Ingabe kukhona inkinga ngesistimu kagesi yenhliziyo yakho? Ake sixoxe nge-Long QT Syndrome (LQTS).

Wake wezwa ngohlelo lukagesi lwenhliziyo? Inhliziyo yethu isebenza njengomshini omncane. Inesistimu kagesi, elawula ukushaya kwenhliziyo. Ngezinye izikhathi, izinguquko ezincane zingavela kulesi simiso sikagesi. Olunye ushintsho olunjalo lubizwa ngokuthi i-Long QT Syndrome, noma `(Long QT Syndrome - LQTS)`. Ungakhathazeki, ake sixoxe ngalokhu kalula.

Iyini i-Long QT Syndrome? Kalula nje...

Cabanga ukuthi ngemva kokushaya kwenhliziyo yakho kanye, kuthatha "ukuphumula" okuncane ngaphambi kokuba iphinde ishaye, njengokushaja kabusha ibhethri. Isikhathi esithathayo "ukuphumula" noma ukushaja kabusha sithatha isikhathi eside kunokujwayelekile, okuyilokho esikubiza ngokuthi i-long QT syndrome.

Uma uke wenza i-ECG, okuwukuhlolwa okubheka ukusebenza kukagesi kwenhliziyo, kunengxenye yayo ebizwa ngokuthi isikhawu se-QT. Lokhu kukhombisa ukuthi kuthatha isikhathi esingakanani ukuthi amakamelo aphansi enhliziyo yakho, abizwa ngokuthi ama-ventricle, afinyele bese ephumula, noma "aphinde ashaje" njengoba ngishilo.

Umsebenzi kagesi wenhliziyo yethu usizwa yizinhlayiya ezincane ezishaja ngogesi ezibizwa ngokuthi 'ama-ion'. Ama-ion anjenge-sodium, i-calcium, i-potassium, ne-chloride anegalelo kulokhu. Lawa ma-ion angena futhi aphume emaseli enhliziyo ngezindlela ezikhethekile ezibizwa ngokuthi 'ama-ion channels'. Manje, kule '(Long QT Syndrome)', kungaba nenkinga ngalezi ziteshi ze-ion, noma kukhona ukuntuleka kwazo. Khona-ke inhliziyo iyalibaziseka "ekushajeni kabusha". Lapho isikhathi se-QT sinwetshwa, kuba nengozi eyengeziwe yokuphazamiseka kwe-rhythm yenhliziyo okusheshayo okuyingozi okubizwa ngokuthi 'i-Torsades de Pointes', okungaba yingozi empilweni.

Ingabe zikhona izinhlobo ze-Long QT Syndrome?

Yebo, kunezinhlobo ezimbili eziyinhloko. Eyokuqala izuzwe njengefa , okusho ukuthi idluliselwa ngezakhi zofuzo. Eyokuqala itholakala , okusho ukuthi ibangelwa ezinye izici, njengemithi ethile.

Izinhlobo zokuzalwa:

Lokhu kubangelwa kakhulu ukukhubazeka kwezakhi zofuzo 'ezindleleni ze-ion' engizishilo.

• Ukukhubazeka kwesiteshi se-ion: Lolu uhlobo oluvame kakhulu. Kunezinhlobo eziningana ezifana ne-`LQT1, LQT2, LQT3, LQT4, LQT5`. Lezi zihlukaniswa ngokohlobo lwesiteshi se-ion esihilelekile. Ingozi yesifo senhliziyo esikhathini esizayo ingahluka kuye ngohlobo.
• I-Jervell ne-Lange-Nielsen Syndrome: Kulesi simo, bobabili abazali bangaba ne-gene engavamile, kodwa bangase bangabi nazimpawu. Lesi yisimo esingavamile kakhulu. Ngenxa yokuthi akunakwenzeka ukuthi bobabili abazali bazoba ne-gene, ingane ngayinye inethuba elingu-25% lokuyizuza njengefa. Abantu abanalesi sifo bangase bazalwe beyizithulu.
• Isifo se-Romano-Ward:Kulesi simo, omunye umzali uvame ukuba ne-"Long QT Syndrome" kanti omunye akanayo. Ingane ngayinye inethuba elingu-50% lokuthola lesi sakhi sofuzo esingavamile. Kulesi simo, ukuzwa kuyinto evamile.
• I-Timothy Syndrome: Lolu uhlobo olungavamile kakhulu. Lungathinta hhayi inhliziyo kuphela, kodwa nezinye izingxenye zomzimba.

Sivame kangakanani lesi simo?

I-Long QT Syndrome empeleni iyisimo esingavamile . Isibonelo, e-United States, kulinganiselwa ukuthi cishe umuntu oyedwa kwabangu-2,000 unalesi simo.

Ziyini izimpawu ze-Long QT Syndrome?

Lezi izimpawu ezivame ukubonakala:

• I-Syncope: Ukulahlekelwa ingqondo ngokuzumayo.
• Ukuquleka: Abanye abantu bangase babe nokuquleka.
• Ukuma kwenhliziyo: Lokhu kuyingozi kakhulu.
• Ukufa okuzumayo:

Lezi zimpawu zenzeka lapho kwenzeka isifo esiyingozi senhliziyo esibizwa ngokuthi 'i-Torsades de Pointes' engikhulume ngaso ekuqaleni. Ngalesi sikhathi, inhliziyo ayikwazi ukupompa igazi ngendlela efanele. Uma ubuchopho bungatholi igazi elanele, kuvela izinto ezifana nokuquleka kanye nokuxhuzula. Uma lesi sifo siqhubeka, ukufa okuzumayo kungenzeka. Kodwa-ke, uma izinga lokushaya kwenhliziyo libuyela esimweni esijwayelekile, izimpawu ziyaphela.

Izimpawu zivame ukuvela uma:

• Ngesikhathi uzivocavoca (noma imizuzu embalwa ngemva kokuzivocavoca).
• Ngesikhathi sokukhuthazeka okukhulu ngokomzwelo , ikakhulukazi lapho wesaba noma wethuka ngokuzumayo.
• Ngesikhathi sokulala noma lapho uvuka ngokuzumayo .

Okubalulekile, abantu abangafika ku-50% abane-Long QT Syndrome bangase bangalokothi babe nezimpawu . Futhi, cishe kubantu abayishumi, ukumiswa kwenhliziyo kuyisibonakaliso sokuqala salesi simo. Lokhu kungaba yingozi uma kungelashwa ngokushesha.

Izimpawu zivame ukuqala lapho umuntu esemdala . Uma unezimpawu, zingase ziphinde zivele. Izimpawu zivame ukuvela ngaphambi kweminyaka engama-30. Kodwa-ke, abanye abantu bangase babe nezimpawu lapho sebeneminyaka engama-50 nangaphezulu.

Yini ebangela i-Long QT Syndrome?

Njengoba ngishilo ngaphambili, lokhu kungazuzwa kubazali bakho (abazalwa nabo) noma kutholakale ngenxa yemithi ethile . Ku-Long QT Syndrome, kukhona ukungajwayelekile kukhodi yezakhi zofuzo zeziteshi zakho ze-ion. Lezi ziteshi ze-ion ezingavamile zibangela ukubambezeleka esigabeni sokululama kwesilinganiso senhliziyo yakho. Lokhu kungaholela ekushayweni kwenhliziyo okusongela impilo.

Eminye imithi ingabangela "i-Long QT Syndrome" kubantu abanomkhuba othile weziteshi zabo ze-ion. Bangase bangazi ngisho nokuthi, kodwa yilapho bethatha leyo mithi lapho "i-Long QT" ivela khona.

Izidakamizwa ezingabangela i-acquired long QT syndrome:

• Ezinye izidakamizwa ze-'Antiarrhythmics' (imithi yokuphazamiseka kwesigqi senhliziyo)
• Ezinye izidakamizwa zokucindezeleka
• Amanye ama-diuretics (imithi ekwenza uchame kakhulu)
• Amanye ama-antibiotic
• Izithibi noma izitha ze-Serotonin receptor
• Abaphikisi be-histamine receptor

Ezinye izimbangela ze-acquired long QT syndrome:

• Ezinye izinhlobo zobuthi
• I-Anorexia nervosa (isifo sengqondo esihlobene ne-anorexia)
• Amanye ama-'bradyarrhythmias' (ukushaya kwenhliziyo okuhamba kancane ngokungavamile)
• Amazinga aphansi e-calcium, i-magnesium, noma i-potassium emzimbeni
• Ukulimala ohlelweni lwakho lwezinzwa

Ubani osengozini enkulu yokuthola lesi simo?

Abantu abalandelayo basengozini enkulu yokuthola i-"Long QT Syndrome":

• Kwabantwana abayizithulu kusukela ekuzalweni.
• Izingane kanye nentsha enomlando womndeni wokufa noma ukuquleka okungachazeki (i-syncope).
• Kwabahlobo begazi abane-"Long QT Syndrome".
• Kulabo abane-'Cardiomyopathy' (isifo semisipha yenhliziyo) noma isifo senhliziyo sokuzalwa.
• Kulabo abanamazinga aphansi ama-electrolyte athile emzimbeni ngenxa yesinye isimo sezokwelapha.
• Kulabo abathatha imithi ethinta iziteshi ze-ion enhliziyweni.

Ingabe kufanele ngihlolwe i-Long QT Syndrome?

I-Long QT Syndrome yisimo sofuzo esingadluliselwa kusuka esizukulwaneni kuya kwesinye. Uma omunye wezihlobo zakho zezinga lokuqala (abazali, abafowenu nodadewenu, izingane) ene-Long QT Syndrome, kungumqondo omuhle ukuthi uhlolwe izakhi zofuzo .

Zonke izihlobo zezinga lokuqala (abafowabo nodadewabo, abazali, nezingane) kufanele zibe ne-ECG. Kubalulekile futhi kunoma ubani onomlando womndeni wokuquleka noma ukuquleka ukuba enze lokhu kuhlolwa.

Isinyathelo sokuqala ukwazisa udokotela wakho ukuthi umndeni wakho unalesi simo. Cishe uzokwenza izivivinyo ezidingekayo ukuhlola inhliziyo yakho. Uma lezo zivivinyo ziqinisekisa ukuthi unalesi simo, kufanele ubonane nodokotela wenhliziyo onolwazi oluhle nge-Long QT Syndrome.

Yiziphi izinkinga ze-Long QT Syndrome?

I-"Long QT Syndrome" ingabangela i-arrhythmia esongela ukuphila ebizwa ngokuthi i-"Torsades de Pointes" engiyikhulumile. Lokhu kungaholela ngisho nasekufeni okuzumayo.

Itholakala kanjani i-Long QT Syndrome?

Odokotela bangakwazi ukuthola i-Long QT Syndrome ngokuhlolwa okulula kwe-ECG. Ukuze bathole lesi simo, balinganisa isikhathi se-QT ku-ECG. Uma isikhathi sakho se-QT singaphezu kwama-millisecond angu-450, ungase ube ne-Long QT Syndrome.

Udokotela wakho uzokubuza ngalezi zinto:

• Ingabe kukhona umlando womndeni we-"Long QT Syndrome"?
• Ingabe kukhona umlando womndeni wokuphelelwa amandla, ukuquleka, noma ukuhlaselwa yinhliziyo okungachazeki?
• Wake waba nokuquleka, ukuxhuzula, noma ukumiswa kwenhliziyo, ikakhulukazi ngesikhathi sokuzivocavoca?

Yiziphi izivivinyo ezisetshenziswa ukuxilonga i-Long QT Syndrome?

Ngaphezu kwe-ECG, lezi zivivinyo zingenziwa futhi:

• Ukuhlolwa kwegazi.
• Ukuhlolwa kofuzo.
• Ukuhlolwa kokucindezeleka kokuzivocavoca.
• I-Ambulatory Monitor: Lokhu kuqapha ukusebenza kwenhliziyo yakho izinsuku eziningana.

Iphathwa kanjani i-Long QT Syndrome?

Kunemithi, amadivayisi, noma ukuhlinzwa okungasiza ukulawula izimpawu nokuvimbela ukufa okuzumayo kwe-Long QT Syndrome. Nakuba lokhu kwelashwa kungeke kuphulukise ngokuphelele lesi simo, kungakuvikela ekushayweni kwenhliziyo okungavamile.

Imithi

Abantu abaningi abane-Long QT Syndrome (ngisho nalabo abangenazo izimpawu) bathatha i-beta-blocker, njenge-nadolol. Eminye imithi inganciphisa isikhathi sakho se-QT noma ithuthukise amazinga akho e-electrolyte. Udokotela wakho uzokutshela ukuthi yimiphi imithi engcono kakhulu kuwe.

Amadivayisi

Ezinye iziguli ezine-"Long QT Syndrome" zingadinga namadivayisi afana nalawa:

• I-ICD (ICD) Engatshalwa: I-ICD (ICD) engatshalwa ingatshalwa kubantu abanomlando wokuma kwenhliziyo noma abasenezimpawu naphezu kokwelashwa nge-beta-blocker. Le divayisi ithola ukungasebenzi kahle kwenhliziyo okusongela impilo futhi inikeze ngokuzenzakalelayo ukushaqeka enhliziyweni ukuze kuvinjelwe ukufa ngokuzumayo.
• I-Pacemaker: Le divayisi isiza abantu abanezinhliziyo ezishaya kancane ngendlela engavamile.

Ukuhlinzwa

Ezinye iziguli ezine-Long QT Syndrome zingase zidingeke ukuthi zenze i- Left Cardiac Sympathetic Denervation (LCSD) noma i-sympathectomy. Lena inqubo engangenisi kakhulu lapho kususwa khona imizwa ethile ohlelweni lwakho lwezinzwa oluzwelayo.

Yiziphi izinkinga/imiphumela emibi yokwelashwa?

Imiphumela emibi noma izinkinga ezingaba khona zincike ohlotsheni lokwelashwa olutholayo.

• I-Beta-blocker:
• Umfutho wegazi ophansi.
• Isiyezi.
• Ikhanda elibuhlungu .
• Ukukhathala.
• I-ICD (I-Implantable Cardioverter Defibrillator):
• Amaphutha edivayisi.
• Iphaphu eligobile.
• Ukopha.
• Izifo.
• I-Pacemaker:
• Iphaphu eligobile.
• Ukopha.
• Izifo.
• Amaphutha edivayisi.
• Ukuhlinzwa Kokunciphisa Ukucindezeleka Kwenhliziyo Kwesobunxele (LCSD)
• I-Horner syndrome.
• Iphaphu eligobile.
• Ukujuluka noma ukujuluka ebusweni.

Ingabe i-Long QT Syndrome ingavinjelwa?

Awukwazi ukukuvimbela uma ukuzuze kubazali bakho. Kodwa-ke, kulabo abangakuzuzeli ifa, ungakuvimbela izimo ezibangela i-acquired (long QT syndrome) kamuva. Isibonelo, qaphela ngemithi ethile.

Kwenzekani uma ngine-Long QT Syndrome?

Uma kwelashwa ngendlela efanele , izinga lokufa kwabantu abane-"Long QT Syndrome" licishe libe ngu-1%. Ngaphandle kokwelashwa, isimo siba sibi kakhulu. Cishe abantu abangu-21% abanezimpawu abangatholi ukwelashwa bafa kungakapheli nonyaka benesifo sokungakwazi ukulala.

Kodwa khumbula, abantu abane-"Long QT Syndrome" bangaphila impilo egcwele . Bangakhulelwa futhi bazale izingane.

I-Long QT Syndrome ihlala isikhathi esingakanani?

I-Inherited Long QT Syndrome iyisimo esihlala isikhathi eside. Kodwa-ke, izimpawu kanye nengozi yezinkinga kungancipha ngokuhamba kwesikhathi.

Ngizinakekela kanjani?

I-Long QT Syndrome ingathinta izici eziningi zokuphila kwakho kwansuku zonke. Kufanele uthintane nodokotela wakho ngaphambi kokwenza izinto ezithile.

Umsindo nokucindezeleka

Abanye abantu abane-Long QT Syndrome bangase bathintwe yimisindo emikhulu engazelelwe noma ukucindezeleka ngokomzwelo. Nakuba ungakwazi ukulawula wonke umsindo nokucindezeleka okukuzungezile, ungazama ukukunciphisa.

• Tshela abangani bakho nomndeni wakho ukuthi akuphephile ukukuvusa ngokuzumayo.
• Esikhundleni se-alamu enkulu ekhala ngasikhathi sinye, sebenzisa i-alamu enomsindo omnandi.
• Yehlisa umsindo wezinto ezinomsindo ezingase zikuthuse.

Ukuzivocavoca umzimba

Ezinye izinhlobo ze-Long QT Syndrome cishe zingabangela izinkinga ngokuzivocavoca. Udokotela wakho uzokutshela ukuthi yimiphi imisebenzi ekufanele, kuye ngohlobo lwesakhi sofuzo esingavamile onaso. Ngenxa yokuthi ukuzivocavoca kungabangela ukungasebenzi kahle kwemisipha okubulalayo, kufanele ukhulume nodokotela wakho ngaphambi kokuba unqume ukudlala imidlalo yokuncintisana. Uma unquma ukudlala imidlalo, kungadingeka uthathe izinyathelo zokuphepha.

Ukubhukuda kungaba yingozi kwabanye abantu abane-"Long QT Syndrome". Uma uquleka ngenkathi ubhukuda, ungaminza.

Imithi

Imithi eminingi ingandisa isikhathi se-QT. Le mithi ingathinta abantu abane-"Long QT Syndrome" kakhulu kunalabo abangenayo. Uma une-"Long QT Syndrome", kufanele wenze okulandelayo:

• Tshela bonke odokotela bakho ukuthi une-"Long QT Syndrome".
• Khuluma nodokotela wakho ngaphambi kokuthatha noma yimiphi imithi oyinikezwe ezinye izimo zezokwelapha.
• Gwema ukuthatha imithi ethengiswa ngaphandle kwemithi kadokotela (i-OTC) (ngaphandle kwe-aspirin noma i-paracetamol) ngaphandle kokukhuluma nodokotela wakho.

Uma une-"Long QT Syndrome", lezi zinhlobo zemithi zingakuthinta:

• Ama-antihistamine (imithi yokwelapha ukungezwani komzimba)
• Ama-antidepressant (imithi yokucindezeleka)
• Imithi yezinkinga zempilo yengqondo
• Imithi yesifo senhliziyo
• `Ama-antibiotic`
• Imithi yokulwa nesikhunta
• `Ama-antiviral`
• Imithi yezifo zamathumbu
• Imithi Yokuquleka
• Imithi yokuhlanza (imithi ebangela ukwanda kokuchama)
• Imithi yokucindezeleka kwegazi okuphezulu
• Imithi yokwelapha ikhanda elibuhlungu
• Imithi yokwehlisa i-cholesterol

Kufanele ngimbone nini udokotela wami?

Kufanele ubonane nodokotela uma uqala ukuba nezimpawu futhi okungenani kanye ngonyaka ngemva kwalokho. Udokotela we-electrophysiologist uyindlela engcono kakhulu.

Uma ingane yakho ine-"Long QT Syndrome", kufanele ubonane nodokotela wakho izikhathi ezingaphezu kwesisodwa ngonyaka ukuqinisekisa ukuthi ithola umthamo ofanele wemithi ngokwesisindo sayo samanje.

Uma unedivayisi efakiwe, kufanele ubonane nodokotela wakho kanye ngonyaka ukuqinisekisa ukuthi isebenza kahle.

Kufanele ngiye nini e-Emergency Treatment Unit (ETU) ?

Umuntu onesifo sokuma kwenhliziyo udinga usizo lwezokwelapha ngokushesha . Othile oseduze kufanele aqale i-CPR (ukuvuselelwa kwenhliziyo) bese eshayela u-911 (noma inombolo yakho yesimo esiphuthumayo yasendaweni).

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Eminye imibuzo ongayibuza udokotela wakho:

• Uhlobo luni lwe-"Long QT Syndrome" enginalo?
• Yikuphi ukwelashwa okukhethekile ongincomayo?
• Kukangaki ngidinga ukukubona?
• Yimiphi imithi nemisebenzi okufanele ngiyigweme ngohlobo lwami oluthile lwe-"Long QT Syndrome"?

Ekugcineni, Umyalezo Wokuya Ekhaya

Kuvamile ukuzizwa ukhathazekile uma uthola ukuthi unesifo senhliziyo. Kodwa ukubuza udokotela wakho imibuzo, futhi uma kudingeka, ukukhuluma nomeluleki kungakusiza uzizwe ukhululekile kakhudlwana. Kubalulekile ukulandela imiyalelo kadokotela wakho, uphuze imithi yakho ngesikhathi, futhi ugcine ama-aphoyintimenti akho.

Kungaba impumuzo futhi ukwazisa umndeni wakho nabangani bakho ukuthi une-Long QT Syndrome. Uma uba nezimpawu ngenkathi benawe, bangashayela usizo oluphuthumayo. Okungcono nakakhulu, uma bazi ukuthi bangayenza kanjani i-CPR, kungasindisa impilo yakho. Ungakhathazeki, ngokuphathwa okufanele, nawe ungaphila impilo enempilo.

I- Long QT Syndrome, i-LQTS, isifo senhliziyo, isikhawu se-QT, izinga lokushaya kwenhliziyo, i-arrhythmia, izifo zofuzo