Ngaba kukho ingxaki kwinkqubo yombane yentliziyo yakho? Masithethe ngeLong QT Syndrome (LQTS).

Ngaba wakha weva ngenkqubo yombane yentliziyo? Intliziyo yethu isebenza njengomatshini omncinci. Inenkqubo yombane, elawula ukubetha kwentliziyo. Ngamanye amaxesha, utshintsho oluncinci lunokwenzeka kule nkqubo yombane. Olunye utshintsho olunjalo lubizwa ngokuba yiLong QT Syndrome, okanye `(Long QT Syndrome - LQTS)`. Ungakhathazeki, masithethe ngale nto ngokulula.

Yintoni i-Long QT Syndrome? Ngamafutshane...

Khawuthelekelele ukuba emva kokuba intliziyo yakho ibethe kube kanye, kuthatha "ukuphumla" okuncinci ngaphambi kokuba iphinde ibethe, njengokutshaja ibhetri. Ixesha elithathayo "ukuphumla" okanye ukuphinda uyitshaje lide kancinci kunesiqhelo, into esiyibiza ngokuba yi-long QT syndrome.

Ukuba ukhe wavavanywa i-ECG, oluvavanyo olujonga umsebenzi wombane wentliziyo, kukho inxalenye yayo ebizwa ngokuba yi-QT interval. Oku kubonisa ukuba kuthatha ixesha elingakanani ukuba amagumbi asezantsi entliziyo yakho, abizwa ngokuba zii-ventricles, afinyeze aze aphumle, okanye "aphinde atshaje" njengoko benditshilo.

Umsebenzi wombane wentliziyo yethu uncediswa ziingceba ezincinci ezitshajiswa ngombane ezibizwa ngokuba yi-'ions'. Ii-ion ezifana ne-sodium, i-calcium, i-potassium, kunye ne-chloride zinegalelo koku. Ezi i-ion zihamba zingena ziphuma kwiiseli zentliziyo ngeetshaneli ezikhethekileyo ezibizwa ngokuba yi-'ion channels'. Ngoku, kule `(Long QT Syndrome)`, mhlawumbi kukho ingxaki ngezi channels ze-ion, okanye kukho ukungabikho kwazo. Emva koko intliziyo iyalibaziseka "ekutshajeni kwakhona". Xa ixesha le-QT liqhubeka, kukho umngcipheko okhulayo wesifo sentliziyo esikhawulezayo esiyingozi esibizwa ngokuba yi-`Torsades de Pointes`, esinokuba yingozi ebomini.

Ngaba kukho iintlobo ze-Long QT Syndrome?

Ewe, zimbini iintlobo eziphambili. Enye ifunyanwa ngenxa yemfuza , oko kuthetha ukuba idluliselwa ngeejini. Enye ifunyanwa ngenxa yezinye izinto, ezifana namayeza athile.

Iintlobo zokuzalwa:

Oku kubangelwa ikakhulu ziziphene zemfuza kwi-'ion channels' endizikhankanyileyo.

• Ukungasebenzi kakuhle kwe-ion channel: Olu lolona hlobo luqhelekileyo. Kukho iintlobo ezahlukeneyo ezifana ne-`LQT1, LQT2, LQT3, LQT4, LQT5`. Ezi zahlulwe ngokwehlobo lwe-ion channel echaphazelekayo. Umngcipheko wesifo sentliziyo kwixesha elizayo unokwahluka ngokuxhomekeke kuhlobo.
• I-Jervell kunye ne-Lange-Nielsen Syndrome: Kule meko, bobabini abazali banokuba ne-gene engaqhelekanga, kodwa basenokungabi nazimpawu. Le yimeko engaqhelekanga kakhulu. Ngenxa yokuba akunakwenzeka ukuba bobabini abazali babe ne-gene, umntwana ngamnye unethuba elingama-25% lokuyizuza njengelifa. Abantu abanale syndrome banokuzalwa bengeva.
• Isifo seRomano-Ward:Kwimeko enjalo, omnye umzali udla ngokuba ne-"Long QT Syndrome" kanti omnye akanayo. Umntwana ngamnye unethuba elingama-50% lokufumana eli jini lingaqhelekanga. Kwimeko enjalo, ukuva kuyinto eqhelekileyo.
• I-Timothy Syndrome: Olu luhlobo olungaqhelekanga kakhulu. Lunokuchaphazela kungekuphela nje intliziyo, kodwa nezinye iindawo zomzimba.

Ixhaphake kangakanani le meko?

I-Long QT Syndrome yimeko engaqhelekanga . Umzekelo, eMelika, kuqikelelwa ukuba malunga nomntu omnye kwabayi-2,000 unale meko.

Ziziphi iimpawu zeLong QT Syndrome?

Ezi ziimpawu ezibonakala rhoqo:

• I-Syncope: Ukulahlekelwa yingqondo ngequbuliso.
• Ukuxhuzula: Abanye abantu banokuba nokuxhuzula.
• Ukubanjwa yintliziyo: Oku kuyingozi kakhulu.
• Ukufa ngequbuliso:

Ezi mpawu zenzeka xa kusenzeka isifo esiyingozi sentliziyo esibizwa ngokuba yi-'Torsades de Pointes' endisikhankanyileyo ngaphambili. Ngeli xesha, intliziyo ayikwazi ukupompa igazi ngokufanelekileyo. Ukuba ingqondo ayifumani igazi elaneleyo, izinto ezifana nokuquleka kunye nokuxhuzula ziyavela. Ukuba esi sifo siyaqhubeka, ukufa ngequbuliso kunokwenzeka. Nangona kunjalo, ukuba isantya sentliziyo sibuyela kwisiqhelo, iimpawu ziyayeka.

Iimpawu zibonakala kakhulu xa:

• Ngexesha lokuzilolonga (okanye imizuzu embalwa emva kokuzilolonga).
• Ngexesha lokuvuselelwa ngamandla ngokweemvakalelo , ingakumbi xa utyhwatyhwa ngequbuliso okanye wothukile.
• Ngexesha lokulala okanye xa uvuka ngequbuliso .

Okubalulekileyo kukuba, ukuya kuthi ga kwi-50% yabantu abane-Long QT Syndrome basenokungaze babe nazimpawu . Kwaye, malunga nomntu omnye kwabalishumi, ukuminxeka kwentliziyo luphawu lokuqala lwesi sifo. Oku kunokuba yingozi ukuba akunyangeki ngokukhawuleza.

Iimpawu zihlala ziqala xa umntu emdala . Ukuba uneempawu, zinokuphinda zibuye. Iimpawu zibonakala kakhulu ngaphambi kokuba ufikelele kwiminyaka engama-30. Nangona kunjalo, abanye abantu banokuba neempawu xa bekwiminyaka engama-50 nangaphezulu.

Yintoni ebangela i-Long QT Syndrome?

Njengoko benditshilo ngaphambili, oku kusenokufunyanwa kubazali bakho (abazalwa nabo) okanye kufumaneke ngenxa yamayeza athile . Kwi-Long QT Syndrome, kukho ukungaqheleki kwikhowudi yejini yeendlela zakho ze-ion. Ezi ndlela ze-ion ezingaqhelekanga zibangela ukulibaziseka kwinqanaba lokubuyela kwisantya sentliziyo yakho. Oku kunokukhokelela kwi-arrhythmia esongela ubomi.

Amanye amayeza anokubangela i-"Long QT Syndrome" kubantu abanotyekelo oluthile kwiindlela zabo ze-ion. Basenokungazi nokuba kunjalo, kodwa kuxa besela loo mayeza apho i-"Long QT" ivele khona.

Amayeza anokubangela i-acquired long QT syndrome:

• Ezinye 'i-Antiarrhythmics' (amayeza okuphazamiseka kwesantya sentliziyo)
• Ezinye izinto ezithintela uxinezeleko
• Ezinye ii-diuretics (amayeza akwenza uchame ngakumbi)
• Amanye amayeza okubulala iintsholongwane
• Izithinteli okanye abachasi be-serotonin receptor
• Ii-anti-histamine receptor

Ezinye izizathu ze-acquired long QT syndrome:

• Ezinye iintlobo zeetyhefu
• I-Anorexia nervosa (isifo sengqondo esinxulumene ne-anorexia)
• Ezinye 'i-bradyarrhythmias' (isantya sentliziyo esicothayo ngokungaqhelekanga)
• Amanqanaba aphantsi e-calcium, i-magnesium, okanye i-potassium emzimbeni
• Ukulimala kwinkqubo yakho yeemvakalelo

Ngubani osengozini enkulu yokuhlaselwa yile meko?

Abantu abalandelayo basengozini enkulu yokuba ne-"Long QT Syndrome":

• Kwabantwana abangevayo kwasekuzalweni.
• Abantwana kunye nabantu abancinci abanembali yosapho yokufa ngequbuliso okanye ukuquleka (syncope).
• Kwizalamane zegazi ezine-"Long QT Syndrome".
• Kwabo bane-'Cardiomyopathy' (isifo semisipha yentliziyo) okanye isifo sentliziyo sokuzalwa.
• Kwabo banezinga eliphantsi lee-electrolytes ezithile emzimbeni ngenxa yesinye isifo.
• Kwabo basebenzisa amayeza achaphazela imijelo ye-ion entliziyweni.

Ngaba kufuneka ndivavanywe i-Long QT Syndrome?

I-Long QT Syndrome yimeko yemfuza enokudluliselwa kwizizukulwana ngezizukulwana. Ukuba omnye wezalamane zakho ezikwinqanaba lokuqala (abazali, abantakwenu, abantwana) une-Long QT Syndrome, licebo elihle ukuba uye kuvavanyo lwemfuza .

Zonke izalamane ezikwinqanaba lokuqala (abantakwabo, abazali, kunye nabantwana) kufuneka zibe ne-ECG. Kubalulekile nokuba nabani na onembali yosapho lwakhe yokuxhuzula okanye ukuquleka ukuba enze olu vavanyo.

Inyathelo lokuqala kukwazisa ugqirha wakho ukuba usapho lwakho lunale meko. Kusenokwenzeka ukuba uza kwenza uvavanyo olufunekayo ukujonga intliziyo yakho. Ukuba olo vavanyo luqinisekisa ukuba unale meko, kuya kufuneka ubonane nogqirha wentliziyo onolwazi oluhle ngeLong QT Syndrome.

Ziziphi iingxaki zeLong QT Syndrome?

I-"Long QT Syndrome" inokubangela i-"Torsades de Pointes" esongela ubomi endiyikhankanyileyo. Oku kunokukhokelela nasekufeni ngequbuliso.

Ifunyanwa njani i-Long QT Syndrome?

Oogqirha banokuxilonga i-Long QT Syndrome ngovavanyo olulula lwe-ECG. Ukuze baxilonge imeko, balinganisa ixesha le-QT kwi-ECG. Ukuba ixesha lakho le-QT lide kunee-450 milliseconds, usenokuba une-Long QT Syndrome.

Ugqirha wakho uza kukubuza ngezi zinto:

• Ingaba kukho imbali yosapho ene-"Long QT Syndrome"?
• Ngaba kukho imbali yosapho lwakho enesiphene esingaziwayo, ukuxhuzula, okanye ukuqiniswa kwentliziyo?
• Ngaba wakha waphelelwa ngamandla, waxhuzula, okanye wabanjwa yintliziyo, ingakumbi ngexesha lokuzilolonga?

Zeziphi iimvavanyo ezisetyenziselwa ukufumanisa i-Long QT Syndrome?

Ukongeza kwi-ECG, ezi mvavanyo zinokwenziwa:

• Uvavanyo lwegazi.
• Uvavanyo lwemfuza.
• Uvavanyo loxinzelelo lokuzivocavoca.
• Isixhobo sokujonga i-Ambulatory Monitor: Esi sisixhobo sokujonga ukusebenza kwentliziyo yakho kangangeentsuku eziliqela.

Inyangwa njani i-Long QT Syndrome?

Kukho amayeza, izixhobo, okanye utyando olunokunceda ukulawula iimpawu kunye nokuthintela ukufa ngequbuliso kweLong QT Syndrome. Nangona olu nyango lungenakunyanga ngokupheleleyo esi sifo, lunokukukhusela kwi-heart rhythms engaqhelekanga.

Amayeza

Abantu abaninzi abaneLong QT Syndrome (kwanabo bangenazo iimpawu) bathatha i-beta-blocker, efana ne-nadolol. Amanye amayeza anokunciphisa ixesha lakho le-QT okanye aphucule amanqanaba akho e-electrolyte. Ugqirha wakho uya kukuxelela ukuba zeziphi amayeza afanelekileyo kuwe.

Izixhobo

Ezinye izigulane ezine-"Long QT Syndrome" nazo zinokufuna izixhobo ezifana nezi:

• I-ICD efakelweyo kwiCardioverter Defibrillator (ICD): I-ICD efakelweyo kwiCardioverter Defibrillator (ICD) inokufakelwa kubantu abanembali yokuma kwentliziyo okanye abasabonakalisa iimpawu nangona benyangwa nge-beta-blocker. Esi sixhobo sibona i-arrhythmias esongela ubomi kwaye ngokuzenzekelayo sibangele umothuko entliziyweni ukuthintela ukufa ngequbuliso.
• I-Pacemaker: Esi sixhobo sinceda abantu abaneentliziyo ezibetha kancinci ngendlela engaqhelekanga.

Utyando

Ezinye izigulana ezine-Long QT Syndrome zinokufuna ukuba zenze i-Left Cardiac Sympathetic Denervation (LCSD) okanye i-sympathectomy. Le yinkqubo engaphantsi kakhulu apho imithambo-luvo ethile kwinkqubo yakho ye-sympathetic nervous system isuswa khona.

Zithini iingxaki/iziphumo ebezingalindelekanga zonyango?

Iziphumo ebezingalindelekanga okanye iingxaki ezinokubakho zixhomekeke kuhlobo lonyango olufumanayo.

• I-Beta-blocker:
• Uxinzelelo lwegazi oluphantsi.
• Isiyezi.
• Intloko ebuhlungu .
• Ukudinwa.
• ICD (I-Implantable Cardioverter Defibrillator):
• Iimpazamo zesixhobo.
• Umphunga owileyo.
• Ukopha.
• Iintsholongwane.
• Isixhobo sokuncedisa ukuphumla:
• Umphunga owileyo.
• Ukopha.
• Iintsholongwane.
• Iimpazamo zesixhobo.
• I-LCSD (Utyando Lokunciphisa Uxinzelelo Lwentliziyo Yasekhohlo):
• Isifo seHorner.
• Umphunga owileyo.
• Ukufutha okanye ukubila ebusweni.

Ngaba i-Long QT Syndrome ingathintelwa?

Awunakukuthintela ukuba ukuzuze njengelifa kubazali bakho. Nangona kunjalo, kwabo bangayifumani njengelifa, ungazithintela iimeko ezibangela i-acquired (long QT syndrome) kamva. Umzekelo, lumka ngamayeza athile.

Kuza kwenzeka ntoni ukuba ndine-Long QT Syndrome?

Ngonyango olufanelekileyo , izinga lokufa kwabantu abane-"Long QT Syndrome" limalunga ne-1%. Ngaphandle konyango, imeko imbi kakhulu. Malunga nama-21% abantu abaneempawu abangafumani nyango bafa kungekapheli nonyaka emva kokuqala kwe-syncope.

Kodwa khumbula, abantu abane-"Long QT Syndrome" banokuphila ubomi obupheleleyo . Banokukhulelwa baze bazale abantwana.

Ihlala ixesha elingakanani i-Long QT Syndrome?

I-Inherited Long QT Syndrome yimeko ehlala ubomi bonke. Nangona kunjalo, iimpawu kunye nomngcipheko weengxaki zinokuncipha ngokuhamba kwexesha.

Ndingazinyamekela njani?

I-Long QT Syndrome inokuchaphazela iinkalo ezininzi zobomi bakho bemihla ngemihla. Kuya kufuneka udibane nogqirha wakho ngaphambi kokuba wenze izinto ezithile.

Ingxolo kunye noxinzelelo

Abanye abantu abane-Long QT Syndrome banokuchaphazeleka ziingxolo ezinkulu ezingalindelekanga okanye uxinzelelo lweemvakalelo. Nangona ungakwazi ukulawula yonke ingxolo kunye noxinzelelo olukungqongileyo, ungazama ukuzinciphisa.

• Xelela abahlobo bakho nosapho ukuba akukhuselekanga ukukuvusa ngequbuliso.
• Endaweni ye-alamu enkulu ekhala ngaxeshanye, sebenzisa i-alamu enesandi esimnandi.
• Yehlisa isandi sezinto ezinomsindo ezinokukoyikisa.

Ukuzilolonga

Ezinye iintlobo zeLong QT Syndrome zinokubangela iingxaki ngokuzivocavoca. Ugqirha wakho uza kukuxelela ukuba zeziphi izinto ezifanelekileyo kuwe, kuxhomekeke kuhlobo lwejini engaqhelekanga onayo. Ngenxa yokuba ukuzilolonga kunokubangela i-arrhythmias ebulalayo, kufuneka uthethe nogqirha wakho ngaphambi kokuba ugqibe ekubeni udlale imidlalo yokhuphiswano. Ukuba ugqiba ekubeni udlale imidlalo, kunokufuneka uthathe amanyathelo okhuseleko.

Ukuqubha kunokuba yingozi kwabanye abantu abane-"Long QT Syndrome". Ukuba uyaquleka ngelixa uqubha, ungatshona.

Amayeza

Amayeza amaninzi anokwandisa ixesha le-QT. La mayeza anokuchaphazela abantu abane-"Long QT Syndrome" ngaphezu kwabo bangenayo. Ukuba une-"Long QT Syndrome", kufuneka wenze oku kulandelayo:

• Xelela bonke oogqirha bakho ukuba une-"Long QT Syndrome".
• Thetha nogqirha wakho ngaphambi kokuba uthathe naliphi na iyeza elinikiweyo kwezinye iimeko zonyango.
• Kuphephe ukuthatha amayeza athengiswa ngaphandle kwemvume kagqirha (i-OTC) (ngaphandle kwe-aspirin okanye i-paracetamol) ngaphandle kokuthetha nogqirha wakho.

Ukuba une-"Long QT Syndrome", olu hlobo lwamayeza lunokukuchaphazela:

• Ii-Antihistamines (amayeza okulwa nee-allergies)
• Amayeza okudakumba (amayeza okudakumba)
• Amayeza eengxaki zempilo yengqondo
• Amayeza esifo sentliziyo
• 'Amayeza okubulala iintsholongwane'
• Izinto ezilwa nokungunda
• `Iintsholongwane`
• Amayeza ezifo zamathumbu
• Imithi yokuxhuzula
• I-Diuretics (amayeza abangela ukwanda kokuchama)
• Amayeza oxinzelelo lwegazi oluphezulu
• Amayeza okunyanga iintloko ezibuhlungu
• Amayeza okwehlisa i-cholesterol

Ndifanele ndimbone nini ugqirha wam?

Ufanele ubone ugqirha xa uqala ukuba neempawu kwaye ubuncinane kanye ngonyaka emva koko. Ingcali ye-electrophysiologist yeyona ndlela ilungileyo.

Ukuba umntwana wakho une-"Long QT Syndrome", kufuneka ubonane nogqirha wakho ngaphezu kwesihlandlo esinye ngonyaka ukuqinisekisa ukuba ufumana idosi efanelekileyo yamayeza ngokobunzima bakhe bangoku.

Ukuba unesixhobo esifakelweyo, kufuneka ubone ugqirha wakho kanye ngonyaka ukuqinisekisa ukuba sisebenza kakuhle.

Ndingaya nini kwiYunithi yoNyango oluNgxamisekileyo (i-ETU) ?

Umntu onesifo sokuma kwentliziyo udinga uncedo lwezonyango ngokukhawuleza . Umntu okufutshane kufuneka aqalise i-CPR (ukuvuselelwa kwentliziyo) aze atsalele umnxeba ku-911 (okanye inombolo yakho yongxamiseko yasekuhlaleni).

Ndingayibuza yiphi imibuzo ugqirha wam?

Eminye imibuzo onokuyibuza ugqirha wakho:

• Luhlobo luni lwe-"Long QT Syndrome" endinalo?
• Loluphi unyango oluthile olundicebisayo?
• Kufuneka ndikubone kangaphi?
• Ngawaphi amayeza kunye nemisebenzi endifanele ndiyiphephe ngohlobo oluthile lwe-"Long QT Syndrome" endinayo?

Ekugqibeleni, umyalezo wokubuyela ekhaya

Kuqhelekile ukuba nexhala xa ufumanisa ukuba unesifo sentliziyo. Kodwa ukubuza ugqirha wakho imibuzo, kwaye ukuba kuyimfuneko, ukuthetha nomcebisi kunokukunceda uzive ukhululekile. Kubalulekile ukulandela imiyalelo kagqirha wakho, uthabathe amayeza akho ngexesha, kwaye ugcine amadinga akho.

Kungakunceda kakhulu ukwazisa usapho lwakho kunye nabahlobo bakho ukuba une-Long QT Syndrome. Ukuba ubonakalisa iimpawu ngelixa bekunye nawe, banokufowunela uncedo olungxamisekileyo. Okungcono nangakumbi, ukuba bayazi indlela yokwenza i-CPR, ingasindisa ubomi bakho. Ungakhathazeki, ngolawulo olufanelekileyo, nawe ungaphila ubomi obusempilweni.

I- Long QT Syndrome, i-LQTS, isifo sentliziyo, i-QT interval, izinga lokubetha kwentliziyo, i-arrhythmia, izifo zemfuza