I-Long QT syndrome (i-LQTS), isifo esithatha njengefa esithinta isistimu kagesi yenhliziyo, ingenye yezimo ezihlobene nokufa okungazelelwe kubadlali abasha. Ama-arrhythmias asongela ukuphila abonakala nge-LQTS cishe angenzeka ngesikhathi sokuzivocavoca, ngakho-ke kubantu abaningi abanalesi sifo kufanele bafinyeleleke. Kodwa-ke, abantu abathintekayo ngokuvamile abavinjelwe emsebenzini.
Ukwazi ukuthi yini evumelekile kuyisihluthulelo sokuhlala kwabo ephephile.
Kuyini i-QT Syndrome?
I-LQTS yinto engavamile yokubeletha ephuza "ukuvuselelwa" kwamangqamuzana ehliziyo ngemva kokuthi 'ixoshwa' yihlelo lombane wenhliziyo. Lokhu kubambezeleka kuboniswa yisikhathi se-QT eside ku- ECG . Ukungajwayelekile kwamandla kagesi okuhlobene ne-LQTS kungaveza ama-arrhythmias enhliziyo (uhlobo lwe- tricycardia ye- ventricular ebizwa nge-torsades de pointses) engabangela ukuvumelanisa noma ukufa okungazelelwe.
Ezigulini eziningi ezine-LQTS, ingozi yokuthuthukisa lezi zinhlelo eziyingozi zanda ngesikhathi sokuzivocavoca.
Ngokujwayelekile, nge-LQTS, azikho izimpawu kuze kube yilapho umuntu othintekile ezwa i-tricycardia ye-ventricular (futhi, ngokuvamile ngesikhathi sokuzama). Uma lokhu ku-arrhythmia kwenzeka, izimpawu zingase zihluke kumasekhondi ambalwa wokuzondeka okukhulu kuze kube yilapho kungenangqondo nokufa okubangelwa ukuboshwa kwenhliziyo.
Ukuxilongwa kwenziwa ngokuhlolisisa i-ECG, ekhombisa isikhathi eside se-QT.
Ngenkathi i-LQTS yimizwa ezuzwe njengefa, kunezinhlobo eziningi zayo (ezihambisana nezakhi zofuzo ezihlukahlukene ezingase zihileleke). Yize ezinye izinto ezihlukahlukene zengozi enkulu yokufa okungazelelwe, ezinye ziyingozi kakhulu.
Ngokuvamile, iziguli ezisengozini enkulu kakhulu ziyoba nomlando oqinile womndeni wabantu abaye babhekana ne-syncope noma ukufa okungazelelwe, kanengi ngesikhathi sokuzivocavoca.
I-LQTS ivame ukuphathwa nge- beta blockers , nokugwema izidakamizwa ezenza ukuqhubeka kwesikhathi eside kwesikhathi se-QT. Uma ingozi yokufa okungazelelwe ihlulelwe ukuthi iphezulu, i- defibrillator engasungulwa ingadingeka.
Yiziphi Izincomo Ezijwayelekile Zokuzivocavoca Kwabadlali Abasha Abaphethe I-LQTS?
Abantu abane-LQTS banconywa ukuba bavimbele imisebenzi yabo emidlalweni ephansi uma omunye okulandelayo esebenza kubo:
- umlando wokulahlekelwa (syncope) noma ukuvuselelwa ekuboshiwe inhliziyo
- Izikhathi ze-QT zinde kakhulu (okungukuthi, isilinganiso esibizwa ngokuthi "isikhathi sokulungiswa kwe-QT - QTc - sinwetshwa okungenani ama-470 msec kumadoda, noma i-480 msec kwabesifazane)
Noma ubani onama-LQTS ongaqiniseki ukuthi angaba umkhawulo wenzani kufanele axoxe nodokotela wabo.
Ngokuvamile, ukuzivocavoca umzimba okufana ne-bowling noma igalofu, nokuzivocavoca okulinganiselayo okufana ne-double tennis, ukuhamba ngebhayisikili kanye nokudoba, kuvunyelwe kwezinye izinsizwa ezincane ezinezinhlaka ze-LQTS.
Njengoba kunikezwe ukuthi kunezinhlobo ezihlukahlukene ze-LQTS ezibonwayo, izincomo zomsebenzi ezahlukene zingase zibe ngcono kwezinye izindawo ezincane. Ngokwesibonelo, abantu abane-LQTS uhlobo lwe-3 babonakala benengozi encane ngesikhathi sokuzivocavoca kunalabo abanezinye izinhlobo; Abantu abane-LQTS uhlobo 1 bangabungozi ikakhulukazi ngesikhathi sokubhukuda noma ukudilika.
Ngakho, abagijimi abangathí sina bangase bafune ukucabangela ukuthi babe ne-genetic subtyping, ukuze bavumele izincomo zabo zokuzivocavoca zihlotshaniswe nokuhluka kwazo ngokoqobo.
NgoNovemba 2015, ukuzivocavoca izincomo zamathikithi okuncintisana nge-LQTS zavuselelwa ngokusemthethweni yi-American Heart Association kanye ne-American College of Cardiology. Ochwepheshe manje batusa ukuthi, uma abadlali abasha abane-LQTS bengenayo izimpawu (ikakhulukazi azikho iziqephu zesikhumba esiphansi noma i-syncope ehlobene nokuzivocavoca), bangabamba iqhaza emidlalweni yokuncintisana IF:
Bona, odokotela babo, nabazali babo noma ababheki (uma bebancane) baqonda izingozi ezingase zihileleke emidlalweni yokuncintisana, futhi bazimisele futhi bayakwazi ukuthatha izinyathelo ezifanele zokuqapha
- zigwema noma yiziphi izidakamizwa ezenza isikhathi se-QT senzeke
- bathola i-defibrillator yangaphandle yokuzenzekelayo yangaphandle (AED) njengengxenye yemishini yabo yokudlala yemidlalo
- Izikhulu zeqembu ziqeqeshwe futhi zilungele ukuthatha isinyathelo esifanele uma kuvela isimo esiphuthumayo, kuhlanganise nokukwazi nokuzimisela ukusebenzisa i-AED
> Imithombo:
> Moss AJ. I-Long QT Syndrome. I-JAMA 2003; 289: 2041.
> Li H, Fuentes-Garcia J, Towbin JA. Imiqondo yamanje e-Long QT Syndrome. I-Pediatr Cardiol 2000; 21: 542.
> Zipes, DP, Ackerman, MJ, Estes NA, 3rd, et al. Umsebenzi Weqhaza 7: I-Arrhythmias. J Am Coll Cardiol 2005; 45: 1354.
> Maron BJ, Zipes DP, Kovacs RJ, et al. Izincomo Zokufaneleka Nokuncishiswa Kwabalandeli Abanokuncintisana Nezinkinga Ze-Cardiovascular Wearmalities. Ukujikeleza kuka-2015; I-DOI: 10.1161 / CIR.0000000000000236.