Ingabe ingane yakho inomthambo enhliziyweni yayo ofana nenkemba? Ake sifunde ngalesi simo esingavamile (Scimitar Syndrome)!

"O, ingane yethu encane ibonakala inenkinga yokuphefumula, iyaphefumula ngisho nalapho incelisa... ngezinye izikhathi isikhumba sayo sibukeka siluhlaza okwesibhakabhaka..." Wake wabhekana nento efana nale? Noma wake wakubona lokhu kwenzeka enganeni yomuntu omaziyo? Ngezinye izikhathi lezi zimpawu zingabangelwa yizinkinga ezithile zenhliziyo izingane ezizalwa nazo. Namuhla sizokhuluma ngesimo senhliziyo esingavamile, kodwa esibalulekile okufanele sazi ngaso.

Iyini i-Scimitar Syndrome?

Lokhu kubizwa ngokuthi i-Scimitar Syndrome . Leli gama lizwakala lingavamile, akunjalo? 'I-Scimitar' ibhekisela enkembeni enesihloko esigobile, eyayisetshenziswa ezikhathini zasendulo eMpumalanga Ephakathi. Kulesi simo, ngezinye izikhathi umthambo wamaphaphu, othwala igazi lisuka ephashini lesokudla uliyise enhliziyweni, uvela ngesimo senkemba egobile kuma-X-ray. Akuyona yini into ethakazelisayo ukuthi leli gama lavela kanjani?

Kalula nje, lokhu kuyisifo senhliziyo esizalwa naso . Okusho ukuthi, isimo esenzeka ingane isesesibelethweni. Odokotela babiza lokhu ngokuthi uhlobo lwesimo esibizwa ngokuthi i-Partial Anomalous Pulmonary Venous Return (PAPVR). Okusho ukuthi, kukhona isici endleleni imithambo yegazi evela emaphashini exhumana ngayo nenhliziyo.

Kunezimpawu eziningana eziyinhloko ezingabonakala kulesi simo:

• Iphaphu langakwesokudla alikakhuli ngokugcwele (iphaphu langakwesokudla eliyi-hypoplastic). Lokhu kusho ukuthi lincane futhi alisebenzi kahle kunephaphu elikwelinye icala.
• Umthambo oyinhloko othwala igazi uye ephashini elingakwesokudla (umthambo wamaphaphu ongakwesokudla) awukhuli kahle (umthambo wamaphaphu ongakwesokudla ongaphansi kwe-hypoplastic). Lokhu kunganciphisa nenani legazi elifinyelela ephashini elingakwesokudla.

Singakuqonda okuncane ngokusebenza okumangalisayo kwezinhliziyo zethu?

Ngakho-ke, ukuze siqonde ngempela ukuthi kwenzekani nge-Scimitar Syndrome, ake siqale sibheke ukuthi inhliziyo enempilo isebenza kanjani, akunjalo? Cabanga ngenhliziyo yethu njengephampu esebenza kahle kakhulu. Iphampa igazi kuwo wonke umzimba wethu, imini nobusuku.

Yilokhu okuvame ukwenzeka:

1. Igazi elisetshenzisiwe, elingenawo umoya-mpilo okwanele elivela kuwo wonke umzimba liyaqoqwa bese libuyiselwa ohlangothini lwesokudla lwenhliziyo.

2. Leli gazi elingenawo umoya-mpilo elivela ohlangothini lwesokudla lwenhliziyo lithunyelwa emaphashini, ngemithambo yamaphaphu .

3. Ngaphakathi emaphashini, leli gazi 'ligcwele umoya-mpilo', njengoba nje amanzi angcolile ehlungwa futhi ehlanzwa.

4. Bese, leli gazi elinomoya-mpilo libuyela ohlangothini lwesobunxele lwenhliziyo ngemithambo yamaphaphu, okuyimithambo ethwala umoya-mpilo usuka emaphashini uye enhliziyweni .

5. Uhlangothi lwesobunxele lwenhliziyo lupompa leli gazi elinomoya-mpilo liye emzimbeni wonke, ngomthambo wegazi omkhulu kunayo yonke , i-aorta .

Lena yindlela iseli ngalinye emzimbeni wethu elithola ngayo umoya-mpilo. Ingabe akuyona inqubo eyinkimbinkimbi kakhulu futhi emangalisayo le?

Ngakho-ke kwenzekani ku-Scimitar Syndrome?

Manje cishe usuyaqonda ukuthi inhliziyo enempilo isebenza kanjani. Okwenzekayo kumuntu one-Scimitar Syndrome ukuthi igazi okufanele liphume emaphashini ukuze liqoqe umoya-mpilo liye ohlangothini lwesobunxele lwenhliziyo, esikhundleni sokuza ngaleyo ndlela , liya ngendlela engafanele bese liya ohlangothini lwesokudla lwenhliziyo.

Cabanga nje uma uxhume kabusha ipayipi eliletha amanzi endlini endaweni engafanele, mhlawumbe ethangini lamanzi elisetshenzisiwe. Khona-ke okufanele kwenzeke ngeke kwenzeke ngendlela efanele. Kungavela isimo lapho amanzi ahlanzekile namanzi angcolile kuhlanganiswa khona.

Kulokhu, imithambo eyodwa noma ngaphezulu (imithambo yamaphaphu) evame ukuthwala igazi elinomoya-mpilo lisuka ephashini lesokudla ixhuma ohlangothini lwesokudla lwenhliziyo esikhundleni sohlangothi lwesobunxele. Lokhu kuvumela elinye igazi elinomoya-mpilo ukuthi ligeleze libuyele emaphashini. Kwenza nohlangothi lwesokudla lwenhliziyo lusebenze kanzima.

Ngezinye izikhathi, iphaphu langakwesokudla alitholi igazi emithanjeni yamaphaphu , kodwa livela ngqo emithanjeni yamaphaphu . I-aorta empeleni iwumthambo wegazi oyinhloko othwala igazi liye emzimbeni wonke. Ngakho-ke kunenkinga encane lapho. Lokhu kungabeka ukucindezeleka okungadingekile enhliziyweni.

Ingabe lesi simo sivame kangakanani? Ingabe kufanele sesabe ngokungadingekile?

Lokhu kungase kuzwakale kusabisa kuwe, kodwa i-Scimitar Syndrome iyisimo esingavamile kakhulu. Ngokwezibalo, ithinta ingane eyodwa kuya kwezintathu kuphela kwabayi-100,000. Kutholakala ukuthi ivame kabili emantombazaneni kunabafana.

Ngakho-ke, ungesabi ngokungadingekile, ngoba lokhu akuvamile. Kodwa-ke, kubaluleke kakhulu ukuqaphela lokhu.

Yiziphi izizathu zalokhu? Kungani lokhu kwenzeka?

Ngokuvamile, ngisho nodokotela banenkinga yokuthola imbangela eqondile ye-Scimitar Syndrome. Kodwa-ke, kunezici ezithile eziyingozi ezingandisa ingozi yengane yokuba nesifo senhliziyo esizalwa naso. Lezi zifaka:

• Izakhi zofuzo: Ngezinye izikhathi izimo zezakhi zofuzo ezidluliselwa ezizukulwaneni zingaba nomthelela.
• Eminye imithi ethathwa ngesikhathi sokukhulelwa: Isibonelo, eminye imithi yokuvuvukala kwezinduna efana ne-`(Isotretinoin - Accutane®)` ingaba yingozi. Yingakho kunconywa ukuthi ungaphuzi noma yimuphi umuthi ngesikhathi sokukhulelwa ngaphandle kweseluleko sikadokotela.
• Izici zemvelo: Uma umama ebhema noma ephuza utshwala ngesikhathi sokukhulelwa, izinto ezinjalo zingathinta ukukhula kwenhliziyo yomntwana.
• Ukutheleleka okuthile kumama: Isibonelo, uma umama ethola ukutheleleka okufana ne-rubella ngesikhathi sokukhulelwa, kungathinta ukukhula kwenhliziyo yengane.
• Izifo ezingamahlalakhona zikamama ezingalawuliwe: Uma izifo ezifana nesifo sikashukela zingalawulwa kahle ngesikhathi sokukhulelwa, zingathinta nengane.

Into ebalulekile ukuthi, isikhathi esiningi, lokhu akulona iphutha lomuntu. Ngakho ungazibeki icala.

Yiziphi izimpawu ezikhona?

Izimpawu ze-Scimitar Syndrome azifani kuwo wonke umuntu. Ezinye izingane zingase zibonise izimpawu ngokushesha nje ngemva kokuzalwa . Izinto okufanele uziqaphele ngalezo zikhathi zifaka:

• Ubunzima bokuphefumula nokuminyana ngesikhathi uncelisa.
• Ukushintsha kombala wesikhumba nezindebe okuluhlaza okwesibhakabhaka/okumpunga (cyanosis): Lokhu kubangelwa ukungatholi umoya-mpilo owanele emzimbeni.
• Ukuphefumula okusheshayo.
• Ukuvuvukala (i-edema) emilenzeni, esiswini, nasemaceleni kwamehlo.

Kodwa-ke, ezinye izingane zingase zingabonisi zimpawu kuze kube yilapho sezikhulile. Ezinye zingase zihlale zingenazo izimpawu zize zibe sezikhulile. Kodwa-ke, uma izimpawu zivela lapho sezikhulile, zingase zibe nalokhu okulandelayo:

• Ukutheleleka kwamaphaphu okuvamile.
• Ubunzima bokuphefumula (ukuphelelwa umoya) ngesikhathi sokuzivocavoca noma ukuzikhandla.

Odokotela bakuthola kanjani lokhu? (Ukuxilongwa)

Odokotela banezivivinyo eziningana zokusiza ekuxilongeni i-Scimitar Syndrome. Lokhu kungabheka izinto ezifana nosayizi wephaphu elingakwesokudla enganeni noma kumuntu omdala kanye nesimo somthambo wamaphaphu ongakwesokudla.

• I-X-ray yesifuba: Lokhu kunganikeza umbono oqondile wenhliziyo, amaphaphu, izindlela zokuphefumula, kanye nemithambo yegazi. Ungase ubone ukuma 'kweScimitar' kulokhu.
• I-CT scan: Lokhu kuthatha ama-X-ray kusuka ema-engeli ahlukene ukudala izithombe ezinemininingwane ezintathu zangaphakathi lomzimba.
• I-MR angiography: Lolu uhlobo lokuhlola olusebenzisa amaza kazibuthe kanye nekhompyutha ukuze kubhekwe imithambo yegazi ngokuningiliziwe.
• I-Transthoracic echocardiogram (TTE): Lokhu kufana nokuskena kwe-ultrasound. Kungabheka eduze ukusebenza kwenhliziyo, amakamelo, ama-valve, kanye nokugeleza kwegazi.
• Ukufakwa kwe-catheter kwenhliziyo: Kule nqubo, kufakwa idayi ekhethekile emithanjeni yegazi bese kuthathwa i-X-ray ukuze kubonwe ukuthi igazi ligeleza kanjani emithanjeni. Iphinde isetshenziswe ukukala ingcindezi enhliziyweni nasemaphashini.

Ungesabi ukubuza ngalezi zivivinyo. Odokotela benza lokhu ukuze baxilonge kahle isimo futhi banikeze ukwelashwa okungcono kakhulu.

Ingabe ikhona indlela yokwelapha lokhu? (Izindlela zokwelapha)

Yebo, ukwelashwa okuyinhloko kwe-Scimitar Syndrome ukuhlinzwa.

• Uma odokotela bebona lesi simo, bavame ukulingeka ukuba benze ukuhlinzwa ezinyangeni ezimbili zokuqala zokuphila . Ngoba uma lesi sici silungiswa ngokushesha, kungcono ukukhula kwengane kanye nempilo yayo yesikhathi esizayo.
• Kwabantu abadala,Akuwona wonke umuntu one-Scimitar Syndrome ozodinga ukuhlinzwa ngaphandle kokuthi abe nezimpawu ezimbi kakhulu (isib. ukutheleleka kwamaphaphu okuvamile, ukuphelelwa umoya okukhulu). Kodwa-ke, uma izimpawu zimbi kakhulu, kungase kudingeke ukuhlinzwa.

Kunezinto eziningana udokotela ohlinza inhliziyo angayenza ngesikhathi sokuhlinzwa:

• Umthambo wamaphaphu, oxhumene endaweni engafanele , udluliselwa ohlangothini lwesobunxele lwenhliziyo. Ngezinye izikhathi kusetshenziswa ama-patches akhethekile kulokhu.
• Ngaphandle kwalokho, umthambo ufakwa ngqo ohlangothini lwesobunxele lwenhliziyo.
• Akuvamile kakhulu, ukuhlinzwa kwe-pneumonectomy kungenziwa ukuze kususwe ngokuphelele iphaphu langakwesokudla elingakhuli kahle futhi elingasebenzi kahle. Kodwa-ke, lokhu kwenziwa kuphela uma kungekho enye indlela.

Ingabe ikhona indlela esingakuvimbela ngayo lokhu?

Eqinisweni, ayikho indlela yokuvimbela ngokuphelele ukukhubazeka kwenhliziyo okuzalwe nakho njenge-Scimitar Syndrome, njengoba kuthonywa futhi yizici zofuzo.

Kodwa-ke, kunezinto umama angazenza ngesikhathi sokukhulelwa ukuze andise amathuba akhe okuba nomntwana onempilo:

• Gwema ngokuphelele utshwala, ugwayi, nezinye izidakamizwa ngesikhathi sokukhulelwa.
• Ngaphambi kokuthatha noma yimuphi umuthi, khuluma nodokotela wakho ngezingozi nezinzuzo zawo.
• Uma ungakawutholi umuthi wokugomela i-rubella , kubalulekile ukuwuthola ngaphambi kokuba ukhulelwe, njengoba ukuthola i-rubella ngesikhathi sokukhulelwa kungathinta inhliziyo yengane.
• Uma unezifo ezingamahlalakhona njengesifo sikashukela , zilawule kahle ngesikhathi sokukhulelwa.
• Thatha i-folic acid kanye namanye amavithamini ngaphambi kokubeletha njengoba kuqondiswe udokotela wakho.

Impilo izoba njani ngalesi simo? (Umbono wesikhathi esizayo)

Abantu abahlinzwa i-Scimitar Syndrome bavame ukuba nemiphumela emihle kakhulu yesikhathi eside . Iningi labantu aliphinde libhekane nezimpawu ngemva kokuhlinzwa.

Kodwa-ke, ngezinye izikhathi, ikakhulukazi ezinganeni nasezinganeni ezincane, umthambo wamaphaphu odluliselwe kwenye indlela ungaba mncane. Uma lokhu kwenzeka, kungadingeka ukwelashwa okwengeziwe.

Abanye abantu abadala abane-Scimitar Syndrome bangaphila impilo enempilo ngaphandle kwezimpawu. Kodwa-ke, kwabanye abantu abadala, lesi simo singaholela kwezinye izinkinga zenhliziyo. Ezimweni ezinjalo, ikhwalithi yokuphila ingathinteka. Uma kuvela izimpawu (njengokuphelelwa umoya okukhulu, ukutheleleka kwamaphaphu), kungadingeka ukuhlinzwa. Kungadingeka futhi ukuthi wenze izivivinyo ezikhethekile ukuze kuhlolwe umfutho wegazi ophezulu emithanjeni yamaphaphu akho.

Zikhona yini ezinye izifo zenhliziyo ezihlobene ne-Scimitar Syndrome?

Yebo, abantu abaningi abane-Scimitar Syndrome bangase babe nezinye izinkinga zenhliziyo nemithambo yegazi. Abanye bazalwa benokukhubazeka okuningi kwenhliziyo. Noma, i-Scimitar Syndrome ingabangela ezinye izinkinga.

Ezinye izimo ezingase zihlotshaniswe zihlanganisa:

• I-Atrial Septal Defect (ASD): Imbobo ephakathi kwamakamelo amabili aphezulu enhliziyo.
• Iphaphu le-Horseshoe: Ukuxhumana okungavamile phakathi kwezingxenye ezimbili zephaphu.
• Umfutho wegazi ophezulu wamaphaphu: Umfutho wegazi ophezulu emithanjeni yamaphaphu.
• Ukususwa kwamaphaphu (iphaphu elihambisanayo): Ingxenye yezicubu zamaphaphu engasebenzi kahle, ehlukanisiwe enegazi elingavamile.
• I-Tetralogy of Fallot: Isifo senhliziyo esivamile esidinga ukwelashwa ukuze kupompelwe igazi emaphashini ezinganeni.
• I-Ventricular Septal Defect (VSD): Imbobo ephakathi kwamakamelo amabili aphansi okupompa enhliziyo.

Ukuzwa lezi zinto kungase kwesabise nakakhulu. Kodwa-ke, kunezindlela zokwelapha zonke lezi zimo. Into ebaluleke kakhulu ukukhuluma nodokotela wakho, uqonde isimo sakho esiqondile, bese uthola ukwelashwa okudingayo.

Imibuzo ebalulekile okufanele uyibuze udokotela wakho

Uma uthola ukuthi wena noma ingane yakho ine-Scimitar Syndrome, ungabuza udokotela wakho imibuzo efana nale:

• Iyini imbangela engaba khona kakhulu ye-Scimitar Syndrome?
• Ingabe mina/ingane yami sidinga ukuhlinzwa?
• Kwenzekani uma ungahlinzwanga?
• Ungayiphatha kanjani impilo yenhliziyo nge-Scimitar Syndrome?
• Uma nginalesi simo, mangakanani amathuba okuba nengane yami nayo ibe naso?
• Uma nginengane yokuqala ene-Scimitar Syndrome, mangakanani amathuba okuba nengane yami yesibili nayo ibe nayo?

Ungesabi noma ungabi namahloni okubuza le mibuzo. Kubaluleke kakhulu ukususa konke ukungabaza engqondweni yakho.

Okokugcina, izinto okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

I-Scimitar Syndrome iyisifo senhliziyo esingavamile, esizalwa naso esithinta kakhulu iphaphu langakwesokudla kanye nemithambo yegazi elixhumanisa namaphaphu.

Ezinye izingane zingase zingabonisi zimpawu nhlobo, kanti ezinye zingase zidinge ukuhlinzwa zingakapheli izinyanga ezimbili zokuqala zokuphila.

Izinsana ezidinga ukuhlinzwa kusenesikhathi zingase zidinge ukwelashwa okwengeziwe kamuva ngoba umthambo wegazi ophambukile uyancipha. Kodwa-ke, abantu abadala nezingane abaningi abahlinzwa i-Scimitar Syndrome baphila impilo enhle kakhulu futhi baphila impilo evamile.

Sithemba ukuthi lolu lwazi luzokusiza. Uma uneminye imibuzo mayelana nalokhu, qiniseka ukuthi ukhuluma nodokotela wakho. Hlala uphilile!

'I-Scimitar Syndrome, isifo senhliziyo, ukukhubazeka kokuzalwa, amaphaphu, ukujikeleza kwegazi, izimpawu zezingane, ukuhlinzwa