I-Tralralogy ye-Fallot (TOF) iyindlela yokuzalwa yesifo socansi esithinta izingane ezingaba ngu-5 000 kuzo zonke izingane ezingu-10 000, ephethe cishe amaphesenti angu-10 azo zonke izifo zenhliziyo ezibangelwa ukufa. Ngenkathi i-TOF ihlale inkinga ebalulekile edinga ukwelashwa okuhlinzekwa, ukuqina kwayo kungahluka kancane. Ngezinye izikhathi kuveza izimpawu ezisongela impilo esanda kuzalwa, futhi kudinga ukwelashwa okuphuthumayo.
Kwezinye izimo, i-TOF kungenzeka ingabonakali izimpawu eziphawulekayo (futhi ingase ihlale ingatholakali) iminyaka eminingana. Kodwa ngokushesha noma ngokuhamba kwesikhathi, i-TOF idala izinkinga zomsongo esongela ukuphila, futhi idinga ukukhanda okuhlinzekwayo.
Kuyini iTetralogy of Fallot?
I-Tetralogy of Fallot iyinhlanganisela yeziphambeko ezine ze-anatomiki zenhliziyo, njengoba kuchazwe uDkt. Etienne-Louis Arthur Fallot ngo-1888. Yilezi:
- umthambo we-pulmonary stenosis (ukugxila komthambo we-pulmonary)
- i-ventalular septal defect (umgodi odongeni phakathi kwama-ventricle angakwesokudla nangakwesokunxele)
- ukuphambuka kwe-aorta kuya kwe-ventricle efanele
- i-ventricular hypertrophy (ukuqina kwe-muscle)
Abafundi bezokwelapha bavame ukwenza iphutha lokuzama ukuqonda i-TOF ngokukhanda lolu hlu lweziphambeko ezine. Ngenkathi benza kanjalo bangabasiza ukuba baphendule imibuzo yokuhlolwa, akusizi kakhulu ekuqondeni ukuthi i-TOF isebenza kanjani, noma kungani abantu abane-TOF behlukahluka kakhulu ezimpawu zabo.
Umsebenzi Wenhliziyo Ojwayelekile
Ukuze "uthole" i-TOF, kuyasiza ukuthi uqale ubuyekeze ukuthi inhliziyo isebenza kanjani ngokujwayelekile. ( Funda lapha mayelana namakamelo enhliziyo namagundvu .) I-oksijeni-empofu, igazi "elisetshenzisiwe" elivela kuwo wonke umzimba libuyela enhliziyweni ngokusebenzisa imithanjeni, futhi lingena ku-atrium elungile, bese i-ventricle efanele. I-ventricle efanele iphoqa igazi nge-artery yamapayipi emaphashini, lapho igcwaliswa khona nge-oxygen.
Igazi elikhona okwenziwe manje selibuyela enhliziyweni ngokusebenzisa imithanga ye-pulmonary futhi ingena kwi-atrium yesokunxele, bese-ke i-ventricle kwesokunxele. I-ventricle yangakwesokunxele (igumbi elikhulu lokuphambanisa) bese liphinda igazi le-oksijeni lingene emthini omkhulu (i-aorta) futhi ngaphandle komzimba. Futhi gcina engqondweni ukuthi ngokuvamile, ama-ventricle angakwesokudla nangakwesobunxele ahlukaniswa omunye komunye ngodonga lwe-muscular okuthiwa i-septum ye-ventricular.
Kungani iTetralogy of Fallot ibangela izinkinga
Ukuze uqonde i-TOF, udinga ngempela ukuqonda izici ezimbili (hhayi ezine) ezibalulekile zalesi simo. Okokuqala, ingxenye enkulu ye-septum ye-ventric ilahlekile ku-TOF (okuthiwa i-ventricular septal defect). Umphumela walesi siphambeko se-septal wukuthi ama-ventricle angakwesokunene nangakwesokunxele awaseziphathe njengamakamelo amabili ahlukene; Kunalokho, isebenza ngezinga elithile njenge-ventricle eyodwa enkulu. I-oksijeni-empofu igazi elibuya emzimbeni, futhi igazi elicebile oksijeni elibuya emaphashini, hlangana ndawonye kule ventricle.
Okwesibili, ngoba ukusebenza kukhona ngempela i-ventricle eyodwa kuphela, lapho lezo zinkontileka ze-ventricle, umthambo we-pulmonary kanye ne-aorta "ngokuncintisana" ngokugeleza kwegazi. Futhi ngenxa yokuthi ku-TOF kukhona inani elithile le-stenosis (ukunciphisa) umthambo we-pulmonary, i-aorta ngokuvamile ithola okungaphezulu kwesabelo sayo.
Uma uhlale ngokuchazwa lokhu, kufanele kube nomqondo kuwe ukuthi isihluthulelo ku-TOF yisilinganiso se-stenosis esivela emthini we-pulmonary. Uma kukhona ukuvinjelwa kwemithi yamapayipi enkulu kakhulu, khona-ke uma izinkontileka ze-ventricle "ezingabodwa," ingxenye enkulu yegazi eliphambanwe lizofaka i-aorta, futhi kancane kancane kuyofaka umthambo we-pulmonary. Lokhu kusho ukuthi igazi elincane kakhulu lifika emaphashini bese liba ne-oxygenated; igazi elijikelezayo liyi-oksijeni-empofu, isimo esiyingozi esibizwa ngokuthi i- cyanosis . Ngakho-ke, amacala obukhulu kunawo wonke we-TOF yilawo angaphansi kwe-pulmonary artery stenosis.
Uma i-pulmonary artery stenosis ayinzima kakhulu, khona-ke inani elilinganiselwe legazi liyaphonsa emaphashini bese liba yi-oxygenated. Laba bantu bane-cyanosis encane, futhi ukuba khona kwe-TOF kungase kungabikho ngesikhathi sokuzalwa.
Isici esichazwe kahle se-TOF, esabonwa ezinganeni eziningi ezinalesi simo, ukuthi izinga le-armonary artery stenosis lingashintsha. Kulezi zimo, i-cyanosis ingafika futhi ihambe. Iziqephu ze-cyanotic zingase zenzeke, isibonelo, lapho ingane ene-TOF iqala ukukhala noma iqala ukukhala, noma lapho ingane ekhulile ene-TOF iqhuba. Lezi "spells" ze-cyanosis ngokuvamile zibizwa ngokuthi "i-tet spell" noma izinhlamvu ze-hypercyanotic. Lezi zinhlobo ze-cyanosis zingase zibe nzima kakhulu, futhi zingadinga ukwelashwa okuphuthumayo.
Izimpawu ze-Tetralogy of Fallot
Izimpawu ikakhulukazi zithembele, njengoba sibonile, ngezinga lokuvimbela elikhona emthonjeni we-pulmonary. Uma ukuvimbela umthambo we-pulmonary fixed is severe, i-cyanosis ejulile ibonakala ekusaneni (isimo esasibizwa ngokuthi "ingane eluhlaza okwesibhakabhaka"). Lezi zinsana zinezinkinga eziphuthumayo ngokushesha, futhi zidinga ukwelashwa okuphuthumayo.
Izingane ezinokuvinjelwa ngokulinganayo komthambo we-pulmonary zivame ukutholakala esikhathini esizayo. Udokotela angase azwe inhliziyo ikhononda futhi ihlelwe ukuhlolwa; noma abazali bangase babone izinhlamvu ze-hypercyanotic lapho ingane ikhungatheka. Ezinye izimpawu ze-TOF zingabandakanya ukugula okunzima, ukwehluleka ukuthuthukisa ngokuvamile, kanye ne- dyspnea .
Izingane ezikhulile ne-TOF zivame ukufundela ukugcoba izimpawu zabo. Ukwehliswa kwegazi kukhulisa ukumelana emithanjeni yegazi, enomphumela wokwenza ukuphikisana nokugeleza kwegazi ku-aorta, ngaleyo ndlela iqondisa ngaphezulu kwegazi le-cardiac ekujikelezeni kwe-pulmonary. Lokhu kunciphisa i-cyanosis kubantu abane-TOF. Ngezinye izikhathi izingane ezine-TOF ziqala ukuxilongwa lapho abazali bazo bekhuluma ngokugqekeza kwabo odokotela njalo.
Izimpawu ze-TOF-cyanosis, ukubekezela ngokweqile kokuzivocavoca umzimba, ukukhathala, kanye ne-dyspnea-zivama ukwedlula isikhathi. Esikhathini esiningi sabantu abane-TOF, ukuxilongwa kuyenziwa ngesikhathi sokukhula ngisho noma kungabonakali ngesikhathi sokuzalwa.
Kulabo bantu abafake i-TOF kuphela emithonjeni encane ye-pulmonary artery stenosis, izinhlamvu ze-cyanosis kungenzeka ukuthi azikho nhlobo, futhi iminyaka ingadlulela ngaphambi kokuba kutholakale ukuxilongwa. Ngezinye izikhathi, i-TOF kungenzeka ingatholakali kuze kube yilapho ikhulile. Naphezu kokuntuleka kwe-cyanosis ejulile, laba bantu basadingeka baphathwe, ngoba bavame ukuhlakulela ukuhluleka kwenhliziyo ngokukhula kwabantu abadala.
Yini Ebangelwa I-Tetralogy of Fallot?
Njengoba kunjalo ngezinhlobo eziningi zesifo senhliziyo esiswini, imbangela ye-TOF ayaziwa. I-TOF ivela ngemvamisa ephakeme ezinganeni ezine- Down syndrome futhi ezinye ezinye izinto ezingavamile. Kodwa-ke, ukubukeka kwe-TOF cishe kuyinto engavamile kakhulu futhi akuzukuzuzwa njengefa. I-TOF nayo ihlotshaniswe no-rubella womama, ukudla okunomsoco omama noma ukusetshenziswa kotshwala, kanye neminyaka yobudala engama-40 noma ngaphezulu. Kodwa-ke, isikhathi esiningi, akekho kulezi zici eziyingozi lapho kuzalwa ingane ene-TOF.
Ukuthola iTetralogy of Fallot
Uma sekusolakala ukuthi inkinga yenhliziyo, ukuxilongwa kwe-TOF kungenziwa nge- echocardiogram noma i- MRI yesifo senhliziyo , okuzobe kubonisa ukuthi i-cardiac anatomy engavamile. I- catheterization ye-cardiac ivame ukusiza ekucaciseni isisu senhliziyo ngaphambi kokukhanda.
Ukwelapha i-Tetralogy ye-Fallot
Ukwelashwa kwe-TOF kuhlinzekwa. Umkhuba wamanje ukwenza ukwelashwa okulungiswa phakathi nonyaka wokuqala wokuphila, cishe phakathi kwezinyanga ezintathu nezinyanga eziyisithupha. "Ukuhlinzwa okulungiswayo" ku-TOF kusho ukuvala i-septal defect defect (ngaleyo ndlela ehlukanisa uhlangothi lwesokudla senhliziyo ukusuka ohlangothini lwesobunxele senhliziyo) nokunciphisa ukuvimbela umthambo we-pulmonary. Uma lezi zinto ezimbili zingagcwaliseka, igazi eligeleza ngaphakathi kwenhliziyo lingasetshenziswa ngokujwayelekile.
Ezimweni lapho ukucindezeleka okukhulu kukhona ekuzalweni, kufanele kwenziwe ukuhlinzwa okunomsoco, ukuqinisa umntwana kuze kube yilapho eqina ngokwanele ukuhlinzwa okulungiswayo. Ukuhlinzwa okunamaphilisi ngokuvamile kuhilela ukwenza i-shunt phakathi kweyodwa yemizila ye-systemic (ngokuvamile i-artery subclavia) kanye neminye imithwalo ye-pulmonary, yokubuyisela inani elithile lokujikeleza kwegazi emaphashini.
Kubantu abadala asebekhulile abanokutholakala ne-TOF, ukuhlinzwa okulungiswayo kuyaphakanyiswa, nakuba ingozi yokuhlinzwa iphakeme kubantu asebekhulile kunabantwana abancane.
Ngezindlela zesimanje, ukuhlinzwa okulungiswa kwe-TOF kungenziwa ngeengozi yokufa kwamaphesenti angu-0 kuya kwangu-3 kuphela ezinganeni nasezinganeni. Inani lokufa lokuhlinzeka kwe-TOF ukulungiswa kubantu abadala, noma kunjalo, lingaba ngaphezu kuka-10. Ngenhlanhla kuyinto engavamile kakhulu ukuba i-TOF "ilahleke" namhlanje ibe umuntu omdala.
Uyini Umphumela Wesikhathi Esivamile We-Tetralogy of Fallot?
Ngaphandle kokulungiswa kokuhlinzwa, cishe isigamu sabantu abane-TOF bafa phakathi neminyaka embalwa yokuzalwa, futhi bambalwa kakhulu (ngisho nalabo abanamafomu "obunzima").
Ngemuva kokuhlinzwa kokuqala kokulungisa, ukusinda isikhathi eside manje sekuhle kakhulu. Iningi labantu abane-TOF elungisiwe lisinda kahle baze babe ngabantu abadala. Ngoba amasu okuhlinzeka okwamanje anamashumi ambalwa eminyaka ubudala, asisazi ukuthi ukusinda kwabo kokugcina kuzoba yini. Kodwa kuyavame ukuba izazi ze-cardiologists zibone iziguli ezine-TOF emashumini eminyaka ayisithupha namashumi ayisikhombisa okuphila.
Noma kunjalo, izinkinga zenhliziyo zivame kakhulu kubantu abadala abane-TOF elungisiwe. I-valve yamapayipi yokuphindaphinda , ukuhluleka kwenhliziyo, kanye ne- arrhythmias ye-cardiac (ikakhulukazi i-trialycardia ye-atrial kanye ne- ventricular tachycardia ) yizinkinga ezivame ukwenzeka lapho iminyaka ihamba. Ngenxa yalesi sizathu, noma ubani oye waqondisa TOF kudingeka abe ngaphansi kwengane yomzimba ongawahlola njalo, ukuze noma yiziphi izinkinga zenhliziyo ezithuthuka ngokuhamba kwesikhathi zingasetshenziswa ngokuhlukunyezwa.
Izwi elivela
Ngokwelashwa kwanamuhla, i-tetralogy ye-Fallot iye yaguqulwa kusukela esimweni senhliziyo esiswini esivame ukuholela ekufeni ngesikhathi seyingane, enkingeni enkulu eqondakalayo, ehambisana nokunakekelwa kwezempilo okuhle nokuqhubekayo, iyahambisana nokuphila okungenani kuze kube sekupheleni komuntu osekhulile. Namuhla, abazali bezingane ezalwa nalesi simo banesizathu sokulindela ukuthi bajabulele izinhlobo eziningi zenjabulo nenhliziyo ebuhlungu abangayilindela nanoma yimuphi omunye ingane.
> Imithombo:
> Khairy P, Aboulhosn J, Gurvitz MZ, et al. I-Arrhythmia Burden in Adult nge-Rurgically yalungisa i-Tetralogy ye-Fallot: Isifundo se-Multi-Institution. Ukujikeleza ngo-2010; 122: 868.
> Park CS, Lee JR, Lim HG, et al. Imiphumela Yesikhathi eside Yokwenza Ukulungiswa Kwempahla Yonke Ye-Tetralogy ye-Fallot. I-Eur J Cardiothorac Surg 2010; 38: 311.
> Ixwayisa i-CA, i-Williams RG, iBashore TM, et al. ACC / AHA 2008 Izinkombandlela Zokuphathwa Kwabantu Abadala abanezifo Zenhliziyo Ebandayo: Umbiko we-American College of Cardiology / American Heart Association Task Force on Guidelines Practice (Ikomidi Lokubhala Ukuthuthukisa Imihlahlandlela Yokuphathwa Kwabantu Abadala Ngezifo Zenhliziyo Ebandayo). Kuthuthukiswa Ekuhlanganyeleni Ne-American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, kanye neNhlangano Yabahlengikazi BamaTrirac. J Am Coll Cardiol 2008; 52: e143.