Long QT Syndrome

Imwe Inowanzokonzera Kungoerekana Yakafa Rufu Kune Vechiduku

Yakareba QT Syndrome (LQTS) chirwere chinokonzerwa nemagetsi emhepo . LQTS inogona kutungamirira kune kamwe kamwe, zvisingatarisirwi, yehupenyu hunotyisa hwetricricular tachycardia inowanzonzi torsades de pointses . Vanhu vane LQTS vari pangozi ye syncope (kurasikirwa kwekuziva) uye kufa kamwe kamwe, kazhinji pazera duku.

Mufananidzo - Torsades de pointses. Nhamba yepamusoro: Kazhinji mwoyo rhythm. Nhamba yepasi: Torsades de pointses. "Torsades de pointes" zvinoreva kutenderera kune imwe nzvimbo. Muchirevo ichi, mwoyo rhythm inokurumidza zvikuru, uye maitiro ematambudziko eEGG anogara achishandura, kazhinji akafanana nesine-wave wave sezvairi mumufananidzo uyu. Apo mwoyo wemagetsi wemhepo unozvibata nenzira iyi, kupinza kwakanaka hakugoneki

Overview

Vanhu vane LQTS vakagara kwenguva refu kweQT pamagetsi avo. Nguva yeKT inomiririra kudzorera, kana kuti "recharge," yesero remwoyo. Mushure mekutsva kwemagetsi emagetsi kunokonzera sero remwoyo (zvichiita kuti rirove), recharging inofanira kuitika kuitira kuti sero rive rakagadzirira kutsvaga kwemagetsi. Nguva yeKT (iyo inoyerwa kubvira pakutanga kwe QRS yakaoma kusvika kumagumo eT wave) ndiyo nguva yakareba yenguva inotora kuti ibudise, uye dzorera zvakare chimiro chemoyo. MuLQTS, nguva yeKT inowedzera. Kukanganisika kwemukati weKT kunokonzera magaramisi anosangana neLQTS.

Zvinokonzera

LQTS idambudziko rakagara nhaka. Majini mazhinji akawanikwa akabatisa nguva yeKT, saka marudzi akasiyana-siyana eLQTS aripo. Mamwe mhuri dzine chiitiko chakakwirira cheLQTS. Nemhaka yokuti majini akawanda anogona kukanganisa nguva yeKT, kusiyana kwakawanda kweLQTS kwakaonekwa.

Zvimwe zveizvi ("classic" LQTS) zvinosanganiswa nehuwandu hwakawanda hwemagadzirirwo ane ngozi uye kufa kamwe kamwe, kunowanzoitika mumhuri dzakawanda dzemhuri. Mimwe mhando dzeLQTS ("LQTS vari vari") inogona kunge isina ngozi. Zvizhinji zvezvipembenene zvemajeni zvakaratidzwa nechikamu chepakutanga chekutanga che QT, uye chironga chemwoyo chinowanzoonekwa chete kana chimwe chinhu chinowedzera (zvakadai sokurapa zvinodhaka, kana kusagadzikana kwemaitiro e electrolyte) kunoita kuti iwedzere nguva ye QT.

Zvisinei, kana nguva dzeKT dzichiwedzera kwenguva refu kuvanhu vane dzakasiyana-siyana zveLQTS, zvirongwa zvinotyisa zvinowanikwa.

Kuwanda

Classic LQTS iripo mune imwe yevanhu 5000. LQTS ndeimwe yezvinowanzokonzera kufa kwechimbichimbi kune vechidiki, zvichiguma pakati pe2000 ne3000 vakafa pagore. Zvimwe zvemaLQTS zvakanyanya kufanana, uye zvichida zvinosvika kusvika 2 - 3% yevanhu.

Zviratidzo

Zviratidzo zveLQTS zvinongoitika kana murwere achiita chiitiko chinotyisa che ventricular tachycardia, uye chiyero chezviratidzo chinoenderana nehurefu hwenguva iyo arrhythmia inopfuurira. Kana ikangogara kwenguva pfupi, mashomanana mashomanana ekuzununguka zvakanyanya angave ari chete chiratidzo. Kana ichiripo kwemaminitsi anopfuura 10 kana zvakadaro, syncope inoitika. Uye kana ikatora kwemaminitsi mashomanana, munhu anenge atambudzwa kazhinji haasati atorazve pfungwa.

Kune avo vane zvimwe zvakasiyana-siyana zveLQTS, zvikamu zvinowanzokonzerwa nekubuda kamwe kamwe kweAdrenaline; yakadaro inogona kuitika panguva yekushanda kwepanyama, kana ichivhiringidzika, kana kana iwe uchitsamwa zvikuru.

Nenzira yakanaka, vanhu vazhinji vane LQTS zvakasiyana-siyana havana kumboona zvirwere zvinopisa upenyu.

Kuziva

Vanachiremba vanofanirwa kufunga nezveRQTS kune ani zvake ane syncope kana kusungwa kwemwoyo, uye mumhuri dzemunhu ane LQTS anozivikanwa.

Chero ani zvake wechidiki ane syncope inowanzoitika panguva yekurovedza muviri, kana mune imwe mamiriro ezvinhu apo kuwedzera kwemazinga adrenaline kwaive kuvepo, inofanira kunge iine LQTS yakanyatsotonga.

Kuongororwa kweLQTS kunowanzoitwa nekucherechedza nguva yakasara yeQT muEECG. Dzimwe nguva chiyero chekugadzirisa chetsika chinodikanwa kuti chibudise chekuita kweEECG. Kuenzanisa kweropa reLQTS uye zvakasiyana-siyana kuri kuita zvakanyanya kushandiswa kupfuura zvaingova gore mashomanana apfuura.

Mishonga

Varwere vazhinji vane LQTS dzakawanda vanorapwa ne beta blockers . Beta vadziviriri vanonyadzisa zvipembenene zveadrenaline izvo zvinokonzera zvikamu zve arrhythmias mune ava varwere.

Zvinosuruvarisa, hazvisati zvichiratidzwa kuti beta-blockers inowedzera kuderedza chiitiko che syncope nekufa kamwe kamwe kune varwere vane LQTS.

Zvinonyanya kukosha kune vanhu vane LQTS uye zvakasiyana-siyana kuti vasapindwa nemishonga yakawanda inokonzera kuwedzerwa kwemukati weKT. Muvanhu ava, madhiragi akadaro anowanzokonzera zvikamu zve torsades de pointses. Zvinodhaka zvinowedzera nguva yeKT ndezvakaipa. Vakuru vezvakaipa ndivo, zvinoshamisa, ma antiarrhythmic zvinodhaka ; mishonga yakasiyana-siyana inopesana nemishonga, uye mishonga inorwisa mabhakitiriya yakadai sa erythromycin, clarithromycin erythromycin, uye azithromycin. CredibleMeds inochengetedza urongwa hwezvinodhaka zvinowanzowedzera nguva yeKT.

Kune vanhu vakawanda vane LQTS, iyo inogadziriswa inogadziriswa ndiyo inonyanya kurapwa. Ichi chigadzirwa chinofanira kushandiswa kune varwere vakapukunyuka kusungwa kwemwoyo, uye zvichida kune varwere vane syncope nekuda kweLQTS kunyanya kana syncope inowanikwa ichiri kutora beta blockers.

Kune vanhu vasingakwanisi kubvumira beta blockers kana kuti vachiri nezviitiko pavanenge vachirapa, vakasiyiwa cardica ruregerero rwekuregererwa (LCSD) inogona kuitwa.

Sodidium channel blockers inogona kushandiswa neLQTS type 3.

Sources

> Chii chakareba QT syndrome? - NHLBI, NIH. National Institutes of Health. http://www.nhlbi.nih.gov/health/health-topics/topics/qt/. Yakaperedzwa musi waSeptember 21, 2011. Yakasvika musi waJuly 27, 2016.

> Alders M, > Christiaans > I. Long QT chirwere . NCBI Bookshelf; June 18, 2015. http://www.ncbi.nlm.nih.gov/books/NBK1129/. Yakasvika musi waJuly 27, 2016.

Moss AJ. Long QT Syndrome. JAMA 2003; 289: 2041.

Li H, Fuentes-Garcia J, Towbin JA. Ikozvino mazano murefu weHT syndrome. Pediatr Cardiol 2000; 21: 542.