Ingaba icala lasekunene lentliziyo yakho libuthathaka? Masifunde ngale ARVD iyingozi (Arrhythmogenic Right Ventricular Dysplasia)?

Owu, ngaba ngamanye amaxesha uziva ngathi isifuba sakho siyabetha ngequbuliso, uzive ngathi unengxaki yokuphefumla, okanye uzive ngathi unesifo sentliziyo? Mhlawumbi ukhe weva ukuba ngamanye amaxesha nomdlali wezemidlalo osemtsha, osempilweni, unokuba nesifo sentliziyo ngequbuliso. Yiloo nto siza kuthetha ngayo namhlanje, kodwa sisifo sentliziyo esingaziwa kangako, esingaqhelekanga. Oku kubizwa ngokuba yi-``Arrhythmogenic Right Ventricular Dysplasia``, okanye ngamafutshane i-ARVD.

Yintoni i-ARVD (Arrhythmogenic Right Ventricular Dysplasia)? Masiyiqonde ngokulula!

Ngamafutshane, i-ARVD sisifo esichaphazela izihlunu zentliziyo, uhlobo lwe-'cardiomyopathy'. Intliziyo yethu inamagumbi amane. Okwenzekayo koku kukuba izihlunu ezikwigumbi elisezantsi kwicala lasekunene lentliziyo yakho, 'i-ventricle yasekunene', zikhula endaweni yamafutha kunye nezicubu ezine-fibrous. Cinga ngayo ngokungathi izihlunu eziphilileyo zikhutshiwe zaza zathatyathelw' indawo yinto efana namafutha.

Kwenzeka ntoni xa oku kusenzeka? Loo ventricle yasekunene iyanda kancinci kancinci, iba ncinci, kwaye ayikwazi ukufinyela ngokufanelekileyo. Oko kuthetha ukuba amandla ayo okupompa igazi entliziyweni ayancipha.

Okubaluleke kakhulu, izicubu ezinamafutha okanye ezine-fibrous ezisandula ukwakheka ziphazamisana nemiqondiso yombane yentliziyo. Yiyo loo nto abantu abaninzi abane-ARVD befumana ukubetha kwentliziyo okungahambelaniyo (arrhythmias). Oku kunokuba yingozi kakhulu, njengoko kwandisa umngcipheko wokuma kwentliziyo ngequbuliso okanye ukufa.

Le ARVD ikwabizwa ngokuba yi-'Arrhythmogenic Right Ventricular Cardiomyopathy' (ARVC). Ngamanye amaxesha inokuchaphazela necala lasekhohlo, ngoko ke ikwabizwa ngokuba yi-'Arrhythmogenic Cardiomyopathy' (ACM).

Zithini izigaba ze-ARVD?

Ukuba une-ARVD, ingadlula kwizigaba ezahlukeneyo ngokuhamba kwexesha.

1. Inqanaba elifihlakeleyo: Ngeli nqanaba, usenokungabi nazimpawu. Nangona kunjalo, usenokuba nokubetha kwentliziyo okungahambelaniyo xa uzilolonga. Okumangalisayo kukuba, ezinye iimvavanyo ezenziwe ngeli nqanaba zinokubonisa ukuba akukho nto ingalunganga.

2. Isigaba sombane: Kweli nqanaba, unamathuba amaninzi okufumana i-ventricular arrhythmias. Ukwasengozini enkulu yokufa ngesiquphe kwentliziyo. I-ECG inokuzibona ezi ngxaki zesantya sentliziyo.

3. Inqanaba lokwakheka: Kule nqanaba, umngcipheko wesantya sentliziyo esingaqhelekanga (izingqisho ze-ventricular) kunye nokufa ngequbuliso kwentliziyo uphezulu nangakumbi. Uvavanyo lwemifanekiso, olufana neeskeni, lunokubonisa ngokucacileyo utshintsho kwisakhiwo sentliziyo yakho.

Ngubani ochaphazeleka kakhulu yile ARVD?

Oogqirha babona le meko rhoqo phakathi kwabantu abaselula.Oko kukuthi, phakathi kwabakwishumi elivisayo okanye abantu abadala abaselula. I-ARVD ikwachongiwe njengembangela yokufa ngesiquphe kwentliziyo kwabanye abadlali abaselula. Ezinye izifundo zibonisa ukuba le meko ixhaphake kancinci kumadoda .

Ngokwendlela exhaphake ngayo, i-ARVD sisifo esingaqhelekanga. Ngokwesiqhelo kuxelwa ukuba sichaphazela umntu omnye kwi-1,000 okanye omnye kwi-5,000 yabantu. Inokwenzeka ngaphandle kokuba nabani na kusapho abe nayo, kodwa idla ngokungena kwiintsapho.

Zithini iimpawu ze-ARVC? Masiqaphele!

Usenokungabi nazo naziphi na iimpawu ze-ARVD kwisigaba sokuqala, kodwa kukho umngcipheko wokufa ngesiquphe kwentliziyo.

Iimpawu eziphambili ezinokubonwa zezi:

• Iingxaki zokungaqini kakuhle kwemithambo yegazi: Ezi zinokuba yingozi kakhulu. Kwisiqingatha sabantu abanezi ngxaki zokungaqini kakuhle kwemithambo yegazi, isiphumo singaba sibi okanye siphantse sibe sibi. Ingxaki eqhelekileyo yi-ventricular tachycardia, eyenzeka kwi-77% yabantu abanale meko.
• I-Supraventricular arrhythmias: Eyona ixhaphakileyo yimeko ebizwa ngokuba yi-atrial fibrillation.
• Ukubetha kwentliziyo: Oku kunokuvakala ngathi kukho into ebetha esifubeni sakho. Oku kubangelwa kukubetha kwentliziyo okungaqhelekanga.
• Ukuba nesiyezi, ukuba nentloko ebuhlungu okanye ukuquleka: Oku kukwabangelwa kukubetha kwentliziyo ngendlela engacwangciswanga.
• Iintlungu zesifuba.
• Ukufa ngesiquphe kwentliziyo: Oku ngamanye amaxesha kunokuba luphawu lokuqala lwe-ARVD.
• Ukuqhawukelwa ngumphefumlo.
• Ukudumba kwemilenze, amaqatha, iinyawo, okanye isisu.
• Ukumelwa yintliziyo.

Ezi mpawu zihlala ziqala phakathi kweminyaka engama-20 ukuya kwengama-50. Oogqirha badla ngokuxilonga i-ARVD besebancinci (ngamaxesha amaninzi ngaphambi kokuba bafikelele kwiminyaka engama-40).

Zithini izizathu ze-ARVD?

Malunga nama-60% abantu abane-ARVD banotshintsho lwemfuza. Abaphandi bafumanise ubuncinane ii-gene ezili-13 ezibangela esi sifo.

Ezi genes zingaqhelekanga zonakalisa iiproteni ezinceda iiseli zemisipha yentliziyo ukuba ziqhagamshelane kwaye zinxibelelane. Oku kunokubangela ukuba iiseli zemisipha yentliziyo kwi-ventricle yasekunene zahlukane zize zife. Oku kunokuba yinto eqhelekileyo kakhulu ngamaxesha oxinzelelo okanye ukuzibhokoxa.

Ubuncinane i-30% ukuya kwi-50% yezigulane ezine-ARVD zinembali yosapho.Kungoko ke, kubaluleke kakhulu ukuba izalamane zomntu one-ARVD zihlolwe ngugqirha (abazali, abantakwabo, abantwana, abazukulwana, oomalume, oomalumekazi, abazukulwana, abatshana). Nokuba akukho zimpawu, izalamane eziselula kufuneka zifune iingcebiso zonyango.

Abaphandi bafumene iindlela ezimbini zokufumana ilifa kwi-ARVD:

1. `I-Autosomal dominant`: Umzali omnye une-gene mutation. Kwiintsapho ezinjalo, abantwana banethuba elingama-50% lokufumana esi sifo njengelifa. Nangona kunjalo, iimpawu kunye nobudala bokuqala kwesi sifo zinokwahluka phakathi kwamalungu osapho. I-ARVD ixhaphake kakhulu kwezinye iindawo zejografi, njenge-Itali.

2. `I-Autosomal recessive` (ayiqhelekanga kakhulu): Bobabini abazali banotshintsho lwezakhi zofuzo, kodwa ababonisi zimpawu. `Isifo sikaNaxos` ngumzekelo woku. Sibangela ukujiya komaleko wangaphandle wesikhumba (`hyperkeratosis`) kunye neenwele ezinde, "ezifana noboya" (`iinwele ezifana noboya`) ezintendeni nasezinyaweni.

I-ARVD inokubangelwa nazizinye izizathu:

• Ukuphazamiseka kokuzalwa kwe-ventricle yasekunene.
• I-'myocarditis' ebangelwa ziintsholongwane okanye ukudumba (ukudumba kwemisipha yentliziyo).
• Izizathu azikafumaneki.

Ifunyanwa njani i-ARVD (Arrhythmogenic Right Ventricular Dysplasia)?

Ugqirha wakho unokuxilonga i-ARVD ngokusekelwe kwimbali yakho yezonyango, uvavanyo lomzimba, kunye novavanyo olwahlukeneyo.

Ugqirha wakho unokuxilonga i-ARVD ukuba uneengxaki ezininzi kwezi zilandelayo:

• Ukusebenza okungaqhelekanga kwe-ventricle yasekunene.
• Ukubakho kwezicubu ezinamafutha okanye ezinamafutha kwimisipha yentliziyo (`myocardium`) ye-ventricle yasekunene.
• Ingxelo engaqhelekanga ye-'ECG/EKG` (i-Electrocardiogram).
• Ukungahambi kakuhle kwesantya sentliziyo (i-arrhythmias): Umzekelo, i-supraventricular tachycardia, i-ventricular tachycardia, okanye i-ventricular fibrillation, ingakumbi ngexesha lokuzilolonga.
• Imbali yosapho ene-ARVD.

Kuxhomekeke ekubeni zingaphi kwezi zinto onazo, ugqirha wakho unokukunika uxilongo "oluqinisekileyo," "olunemingcele," okanye "olunokwenzeka." Kwezinye iimeko, kodwa hayi rhoqo, ugqirha wakho unokukuthumela kuvavanyo lwemfuza .

Ziziphi iimvavanyo ezisetyenziselwa ukufumanisa i-ARVD?

• I-Electrocardiogram (ECG/EKG): Uvavanyo olujonga umsebenzi wombane wentliziyo.
• I-Transthoracic echocardiogram: Oku kufana ne-ultrasound yentliziyo. Ingajonga amagumbi entliziyo, iivalvu, kunye nokukwazi kwayo ukupompa.
• `Isixhobo sokujonga i-Holter`: Oku kufana nomatshini omncinci we-`ECG` owunxiba iiyure ezingama-24 ukuya kuma-48. Urekhoda ukubetha kwentliziyo yakho imini yonke.
• I-angiogram ye-ventricular yasekunene: Le ayisetyenziswa rhoqo.
• `Uvavanyo lwe-electrophysiology`:Uvavanyo olukhethekileyo lokujonga iingxaki kwinkqubo yombane yentliziyo.
• I-Cardiac magnetic resonance imaging (MRI): Oku kunokuvelisa imifanekiso eneenkcukacha zentliziyo. Oku kubaluleke kakhulu ekufumaneni izinto ezifana ne-fatty deposits kwi-ventricle yasekunene kwi-ARVD.
• `I-cardiac computed tomography (CT):` Le yenye indlela yokufumana imifanekiso yentliziyo.
• `I-Biopsy` (ukuthatha isampuli yethishu): Oku kwenziwa rhoqo. Iqhekeza elincinci lethishu liyathathwa entliziyweni lize lihlolwe.

Ziziphi iindlela zonyango lwe-ARVD?

Okwangoku akukho nyango lwe-ARVD. Nangona kunjalo, ugqirha wakho uza kuzama ukwenza oku kulandelayo:

• Lawula ukungahambelani kwesantya sentliziyo yakho (`ventricular arrhythmias`).
• Thintela ukuqhekeka kwegazi.
• Lawula ukusilela kwentliziyo yakho.

Iindlela zonyango lwe-ARVD zezi:

• Amayeza okulwa nokubetha kwentliziyo okungaqhelekanga: Athintela ukubetha kwentliziyo okungaqhelekanga ixesha elide kunye/okanye ukufa ngequbuliso. Olu lolona nyango lusetyenziswa kakhulu ngoogqirha. Unganikwa amayeza anjenge-sotalol okanye i-amiodarone.
• Amayeza oxinzelelo lwegazi: Amayeza anjenge-diuretics (asusa ulwelo olugqithisileyo emzimbeni) okanye ii-beta-blockers (ezinciphisa umthwalo womsebenzi wentliziyo).
• I-Anticoagulant (i-blood thinner): Amayeza afana ne-warfarin anikwa ukuthintela ukujiya kwegazi.
• Ukususwa kwe-catheter ngeRadiofrequency: Unyango lokuphazamiseka kwesigqi sentliziyo rhoqo okungalawulwa ngamayeza. Oku kuquka ukutshabalalisa indawo encinci yentliziyo ebangela ukubetha kwentliziyo okungaqhelekanga.
• I-ICD (isixhobo esifakelwayo se-cardioverter-defibrillator): Isixhobo esincinci esifakwe entliziyweni yabantu abasengozini yokufa ngequbuliso. Ukuba sifumanisa isigqi sentliziyo esiyingozi, siya kuyibona size sibangele ukubetha kombane entliziyweni ukuze kulungiswe loo nto.
• Ukufakelwa kwentliziyo: Oku kwenziwa xa zonke ezinye iindlela zonyango zingaphumelelanga. Lipesenti encinci kuphela yabantu abanesi sifo, malunga ne-2% ukuya kwi-4%, abafuna intliziyo entsha.

Khumbula, ungadinga unyango olungaphezulu kolunye lwesi sifo ubomi bakho bonke.

Ngaba kukho naziphi na iingxaki okanye iziphumo ebezingalindelekanga zonyango?

Ewe, kukho izinto ezithile ekufuneka uzilumkele ngonyango oluthile.

• Ukuba uthatha i-warfarin, kuya kufuneka uhlolwe igazi rhoqo . Ugqirha wakho kuya kufuneka aqinisekise ukuba ufumana idosi efanelekileyo. Idosi yakho inokulungiswa ngokusekelwe kwiziphumo zovavanyo.
• Nangona unyango lwe-catheter ablation luphumelela ekuqaleni kubantu abaninzi, njengoko esi sifo siqhubeka sisiba mandundu ngokuhamba kwexesha, malunga nama-60% abantu banokuphinda bave nokuphazamiseka kwesantya sentliziyo yabo.
• Iintambo ezikwisixhobo se-ICD zinokuhambahamba okanye zingasebenzi kakuhle, ngoko ke kufuneka uhlale uzijonga.

Ndingazinyamekela njani? Zeziphi iinguqu zendlela yokuphila ezifunekayo?

Kubaluleke kakhulu ukwenza utshintsho oluthile kwindlela ophila ngayo ukuze intliziyo yakho ikhululeke.

• Nciphisa ukusetyenziswa kotywala.
• Yeka ukusebenzisa iimveliso zecuba ngokupheleleyo.
• Yitya ukutya okunempilo (amafutha aphantsi, iziqhamo nemifuno emininzi)
• Nciphisa ukutya neziselo ezine-caffeine (ezifana nekofu, iti, itshokolethi).
• Gcina ubunzima bomzimba bufanelekile.
• Nciphisa umsebenzi onzima womzimba.

Eyona nto ibalulekileyo: Soloko ubuza ugqirha wakho ngaphambi kokuba uqale ukuzilolonga. Imidlalo yokhuphiswano ayikhuthazwa. Imidlalo engenamandla kakhulu isenokulunga.

Nxibelelana nogqirha wakho rhoqo ngalo lonke ixesha lonyango lwakho. Baza kuqinisekisa ukuba ufumana idosi efanelekileyo yamayeza. Kwakhona, ngokuya rhoqo kwintlanganiso yokulandelela, ungafumana naluphi na utshintsho kwimeko yakho kwangethuba.

Ngaba umngcipheko ungancitshiswa?

Ewe, ukuya kuthi ga kwinqanaba elithile. Ukuba kukho umntu kusapho lwakho one-ARVD, ukuvavanywa ngokukhawuleza yeyona ndlela ilungileyo yokunciphisa umngcipheko wakho. Uvavanyo olulula nolungenabuhlungu lunokukuxelela ukuba usemngciphekweni wokuba nentliziyo engaqhelekanga. Ukuba unjalo, ugqirha wakho unokukunceda uphuhlise isicwangciso sonyango esifanelekileyo kuwe.

Ingakanani iminyaka yokuphila nge-ARVD?

Ulindelo lobomi be-ARVD luyahluka ngokuxhomekeke ekubeni lufunyaniswe nini. Eyona nto ingcono onokuyenza kukufumanisa isifo kwangethuba kangangoko kwaye ufumane unyango lokuthintela ukubetha kwentliziyo okungaqhelekanga. Esi sifo siba mandundu ngokuhamba kwexesha.

Ukuba i-ARVD ayinyangwa, i-ventricle yakho yasekunene inokungaphumeleli. Emva koko i-ventricle yakho yasekhohlo nayo inokungaphumeleli. Oku kunokukhokelela kwiimeko ezifana 'nokusilela kwentliziyo' kunye 'ne-atrial fibrillation'. Abaphandi bakholelwa ukuba imeko iba mandundu ukuba i-ARVD ichaphazela zombini ii-ventricle.

Kodwa, ngonyango, ungafumana uncedo olufunekayo ukunciphisa uxinzelelo entliziyweni yakho nokuthintela ukuhlaselwa yintliziyo okuyingozi. Ezinye izifundo zibonisa ukuba abanye abantu bafunyanwa benesifo emva kweminyaka engama-65. Kukwaqikelelwa ukuba malunga nomntu omnye kwabahlanu abane-ARVC uqala iimpawu emva kweminyaka engama-50. Ke ngoko, kunokwenzeka ukuba uphile ubomi obude nge-ARVC.

Kule mihla, ubuchwepheshe obuphambili bokufota njenge-CT kunye ne-MRI buvumela ukuxilongwa kwangethuba kunye nonyango, ngoko ke ikamva elide labantu abane-ARVD lilungile.

Nangona kunjalo, ukufa ngequbuliso kunokwenzeka kwabo bangaxilongwanga okanye abangafumani nyango lufunekayo. Kuthiwa i-ARVD yimbangela ye-11% yokufa ngequbuliso kwentliziyo kubantu abangaphantsi kweminyaka engama-35 ubudala kunye ne-22% yokufa ngequbuliso kwentliziyo kubadlali.

Ndifanele ndimbone nini ugqirha? Ndifanele ndiye nini kwigumbi longxamiseko?

Ukuba une-ARVD, kuya kufuneka uqhubeke nokuhlolwa ngugqirha ubomi bakho bonke.Ugqirha wakho kuya kufuneka ahlole rhoqo ukuqinisekisa ukuba ufumana unyango olufanelekileyo. Ukuba une-ICD, kuya kufuneka ihlolwe rhoqo .

Yintoni omawuyenze xa kukho imeko engxamisekileyo?

Ukuba umntu uquleka ngequbuliso aze angaphenduli kwiifowuni zakho, fowunela ngoko nangoko ku-1990 (inkonzo ye-ambulensi engxamisekileyo yaseSri Lanka). Emva koko, ukuba kunokwenzeka, qalisa i-CPR (ukuvuselelwa kwentliziyo), okanye ubuncinci i-CPR eyenziwe ngezandla kuphela.

Ukuba usemngciphekweni wokubanjwa yintliziyo ngequbuliso, licebo elihle ukuba abo basekhaya nawe bafumane uqeqesho lweCPR.

Ndingayibuza yiphi imibuzo ugqirha wam?

• Ngaba ndingakwazi ukuzilolonga okanye ukuthatha inxaxheba kwezemidlalo? Ukuba kunjalo, loluphi uhlobo lwemidlalo?
• Loluphi unyango olungcono kum?
• Ngaba ndifuna i-ICD ngeli xesha?

Ngenxa yokuba i-ARVD inokuba nzima ukuyifumanisa ngaphambi kokuba kuvele iimpawu, kubalulekile ukwazi imbali yosapho lwakho. Ukuba usemngciphekweni ngenxa yembali yosapho lwakho yesi sifo, ugqirha wakho unokukuvavanya.

Umyalezo Wokuya Ekhaya

Kulungile, ngoku uyaqonda ngcono into esithethe ngayo namhlanje, i-ARVD. Nangona ingaqhelekanga, inokuba yingxaki yentliziyo enzulu. Idla ngokubangelwa zizinto zemfuza . Ke ngoko, ukuba kukho umntu kusapho lwakho onale meko, kubaluleke kakhulu ukuba abanye bavavanywe.

Ngokufumanisa isifo kwangethuba, ukufumana unyango olufanelekileyo, nokwenza utshintsho olufunekayo kwindlela yokuphila, ungaphila kakuhle nesi sifo. Hlala unxibelelana nogqirha wakho kwaye ulandele iingcebiso zakhe. Kwakhona, ukuqinisekisa ukuba abantu obathandayo bayayazi i-'CPR' kunokukunceda usindise ubomi bakho xa kukho imeko engxamisekileyo. Musa ukoyika, ukuqonda yeyona nto inamandla amakhulu!

` I-ARVD, i-Arrhythmogenic Right Ventricular Dysplasia, isifo sentliziyo, uhlaselo lwentliziyo, i-cardiomyopathy, ukufa kwentliziyo ngesiquphe, isifo semfuza, uhlaselo lwentliziyo