Ingabe uhlangothi lwesobunxele lwenhliziyo yengane yakho lubuthakathaka? Ake sixoxe nge-Hypoplastic Left Heart Syndrome (HLHS)

Kunzima ukuchaza umuzwa owuzwayo lapho udokotela wakho ekutshela ukuthi ingane yakho esanda kuzalwa inesifo senhliziyo esibi kakhulu. Kanye nokwesaba, ukushaqeka nokudabuka, umbuzo othi "Kuzokwenzekani enganeni yethu?" uzwakala engqondweni yakho. Yisikhathi esinzima kakhulu. Kodwa into ebaluleke kakhulu ngesikhathi esinjengalesi ukuqaphela ngokugcwele lesi simo. Namuhla sizokhuluma nge-Hypoplastic Left Heart Syndrome (HLHS), isimo senhliziyo esibi nesingavamile esibangelwa ukuzalwa. Kulesi sihloko, sizokhuluma ngalokhu kalula, ngendlela ongayiqonda.

Iyini ngempela i-Hypoplastic Left Heart Syndrome (HLHS)?

Kalula nje, i-Hypoplastic Left Heart Syndrome (HLHS) yisimo esenzeka lapho uhlangothi lwesobunxele lwenhliziyo lungakhuli kahle ngesikhathi umntwana ekhula esibelethweni. Lesi yisifo senhliziyo esizalwa naso. Igama elithi "hypoplastic" lisho "ukungakhuli kahle."

Inhliziyo yethu ifana nendlu encane enamakamelo amane. Amakamelo amabili phezulu namakamelo amabili phansi. Izingxenye zohlangothi lwesobunxele lwenhliziyo yomntwana one-HLHS, ikakhulukazi:

• I-ventricle yesobunxele: Lena yigumbi eliyinhloko lokupompa lenhliziyo. Lipompa igazi elihlanzekile, elinomoya-mpilo emzimbeni wonke. Ku-HLHS, lincane kakhulu futhi alikakhuli kahle.
• I-Aorta: Umthambo wegazi oyinhloko othwala igazi lisuka e-ventricle yesobunxele liye kuwo wonke umzimba. Lokhu futhi kuvame ukuba mncane futhi kube kuncane.
• Amavalvu e-mitral kanye ne-aortic: Lawa afana neminyango. Avumela igazi ukuthi ligeleze ohlangothini olulodwa kuphela. Ku-HLHS, lawa mavalvu angase angakhuli kahle noma angase avalwe ngokuphelele.

Ngenxa yokuthi lezi zingxenye ezinkulu azikhuli kahle, uhlangothi lwesobunxele lwenhliziyo alukwazi ukupompa igazi elinomoya-mpilo elanele emzimbeni wonke. Lesi yisimo esibi kakhulu. Ngezinye izikhathi, kungaba nembobo encane odongeni phakathi kwamakamelo amabili aphezulu enhliziyo, ebizwa ngokuthi i- atrial septal defect .

Uyini umehluko phakathi kwalokhu nenhliziyo enempilo?

Enhliziyweni enempilo, zombili izinhlangothi zesokudla nesobunxele zisebenza ndawonye. Cabanga ukuthi uhlangothi lwesokudla luthatha igazi elingenawo umoya-mpilo emzimbeni bese lulithumela emaphashini ukuze lithole umoya-mpilo. Igazi elicebile ngomoya-mpilo emaphashini libuyela ohlangothini lwesobunxele lwenhliziyo. Bese ipompo enamandla ohlangothini lwesobunxele (i-ventricle yesobunxele) ipompa lelo gazi liye kuwo wonke umzimba.

Kodwa enganeni ene-HLHS, uhlangothi lwesobunxele lwenhliziyo lubuthakathaka kakhulu. Ngakho alukwazi ukwenza lowo msebenzi. Pho kwenzekani? Uhlangothi lwesokudla lwenhliziyo kufanele lwenze yomibili le misebenzi lodwa . Lokho kusho ukuthi i-ventricle yesokudla kufanele ipompe igazi liye emaphashini, kuyilapho ipompa igazi liye kuwo wonke umzimba.

Kwenzeka kanjani lokhu? Uma umntwana esesibelethweni, kuba nokuxhumana kwesikhashana phakathi kwemithambo yegazi emibili eyinhloko enhliziyweni. Kubizwa ngokuthi i-ductus arteriosus.Kufana 'nendlela enqamulelayo'. Le 'ndlela enqamulelayo' ibalulekile ukuze ingane ene-HLHS iphile ngemva kokuzalwa. Ngoba igazi ligeleza ngaleli payipi liye emzimbeni. Ngokuvamile, leli payipi livala ezinsukwini ezimbalwa ngemva kokuzalwa kwengane. Kodwa uma leli payipi livala enganeni ene-HLHS, ayikho indlela yokuthi igazi ligeleze liye emzimbeni. Ngakho-ke, uma lesi simo singelashwa, ingane izoba nezinsuku ezimbalwa kuphela zokuphila.

Wazi kanjani ukuthi ingane yakho inalesi simo? Ziyini izimpawu?

Uma umntwana osanda kuzalwa ene-HLHS, ngezinye izikhathi izimpawu azibonakali ekuqaleni. Kodwa lezi zimpawu zingavela emahoreni ambalwa noma ezinsukwini ezimbalwa. Kubaluleke kakhulu ngabazali ukuqaphela lokhu.

Kungani lokhu kwenzeka ezinganeni? Iyini imbangela?

Lo mbuzo usemqondweni wabazali bonke. Kujwayelekile ukuzibuza ukuthi, "Kungani lokhu kwenzeke enganeni yethu? Ingabe senze into engalungile?"

Kodwa iqiniso liwukuthi, ezimweni eziningi, akukho imbangela ethile ye-HLHS . Okusho ukuthi, akuyona into ebangelwa umama noma ubaba. Ngezinye izikhathi, izici zofuzo zingadlala indima. Okusho ukuthi, izinguquko ezithile zofuzo emndenini zingandisa ingozi. Izingane ezinezinye izimo zofuzo, njenge-Turner syndrome noma i-trisomy 18, nazo zisengozini ye-HLHS.

Odokotela basithola kanjani lesi sifo ngokunembile?

Ngenhlanhla, ngobuchwepheshe obuthuthukisiwe banamuhla, lesi sifo singatholakala ngaphambi kokuba umntwana azalwe, okungukuthi, ngesikhathi sokukhulelwa . Uma kutholakala ukungajwayelekile enhliziyweni ngesikhathi sokuhlolwa kwe-ultrasound okuvamile ngaphambi kokubeletha, udokotela wakho uzokudlulisela kuchwepheshe futhi akucele ukuthi wenze i- echocardiogram yengane . Lokhu kukuvumela ukuthi ubone isakhiwo kanye nomsebenzi wenhliziyo yengane ngokucacile.

Ukuze ahlole lesi simo ngemva kokuzalwa komntwana, udokotela uzobheka izimpawu zomntwana bese elalela isifuba nge-stethoscope ukuze abone ukuthi kukhona yini umsindo wenhliziyo ongajwayelekile (ukububula kwenhliziyo). Ngemuva kwalokho, kuzokwenziwa izivivinyo eziningana ukuqinisekisa lesi simo, njengokuthi:

• I-X-ray yesifuba: I-X-ray yesifuba ingahlola usayizi nesimo senhliziyo namaphaphu.
• I-Echocardiogram: Lesi isivivinyo esibaluleke kakhulu. Kufana nokuskena kwevidiyo kwenhliziyo. Amakamelo enhliziyo, ama-valve, kanye nemithambo yegazi konke kubonakala kahle. Yilokhu okuqinisekisa ukuba khona kwe-HLHS.
• I-Electrocardiogram (EKG): Ukuhlolwa okulinganisa umsebenzi kagesi wenhliziyo.
• Ukuhlolwa kwe-pulse oximetry: Idivayisi encane efana ne-clip ibekwa emunweni noma endlebeni yengane ukuze kulinganiswe inani lomoya-mpilo egazini. Leli nani liphansi ezinganeni ezine-HLHS.

Ingabe ikhona indlela yokwelapha lesi simo? Ingabe ingane ingasindiswa?

Yebo, lesi yisimo esibucayi kakhulu, kodwa kunezindlela zokwelapha. Kodwa lesi akusona isifo esingelapheka ngemithi. Ukuze kusindiswe impilo yengane, uchungechunge oluyinkimbinkimbi lokuhlinzwa olunezigaba eziningana kumele lwenziwe.

Isinyathelo Sokuqala: Ukuqinisa Umntwana

Ngaphambi kokuhlinzwa, umntwana udinga ukuzinza. Isinyathelo sokuqala ukugcina i-ductus arteriosus , umthambo wegazi 'onqamulelayo' esikhulume ngawo, uvulekile. Umuthi obizwa ngokuthi i-prostaglandin unikezwa ngemithambo yegazi. Eminye imithi ingasetshenziswa futhi ukusiza inhliziyo yengane ukushaya, futhi kungasetshenziswa umshini wokuphefumula ukusiza ingane ukuthi iphefumule.

Isinyathelo Sesibili: Uchungechunge Lokuhlinzwa Lwezigaba Ezintathu

Umgomo oyinhloko walolu chungechunge lokuhlinzwa ukugwema ngokuphelele uhlangothi lwesobunxele olubuthakathaka nokunikeza uhlangothi lwesokudla oluqinile lwenhliziyo (i-ventricle yesokudla) umsebenzi wokupompa igazi liye emzimbeni wonke. Kufana nokuqondisa ukugeleza kwegazi ohlangothini oluhluke ngokuphelele.

1. Inqubo kaNorwood: Lokhu kwenziwa phakathi namasonto amabili okuqala okuzalwa komntwana. Lokhu ukuhlinzwa okuyinkimbinkimbi kakhulu nokungathi sína. Lapha, odokotela abahlinzayo:

• I-aorta engakathuthuki iyakhiwa kabusha.
• Kufakwa ipayipi ekhethekile (i-shunt) ukuze ithumele igazi lisuka kwi-ventricle yesokudla ngqo emaphashini.
• Ngale ndlela, "uhlelo lwe-tubular" lwenhliziyo luyaguqulwa ukuze i-ventricle yesokudla ikwazi ukupompa igazi liye emzimbeni wonke nasemaphashini.

2. Ukuhlinzwa kwe-Bidirectional Glenn shunt: Lokhu kwenziwa lapho umntwana ephakathi kwezinyanga ezi-4 nezi-6 ubudala . Lapha:

• Ipayipi (i-shunt) efakwe ngesikhathi sokuhlinzwa kokuqala iyasuswa.
• Igazi elingenawo umoya-mpilo ovela engxenyeni engenhla yomzimba (evela emthanjeni obizwa ngokuthi i-vena cava ephezulu) lixhunywe ngqo emaphashini.
• Lokhu kunciphisa kakhulu umthwalo womsebenzi e-ventricle yesokudla, ngoba manje akudingeki ukuthi ipompe igazi lisuka emzimbeni ongaphezulu, liya ngqo emaphashini.

3. Inqubo yeFontan: Lesi yisigaba sokugcina. Senziwa phakathi kweminyaka eyi-18 neminyaka engu-4 . Lapha:

• Igazi elingenawo umoya-mpilo okwanele elivela engxenyeni engezansi yomzimba (elivela emthanjeni obizwa ngokuthi i-vena cava engaphansi) lixhunywe ngqo emaphashini.
• Ngemva kwalokhu kuhlinzwa, lonke igazi elingenawo umoya-mpilo emzimbeni liya ngqo emaphashini. I-ventricle yesokudla yenhliziyo kufanele ipompe igazi elihlanzekile, elinomoya-mpilo elivela emaphashini liye emzimbeni wonke.

Ngemva kokuba lokhu kuhlinzwa okuthathu sekuqediwe, uhlelo lokujikeleza kwegazi lomntwana luqala ukusebenza ngendlela entsha ngokuphelele.

Yiziphi izinkinga zokwelashwa?

Nakuba lokhu kuhlinzwa kusindisa impilo, kuyinkimbinkimbi kakhulu futhi kungaba nezingozi nezinkinga. Izinkinga zingavela ngesikhathi sokuhlinzwa nangemva kokuhlinzwa.

• I-ventricle yesokudla iba buthakathaka ngenxa yomthwalo osindayo okufanele iwuthwale.
• Ukuvuza kwegazi okuvela kuma-valve enhliziyo.
• Izifo zesibindi.
• Izigqi zenhliziyo ezingavamile.
• Ukujiya kwegazi.
• Izifo.
• Ubunzima bokudla.
• Ukuquleka.
• Imiphumela ezinso.

Udokotela uzoxoxa nawe ngazo zonke lezi zingozi ngokuningiliziwe. Ithimba lezokwelapha lizoqapha ingane ngokucophelela njengoba behamba kulolu hambo.

Ingabe Ukufakelwa Inhliziyo Kuyindlela Enhle?

Kwezinye izimo, uma isimo senhliziyo yengane siyinkimbinkimbi kakhulu noma uma odokotela becabanga ukuthi ingane ngeke isinde ekuhlinzweni, bangase batuse ukufakelwa inhliziyo . Kodwa kufanele balinde kuze kube yilapho inhliziyo ehambisanayo itholakala. Futhi, ngemva kokufakelwa inhliziyo, ingane kuzodingeka ithathe imithi yokucindezela amasosha omzimba impilo yayo yonke.

Uma umntwana enalesi simo, yini okufanele siyazi njengabazali?

Lolu hambo luyinselele, kodwa ngokuphathwa kahle kwezokwelapha nokunakekelwa, izingane ezine-HLHS zingaphila impilo enhle.

• Ukuhlolwa kwezokwelapha impilo yonke: Ngemva kokuhlinzwa, ingane kuzodingeka ivakashele udokotela wenhliziyo impilo yayo yonke. Kufanele ihlolwe okungenani kanye ngonyaka.
• Imithi: Izingane eziningi kuzodingeka zithathe imithi yenhliziyo impilo yazo yonke.
• Ama-antibiotic ngaphambi kokunye ukuhlinzwa: Ngaphambi kwanoma yikuphi okunye ukuhlinzwa, njengokukhipha amazinyo, udinga ukuthatha ama-antibiotic ukuvimbela izifo zenhliziyo (i-endocarditis).
• Umsebenzi womzimba: Umsebenzi womzimba wengane yakho kungadingeka ulinganiselwe. Imisebenzi enzima, njengemidlalo yokuncintisana, ngokuvamile ayinconywa. Cela iseluleko kudokotela wakho ngalokhu.
• Impilo yakho yengqondo: Lolu hambo lukhathaza kakhulu abazali. Lungaba lucindezelayo. Ngakho-ke, khuluma ngemizwa yakho nomyeni/umkakho nomndeni wakho. Funa iseluleko uma kudingeka. Kuphela uma unamandla lapho uzokwazi ukunakekela kahle ingane yakho.