Brugada syndrome haisati yambozivikanwa, inokonzerwa nemafungiro emagetsi emwoyo ayo anogona kutungamirira ku ventricular fibrillation uye kufa kamwe kamwe mune zvingangove zvine utano hwevechidiki. Kusiyana nedzimwe mimwe mamiriro ezvinhu anokonzera kufa kamwe kamwe muvechiduku, ma arrhythmias akabudiswa neBrugada syndrome anowanzoitika panguva yokurara, uye kwete panguva yekurovedza muviri.
Occurrence
Vanhu vazhinji vanoona kuti vane chirwere cheBrugada vari vaduku kune vakwegura, vane mavhareji emakore panguva yekuongororwa ndeye 41. Brugada syndrome inoonekwa kakawanda kune varume kupfuura vakadzi-mune zvimwe zvidzidzo kuwanda kwevarume zvipfumbamwe zvakapfuura kupfuura vakadzi. MuUnited States, chirwere cheBrugada chinofungidzirwa kuti chinoitika munenge imwechete mu10 000 vanhu. Zvisinei, yakawanda-inogona kunge yakakwirira seyese mu100-muvanhu veSouth Asia yekuzvarwa. Chinhu chisina kuchena chemwoyo ndeyemagetsi; mwoyo yevanhu vane Brugada syndrome yakarongeka.
Zviratidzo
Dambudziko rinoparadza zvikuru rinokonzerwa neBrugada syndrome rinouraya kamwe kamwe panguva yokurara. Zvisinei, vanhu vane chirwere cheBrugada vanogona kuwana zvikamu zvekudzivirira, chizivo , kana syncope (kurasikirwa kwekuziva) zvisati zvaitika kune chiitiko chinouraya. Kana zviitiko izvi zvisiri kuuraya zvichivaendesa kuna chiremba, chirwere chinogona kugadzirwa uye chirwere chinotangwa kuti chidzivise kufa kwekuerekana kamwe kamwe.
Brugada syndrome yave ichionekwa sechikonzero chekusanzwisisika "kamwe-kamwe kusina kutsanangurwa kwezuva rakabuda kamwe-kamwe nekushaya rufu," kana SUNDS. SUNDS yakatanga kurondedzerwa makumi emakore akapfuura sechiitiko chinokonzera varume vaduku muSouth-Asia. Iko kubva kare yave ichinyatsozivikanwa kuti varume vechidiki vechiAsia vane Brugada syndrome, iyo yakazara zvikuru munzvimbo iyoyo yenyika kupfuura mune dzimwe nzvimbo dzakawanda.
Zvinokonzera
Brugada syndrome inoratidzika kunge yakakonzerwa nechimwe kana kupfuura zvimwe zvipembenene zvinokanganisa masero emwoyo, kunyanya, mumagenzura anotungamirira sodium channel. Izvo zvakatora nhaka sechitarisiko chakakosha , asi haasi munhu wese ane geni risina kujairika kana mazamu akabatwa nenzira imwechete.
Chiratidzo chemagetsi chinodzora mwoyo rhythm inoumbwa nemitsara mumoyo we membrane cell membranes, iyo inobvumira kutengesa zvidimbu (zvinonzi ions) kuti dziyerere mberi nepamusoro pese membrane. Kuyerera kweiyoni kuburikidza nenzira idzi kunounza chiratidzo chemagetsi emwoyo. Imwe yenzira dzinonyanya kukosha ndeyeyerodium, iyo inobvumira sodium kupinda masero emwoyo. MuBrugada syndrome, gwara re sodium rinodziviswa zvishoma, kuitira kuti chiedza chegesi chakagadzirwa nemwoyo chichinje. Iko kushandura kunotungamirira kune kusagadzikana kwemagetsi izvo, mune mamwe mamiriro ezvinhu, zvinogona kubudisa ventricular fibrillation.
Mukuwedzera, vanhu vane Brugada syndrome vanogona kunge vane dysautonomia- kusanzwisisana pakati pekunzwira tsitsi nekutaura kwemazwi. Iko kunotsanangurwa kuti kuwedzera kwemazwi anonyadzisa kunowanikwa panguva yekurara kunogona kuwedzererwa kune vanhu vane Brugada syndrome, uye kuti izwi iri rakasimba parasympathy rinogona kuita kuti nzira dzisina kunaka dzisagadzikane, uye dzinobudisa kufa kamwe kamwe.
Zvimwe zvinhu zvinogona kukonzera chironga chinouraya kuvanhu vane Brugada syndrome vanosanganisira fever, kushandisa cocaine, uye kushandiswa kwemishonga yakasiyana-siyana, kunyanya zvimwe zvinodhaka zvinodhaka.
Kuziva
Izvo zvinokonzera magetsi zvinokonzerwa neBrugada syndrome zvinogona kuita chimiro chemuitiro paEECG , muenzaniso unonzi Brugada pattern. Iyi muenzaniso inosanganisira bhizimusi rekodhi rebazi , rakakonzerwa nekukwirira kwezvikamu zve ST mumutungamiri V1 uye V2.
Haasi munhu wose ane Brugada syndrome ane "inowanzo" yeBrugada pattern paEECG yavo, kunyange zvazvo inogona kunge ine zvimwe zvinyengeri zvingachinja.
Saka, kana chirwere cheBrugada chikafungidzirwa (nokuti, somuenzaniso, syncope yakamboitika kana imwe nhengo yemhuri yafa kamwe kamwe muhope dzavo), chero humwe hutachiona hweEGG hunofanira kutumirwa kune nyanzvi ye electrophysiology, kuongororai kana "chiitiko" cheBrugada chinogona kuva iripo.
Kana munhu weEEC ari kuratidza muenzaniso weBrugada, uye kana iye akave ane zvikamu zvisingatsanangurwi mutsivi kana kuti syncope, yakapukunyuka kusungwa kwemoyo , kana ine nhoroondo yemhuri yekufa kamwe kamwe pasi pemakore makumi maviri nemashanu, njodzi yekufa kamwe kamwe yakakwirira. Zvisinei, kana ruvara rweBrugada rwuripo uye hapana chimwe chezviitiko zvimwe zvinokonzera kuitika, njodzi yekufa kamwe kamwe inoratidzika zvikuru.
Vanhu vane chirwere cheBrugada vane ngozi yakawanda yekufa kamwe-kamwe vanofanira kuitirwa utsinye. Asi kune avo vane maitiro eBuddha paEECG asi pasina zvimwe zvinokonzera njodzi, kusarudza kuti kusuruvara kuve kusati kwave kwakajeka-kutemwa.
Kuongorora kweElectrophysiologic kwave kushandiswa kubatsira nechisarudzo ichi chakaoma kurapwa, nekujekesa njodzi yemunhu yekufa kamwe kamwe. Unyanzvi hwekuedza kwe electrophysiologic kuongorora zvakananga kuti njodzi inonyanya kuderera kupfuura yakakwana. Kunyange zvakadaro, mapoka makuru emagariro evanhu iye zvino anotsigira kuita chiyero ichi kuvanhu vane bhagada purogiramu yavo yeEGG pasina zvimwe zvinokonzera ngozi.
Kuongorora zvirwere kunogona kubatsira kutsigira kuongororwa kweBrugada syndrome, asi kazhinji hazvibatsiri pakufungidzira dambudziko remurwere wekuerekana kamwe kufa. Uyezve, kuongorora mazita eBrugada syndrome kwakaoma zvikuru, uye kazhinji hazvibvumi mhinduro dzakajeka. Nokudaro nyanzvi dzakawanda hadzikurudziri nguva dzose maitiro ekuongorora maitiro kune vanhu vane chirwere ichi.
Nemhaka yokuti chirwere cheBrugada chinonzi genetic disorder inowanzotora nhaka, mazano anowanzokumbira kuongorora zvose zvekutanga-degree hama dzevamwe vanorwara vane chirwere ichi. Kuongorora kunofanirwa kuenzanisa nekuongorora ECG, uye kutora nhoroondo yezvokurapa zvakanyatsotarisa kutsvaga zvikamu zve syncope kana hutsinye hwakasimba.
Kurapa
Iyo nzira chete inopupurirwa yekudzivirira kufa kwechimbichimbi muhutano hweBrugada iri kuisa chinodzivirira chinogadziriswa. Kazhinji, zvinodhaka zvinodhaka zvinofanira kudziviswa. Nemhaka yemishonga yemishonga iyi inoshanda mumitambo iri mumoyo memakumbo emumakumbo, havangokundikani kuderedza dambudziko re ventricular fibrillation muBrugada syndrome, asi zvakare inogona kuwedzera ngozi iyo.
Kunyangwe mumwe munhu ane chirwere cheBrugada achifanira kugadziriswa chinogadziriswa chinogadziriswa chinoenderana kana ngozi yavo yekufa kamwe-kamwe inopomerwa kuti yakakwirira kana yakaderera. Kana njodzi yacho yakakwirira (zvichienderana nezviratidzo kana kuti electrophysiologic test), munhu anozvidzivirira anofanirwa kukurudzirwa. Asi zvinogadziriswa zvinodhura zvinodhura uye zvinotakura zvinetso zvayo , saka kana ngozi yekufa kamwe-kamwe inonzi yakaderera, izvi zvigadzirwa hazvibudiswi panguva ino.
Ita mazano
Chero nguva iyo wechidiki akaonekwa kuti ane chirwere chemwoyo chinogona kubereka kufa kamwe kamwe, mubvunzo wekuti wakachengeteka kuita basa rinofanira kubvunzwa. Ichi ndechokuti akawanda magythmias anobudisa kufa kamwe kamwe mumajaya vanhu vanowanzoitika panguva yekuedza.
MuBrugada syndrome, zvakasiyana, zvirongwa zvinouraya zvinowanzoitika panguva yokurara kupfuura panguva yekurovedza muviri. Kunyange zvakadaro, inofungidzirwa (isina zvishoma kana pasina chinangwa chinangwa) kuti kuedza kwakasimba kunogona kuisa njodzi yakakwirira-kupfuura-inowanikwa kuvanhu vane chirwere ichi. Nokuda kweizvi chikonzero cheBrugada syndrome chinowanikwa mumitemo yakarongeka inoumbwa nevanodzidza panels iyo yakanyorera maitiro ekuongororwa kwevatambi vechidiki vane mamiriro emwoyo.
Pakutanga, mirayiridzo pamusoro pekurovedza muviri neBrugada syndrome yakanga ichidzivirira. Musangano weBethesda weBrazil wa2005 pamusoro peEgligility Recommendations yeVakwikwidza Vakatambira neAIDS Inoratidzira kuti vanhu vane Brugada syndrome varege kudzivirira muviri wose.
Zvisinei, izvi zvinoregererwa zvachose zvakazoonekwa sezvo zvakanyanya. Tichifunga nezvekuti ma arrirthmias anoonekwa neBrugada syndrome haawanzoitiki panguva yekurovedza muviri, izvi zvinorayirwa zvakasunungurwa muna 2015 pasi pemitemo mitsva inobva kuAmerican Heart Association uye American College of Cardiology.
Maererano nezvakaitwa munguva pfupi yapfuura, 2015, kana vatambi vechidiki vane Brugada syndrome vasina zviratidzo zvine chokuita nekurovedza muviri, zvinonzwisisika kuti vabatane mumakwikwi ekukwikwidza kana:
- Ivo, vanachiremba, nevabereki kana vatarisi vanonzwisisa zvinogona kuitika, uye vakabvumirana kutora matanho anodiwa.
- Anowanzobuda kunze defibrillator (AED) chikamu chechidimbu chemidziyo yavo yemitambo.
- Vakuru vakuru vanogona uye vanoda kushandisa AED uye vanoita CPR kana zvichifanira.
Summary
Brugada syndrome inowanzosangana nemararamiro anokonzerwa nekufa kamwe kamwe, kazhinji panguva yokurara, mune vamwe vane utano hwakanaka vechidiki. Icho chinyengeri ndechokuongorora chirwere ichi chisati chave chiitiko chisingaregereki chinoitika. Izvi zvinoda vanachiremba kuti vave vakangwarira-kunyanya kune ani zvake ave ane syncope kana zviitiko zvisingatsanangurwi zvoupamhi-kune zvakajeka ECG zvitsva zvinowanikwa muBrugada syndrome.
Vanhu vane chirwere cheBrugada syndrome vanogona kunge vachingogara vachidzivisa zvinogumbura nekurapa kwakakodzera, uye vanogona kutarisira kurarama hupenyu huri hupenyu.
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> Maron BJ, Zipes DP, Kovacs RJ, et al. Kukodzera uye Kukanganiswa Zvikurudziro zveVakwikwidzi Vanomhanya neCardiovascular Abnormalities. Circulation 2015; DOI: 10.1161 / CIR.0000000000000236.
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