I-Tetralogy ye-Fallot (TOF) yindlela yesifo senhliziyo esiswini esithintelayo malunga nantlanu kwiintsana ezili-10 000, esabiza malunga neepesenti ezili-10 zonke izifo zesifo senhliziyo. Nangona i-TOF ihlala iyingxaki ebalulekileyo efuna unyango otyando, ubunzima bayo buyahlukahluka kakhulu. Ngamanye amaxesha kuvelisa iimpawu ezisongela ubomi ebusana, kwaye kufuna unyango oluphuthumayo.
Kwezinye iimeko, i-TOF ingenakho ukuvelisa iimpawu ezibalulekileyo (kwaye ingahlala ingaziwa) iminyaka emininzi. Kodwa ngokukhawuleza okanye kamva, i-TOF ihlala idala iingxaki zengozi yokuphila, kwaye idinga ukulungiswa kokuhlinzwa.
Yintoni iTetralogy yobuxoki?
I-Tetralogy of Fallot iyintlangano yesine i-astrocyat defects, njengoko kuchazwe nguDkt. Etienne-Louis Arthur Fallot ngo-1888. Zizo:
- i-pulmonary artery stenosis (ukunciphisa umthambo we-pulmonary)
- siphene esikhulu se-septal (isango eludongeni phakathi kwama-ventricle asekunene nakwesobunxele)
- ukuphambuka kwe-aorta ukuya kwi-ventricle efanelekileyo
- i-hypertrophy (i-muscle thickening)
Abafundi bezonyango bahlala benza impazamo yokuzama ukuqonda i-TOF ngokukhumbula ngeloluhlu lweziphene ezine. Ngoxa bekwenza njalo kunokubanceda baphendule imibuzo yokuvavanya, akubancedi kakhulu ekuqondeni indlela i-TOF isebenza ngayo, okanye kutheni abantu abane-TOF banokungafani kakhulu kwiimpawu zabo.
Umsebenzi oqhelekileyo wenhliziyo
Ukuze "ufumane" i-TOF, kukulungele kuqala ukuphonononga indlela intliziyo isebenza ngayo ngokuqhelekileyo. ( Funda apha malunga neenkamelo zentliziyo kunye neepavini .) I-oksijeni-ihlwempu, "isetyenziswe" igazi ukusuka kuwo wonke umzimba uphindela entliziyweni ngokusebenzisa imithanjeni, kwaye ingena kwi-atrium efanelekileyo, kwaye i-ventricle efanelekileyo. I-ventricle efanelekileyo igxotha igazi nge-pulmonary umthambo kwimiphunga, apho izaliswa nge-oxygen.
Igazi langoku-oksijini libuyela entliziyweni ngeemvumba zeplmonta kwaye lingene kwi-atrium ekhohlo, kwaye ke i-ventricle ekhohlo. I-ventricle engasekhohlo (igumbi eliphambili lokupompa) liphompoze igazi le-oksijeni kwi-artery ephambili (i-aorta) kwaye yaya emzimbeni. Kwakhona ugcine engqondweni ukuba ngokuqhelekileyo, i-ventricle efanelekileyo kunye nekhohlo iyahlukana komnye ngodonga lwe-muscular olubizwa ngokuba yi-septum ye-ventricular.
Kutheni iTetralogy of Fallot ibangela iingxaki
Ukuze uqonde i-TOF, ufuna ngokwenene ukuqonda kuphela ezimbini (ezingekho ezine) ezibalulekileyo ezi zinto zibalulekileyo. Okokuqala, inxalenye enkulu ye-septum ye-ventum ilahlekile kwi-TOF (into ebizwa ngokuthi yi-ventricular defal defect). Isiphumo sale siphene se-septal kukuba i-ventricles efanelekileyo neyekhohlo ayisekho njengamagumbi ahlukeneyo amabini; Kunoko, zisebenza kwinqanaba elithile njenge-ventricle enkulu. Igazi le-oksijini-elibi libuya emzimbeni, kunye negazi elityebileyo legazi elibuya kwimiphophu, xubana kunye nale ventricle.
Okwesibini, ngenxa yokuba kusetyenziswe khona i-ventricle enkulu kuphela, xa loo mvumelwano ye-ventricle, i-pulmonary artery kunye ne-aorta "ngokukhawuleza" yokuphuma kwegazi. Kwaye kuba ku-TOF kukho inani elithile le-stenosis (ukunciphisa) umthambo we-pulmonary, i-aorta ivame ukufumana ngaphezulu kwesabelo sayo.
Ukuba uthe wahlala nale nkcazo ngoku, kufuneka ube nengqiqo kuwe ukuba isitshixo ku-TOF yinqanaba le-stenosis ekhoyo kwi-pulmonary artery. Ukuba kukho umda omkhulu we-pulmonary obstruction, ngoko ke xa iivumelwano ze-ventricle "ezingatshatanga", inxalenye enkulu yegazi elipompile liza kungena kwi-aorta, kwaye ingancinci iya kufaka umthambo we-pulmonary. Oku kuthetha ukuba igazi elincinci lifikelela kumaphaphu kwaye liba yomoya-mpilo; Igazi elijikelezayo yilapho i-oxygen-ihlwempu, imeko eyingozi eyaziwa ngokuba yi- cyanosis . Ngoko, iimeko ezimbi kakhulu ze-TOF zizo ziphi i-pulmonary artery stenosis.
Ukuba i-pulmonary artery stenosis ayinamandla kakhulu, ngoko ke inani elithile elifanelekileyo legazi liye linyanyiswe kwimiphunga kwaye liba yomoya. Aba bantu bane-cyanosis encinci, kwaye ubukho be-TOF buya kuphinda bukholelwe ekuzalweni.
Inkalo echazwe kakuhle ye-TOF, ebonwa kubantwana abaninzi abaneli meko, kukuba iqondo le-pulmonary artery stenosis lingashintsha. Kule meko, i-cyanosis ifike kwaye ihambe. Iziqwenga ze-cyanotic zenzeka, umzekelo, xa umntwana okhuselekileyo ene-TOF eqala ukukhala okanye eqala ukukhala, okanye xa umntwana osekhulile ene-TOF. Ezi "spell" ze-cyanosis zidla ngokuba zibizwa ngokuthi "i-tet spell" okanye i-hypercyanotic spells. Ezi zihlunu ze-cyanosis ziba nzima, kwaye zifuna unyango lwezonyango.
Iimpawu zeTetralogy of Fallot
Iimpawu zixhomekeka kakhulu, njengokuba sibonile, kwinqanaba lomqobo okhoyo kwi-pulmonary artery. Xa i-pulmary pulterary obstruction ishintshi, i-cyanosis ebonakalayo ibonakala kwintsana (imeko ebizwa ngokuba "ngumntwana ohlaza okwesibhakabhaka"). Ezi ntsana zinexinzelelo olukhawulezayo kwaye lifuna unyango oluphuthumayo.
Abantwana abanomqobo othotyelwayo we-pulmonary artery bahlala bexilongwa kwixesha elizayo. Ugqirha unokuva intliziyo igungqele kwaye ilandele iimvavanyo; okanye abazali bangazibona i-hypercyanotic spells xa umntwana ephazamiseka. Ezinye iimpawu ze-TOF zingabandakanya ukutya okunzima, ukungaphumeleli ukukhula ngokuqhelekileyo kunye ne- dyspnea .
Abantwana abakhulileyo kunye ne-TOF bafunda ngokuphindaphindiweyo ukuba banciphise iimpawu zabo. Ukuxubha kwandisa ukungatshatyalaliswa kwimithambo yegazi, enefuthe lokubangela ukunganyangeki kwegazi kwi-aorta, ngaloo ndlela iqondisa ngaphezulu kwegazi legazi ngaphakathi kwimijikelezo ye-pulmonary. Oku kunciphisa i-cyanosis kubantu abane-TOF. Ngamanye amaxesha abantwana abane-TOF baqala ukuxilongwa xa abazali babo bethetha ngokugqithisa kwabo ugqirha.
Iimpawu ze-TOF-cyanosis, ukunyamezela kokunyamezela, ukukhathala, kunye ne-dyspnea-ziba nzima ngakumbi xa ixesha lihamba. Kwininzi enkulu yabantu abane-TOF, ukuxilongwa kwenziwa ngexesha lokuqala kwasebuntwaneni nokuba ingaqatshelwa ekuzalweni.
Kubantu abahlala kwi-TOF kubandakanya kuphela i-pulmonary artery stenosis, i-cyanosis ayinakwenzeka nakweyonke, kwaye iyakudlula iminyaka ngaphambi kokuba i-diagnostic yenziwe. Ngamanye amaxesha, i-TOF ingasayi kufumaniswa ukuba ikhulile. Nangona kunqongophala kwe-cyanosis enzima, aba bantu basadingeka baphathwe, kuba bahlala behlakulela ukungaphumeleli kwenhliziyo xa bekhulile.
Yintoni eyenza iTetralogy yobuxoki?
Njengoko kunjalo ngeendlela ezininzi zesifo senhliziyo esiswini, imbangela ye-TOF ayaziwa. I-TOF iqhutyelwa ngamaxesha aphezulu kubantwana abane- Down syndrome kunye nezinye izinto ezingafaniyo zofuzo. Nangona kunjalo, ukubonakala kwe-TOF kusondeleko kwindalo kwaye ayizuze ilifa. I-TOF iye yadibaniswa ne-rubella yomama, isondlo esingasweleko somama okanye ukusetyenziswa kotywala, kunye neminyaka yobudala engama-40 okanye ngaphezulu. Uninzi lwexesha, nangona kunjalo, akukho nenye yale mingcipheko ekhoyo xa umntwana ezalwa ene-TOF.
Ukuchonga i-Tetralogy ye-Fallot
Xa kunesityholo senhliziyo esicatshulwayo, ukuxilongwa kwe-TOF kungenziwa nge- echocardiogram okanye i- MRI ye-cardiac , nokuba yeyiphi enye eya kubonisa i-cardiac anatomy. I- catheterization ye-cardia ihlala isinceda ekucaciseni i-cardiac anatomy ngaphambi kokulungiswa komzimba.
Ukuphathwa kweTetralogy ye Fallot
Ukunyangwa kwe-TOF kutyalwa. Ukusebenza okwangoku kukuba kwenziwe utyando lokulungisa ngunyaka wokuqala wobomi, ngokufanelekileyo phakathi kweenyanga ezintathu nantandathu. "Ukuhlinzwa okulungiswayo" kwi-TOF kuthetha ukuvala isiphako se-septal defect (ngokunjalo ukwahlula ngakwesokudla kwintliziyo ukusuka kwicala lasekhohlo lentliziyo) kunye nokukhupha umthamo we-pulmonary. Ukuba ezi zinto zimbini ziya kufezwa, ukuphuma kwegazi entliziyweni kunokwenziwa ngokuqhelekileyo.
Kwiimeko apho kubakho ukukhathazeka okubalulekileyo ekuzalweni, kufuneka kwenziwe utyando olunyanzelisayo, ukuzinzisa umntwana ade anqine ngokwaneleyo ukulungiselela ukulungiswa. Ukuhlinzwa ngokugqithisileyo kubandakanya ukudala i-shunt phakathi kweyodwa yeerriyriystemic (ngokuqhelekileyo i-subclavian artery) kunye nenye yemithambo ye-pulmonary, ukubuyisela umlinganiselo othile wegazi kwimipuphu.
Kubantu abadala abafumene i-TOF, utyando olusilungisiweyo lukwacetyiswa, nangona ubungozi obuncipheko buphezulu kubantu abadala kunabantwana abancinci.
Ngeendlela zanamhlanje, utyando olulungiswayo lwe-TOF lunokwenziwa ngengozi yokufa kwe-0 ukuya kwi-3 kuphela kwiintsana nabantwana. Isantya sokufelwa kwe-TOF ukulungiselela abantu abadala, nangona kunjalo, sinokuphakama ngaphezu kwe-10. Ngethamsanqa kunzima kakhulu ukuba i-TOF "ilahleke" namhlanje ibe ngumdala.
Yintoni Isiphumo Sesikhathi Eside Sokuba iTetralogy of Fallot?
Ngaphandle kokulungiswa kocwangco, phantse isiqingatha sabantu abane-TOF bafa kwiminyaka embalwa yokuzalwa, kwaye bambalwa kakhulu (nokuba nabo baneengxaki "ezinzima") zihlala ezingama-30.
Emva kokuhlinzwa ngokukhawuleza kwangaphambili, ukusinda kwexesha elide kulunge ngoku. Uninzi lwabantu abaninzi abane-TOF echanekileyo baphila kakuhle baze babe ngabantu abadala. Ngenxa yokuba iindlela zobugcisa zanamhlanje ziyiminyaka embalwa kuphela ubudala, asikwazi ukuba yintoni abayosinda kuyo. Kodwa kuyaqheleka ukuba izazi ze-cardiologists zibone izigulane ezine-TOF kwiminyaka yesithandathu okanye yesibhobho yobomi.
Sekunjalo, iingxaki zomzimba ziqhelekileyo kubantu abadala abakulungiselele i-TOF. I-valveary valve regurgation , ukungaphumeleli kwintliziyo, kunye ne- cardiac arrhythmias (ingakumbi i-tachycardia ye-atrial kunye ne- tachycardia ye-ventricular ) yizona ngxaki eziqhelekileyo ezenzekayo njengoko iminyaka ihamba. Ngenxa yeso sizathu, nabani na oye walungisa i-TOF kufuneka babe phantsi kwengqalelo ye-cardiologist onokuyihlolisisa rhoqo, ukwenzela ukuba nayiphi na ingxaki zengqondo eziza kuphuhliswa zijongane ngonya.
ILizwi
Ngonyango lwangoku, i-tetralogy ye-Fallot iye yaguqulwa kwimeko yesifo senhliziyo esazalwa yintsholongwane eyayivame ukuphumela ekufeni ngexesha lobuntwaneni, kwingxaki enkulu echanekileyo, ehambisana nokunyamekela unyango, iyahambisana nokuphila okungenani kude kube ngumdala. Namhlanje, abazali beintsana ezizalwe nale meko banesizathu sokulindela ukufumana ezininzi iintlobo zentlalo yolonwabo kunye neentlungu ezingayilindela nangomnye umntwana.
> Imithombo:
> I-Khairy P, uAboulhosn J, uGurvitz MZ, et al. I-Arrhythmia Umthwalo kubantu abadala abakulungiselele ukulungiswa kweTetralogy of Fallot: i-Multi-institutional Study. Ukuhamba ngo-2010; 122: 868.
> Park CS, Lee JR, Lim HG, et al. Isiphumo sesikhathi esizeleyo sokuPhathwa kweNkcitho yeTetralogy yobuxoki. Eur J Cardiothorac Surg 2010; 38: 311.
> Isexwayisa CA, Williams RG, Bashore TM, et al. I-ACC / AHA 2008 Izikhokelo zoLawulo lwaBadala abaneSifo seNtsholongwane yeNtliziyo: Ingxelo ye-American College of Cardiology / i-American Heart Association Umsebenzi weKomiti yeziKhokelo (iKomiti yokuBhala ukuPhucula iziKhokelo zoLawulo lwaBadala abaneNtsholongwane yesifo senhliziyo). Ukuphuhliswa koBambiswano kunye ne-American Society of Echocardiography, Intliziyo yeRhythm Society, uMbutho weZizwe eziMdala weZizwe eziKhulayo zeNtliziyo, i-Society for Cardiovascular Angiography kunye neNongenelo, kunye noMbutho wezilwanyana zeTrans. J Am Coll Cardiol 2008; 52: e143.