Isizathu Esivamile Sokufa Okungazelelwe Kwabancane
I-Long QT Syndrome (i-LQTS) yinkinga ezuzwe njengefa lenqubo kagesi yenhliziyo . I-LQTS ingabangela uhlobo olungazelelwe, olungalindelekile, olusongela impilo ye -tachycardia ye- ventricular ngokuvamile ebizwa ngokuthi i- torsades de pointes . Abantu abane-LQTS basengozini ye- syncope (ukulahlekelwa kwengqondo) nokufa okungazelelwe, kaningi esemncane.
Umfanekiso - Torsades de amaphuzu. Isithunzi esiphakeme: Isigqi senhliziyo evamile. Isibalo esingezansi: Torsades de amaphuzu. "Amaphesenti ama-pointes" asho ukuqhuma okuzungeze iphuzu. Kulesi sigamu, isigqi senhliziyo siphuthuma kakhulu, futhi ukuma kwezimo eziyinkimbinkimbi ku-ECG kushintsha njalo, kuvame ukufana nephethini lomsindo njengalesi sithombe. Lapho uhlelo lwegesi lwenhliziyo luziphatha ngale ndlela, ukuqhuma okuphumelelayo akunakwenzeka
Sibutsetelo
Abantu abane-LQTS baye bachitha isikhathi se-QT kuma- ECG abo. Isikhathi se-QT simelela ukubuyiswa kabusha, noma "ukushisa kabusha," kweseli yenhliziyo. Ngemuva kokucindezeleka kagesi kwenhliziyo kuvuselela iseli senhliziyo (ngaleyo ndlela ibangele ukushaya), ukuvuselelwa kufanele kwenzeke ukuze iseli lilungele ukushaywa kombane okulandelayo. Isikhathi se-QT (esilinganiswa kusukela ekuqaleni kobunzima be-QRS kuze kube sekupheleni kwe-T wave) yisikhathi esiphelele sithatha ukukhipha, bese ushona iseli senhliziyo. Ku-LQTS, isikhathi se-QT sinwetshwa isikhathi eside. Ukungahambi kahle esikhathini sokuqala kwe-QT kunesibopho sama-arrhythmiya ahlobene ne-LQTS.
Izimbangela
I-LQTS yinkinga ezuze njengefa. Izakhi zofuzo eziningana zitholwe ukuthi zithinta isikhathi se-QT, ngakho-ke kukhona izinhlobo eziningana ze-LQTS. Eminye imindeni inezimo eziphakeme kakhulu ze-LQTS. Ngenxa yokuthi izakhi eziningi zegciwane zingathinta isikhathi se-QT, ukuhlukahluka okuningi ku-LQTS kuye kwabonakala.
Ezinye zazo ("i-classic" LQTS) zihlotshaniswa nesigameko esiphakeme sama-arrhythmias ayingozi nokufa okungazelelwe, okuvame ukuvela kumalungu omndeni amaningana. Ezinye izinhlobo zama-LQTS ("ama-LQTS variants") angaba yingozi kakhulu. Eziningi zalezi zinhlobonhlobo zofuzo zibhekwa yisikhathi esivamile sokuqala se-QT, futhi ama-arrhythmias enhliziyo ngokuvamile abonakala kuphela uma esinye isici esengeziwe (njengokuphathwa kwezidakamizwa, noma ukungalingani okuphawulekayo kwe-electrolyte) kuthatha isikhathi eside se-QT.
Kodwa-ke, uma izikhathi ze-QT ziqhubeka zihlala isikhathi eside kubantu abanezinhlobo ezihlukahlukene ze-LQTS, kuvele ama-arrhythmias ayingozi.
Ukuvama
I-LQTS yama-Classic ikhona cishe kubantu abayi-5000. I-LQTS ingenye yezimbangela ezivamile zokufa ngokungazelelwe kubantu abasha, okuholela ekufeni kwabantu abaphakathi kuka-2000 no-3000 ngonyaka. Izinguquko ze-LQTS zivame kakhulu, futhi cishe zithinta abantu abangaba ngu-2 kuya ku-3%.
Izimpawu
Izimpawu ze-LQTS zenzeka kuphela uma isiguli siqala isiqephu se-tricycardia eyingozi, kanti izinga lezimpawu lithinta ubude besikhathi lapho i-arrhythmia iqhubeka. Uma ihlala isikhashana nje, imizuzwana embalwa yokungena ngokweqile kungase ibe yodwa uphawu. Uma iqhubekela phambili kumasekhondi angaba ngu-10 noma kunjalo, i-syncope ivele. Futhi uma ihlala isikhathi esingaphezu kwamaminithi ambalwa, isisulu ngokuvamile asiphinde sithole ulwazi.
Kulabo abanezinhlobo ezithile ze-LQTS, iziqephu zivame ukubangelwa ukuqhuma okungazelelwe kwe-adrenaline; okungahle kwenzeke ngesikhathi sokuzikhandla ngokomzimba, lapho uhlaselwa kakhulu, noma uma ulaka kakhulu.
Ngenhlanhla, iningi labantu abanezinhlobo ze-LQTS azitholi izimpawu ezisongela ukuphila.
Ukuxilongwa
Odokotela kufanele bacabange nge-LQTS kunoma ubani oye waba ne-syncope noma ukuboshwa komzimba, nasemalungwini omndeni womuntu one-LQTS owaziwayo.
Noma yimuphi umuntu osemusha onesi-syncope okwenzeka ngesikhathi sokuzivocavoca, noma kunoma yiziphi ezinye izimo lapho amazinga e-adrenaline ayengaba khona, kufanele ukuthi i-LQTS iqondiswe ngokuqondile.
Ukuxilongwa kwe-LQTS kuvame ukwenziwa ngokubheka isikhala se-QT esingavamile ku-ECG. Ngezinye izikhathi ukuhlolwa kwe-treadmill kuyadingeka ukuze kukhishwe okungavamile kwe-ECG. Ukuvivinya nge-Genetic ye-LQTS kanye nokuhlukahluka kwayo kusetshenziselwa kakhulu kunalokho nje kuphela eminyakeni embalwa eyedlule.
Ukwelapha
Iziguli eziningi ezine-LQTS ziningi ziphathwa nge- beta blockers . Abavimbela i-Beta bahlukumeza ukuphakama kwe-adrenaline okubangela iziqephu ze-arrhythmias kulezi ziguli.
Ngeshwa, akukaze kuboniswe ukuthi i-beta-blockers iyanciphisa kakhulu ukukhubazeka jikelele kwe-syncope nokufa okungazelelwe kuziguli ezine-LQTS.
Kubaluleke kakhulu kubantu abane-LQTS nezinhlobonhlobo zayo ukugwema izidakamizwa eziningi ezenza isikhathi eside se-QT. Kulaba bantu, izidakamizwa ezinjalo cishe zivusa iziqephu ze-torsades of pointes. Izidakamizwa ezenza isikhathi eside se-QT zivamile ngeshwa. Abahlukumezi abakhulu, ngokudabukisayo, izidakamizwa ezithathelwanayo ; imithi eminingi yokulwa nokucindezeleka, nemithi elwa namagciwane efana no-erythromycin, i-clarithromycin erythromycin, ne-azithromycin. I-CredibleMeds igcina uhlu lwezidakamizwa ezivame ukwandisa isikhathi se-QT.
Kubantu abaningi abane-LQTS, i- defibrillator engasetshenzisiwe iyindlela yokwelashwa engcono kakhulu. Le divayisi kufanele isetshenziswe ezigulini eziye zagcinwa ukuboshwa kwe-cardiac, futhi mhlawumbe ezigulini eziye zaba nesifo se-syncope ngenxa ye-LQTS ikakhulukazi uma i-syncope ivela ngenkathi ithatha kakade ukuthatha i-beta blockers.
Kubantu abangakwazi ukubekezelela abavimba be-beta noma abasenayo imicimbi ngenkathi bethola ukwelashwa, ukuhlinzwa okungahambi kahle kwe-cardica ukuguqulwa (LCSD) kungenziwa.
Ama-blockers wesiteshi se-sodium angasetshenziswa ne-LQTS uhlobo lwe-3.
Imithombo
> Iyini i-QT syndrome ende? - NHLBI, NIH. Izikhungo zezempilo zikazwelonke. http://www.nhlbi.nih.gov/health/health-topics/topics/qt/. Kubuyekezwe ngomhla ka-21 Septhemba 2011. Kufinyelelwe ngoJulayi 27, 2016.
> Alders M, > Christiaans > I- Long Long QT isifo . I-NCBI Bookshelf; Juni 18, 2015. http://www.ncbi.nlm.nih.gov/books/NBK1129/. Ifinyelele ngo-Julayi 27, 2016.
Moss AJ. I-Long QT Syndrome. I-JAMA 2003; 289: 2041.
Li H, Fuentes-Garcia J, Towbin JA. Imiqondo yamanje yamanje kwi-QT syndrome. I-Pediatr Cardiol 2000; 21: 542.