Brugada syndrome chirwere chisingawanzozivikanwa chinoratidzwa nekubatanidzwa kwemoyo. Zvinowanzo kuguma nekuwedzera kwemoyo (arrhythmia). Uku kuwanda kwemoyo kunogona kukonzeresa kuvepo kwekurohwa, kufenda kana kutofa. (2)

Vamwe varwere vanogona kunge vasina chero zviratidzo. Nekudaro, kunyangwe ichi chiri chokwadi uye zvakajairika mumutsipa wemwoyo, shanduko kamwe kamwe muchiitiko chemagetsi chemwoyo chinogona kuva nenjodzi.

Icho hutachiona hutachiona hunogona kutapurirwa kubva kuchizvarwa kusvika kuchizvarwa.

Kuwanda chaiko (huwandu hwezviitiko zvechirwere panguva yakapihwa, mune yakapihwa huwandu hwevanhu) hazvizivikanwe. Nekudaro, fungidziro yayo ndeye 5 / 10. Izvi zvinoita kuti ive chirwere chisingawanzo chinogona kuuraya varwere. (000)

Brugada syndrome inonyanya kubata vadiki kana vezera repakati zvidzidzo. Hukuru hwechirume hunoonekwa mune hurwere uhwu, pasina kuve nehutsanana husina kunaka hwehupenyu. Kunyangwe ichi chikuru chechirume, vakadzi vanogona zvakare kukanganiswa neBrugada syndrome. Iyi nhamba yakakura yevarume vanobatwa nechirwere ichi inotsanangurwa neakasiyana echirume / echikadzi mahormone system. Chokwadi, testosterone, yemurume chete mahormone, yaizove nebasa rakakosha mukusimudzira hutachiona.

Iyi yechirume / yevakadzi inotungamira inofungidzirwa inofungidzirwa neiyo 80/20 chiyero chevarume. Mune huwandu hwevarwere gumi vane Brugada syndrome, vasere vanowanzova varume uye vaviri vakadzi.

Zvidzidzo zveEpidemiological zvakaratidza kuti chirwere ichi chinowanikwa nehuwandu hwehuwandu hwevarume muJapan nekuSoutheast Asia. (2)

MuBrugada syndrome, zviratidzo zvekutanga zvinowanzoonekwa kusati kwatanga kweasina mwero wakakwira wemoyo. Izvi zviratidzo zvekutanga zvinofanirwa kuzivikanwa nekukurumidza sezvazvinogona kuitira kuti zvidzivise matambudziko, uye kunyanya kusungwa kwemoyo.

Izvi zvinoratidzira zvekutanga zvekiriniki zvinosanganisira:

• kusagadzikana kwemagetsi kwemoyo;
• palpitations;
• chizoro.

Icho chokwadi chekuti chirwere ichi chine mavambo enhaka uye kuvapo kwezviitiko zvechirwere ichi mukati memhuri kunogona kusimudza mubvunzo wekuvapo kwechirwere munyaya iyi.

Zvimwe zviratidzo zvinogona kudaidzira kukura kwechirwere. Chokwadi, vangangoita 1 muvashanu varwere vane chirwere cheBrugada vakawana atrial fibrillation (hunhu hwechiitiko chakasarudzika chetsandanyama yemoyo) kana kutoratidza mwero wakakura kwazvo wemoyo kurova.

Kuvapo kwefivha muvarwere kunowedzera mukana wavo wekuwedzera zviratidzo zvine hukama neBrugada syndrome.

Mune zvimwe zviitiko, iyo isina kujairika moyo rhythm inogona kuramba ichitungamira kune ventricular fibrillation. Ichi chiitiko chekupedzisira chinoenderana neakateedzana zvisingaite nekukurumidza uye zvisina kurongeka mwoyo contractions. Kazhinji, kurova kwemoyo hakudzokere kune zvakajairwa. Iyo magetsi munda weiyo moyo mhasuru inowanzo kukanganiswa ichikonzera kumisa mukushanda kwepombi yemoyo.

Brugada syndrome inowanzo tungamira mukusungwa kamwe kamwe kwemoyo uye nekudaro kune kufa kwechinyorwa. Izvo zvidzidzo zvakakanganiswa, kazhinji, vechidiki vane mararamiro ane hutano. Kuongororwa kunofanirwa kushanda nekukurumidza kuitira kuti umise kurapwa nekukurumidza uye nekudaro urege kuuraya. Nekudaro, izvi kuongororwa zvinowanzo kunetsa kumisikidza kubva pakuona uko zviratidzo zvisingawanzoonekwa. Izvi zvinotsanangura kufa kamwe kamwe mune vamwe vana vane Brugada syndrome iyo isingaratidze zviratidzo zvinooneka zvinotyisa. (2)

Iko mhasuru chiitiko chemoyo wevarwere vane Brugada syndrome zvakajairika. Izvo zvisizvo zviri mune yemagetsi chiitiko chacho.

Pamusoro pemwoyo, pane madiki pores (ion chiteshi). Aya ane kugona kuvhura nekuvhara pamwero wenguva dzose kubvumira calcium, sodium uye potassium ion kuti ipfuure mukati memasero emoyo. Aya mafambiro eiononi anobva ari panotangira chiitiko chemagetsi chemoyo. Chiratidzo chemagetsi chinogona kutambanudza kubva kumusoro kwetsandanyama yemoyo zvichidzika uye zvobva zvabvumira moyo kubata uye kuita basa rayo reropa "pombi".

Kwakatangira Brugada syndrome majini. Kusiyana kwakasiyana kwemajini kunogona kuve chikonzero chekusimudzira chirwere.

Iyo geni inowanzo kuve nechekuita mukurwara ndeye SCN5A geni. Iri jeni rinouya mukutamba mukuburitswa kweruzivo kubvumira kuvhurwa kwenzvimbo dze sodium. Shanduko mukati meiyi geni yekufarira inokonzeresa kugadzirwa kweprotein inobvumira kuvhurwa kweiyi ion chiteshi. Mupfungwa iyi, kuyerera kweioniyamu ions kwakadzikira zvakanyanya, kuchikanganisa kurova kwemoyo.

Kuvapo kweimwe chete yemaviri makopi eiyo SCN5A geni kunoita kuti zvikwanise kukonzera kusagadzikana mukuyerera kweion. Kana, muzviitiko zvakawanda, munhu akabatwa ane mumwe wevabereki vaviri vane geni shanduko yeiyo geni.

Uye zvakare, mamwe magene uye zvekunze zvinhu zvinogona zvakare kunge kuri pakutanga kwekusanzwisisika padanho remagetsi chiitiko chemoyo mhasuru. Pakati pezvinhu izvi, tinoona: mamwe madhiragi kana kusaenzana mune sodium mumuviri. (2)

Chirwere chinoparadzirwa by autosomal inonyanya kutamisa. Either, kuvapo kweimwe chete yemaviri makopi ejeni rekufarira kwakaringana kuti munhu agadzire iyo phenotype inosangana nechirwere. Kazhinji, munhu akabatwa ane mumwe wevabereki vaviri vane geni rakachinja. Nekudaro, mune zvisingawanzoitika zviitiko, shanduko nyowani dzinogona kuoneka mune iri geni. Aya kesi ekupedzisira ane chekuita nezvevaya vasina nyaya yechirwere mukati memhuri yavo. (3)

Izvo zvinhu zvine njodzi zvinosanganisirwa nechirwere ndezvemajini.

Muchokwadi, kutapurirana kweBrugada syndrome kwakanyanya autosomal. Either, kuvepo kweimwe chete yemaviri makopi ejeni rakachinjika kunodiwa kuti chidzidzo chipupurire chirwere. Mupfungwa iyi, kana mumwe wevabereki vaviri akaunza shanduko mune geni rekufarira, kutwasuka kwechirwere ichi kunogona.

Kuongororwa kwechirwere kunobva pane yekutanga kusiyanisa kuongororwa. Chokwadi, chiri kutevera kuongororwa kwechiremba nachiremba mukuru, achiona zviratidzo zvechirwere munyaya iyi, kuti kukura kwechirwere kunogona kubviswa.

Kutevera izvi, kushanya kuna chiremba wezvemwoyo kunogona kukurudzirwa kuti usimbise kana kuti kwete kusiyanisa kuongororwa.

Iyo electrocardiogram (ECG) ndiyo yegoridhe chiyero pakuongorora iyi syndrome. Uyu muyedzo unoyera kuyerwa kwemoyo pamwe nemagetsi chiitiko chemoyo.

Muchiitiko chekuti Brugada syndrome inofungidzirwa, kushandiswa kwemishonga yakadai se: ajmaline kana kunyange flecainide inoita kuti zvikwanise kuratidza kukwirira kwechikamu che ST mune varwere vanofungidzirwa kuti vane chirwere.

Echocardiogram uye / kana Magnetic Resonance Imaging (MRI) inogona kudikanwa kuti uongorore kuvepo kwemamwe matambudziko emoyo. Uye zvakare, iyo yeropa bvunzo inogona kuyera iwo mazinga e potasium uye calcium muropa.

Genetic bvunzo dzinogona kuona kuti panogona kuvepo nekusajairika muSCN5A geni inobatanidzwa muBrugada syndrome.

Iyo yakajairwa kurapwa yeiyi mhando yehutachiona yakavakirwa pakuiswa kweiyo yemoyo defibrillator. Iyo yekupedzisira yakafanana neye pacemaker. Ichi chishandiso chinoita kuti zvikwanisike, mune chiitiko cheasina kujairika kurohwa frequency, kuendesa magetsi emagetsi achibvumira murwere kuti adzokerezve akajairwa moyo mutinhimira.

Parizvino, hapana mushonga wemishonga uripo pakurapa chirwere. Uye zvakare, mamwe matanho anogona kutorwa kudzivirira kusagadzikana kwemutinhimira. Izvi zvinonyanya kuitika nekubviswa nekuda kwemanyoka (kukanganisa muyero wesodium mumuviri) kana kunyangwe fivha, nekutora mishonga yakakwana. (2)