Ingabe inhliziyo yesobunxele yomntwana wakho ayikhuli kahle? (Hypoplastic Left Heart Syndrome - HLHS) Ake sixoxe ngalokhu!

Namuhla sizokhuluma ngesimo senhliziyo esiyinkimbinkimbi kancane, futhi esingavamile kakhulu, kodwa esibaluleke kakhulu. Lesi yisimo esibizwa ngokuthi i-Hypoplastic Left Heart Syndrome , noma (HLHS) ngamafuphi. Ungase uzizwe wesaba uma uzwa lokhu, kodwa ungakhathazeki. Ake sixoxe ngalokhu kalula, ngendlela ongayiqonda. Ngoba, kubaluleke kakhulu ukuqaphela izinto ezinjengalezi.

Iyini i-hypoplastic left heart syndrome (HLHS)?

Kalula nje, (i-HLHS) yisifo senhliziyo esizalwa naso esiba khona lapho kuzalwa . Kulokhu, ezinye izingxenye zohlangothi lwesobunxele lwenhliziyo yengane azikhuli kahle. Cabanga ngakho njengephampu. Uhlangothi lwesobunxele lwale phampu yilona oluthumela igazi elinomoya-mpilo emzimbeni wonke. Ngakho-ke, uma lolu hlangothi lwesobunxele lungakhuli kahle, kuvela inkinga. Kulokhu, lezi zingxenye zohlangothi lwesobunxele lwenhliziyo zithinteka kakhulu:

• I-ventricle yesobunxele : Leli yigumbi eliyinhloko ohlangothini olungezansi lwesobunxele lwenhliziyo. Lipompa igazi elinomoya-mpilo liye e-aorta . Ku-HLHS, livame ukuba lincane kakhulu ukuthi lingasebenza kahle.
• I-Aorta : Lona umthambo wegazi omkhulu kunayo yonke emzimbeni wethu. Uthwala igazi lisuka enhliziyweni liye kuwo wonke umzimba. Ku-(HLHS), nalo ungase ungakhuli kahle.
• Amavalvu e-mitral kanye ne-aortic: Lawa afana neminyango. Avumela igazi ukuthi ligeleze ohlangothini olulodwa kuphela. Ivalvu ye-aortic ivumela igazi ukuthi ligeleze lisuka kwi-ventricle yesobunxele liye kwi-aorta. Ivalvu ye-mitral ivumela igazi ukuthi ligeleze lisuka ekamelweni eliphezulu (atrium) ohlangothini lwesobunxele lwenhliziyo liye ekamelweni elingezansi (mitral valve). Ku-HLHS, leli valve lingase lingasebenzi kahle noma lingase libe lincane kakhulu.

Ngezinye izikhathi, izingane ezine-HLHS zingase zibe nembobo encane odongeni phakathi kwamakamelo amabili aphezulu enhliziyo (i-atrial septal defect) . Ngokuvamile, lokhu kufanele kube udonga olujiyile.

Uyini umehluko phakathi kwenhliziyo evamile nenhliziyo ene-(HLHS)?

Inhliziyo yethu evamile inezinhlangothi ezimbili. Uhlangothi lwesokudla lupompa igazi elingenawo umoya-mpilo lisuka emzimbeni liye emaphashini ukuze liqoqe umoya-mpilo. Bese, igazi elinomoya-mpilo liya ohlangothini lwesobunxele bese lipompelwa kulo lonke umzimba. Kodwa-ke, enganeni ene-HLHS, uhlangothi lwesobunxele lwenhliziyo luncane kakhulu kangangokuthi alukwazi ukupompelela igazi elanele kulo lonke umzimba. Bese, i-ventricle yesokudla iyathatha izintambo. Izama ukupompelela igazi emaphashini nakuwo wonke umzimba. Lokhu kwenziwa nge-ductus arteriosus.Ngomthambo wegazi okhethekile obizwa ngokuthi i-ductus arteriosus. Lo (ductus arteriosus) umthambo wegazi onawo wonke umntwana esibelethweni. Ngokuvamile uvaleka ezinsukwini ezimbalwa ngemva kokuzalwa. Kodwa enganeni ene-(HLHS), uma lo mthambo wegazi uvaleka, uma ungelashwa, ungaba yingozi empilweni. Ngenxa yokuthi uhlangothi lwesobunxele alusebenzi, okuwukuphela kwendlela igazi elifika ngayo emzimbeni ivinjiwe.

Kuvamile kangakanani (i-HLHS)?

(HLHS) yisimo esingavamile kakhulu. Ngokwezibalo e-United States, sithinta cishe ingane eyodwa kwezingu-3,800 ezizalwa minyaka yonke. (HLHS) iphakathi kuka-2% no-3% wazo zonke izifo zenhliziyo ezizalwa nazo (CHD). Sivame kakhulu kubafana kunamantombazane.

Ziyini izimpawu zomntwana one-(HLHS)?

Izimpawu zomntwana one-(HLHS) zingase zingaveli ngokushesha. Zingase zivele zingakapheli amahora noma izinsuku ezimbalwa ngemva kokuzalwa. Izimpawu eziyinhloko yilezi:

• I-Cyanosis : Umbala oluhlaza okwesibhakabhaka ompunga wesikhumba, izindebe, kanye nezinzipho. Lokhu kungabonakala njengombala ompunga ezinganeni ezinesikhumba esimnyama. Kungabonakala njengombala oluhlaza okwesibhakabhaka ezinganeni ezinesikhumba esikhanyayo. Lokhu kubangelwa ukungatholi umoya-mpilo owanele emzimbeni.
• Ubunzima bokuphefumula : Ingane ingase izwe sengathi kunzima ukuphefumula.
• Ubunzima bokuphuza ubisi: Ingane ingase ingafuni ukuphuza ubisi, noma ingase ikhiphe amathe lapho iphuza ubisi.
• Ukuzola : Ingane ingase ibonakale ilele futhi ikhathele.
• Ukushaya kwenhliziyo okusheshayo.
• Ukujuluka , isikhumba esithambile, noma ukuzizwa ubanda.
• Ukushaya kwenhliziyo okubuthakathaka.

Ngisho noma ubona eyodwa noma ezimbili zalezi zimpawu, kubaluleke kakhulu ukufuna iseluleko sezokwelapha ngokushesha. Ungesabi, kodwa thatha isinyathelo ngokushesha.

Yiziphi izimbangela ze-(HLHS)?

Ezimweni eziningi, asikho isizathu esithile se-HLHS. Ngezinye izikhathi kungase kube ngenxa yezakhi zofuzo . Izingane ezinezinguquko ezithile zezakhi zofuzo, njenge- GJA1 noma i-NKX2-5, zisengozini enkulu yokuthola i-HLHS. Futhi, izingane ezinezimo ezithile zezakhi zofuzo, njenge -Turner syndrome noma i-Trisomy 18, zingaba ne-HLHS.

I-HLHS ihlolwa kanjani?

Odokotela bangaxilonga i-HLHS ngokuhlola izithombe okungabandakanyi ukutheleleka. Lokhu kungenziwa ngesikhathi sokukhulelwa noma ngemva kokuzalwa komntwana. Ngesikhathi sokukhulelwa:

• I-ultrasound yangaphambi kokubeletha: Lokhu ukuskena okuvame ukwenziwa ngesikhathi sokukhulelwa.
• I-echocardiogram yengane esanda kuzalwa: Lokhu kuskena okukhethekile okungahlola izinkinga zenhliziyo ngesikhathi ingane isesesibelethweni.

Ngemva kokuzalwa kwengane: Odokotela baxilonga lesi simo ngokubheka izimpawu nokubheka imiphumela yokuhlolwa. Ngezinye izikhathi, lapho ulalele inhliziyo yengane nge-stethoscope, ungase uzwe ukububula kwenhliziyo . Lokhu kusho ukuthi igazi aligelezi kahle.

Yiziphi izivivinyo ezisetshenziswa ukuxilonga (i-HLHS)?

• I-X-ray yesifuba: Lokhu kungahlola usayizi kanye nesimo senhliziyo namaphaphu engane.
• I-Echocardiogram: Lokhu futhi ukuhlolwa kwe-ultrasound. Izakhiwo zangaphakathi zenhliziyo zingabonakala ngokucacile.
• I-Electrocardiogram (EKG/ ECG ): Lokhu kusetshenziselwa ukukala izinguquko zikagesi ezenzeka ngesikhathi sokushaya kwenhliziyo.
• Ukuhlolwa kwe-pulse oximetry: Lokhu kunganquma inani lomoya-mpilo egazini lomntwana.

Ingabe kukhona ukwelashwa kwe-(HLHS)?

Yebo, kunezindlela zokwelapha. Okokuqala, ingane idinga ukunikezwa umuthi obizwa ngokuthi i-prostaglandin . Lokhu kugcina i-ductus arteriosus ivulekile. Lokhu kudala indlela yokuthi igazi ligeleze emzimbeni. Ngaphezu kwalokho, eminye imithi inganikezwa ukusiza inhliziyo yengane ukuthi isebenze kahle. Ingane ingadinga nosizo lokuphefumula. Ngemuva kwalokho, kungase kwenziwe ukuhlinzwa okuningana. Lokhu kuhlinzwa kusetshenziselwa ukuqondisa ukugeleza kwegazi emaphashini nasemzimbeni. Ngemva kwalokhu kuhlinzwa, umsebenzi wenhliziyo cishe udluliselwa ngokuphelele kwi-ventricle yesokudla. Yiyona epompa igazi liye kuwo wonke umzimba.

Yikuphi ukuhlinzwa okwenziwayo (i-HLHS)?

Odokotela abahlinzayo benza izinqubo ezintathu eziyinhloko: inqubo yeNorwood , inqubo yeGlenn , kanye nenqubo yeFontan . Lezi zinqubo zenziwa ngokulandelana. 1. Inqubo yeNorwood: Le nqubo yenziwa phakathi namasonto amabili okuqala okuphila kwezingane ezine-HLHS. Kule nqubo, odokotela abahlinzayo:

• I-aorta yengane engakakhuli kahle ishintshwa ukuze igazi ligeleze emzimbeni.
• I-shunt , ipayipi elincane, lifakwa ukuze liguqule igazi lisuke e-ventricle yesokudla, kungaba lisuka e-aorta, liye emithanjeni yamaphaphu eholela emaphashini.
• Kudala ukuxhumana phakathi kwamakamelo aphezulu (i-atria) enhliziyo. Lokhu kusiza ukuhlinzeka umzimba ngegazi elinomoya-mpilo elivela emaphashini.

2. Ukuhlinzwa kwe-shunt ye-Glenn okuqondiswayo: KomntwanaUkuhlinzwa kwesibili kufanele kwenziwe ezinyangeni ezi-4 kuya kwezi-6. Ngesikhathi sokuhlinzwa kukaGlenn:

• I-shunt endala iyasuswa.
• Kufakwa i-shunt entsha bese ixhumanisa i-vena cava (SVC) yengane (ethwala igazi elingenawo umoya-mpilo lisuka emzimbeni ongaphezulu liye enhliziyweni) ne-pulmonary artery.
• Le shunt yehlisa umthwalo ku-ventricle yesokudla, ngoba ivumela igazi ukuthi liye ngqo emaphashini.

3. Inqubo ye-Fontan: Inqubo yokugcina yenziwa phakathi kweminyaka eyi- 18 neminyaka engu-4 . Le nqubo idlulisela lonke igazi elibuya emzimbeni liye emaphashini, idlule inhliziyo. Ngesikhathi senqubo ye-Fontan:

• I -vena cava engaphansi kwengane (IVC) (ethwala igazi elingenawo umoya-mpilo lisuka emzimbeni ongezansi liye enhliziyweni) ixhumeka emthanjeni wamaphaphu.

Ingabe kukhona izinkinga ekwelashweni?

Yebo, ezinye izingane zingalahlekelwa ngisho nokuphila kwazo ngenkathi zisuka kolunye ukuhlinzwa ziye kolunye. Futhi, kungaba nezinkinga ezithile ngemva kokuhlinzwa ngakunye. Isibonelo:

• Izinkinga nge-ventricle yesokudla engasebenzi kahle.
• Ukuvuza kwe-valve ye-aorta.
• Isifo sesibindi.
• Izigqi zenhliziyo ezingavamile.
• Amahlule egazi.
• Ukutheleleka.
• Ubunzima bokudla.
• Ukuquleka.
• Izinkinga zezinso.
• Ukuboshwa kwenhliziyo.

Lezi izinto ezesabekayo ukuzizwa, kodwa odokotela bazokhuluma nawe ngalezi zingozi futhi bazame ukukunikeza ukwelashwa okungcono kakhulu.

Ingabe ukufakelwa inhliziyo kuyindlela enhle yokwelapha?

Ngezinye izikhathi, odokotela abahlinzayo bangase batuse ukufakelwa inhliziyo esikhundleni sokwenza lezo zinqubo ezintathu zokuhlinzwa. Kodwa-ke, izingane eziye zafakelwa inhliziyo kuzodingeka ziphuze imithi (imithi yokucindezela amasosha omzimba) impilo yazo yonke.

Ingabe i-HLHS ingelashwa ngaphambi kokuzalwa?

Okwamanje akunakwenzeka ukwelapha ngokuphelele i-HLHS ngokuhlinzwa ngesikhathi sokukhulelwa. Kodwa-ke, odokotela abahlinzayo be-fetus ngezinye izikhathi bangenza ezinye izindlela zokuvimbela eminye yemiphumela emibi engase ivele enganeni ekhulayo ene-HLHS (isb., i-aortic valve encane). Kodwa lokhu kwenzeka kuphela ezimweni ezikhethekile kakhulu.

Ingabe ingozi ye-(HLHS) ingancishiswa?

Njengoba imbangela yamacala amaningi e-HLHS ingaziwa, kunzima ukusho ukuthi ungayivimbela kanjani ngokuphelele. Kodwa-ke, kubalulekile njalo ukulandela imikhuba enempilo ngesikhathi sokukhulelwa:

• Ukugwema utshwala nokubhema.
• Ukulawula ezinye izimo zezokwelapha njenge -Diabetes Mellitus .
• Ukudla ukudla okunempilo.
• Ukuthatha i-vitamin yokukhulelwa equkethe okungenani ama-microgram angu-400 (400 mcg) we-folic acid nsuku zonke .

Uma wena noma umlingani wakho ninomlando womndeni we-HLHS, kungumqondo omuhle ukukhuluma nomeluleki wezakhi zofuzo ngaphambi kokukhulelwa.

Uma ingane yami inayo (i-HLHS), yini okufanele ngiyilindele?

Ngemva kokwelashwa kwe-HLHS, ingane yakho kuzodingeka ibone udokotela wenhliziyo okungenani kanye ngonyaka impilo yayo yonke. Lokhu kuzoqinisekisa ukuthi inhliziyo yayo, amaphaphu, nezinye izitho zomzimba zisebenza kahle. Njengoba ingane yakho ikhula, kuzodingeka ibone uchwepheshe wesifo senhliziyo sokuzalwa sabantu abadala . Izingane eziningi ezine-HLHS kuzodingeka zithathe imithi yenhliziyo impilo yazo yonke. Kuzodingeka futhi zithathe ama-antibiotic ngaphambi kwanoma yikuphi ukuhlinzwa, okuhlanganisa nokuhlinzwa kwamazinyo. Lokhu kunganciphisa ingozi ye -endocarditis (ukutheleleka kwengaphakathi lenhliziyo).

Iyini inhloso yesimo (se-HLHS)?

Uma ingelashwa, i-HLHS yisimo esingaholela ekufeni zingakapheli izinsuku noma amasonto okuzalwa. Ngokwelashwa, isimo sincike enkingeni yesifo senhliziyo enganeni. Buza udokotela wengane yakho ngezingozi zokuhlinzwa ngakunye. Ezinye izingane zingase zibe nekhono lokuzivocavoca elinciphile impilo yazo yonke. Odokotela bavame ukuncoma ukunciphisa imisebenzi yomzimba enzima, njengemidlalo yokuncintisana.

Lingakanani izinga lokusinda kwezinsana ezine-(HLHS)?

Lokhu kuyiqiniso elidabukisayo, kodwa kubalulekile ukwazi. Phakathi kuka-20% no-60% wezingane ezine-(HLHS) zisinda onyakeni wokuqala wokuphila. Ngemva kwalokho, izinga lokusinda eminyakeni emihlanu, eyishumi, neyishumi nanhlanu ezayo licishe libe ngu-40%. Izingane ezizalwa ngesisindo sokuzalwa esijwayelekile, ngaphandle kokuzalwa ngaphambi kwesikhathi, zinemiphumela engcono kunezingane ezizalwa zinesisindo esiphansi sokuzalwa. Ucwaningo olulodwa luthole ukuthi izingane eziningi ezisinda onyakeni wokuqala zazisaphila zineminyaka engu-18.

Kunzima ukubona lezi zibalo. Kodwa khumbula, isayensi yezokwelapha iyathuthuka nsuku zonke. Ukwelashwa okusha, ubuchwepheshe obusha buza. Ngakho-ke kubalulekile ukugcina ithemba liphila.

Ngingayinakekela kanjani ingane yami?

Izingane ezizalwa nalesi simo zingaphila impilo enempilo ngokunakekelwa isikhathi eside ngudokotela wenhliziyo. Unganakekela ingane yakho ngokuthi:

• Nikeza ingane yakho umuthi wokugomela umkhuhlane (umkhuhlane) kanye nomuthi wokugomela i-COVID-19 njalo ngonyaka.
• Hamba nengane yakho iyobonana nodokotela wenhliziyo njalo ezinyangeni eziyisithupha noma kanye ngonyaka .
• Nikeza ingane yakho imithi oyinikezwe ngesikhathi .
• Nciphisa umsebenzi onzima womzimba wengane yakho , njengoba kunconywa udokotela wakho.
• Uma ingane yakho inezinkinga zokufunda , yisize.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Uma ingane yakho ine-HLHS, ungabuza udokotela imibuzo efana nale:

• Ziyini izingozi zokuhlinzwa?
• Ingabe kukhona izinkinga okufanele uziqaphele ngemva kokuhlinzwa?
• Yimiphi imithi edingwa yingane yami? Ingabe ikhona imiphumela emibi kuleyo mithi?
• Lesi simo (i-HLHS) sizoyithinta kanjani impilo yengane yami?
• Yikuphi ukunakekelwa okulandelayo ingane yami edingayo ngemva kokuhlinzwa?
• Uma nginomunye umntwana, ingabe ikhona ingozi yokuthi lowo mntwana naye uzoba nomntwana (i-HLHS)?

Ukuba nengane ene-HLHS kungaba yinto ekhathazayo kakhulu ngokomzwelo futhi enesizungu . Uma ubhekene nokucindezeleka nokungaqiniseki kwesifo senhliziyo sengane yakho, kubalulekile ukuthola iqembu lokusekela noma enye indawo yokuthola ukwesekwa ngokomzwelo. Ukuhlala uqinile ngokwengqondo kuzokusiza ukubhekana nobunzima nobunzima bempilo yengane yakho.

Umlayezo Wokuya Nawe Ekhaya

(HLHS) yisifo senhliziyo esibi kakhulu, esizalwa naso. Kodwa-ke, uma sitholakala kusenesikhathi futhi selashwa kahle, izingane zinethuba lokuphila impilo enhle. Lokhu kudinga ukuhlinzwa okuningana okuyinkimbinkimbi kanye nokubhekwa kwezokwelapha isikhathi eside. Into ebaluleke kakhulu ukwazi ukuthi awuwedwa. Odokotela, abahlengikazi, kanye nabanye abasebenzi bezempilo bakhona ukukusiza wena nengane yakho. Kubaluleke kakhulu futhi ukuthola ukwesekwa kwabanye abazali abaye baba nokuhlangenwe nakho okufanayo. Ungalahli isibindi. Iminikelo oyenzela ingane yakho ayinakuqhathaniswa.