Ingabe uhlangothi lwesokudla lwenhliziyo yengane yakho alukhuli kahle? (Hypoplastic Right Heart Syndrome)

Kuthiwani uma ingane yakho encane izalwa inenkinga yenhliziyo? Cabanga ukuthi lokho kungaba buhlungu kangakanani. Ezinye izingane zizalwa zinohlangothi lwesokudla lwenhliziyo yazo olungakhuli kahle. Odokotela babiza lokhu ngokuthi yi-Hypoplastic Right Heart Syndrome. Lokhu kunciphisa inani lomoya-mpilo umntwana alitholayo. Ake sixoxe ngalokhu ngokuningiliziwe, akunjalo?

Iyini i-Hypoplastic Right Heart Syndrome?

Kalula nje, i-Hypoplastic Right Heart Syndrome yisifo senhliziyo esingavamile esizalwa naso ezinye izingane . Okwenzekayo kulokhu ukuthi ezinye izingxenye zohlangothi lwesokudla lwenhliziyo yengane azikhuli ngokugcwele. Cabanga ngakho njengephampu encane. Uhlangothi lwesokudla lwenhliziyo yilona oluthatha igazi elingenawo umoya-mpilo emzimbeni bese lulithumela emaphashini ukuze lithole umoya-mpilo. Ngakho-ke, uma lolu hlangothi lwesokudla lungakhuli kahle, ngeke lwenze umsebenzi walo kahle.

Kulesi simo, izingxenye ezilandelayo zenhliziyo zingathinteka:

• I-valve ye-Tricuspid: Lokhu kufana nesango eliphakathi kwe-atrium yangakwesokudla ne-ventricle yangakwesokudla yenhliziyo. Uma ingasebenzi kahle, ivimbela ukugeleza kwegazi.
• I-valve yamaphaphu: Lena yi-valve lapho umthambo wamaphaphu, othwala igazi lisuka kwi-ventricle yesokudla liye emaphashini, uqala khona.
• I-ventricle yesokudla: Lena yigumbi eliyinhloko lokupompa igazi ohlangothini lwesokudla lwenhliziyo. Uma liba lincane, lizopompa igazi elincane.
• Umthambo we-pulmonary: Lona umthambo omkhulu othwala igazi lisuka kwi-ventricle yesokudla liye emaphashini.

Kuye ngokuthi isimo sibi noma sincane kangakanani, ukusebenza kwalezi zingxenye zenhliziyo kungancipha noma kwandiswe.

Ngezinye izikhathi, izingane ezine-HRHS zingaba nezinye izinkinga zenhliziyo. Isibonelo:

• I-Atrial septal defect (ASD): Imbobo odongeni phakathi kwamakamelo amabili aphezulu (i-atria) enhliziyo.
• I-Ventricular septal defect (VSD): Imbobo odongeni phakathi kwamakamelo amabili aphansi (ama-ventricles) enhliziyo.
• I-Atrioventricular canal defect: Imbobo enkulu phakathi kwenhliziyo kanye nezinye izinkinga eziningana.

Sibi kangakanani lesi simo?

I-Hypoplastic right heart syndrome (HRHS) ingaba yingozi kakhulu kwabanye abantu kunabanye . Abantu abanezimo ezinzima, njengamazinga aphansi kakhulu omoya-mpilo , badinga ukwelashwa ngokushesha . Kodwa-ke, abanye abantu bangase bangabi nayo inkinga enkulu kangako, kodwa banezinkinga ezincane kuphela. Abantu abanjalo bangase bangatholakali kuze kube yilapho sebekhulile.

Sivame kangakanani lesi simo?

Lesi yisimo esingavamile kakhulu ngempela.Abacwaningi balinganisela ukuthi abantu abangaphansi kuka-50,000 e-United States bane-HRHS. Kuyivelakancane kune-Hypoplastic Left Heart Syndrome, isimo lapho uhlangothi lwesobunxele lwenhliziyo lungakhuli kahle.

Ziyini izimpawu ze-hypoplastic right heart syndrome (HRHS)?

Okulandelayo yizimpawu zalesi simo:

• Ukushintsha kombala wesikhumba sezandla nezinyawo okuluhlaza okwesibhakabhaka. Lokhu kubizwa ngokuthi i-cyanosis . Kusho ukuthi umzimba awutholi umoya-mpilo owanele.
• Ubunzima bokuphefumula. Lokhu kubizwa ngokuthi i-dyspnea .
• Ukuphefumula okusheshayo. Lokhu kubizwa ngokuthi i-tachypnea .
• Ingane ayiphuzi ubisi ngendlela efanele.
• Ukukhathala ngokushesha, ukuzizwa ulele njalo.

Uma ingane yakho inesinye noma ngaphezulu salezi zimpawu, kungcono ukubona udokotela ngokushesha.

Yini ebangela lokhu?

Eqinisweni, imbangela eqondile ye-hypoplastic right heart syndrome (HRHS) ayikatholakali okwamanje .

Kodwa-ke, abacwaningi bakholelwa ukuthi kungenzeka ukuthi kukhona izakhi zofuzo ezihilelekile , njengoba amalungu amaningana omndeni ofanayo kubikwe ukuthi ane-HRHS lapho ezalwa. Ngakho-ke, kudingeka ucwaningo olwengeziwe.

Yiziphi ezinye izinkinga ezingabangelwa yilokhu?

I-Hypoplastic Right Heart Syndrome (HRHS) ingabangela izinkinga ezifana nalezi:

• Amazinga aphansi omoya-mpilo emzimbeni. Lokhu kubizwa ngokuthi i-hypoxemia .
• Ukwehluleka kwenhliziyo ohlangothini lwesokudla.
• Ukushaya kwenhliziyo okungajwayelekile. Lokhu kubizwa ngokuthi i-arrhythmia .

Ebancane, ngisho nakulabo abangathinteki kakhulu yi-HRHS, ukuphuphuma kwesisu kungenzeka ngenxa yamazinga aphansi omoya-mpilo emzimbeni.

Lesi sifo sitholakala kanjani?

Ngezinye izikhathi, umhlinzeki wezempilo angathola i-HRHS ngenkathi umntwana esesesibelethweni (ukukhula kwengane) . Basebenzisa i-ultrasound scan ukuze babone inhliziyo yengane. Uma ufuna ukubhekisisa, ungenza ne-echocardiogram yengane . Zombili lezi zivivinyo azihlaseli.

Yiziphi izivivinyo ezenziwa ngemva kokuzalwa komntwana?

Ngemva kokuzalwa komntwana, lezi zivivinyo ezingahlaseli zingenziwa ukuthola ukuthi umntwana une-HRHS yini:

• I-pulse oximeter (i-pulse ox): Lena idivayisi encane efana ne-clip elinganisa amazinga e-oxygen ngokuyinamathisela emunweni noma endlebeni yakho.
• I-Electrocardiogram (EKG/ECG): Ukuhlolwa okukala ukusebenza kukagesi kwenhliziyo.
• I-Echocardiogram (i-Echocardiogram - i-echo): Lolu uhlobo lokuhlola olusebenzisa amaza omsindo, afana ne-ultrasound, ukuthatha izithombe zenhliziyo. Lungabonisa isakhiwo senhliziyo, ukuthi ama-valve asebenza kanjani, nokuthi igazi ligeleza kanjani.
• I-MRI Yenhliziyo (I-Magnetic Resonance Imaging): Isebenzisa amasimu kazibuthe namagagasi omsakazo ukukhiqiza izithombe ezinemininingwane zenhliziyo.

Yiziphi izindlela zokwelapha lokhu?

Ukwelashwa kwe-hypoplastic right heart syndrome (HRHS) kungafaka imithi, izinqubo ezingangenisi kakhulu, noma ukuhlinzwa . Umgomo oyinhloko wazo zonke lezi zindlela zokwelapha ukusiza amaphaphu engane ukuthi athole igazi nomoya-mpilo owanele .

Umuthi

• I-Prostaglandin isetshenziselwa ukusiza ukugcina i-ductus arteriosus yengane ivulekile. Le ductus arteriosus iyipayipi umntwana alisebenzisayo ukuthola igazi elinomoya-mpilo kumama esesibelethweni. Njengoba umntwana eqala ukuphefumula ngamaphaphu ngemva kokuzalwa, leli payipi livame ukuvala zingakapheli izinsuku ezimbalwa. Kodwa-ke, enganeni ene-HRHS, ngoba uhlangothi lwesokudla lwenhliziyo alusebenzi kahle, ukugcina leli payipi livulekile kusiza ukugeleza kwegazi emaphashini.

Izinqubo ezingangenisi kakhulu

• Udokotela angasebenzisa ithubhu elincane elibizwa ngokuthi i-catheter ukufaka i-stent (ithubhu elincane elifana ne-mesh yensimbi) ku-ductus arteriosus yengane. Lokhu kugcina ithubhu livulekile futhi kuvumela igazi ukuthi ligeleze kahle emaphashini.
• Okunye ukubhoboka kwevalvu yamaphaphu . Kulokhu, i-catheter idluliselwa emthanjeni wegazi iye kuvalvu yamaphaphu engavuli kahle, okudala imbobo kuvalvu. Lokhu kuvumela igazi ukuthi ligeleze liye emthanjeni wamaphaphu. Abanye odokotela basebenzisa nenqubo ebizwa ngokuthi i-ablation kulokhu.

Ukuhlinzwa

Amacala abucayi e-HRHS adinga ukuhlinzwa ukuze elashwe. Lokhu kuvame ukwenziwa ngezigaba eziningana.

• I-Blalock-Taussig-Thomas (BTT) shunt: Lokhu kusetshenziselwa ukubuyisela ukugeleza kwegazi emaphashini engane ene-HRHS. Kusebenzisa indwangu yokwenziwa ukudala uxhumano phakathi komthambo we-subclavian (umthambo ose-collarbone) nomthambo we-pulmonary.
• Ngemva kwezinyanga noma iminyaka embalwa, udokotela ohlinzayo angenza inqubo yeGlenn kanye/noma inqubo yeFontan . Ngayinye yalezi zinqubo idala indlela entsha yokuthi igazi ligeleze eduze kwe-ventricle yangakwesokudla engasebenzi kahle. Ngokuvamile, i-ventricle yangakwesokudla ipompa igazi emthanjeni wamaphaphu.

Izinqubo zikaGlenn noFontan zivumela igazi ukuthi ligeleze ngqo lisuka emthanjeni omkhulu liye emthanjeni wamaphaphu, lapho liya khona emaphashini ukuze lithathe umoya-mpilo.

Ingabe kukhona izinkinga ekwelashweni?

Njengakuzo zonke izinhlobo zokuhlinzwa, kunezinkinga ezithile ezingaba khona ngalezi zindlela zokwelapha. Izinkinga zingahluka kuye ngokwelashwa. Isibonelo:

• I-stent ingase isuke endaweni yayo yokuqala.
• Ukuhlinzwa kwe -BTT shunt kungabangela ukuthi imithambo yegazi inciphe noma yonakalise inzwa.
• Ukubhoboka kwevalvu yamaphaphu kungabangela ukushaya kwenhliziyo okungajwayelekile (i-arrhythmia) noma kulimaze umthambo wegazi.
• Ukuhlinzwa okukhulu kungabangela umfutho wegazi ophakeme, ukuphuma kwegazi emithanjeni yegazi, ukumiswa kwenhliziyo, isifo sezinso noma sesibindi, noma ukushaya kwenhliziyo okungajwayelekile.

Kodwa ungakhathazeki. Odokotela bazokwazisa ngakho konke lokhu futhi bathathe izinyathelo ezidingekayo.

Kuthatha isikhathi esingakanani ukuphulukiswa?

Isikhathi sokululama siyahlukahluka kuye ngokwelashwa. Ukuhlinzwa okukhulu kuthatha isikhathi eside kakhulu. Ingane yakho ingase ihlale esibhedlela izinsuku eziningana, mhlawumbe isikhathi esingaphezu kwesonto. Kuzodingeka iqashwe ngokucophelela ngemva kokubuyela ekhaya.

Yini engingayilindela uma ingane yami ikulesi simo?

Ngenxa yamazinga aphansi omoya-mpilo ekuqaleni kokuphila, abantu abane-hypoplastic right heart syndrome (HRHS) bangabhekana nezinselele ezifana nalezi:

• Izinkinga zolimi.
• Izinkinga zememori.
• Ingozi eyengeziwe yesifo senhliziyo, isibindi, noma izinso lapho umuntu ekhula.
• Izinkinga zemizwa noma izinkinga zokukhathazeka.
• Ubunzima bokuhlela okuthile noma ukuxazulula inkinga.

Kodwa, kukhona ochwepheshe abangasiza ngakho konke lokhu. Ngakho ungakhathazeki.

Iyini inkomba yalesi simo?

Akuvamile kakhulu ukuthi abantu abane-hyperplastic right heart syndrome (HRHS) baphile baze babe ngabantu abadala ngaphandle kokuhlinzwa . Kodwa-ke, abantu abane-HRHS encane bangase bangazi nokuthi banalesi simo baze babe ngabantu abadala.

Unakekela kanjani ingane ene-HRHS?

Ukunakekelwa ingane yakho ekudingayo kuzoncika ekutheni isimo sayo sibi kangakanani . Uma ihlinzwe ngokushesha ngemva kokuzalwa, kuzodingeka iqashwe ngokucophelela kakhulu ngemva kokufika ekhaya. Udokotela wakho noma umbelethisi wakho uzokunikeza iseluleko esihle sendlela yokunakekela ingane yakho.

Kunini lapho udinga ukubonana nodokotela?

Ingane eke yahlinzwa kuzodingeka ibone udokotela ukuze ithole ama-aphoyintimenti okulandelela . Bazokutshela ukuthi yini okufanele uyiqaphele nokuthi yini okufanele uyitshele udokotela ngokushesha uma ubona noma yini. Uma unemibuzo noma ukukhathazeka ngesimo sengane yakho, shayela udokotela ngokushesha.

Uma ingane yakho inezinkinga zokufunda noma ukulawula imizwa yayo, ungathola nabanye ochwepheshe abangasiza kulezo zindawo. Udokotela wakho uzokunikeza isiqondiso esidingekayo.

Yimiphi imibuzo okufanele ngiyibuze udokotela?

Nansi eminye imibuzo ongayibuza udokotela wengane yakho:

• Ingabe ingane yami idinga ukuhlinzwa?
• Kudingeka uhlinzwe isikhathi esingakanani?
• Zingaki ukuhlinzwa okuzodingeka, futhi kuzokwenziwa nini?
• Uyini umbono ngengane yami ngalesi simo esithile?

Ngiyaqonda ukuthi kunzima kangakanani ukuthola ukuthi ingane yakho inesifo senhliziyo. Kodwa-ke, izingane ezizalwa namuhla zinezindlela zokwelapha ezazingatholakali ezizukulwaneni ezedlule. Ungumuntu onamandla kakhulu ongaba yiwo enganeni yakho ngalesi sikhathi. Funda nge-HRHS. Buza udokotela wakho nganoma yini engacacile. Khuluma nodokotela wakho ukuze unqume ukuthi yini engcono kakhulu enganeni yakho.

Okokugcina, khumbula lokhu (Umyalezo Wokuya Ekhaya)

I-Hypoplastic Right Heart Syndrome (HRHS) yisimo esingavamile esizalwa naso izingane, lapho uhlangothi lwesokudla lwenhliziyo lungakhuli kahle. Lokhu kunciphisa inani lomoya-mpilo otholwa umzimba. Ukuxilongwa kusenesikhathi kanye nokwelashwa okufanele (imithi, ukuhlinzwa okuncane, noma ukuhlinzwa okukhulu) kungathuthukisa impilo yengane . Esimweni esinjalo, kubaluleke kakhulu kuwe njengomzali ukwaziswa nokusebenzisana nodokotela bakho. Khumbula, awuwedwa. Ungathola usizo oludingayo.

Isifo Senhliziyo Esingenasifo Sokunene, i-HRHS, Isifo Senhliziyo Sezingane Ezincane, Ukukhubazeka Kwenhliziyo Okuzelwe, i-Cyanosis, Ubunzima Bokuphefumula, Ukuhlinzwa Kwenhliziyo