaphantsi kakhulu. Sekunjalo, iindawo eziphambili zengcali zixhasa ukuqhuba olu vavanyo kubantu abanomzekelo waseBrugada kwi-ECG yabo ngaphandle kweengxaki ezingaphezulu.
Ukuvavanya i-genetic kunokunceda ukuqinisekisisa ukuxilongwa kwe-Brugada syndrome, kodwa ngokuqhelekileyo akunakunceda ukuqikelela ingozi yomguli wokufa ngokukhawuleza. Ukongezelela, ukuhlolwa kofuzo kwi-Brugada syndrome kuyinkimbinkimbi kwaye kaninzi ayiphumeli iimpendulo ezicacileyo. Ngoko ezininzi iingcali azicebisi ukuhlolwa kofuzo oluqhelekileyo kubantu abanalo mqathango.
Ngenxa yokuba i-Brugada syndrome yintlupheko yofuzo edlalwa njengefa, iziphakamiso zangoku zifuna ukuhlolisisa zonke izihlobo ze-first-degree of any diagnostic. Ukuhlola kufuneka kuquke ukuhlola i-ECG, kwaye uthathe imbali yonyango ngokucophelela ifuna izigcawu ze syncope okanye ubunzima obukhulu.
Unyango
Inye indlela eqinisekisiweyo yokuthintela ukufa ngokukhawuleza kwi-Brugada syndrome ifaka i- defibrillator enokutsha . Ngokuqhelekileyo, iziyobisi ezinqabileyo kufuneka ziphetshwe. Ngenxa yendlela leziyobisi zisebenza ngayo kumashaneli emagqabini e-cell, ayithinteli nje ukunciphisa umngcipheko we-fibrillation ye-ventric e-Brugada syndrome, kodwa inokwandisa ingozi.
Ingaba umntu onentsholongwane kaBrugada kufuneka afumane i-defibrillator engenakho ukuxhomekeka ekubeni kuxhomekeke ekubeni umngcipheko wokufa ngokukhawuleza ugqithiselwa ukuba uphakeme okanye uphantsi. Ukuba umngcipheko uphakeme (ngokusekelwe kwiimpawu okanye ukuhlolwa kwe-electrophysiologic), kufuneka i-defibrillator iphakanyiswe. Kodwa i-defibrillators ezixhomekeke kwimida zibiza kwaye zithwala iingxaki zabo , ngoko ke ukuba ingozi yokufa ngokukhawuleza ikwahlulelwa ukuba iphantsi, ezi zixhobo azikhuthazwa njengamanje.
Iingcebiso Zomsebenzi
Naliphi na ixesha umntu omtsha ofumanisa ukuba unesimo senhliziyo esingavelisa ukufa ngokukhawuleza, umbuzo wokuba ukhuselekile ukwenza umthambo kufuneka uceliwe. Oku kungenxa yokuba ezininzi i-arrhythmias ezivelisa ukufa ngokukhawuleza kubantu abancinci ziyakwenzeka xa ziqhubeka.
Kwi-Brugada syndrome, ngokuphambene, i-arrhythmias ezibulalayo zinokuthi zenzeke ngexesha lokulala kunexesha lokuzivocavoca. Sekunjalo, kucingwa (nangobungqina obuncinane okanye abukho ubungqina obunobunzima) ukuba ukunyamezela kunokuba ngumngcipheko ophezulu kunabo bonke abantu abaneli meko. Ngenxa yeso sizathu i-Brugada syndrome ifakwe kwizikhokelo ezisemthethweni eziveliswe ngamaphaneli oobuchule abaye bajongana neziphakamiso zoqeqesho kwiintlabathi ezincinane ezineemeko zomzimba.
Ekuqaleni, izikhokelo malunga nokuzilolonga kunye ne-Brugada syndrome zazingqongqo. Ingqungquthela ye-Bethesda ye-36 ye-36 ye-Eligibility Iziphakamiso ze-Athletes ezikhuphisanayo kunye nee-Cardiovascular abnormalities zinconywa ukuba abantu abane-syndrome ye-Brugada bagweme ukuzivocavoca okukhulu.
Nangona kunjalo, lo mqathango uqobo luye lwaqatshelwa ngokuba lukhulu kakhulu. Ngenxa yokuba i-arrhythmias ebonwayo ne-Brugada syndrome ngokuqhelekileyo ayifani ngexesha lokuzivocavoca, ezi ziphakamiso zanikelwa ngo-2015 phantsi kwezikhokelo ezintsha ezivela kwi-American Heart Association kunye ne-American College of Cardiology.
Ngokweziphakamiso zamuva zango-2015, ukuba abadlali abancinci abane-Brugada syndrome abazange babe neempawu ezinxulumene nokuzivocavoca, kunengqiqo ukuba bathathe inxaxheba kwimidlalo yokukhuphisana ukuba:
- Bona, oogqirha babo, nabazali okanye abagcini bayiqonda ingozi ebandakanyekayo, kwaye baye bavuma ukuthatha izilumkiso eziyimfuneko.
- I- defibrillator yangaphandle yendalo yangaphandle (AED) yinto eqhelekileyo yezoxhobo zokusebenza zemidlalo.
- Amagosa eqela akwazi kwaye azimisele ukusebenzisa i-AED kwaye enze i-CPR xa kuyimfuneko.
Isishwankathelo
I-Brugada syndrome yimeko engavamile yokuvelisa izityalo zofuzo, ngokuqhelekileyo ngexesha lokulala, ngokunye abantu abatsha abanobomi. Ubuqhetseba kukuxilonga le meko ngaphambi kokuba kwenzeka isiganeko esingenakwenzeka. Oku kufuna ukuba ogqirha baqaphele-ikakhulukazi nakubani na oye waba ne-syncope okanye iziganeko ezingabonakali zezihlokwana-kwiziphumo ezifihlakeleyo ze-ECG ezibonakala kwi-syndrome yeBrugada.
Abantu abafunyaniswa ukuba bane-syndrome yeBrugada banokuthi bahlale bephephe isiphumo esibulalayo ngokunyanga, kwaye banokulindela ukuba baphile ubomi obuqhelekileyo.
> Imithombo:
> Brugada P, uBrugada J. I-Bundle yeSigqeba segatsha lasekunene, iSigqeba esiqhubekayo ISigaba seNkcenkcesha kunye nokuFaleka kweCaracac ngokukhawuleza: i-Clinic Distinct and Electrocardiographic Syndrome. Ingxelo yeMatticenter. J Am Coll Cardiol 1992; 20: 1391.
> Maron BJ, Zipes DP, Kovacs RJ, et al. Izilungelelwaniso kunye neziNcomelo zokuKhutshwa kweMidlalo yeCompetitive Athletes ne-Cardiovascular abnormalities. Uhambo luka-2015; INGXELO: 10.1161 / CIR.0000000000000236.
> Priori SG, Wilde AA, Horie M, et al. I-HRS / i-EHRA / i-APHRS Ingcali Ingxelo yokuBoniswa kweNgcaciso noLawulo lwabaPhezulu abaneMveli ye-Primary Arrhythmia Syndromes: Uxwebhu olwamkelwa yi-HRS, i-EHRA kunye ne-APHRS ngo-Meyi 2013 kunye ne-ACCF, i-AHA, i-PACES, ne-AEPC ngoJuni 2013. IsiHlomelo senhliziyo 2013 ; 10: 1932.
> Zipes, DP, Ackerman, MJ, Estes NA, 3rd, et al. Umsebenzi wamaqela 7: Arrhythmias. J Am Coll Cardiol 2005; 45: 1354.