Ingabe uhlangothi lwesokudla lwenhliziyo yakho lubuthakathaka? Ake sifunde ngale ARVD eyingozi (Arrhythmogenic Right Ventricular Dysplasia)?

O, ingabe ngezinye izikhathi uzizwa sengathi isifuba sakho siyashaya ngokuzumayo, uzizwa sengathi unenkinga yokuphefumula, noma uzizwa sengathi unesifo senhliziyo? Cishe uke wezwa ukuthi ngezinye izikhathi ngisho nomdlali osemusha onempilo angaba nesifo senhliziyo ngokuzumayo. Yilokho esizokhuluma ngakho namuhla, kodwa kuyaziwa kancane, isifo senhliziyo esingavamile. Lokhu kubizwa ngokuthi "i-Arrhythmogenic Right Ventricular Dysplasia``, noma i-ARVD ngamafuphi.

Iyini i-ARVD (i-Arrhythmogenic Right Ventricular Dysplasia)? Masiyiqonde kalula!

Kalula nje, i-ARVD yisifo esithinta imisipha yenhliziyo, uhlobo lwe-'cardiomyopathy'. Inhliziyo yethu inamakamelo amane. Okwenzekayo kulokhu ukuthi imisipha ekamelweni elingezansi ngakwesokudla kwenhliziyo yakho, 'i-ventricle efanele', ikhula esikhundleni samafutha nezicubu ezine-fibrous. Cabanga ngakho njengokungathi imisipha enempilo isusiwe futhi yathathelwa indawo yinto efana namafutha.

Kwenzekani uma lokhu kwenzeka? Leyo ventricle yangakwesokudla iyanwebeka kancane kancane, iba mncane, futhi ayikwazi ukufinyela kahle. Lokho kusho ukuthi ikhono layo lokupompa igazi liye enhliziyweni liyabuthaka.

Okubaluleke kakhulu, izicubu ezinamafutha noma ezine-fibrous ezisanda kwakheka ziphazamisa izimpawu zikagesi zenhliziyo. Yingakho abantu abaningi abane-ARVD beba nokushaya kwenhliziyo okungajwayelekile (ama-arrhythmia). Lokhu kungaba yingozi kakhulu, njengoba kwandisa ingozi yokuma kwenhliziyo noma ukufa ngokuzumayo.

Le ARVD ibizwa nangokuthi i-'Arrhythmogenic Right Ventricular Cardiomyopathy' (ARVC). Ngezinye izikhathi ingathinta nohlangothi lwesobunxele, ngakho-ke ibizwa nangokuthi i-'Arrhythmogenic Cardiomyopathy' (ACM).

Yiziphi izigaba ze-ARVD?

Uma une-ARVD, ingadlula ezigabeni eziningana ngokuhamba kwesikhathi.

1. Isigaba esifihliwe: Phakathi nalesi sigaba, ungase ungabi nazimpawu. Kodwa-ke, ungase ube nokushaya kwenhliziyo okungajwayelekile lapho uzivocavoca. Ngokumangalisayo, ezinye izivivinyo ezenziwe phakathi nalesi sigaba zingase zibonise ukuthi akukho okungahambi kahle.

2. Isigaba sikagesi: Kulesi sigaba, kungenzeka kakhulu ukuthi ube nokuphazamiseka kwe-ventricular arrhythmias. Usengozini enkulu yokufa ngokuzumayo kwenhliziyo. I-ECG ingathola lezi zinkinga zesigqi senhliziyo.

3. Isigaba sokwakheka: Kulesi sigaba, ingozi yezigqi zenhliziyo ezingavamile (izigqi ze-ventricular) kanye nokufa kwenhliziyo ngokuzumayo iphakeme kakhulu. Ukuhlolwa kwezithombe, njengezikeni, kungabonisa ngokucacile izinguquko esakhiweni senhliziyo yakho.

Ubani othinteka kakhulu yi-ARVD?

Odokotela babona lesi simo kaningi phakathi kwentsha.Okusho ukuthi, phakathi kwentsha noma abantu abadala abasebasha. I-ARVD ibonwe njengembangela yokufa kwenhliziyo ngokuzumayo kwabanye abadlali abasebasha. Ezinye izifundo ziphakamisa ukuthi lesi simo sivame kakhulu kwabesilisa .

Ngokuphathelene nokuthi ivame kangakanani, i-ARVD iyisifo esingavamile. Ngokuvamile kubikwa ukuthi ithinta umuntu oyedwa kwabayi-1,000 noma oyedwa kwabayi-5,000. Ingenzeka ngaphandle kokuba noma ubani emndenini abe nayo, kodwa ivame ukwenzeka emindenini.

Ziyini izimpawu ze-ARVC? Masiqaphele!

Ungase ungabi nazo izimpawu ze-ARVD ezigabeni zokuqala, kodwa kunengozi yokufa kwenhliziyo ngokuzumayo.

Izimpawu eziyinhloko ezingabonakala yilezi:

• Ukuphazamiseka kwe-ventricular: Lokhu kungaba yingozi kakhulu. Cishe ingxenye yabantu abanalezi zinkinga zokuphazamiseka kwe-ventricular, umphumela ungaba yingozi noma ucishe ubulale. Isigqi esivame kakhulu yi-ventricular tachycardia, eyenzeka kubantu abangu-77% abanalesi simo.
• I-Supraventricular arrhythmias: Okuvame kakhulu yisimo esibizwa ngokuthi i-atrial fibrillation.
• Ukushaya kwenhliziyo okungavamile: Lokhu kungazwakala sengathi kukhona okushayayo esifubeni sakho. Lokhu kubangelwa ukushaya kwenhliziyo okungavamile.
• Ukuzungeza, ukuba nekhanda elikhanyayo noma ukuquleka: Lokhu kubangelwa nokushaya kwenhliziyo okungajwayelekile.
• Ubuhlungu besifuba.
• Ukufa kwenhliziyo okungazelelwe: Lokhu kungaba uphawu lokuqala lwe-ARVD ngezinye izikhathi.
• Ukuphelelwa umoya.
• Ukuvuvukala kwemilenze, amaqakala, izinyawo, noma isisu.
• Ukwehluleka kwenhliziyo.

Lezi zimpawu zivame ukuqala phakathi kweminyaka engama-20 nengama-50. Odokotela bavame ukuxilonga i-ARVD besebancane (ngokuvamile ngaphambi kweminyaka engama-40).

Yiziphi izimbangela ze-ARVD?

Cishe abantu abangu-60% abane-ARVD banoguquko lwezakhi zofuzo. Abacwaningi bathole okungenani izakhi zofuzo ezingu-13 ezibangela lesi sifo.

Lezi zakhi zofuzo ezingavamile zilimaza amaprotheni asiza amangqamuzana emisipha yenhliziyo ukuthi axhumane futhi axhumane. Lokhu kungabangela ukuthi amangqamuzana emisipha yenhliziyo ase-ventricle yesokudla ahlukane futhi afe. ​​Lokhu kungaba yinto evamile ikakhulukazi ngezikhathi zokucindezeleka noma ukuzikhandla.

Okungenani ama-30% kuya kuma-50% eziguli ezine-ARVD zinomlando womndeni.Ngakho-ke, kubaluleke kakhulu ukuba izihlobo zezinga lokuqala nelesibili (abazali, abafowabo nodadewabo, izingane, abazukulu, omalume, o-anti, abashana, abashana) bomuntu one-ARVD bahlolwe udokotela. Ngisho noma kungekho zimpawu, izihlobo ezisencane kufanele zifune iseluleko sezokwelapha.

Abacwaningi bathole amaphethini amabili ofuzo kuma-ARVD:

1. `I-Autosomal dominant`: Umzali oyedwa une-gene mutation. Emindenini enjalo, izingane zinethuba elingu-50% lokuthola lesi sifo njengefa. Kodwa-ke, izimpawu kanye nobudala bokuqala kwalesi sifo kungahluka phakathi kwamalungu omndeni. I-ARVD ivame kakhulu kwezinye izindawo, njenge-Italy.

2. `I-Autosomal recessive` (ayivamile kakhulu): Bobabili abazali bane-gene mutation, kodwa ababonisi izimpawu. `Isifo se-Naxos` siyisibonelo salokhu. Sibangela ukuqina kwengqimba yangaphandle yesikhumba (`i-hyperkeratosis`) kanye nezinwele ezijiyile, "ezifana noboya" (`izinwele ezifana noboya`) ezintendeni nasematheni.

I-ARVD ingabangelwa nayizinye izizathu:

• Ukukhubazeka kokuzalwa kwe-ventricle yesokudla.
• I-'myocarditis' ebangelwa yigciwane noma ukuvuvukala (ukuvuvukala kwemisipha yenhliziyo).
• Izizathu azikatholakali okwamanje.

I-ARVD (Arrhythmogenic Right Ventricular Dysplasia) itholakala kanjani?

Udokotela wakho angaxilonga i-ARVD ngokusekelwe emlandweni wakho wezokwelapha, ukuhlolwa ngokomzimba, kanye nokuhlolwa okuhlukahlukene.

Udokotela wakho angase ahlole ukuthi une-ARVD uma unenhlanganisela yezinkinga eziningana ezilandelayo:

• Ukusebenza okungavamile kwe-ventricle yesokudla.
• Ukuba khona kwezicubu ezinamafutha noma ezinama-fibro-fatty emisipheni yenhliziyo (`myocardium`) ye-ventricle yesokudla.
• Umbiko ongavamile we-'ECG/EKG' (i-Electrocardiogram).
• Ukungalingani kwesigqi senhliziyo (ama-arrhythmia): Isibonelo, i-supraventricular tachycardia, i-ventricular tachycardia, noma i-ventricular fibrillation, ikakhulukazi ngesikhathi sokuzivocavoca.
• Umlando womndeni we-ARVD.

Kuye ngokuthi zingaki kulezi zici onazo, udokotela wakho angakunikeza ukuxilongwa kokuthi "kuqinisekile," "kunemingcele," noma "kungenzeka." Kwezinye izimo, kodwa hhayi njalo, udokotela wakho angakuthumela nokuhlolwa kwezakhi zofuzo .

Yiziphi izivivinyo ezisetshenziswa ukuxilonga i-ARVD?

• I-Electrocardiogram (ECG/EKG): Ukuhlolwa okubheka ukusebenza kukagesi kwenhliziyo.
• I-Transthoracic echocardiogram: Njenge-ultrasound yenhliziyo, ingabheka amakamelo enhliziyo, ama-valve, kanye nekhono lokupompa.
• `Isibonisi se-Holter`: Lokhu kufana nomshini omncane we-`ECG` owugqoka amahora angama-24 kuya kwangu-48. Uqopha ukushaya kwenhliziyo yakho usuku lonke.
• I-angiogram ye-ventricular yesokudla: Lokhu akuvamile ukusetshenziswa.
• `Ukuhlolwa kwe-Electrophysiology`:Ukuhlolwa okukhethekile kokuhlola izinkinga ngohlelo lukagesi lwenhliziyo.
• Ukufanekisa kwe-cardiac magnetic resonance (MRI): Lokhu kungaveza izithombe ezinemininingwane zenhliziyo. Lokhu kubaluleke kakhulu ekutholeni izinto ezifana nokufakwa kwamafutha e-ventricle efanele ku-ARVD.
• `I-cardiac computed tomography (CT)`: Lena enye indlela yokuthola izithombe zenhliziyo.
• `I-Biopsy` (ukuthatha isampula yezicubu): Lokhu futhi akwenziwa njalo. Ingcezu encane yezicubu ithathwa enhliziyweni bese ihlolwa.

Yiziphi izindlela zokwelapha i-ARVD?

Okwamanje akukho ukwelashwa kwe-ARVD. Kodwa-ke, udokotela wakho uzozama ukwenza okulandelayo:

• Lawula ukungalingani kwesigqi senhliziyo yakho (`i-ventricular arrhythmias`).
• Vimbela ukuqhekeka kwegazi.
• Phatha ukwehluleka kwenhliziyo yakho.

Izindlela zokwelapha i-ARVD yilezi:

• Imithi yokulwa nokushaya kwenhliziyo: Ivimbela ukushaya kwenhliziyo okungajwayelekile isikhathi eside kanye/noma ukufa okuzumayo. Lena yindlela yokwelapha evame ukusetshenziswa odokotela. Ungase unikezwe imithi efana ne-sotalol noma i-amiodarone.
• Imithi yokucindezela kwegazi: Imithi efana ne-diuretics (esusa uketshezi oluningi emzimbeni) noma ama-beta-blockers (anciphisa umthwalo womsebenzi wenhliziyo).
• I-Anticoagulant (i-blood thinner): Imithi efana ne-warfarin inikezwa ukuvimbela ukujiya kwegazi.
• Ukususwa kwe-catheter ngemisebe: Ukwelashwa kokuphazamiseka kwesigqi senhliziyo okuvamile okungalawulwa ngemithi. Lokhu kuhilela ukubhubhisa indawo encane yenhliziyo ebangela ukushaya kwenhliziyo okungajwayelekile.
• I-ICD (i-ICD) efakwa enhliziyweni yabantu abasengozini yokufa ngokuzumayo. Uma ithola isigqi senhliziyo esiyingozi, izosithola bese ibangela ukushaqeka kukagesi enhliziyweni ukuze kulungiswe.
• Ukufakelwa inhliziyo: Lokhu kwenziwa lapho zonke ezinye izindlela zokwelapha zehlulekile. Amaphesenti amancane kuphela abantu abanalesi sifo, cishe u-2% kuya ku-4%, abadinga inhliziyo entsha.

Khumbula, ungadinga ukwelashwa okungaphezu kokukodwa kwalesi sifo impilo yakho yonke.

Ingabe kukhona izinkinga noma imiphumela emibi yokwelashwa?

Yebo, kunezinto ezithile okufanele uziqaphele ngemithi ethile.

• Uma uthatha i-warfarin, kuzodingeka ukuthi uhlolwe igazi njalo . Udokotela wakho kuzodingeka aqinisekise ukuthi uthola umthamo ofanele. Umthamo wakho ungalungiswa ngokusekelwe emiphumeleni yokuhlolwa.
• Nakuba ukwelashwa nge-catheter ablation ekuqaleni kuphumelela kubantu abaningi, njengoba lesi sifo siba sibi ngokuhamba kwesikhathi, cishe abantu abangu-60% bangase babhekane nokuphindaphinda kokuphazamiseka kwenhliziyo yabo.
• Izintambo ezikudivayisi ye-ICD zingahambahamba noma zingasebenzi kahle, ngakho-ke kudingeka uzihlole njalo .

Ngizinakekela kanjani? Yiziphi izinguquko zendlela yokuphila okufanele ngizenze?

Kubaluleke kakhulu ukwenza izinguquko ezithile endleleni yakho yokuphila ukuze ukhulule inhliziyo yakho.

• Nciphisa ukusetshenziswa kotshwala.
• Yeka ukusebenzisa imikhiqizo kagwayi ngokuphelele.
• Yidla ukudla okunempilo (amafutha aphansi, izithelo nemifino eminingi)
• Nciphisa ukudla neziphuzo eziqukethe i-caffeine (njengekhofi, itiye, ushokoledi).
• Gcina isisindo somzimba esifanele.
• Nciphisa umsebenzi onzima womzimba.

Okubaluleke kakhulu: Hlola njalo nodokotela wakho ngaphambi kokuba uqale ukuzivocavoca. Imidlalo yokuncintisana ayikhuthazwa. Imidlalo esezingeni eliphansi ingase ilunge.

Xhumana nodokotela wakho njalo phakathi nokwelashwa kwakho. Bazoqinisekisa ukuthi uthola umthamo ofanele wemithi. Futhi, ngokuya emihlanganweni yokulandelela evamile, ungabona noma yiziphi izinguquko esimweni sakho kusenesikhathi.

Ingabe ingozi ingancishiswa?

Yebo, ngezinga elithile. Uma othile emndenini wakho ene-ARVD, ukuhlolwa ngokushesha okukhulu kuyindlela engcono kakhulu yokunciphisa ingozi yakho. Ukuhlolwa okulula, okungenabuhlungu kungakutshela ukuthi usengozini yokushaya kwenhliziyo okungavamile. Uma kunjalo, udokotela wakho angakusiza ukuthi wakhe uhlelo lokwelashwa olufanele wena.

Iyini isikhathi sokuphila nge-ARVD?

Isikhathi sokuphila se-ARVD siyahlukahluka kuye ngokuthi sitholakale nini. Into engcono kakhulu ongayenza ukuxilonga lesi sifo kusenesikhathi futhi uthole ukwelashwa ukuvimbela ukushaya kwenhliziyo okungajwayelekile. Lesi sifo siba sibi kakhulu ngokuhamba kwesikhathi.

Uma i-ARVD ingelashwa, i-ventricle yakho yesokudla ingahluleka. Khona-ke i-ventricle yakho yesobunxele nayo ingahluleka. Lokhu kungaholela ezimweni ezifana 'nokwehluleka kwenhliziyo' kanye 'ne-atrial fibrillation'. Abacwaningi bakholelwa ukuthi isimo siba sibi kakhulu uma i-ARVD ithinta womabili ama-ventricle.

Kodwa, ngokwelashwa, ungathola usizo oludingayo ukuze unciphise ukucindezeleka enhliziyweni yakho futhi uvimbele ukuhlaselwa yinhliziyo okuyingozi. Ezinye izifundo ziphakamisa ukuthi abanye abantu batholakala benalesi sifo ngemva kweminyaka engu-65. Kulinganiselwa nokuthi cishe umuntu oyedwa kwabahlanu abane-ARVC uqala izimpawu ngemva kweminyaka engu-50. Ngakho-ke, kungenzeka ukuphila impilo ende nge-ARVC.

Kulezi zinsuku, ubuchwepheshe obuthuthukisiwe bokuthwebula izithombe njenge-CT kanye ne-MRI buvumela ukuxilongwa nokwelashwa kusenesikhathi, ngakho-ke umbono wesikhathi eside kubantu abane-ARVD muhle.

Kodwa-ke, ukufa okuzumayo kungenzeka kulabo abangaxilongwanga noma abangatholi ukwelashwa okudingekayo. Kuthiwa i-ARVD iyimbangela engu-11% yokufa okuzumayo kwenhliziyo kubantu abangaphansi kweminyaka engu-35 kanye no-22% wokufa okuzumayo kwenhliziyo kubadlali.

Kufanele ngimbone nini udokotela? Kufanele ngiye nini egumbini lezimo eziphuthumayo?

Uma une-ARVD, kuzodingeka uqhubeke nokuhlolwa kwezokwelapha impilo yakho yonke.Udokotela wakho kuzodingeka ahlole njalo ukuqinisekisa ukuthi uthola ukwelashwa okufanele. Uma une-ICD, kuzodingeka ihlolwe njalo .

Yini okufanele uyenze uma kunesimo esiphuthumayo?

Uma othile ewa ngokuzumayo futhi engaphenduli izingcingo zakho, shayela ngokushesha ku-1990 (insizakalo ye-ambulensi ephuthumayo yaseSri Lanka). Bese, uma kungenzeka, qala i-CPR (ukuvuselelwa kwenhliziyo), noma okungenani i-CPR esetshenziswa ngezandla kuphela.

Uma usengozini yokuma kwenhliziyo ngokuzumayo, kuwumqondo omuhle ukuba nalabo abasekhaya bathole ukuqeqeshwa kwe-CPR.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

• Ngingakwazi yini ukuzivocavoca noma ukuhlanganyela kwezemidlalo? Uma kunjalo, hlobo luni lwezemidlalo?
• Yikuphi ukwelashwa okungcono kakhulu kimi?
• Ingabe ngidinga i-ICD ngalesi sikhathi?

Ngenxa yokuthi i-ARVD ingaba nzima ukuyixilonga ngaphambi kokuba kuvele izimpawu, kubalulekile ukwazi umlando womndeni wakho. Uma usengozini ngenxa yomlando womndeni walesi sifo, udokotela wakho angakuhlola.

Umlayezo Wokuya Nawe Ekhaya

Kulungile, manje usunokuqonda okungcono ngalokho esikhulume ngakho namuhla, i-ARVD. Nakuba kungavamile, kungaba yisifo senhliziyo esibi kakhulu. Ngokuvamile sibangelwa izici zofuzo . Ngakho-ke, uma othile emndenini wakho enalesi simo, kubaluleke kakhulu ukuthi abanye bahlolwe.

Ngokuthola lesi sifo kusenesikhathi, ukuthola ukwelashwa okufanele, nokwenza izinguquko ezidingekayo endleleni yokuphila, ungaphila kahle nalesi sifo. Hlala uxhumana nodokotela wakho futhi ulandele iseluleko sakhe. Futhi, ukuqinisekisa ukuthi abathandekayo bakho bayazi nge-'CPR' kungasiza ekusindiseni impilo yakho esimweni esiphuthumayo. Ungesabi, ukuqwashisa kungamandla amakhulu!

I- ARVD, i-Arrhythmogenic Right Ventricular Dysplasia, isifo senhliziyo, ukuhlaselwa yinhliziyo, i-cardiomyopathy, ukufa kwenhliziyo okuzumayo, isifo sofuzo, ukuhlaselwa yinhliziyo