Tetralogy of Fallot (TOF) chirwere chekuberekwa kwepfungwa chinokanganisa nevana vashanu pavana 10 000 vacheche, vanoverengera vanenge 10 kubva muzana yehutano hwepfungwa dzose. Kunyange zvazvo TOF inogara iri dambudziko guru rinoda kurapwa, kupisa kwayo kunogona kusiyanisa zvakanyanya. Dzimwe nguva inounza zviratidzo zvinotyisa zvehupenyu muchecheche, uye inoda kurapwa nokukurumidza.
Mune dzimwe nguva, TOF inogona kusaita zviratidzo zvikuru (uye inogona kuramba isingazivikanwi) kwemakore akawanda. Asi nokukurumidza kana kuti gare gare, TOF inogara ichikonzera matambudziko ehupenyu hunotyisa, uye inoda kugadziriswa chirwere.
Chii Chinonzi Tetralogy yeNhema?
Tetralogy of Fallot iyi inosanganiswa neine mwoyo yematomic matambudziko, sezvakatsanangurwa Dr. Etienne-Louis Arthur Fallot muna 1888. Izvi ndezvi:
- pulmonary artery stenosis (kupera kwemapurmonary arteri)
- guru ventricular septal defect (gomba murusvingo pakati pezvinyorwa zvekurudyi nechezasi)
- kutsauka kweaorta kune ventricle yakarurama
- zvakarurama ventricular hypertrophy (muscle thickening)
Vadzidzi vezvokurapa vanowanzoita chikanganiso chekuedza kunzwisisa TOF nekuyeuchidza urongwa hwezvikanganiso zvina. Apo vachiita izvi vanogona kuvabatsira kuti vapindure mibvunzo yekuedza, hazvivabatsire zvikuru mukunzwisisa kuti TOF inoshanda sei, kana kuti sei vanhu vane TOF vane kusiyana kwakanyanya muzviratidzo zvavo.
Kazhinji Moyo Wemabasa
Kuti "uwane" TOF, zvinobatsira kutanga kutarisa kuti moyo unowanzoshanda sei. ( Verenga pano pamusoro pemakamuri emwoyo nemagavhu .) Oxygen-murombo, "yakashandiswa" ropa kubva kumativi ose mumuviri unodzokera kumwoyo kuburikidza nemavhuna, uye inopinda paanrium yakarurama, uye ipapo inonzi ventricle. Izvo ventricle yakarurama inoputira ropa kuburikidza nemishonga yepermonary kumapapu, uko inowedzerwa neokisijeni.
Iko-ikozvino-oxygenated ropa inodzokera kumwoyo kuburikidza nemaspine emapinmoni uye inopinda kuruboshwe rwemarerium, uye ipapo kuruboshwe rwemhepo. Nzvimbo yakasara yekasirikiti (iyo ikamuri huru yekuputira) inoputira ropa reokisijeni muyeri huru (aorta) uye kunze kwomuviri. Uyewo chengetedza mupfungwa kuti kazhinji, kodzero dzakasara uye dzakasara dzemhepo dzakaparadzana dzakaparadzana kubva kune rimwe nerusvingo runonzi muscular inonzi septric ventricular.
Chikonzero nei Tetralogy yeNhema inokonzera Matambudziko
Kuti unzwisise TOF, unonyatsoda kunzwisisa zviviri chete (kwete zvina) zvinokosha zvechiitiko ichi. Chokutanga, chikamu chakawanda chevhuvhuta chinonzi ventricular chinoshaikwa mu TOF (zvinonzi ventricular septal defect). Chigumisiro chechiremera ichi che septal ndechekuti makiromita akanaka uye akasiyiwa haachaiti semakamuri maviri akasiyana; panzvimbo pacho, inoshanda kune imwe nzvimbo seyero rimwe guru. Oxygen-ropa risina kudzoka kubva mumuviri, uye ropa re-oksijeni rinodzoka kubva kumapapu, kusanganiswa pamwe chete mune iyi ventricle.
Chechipiri, nokuti kushanda ikoko kunongova imwe chete yemhepo huru, kana zvibvumirano zvemhepo, iyo mishonga yemapuraneti uye aorta inowanzo "kukwikwidza" kweropa ruchiyerera. Uye nokuti muA TOF pane imwe nzvimbo ye stenosis (narrowing) yemapurmonary artery, aorta kazhinji inogamuchira zvinopfuura chikamu chayo.
Kana iwe wakaramba uine tsananguro iri kure, inofanira kuve yakanakira kwauri kuti kiyi ye TOF ndeyero ye stenosis iri mune pulmonary artery. Kana pane huwandu hwemapuraneti inopesana, apo apo "vasina" mishonga yemagetsi, chikamu chikuru cheropa rakaputika richapinda muaorasi, uye zvishomanana zvinopinda mumapirmaria arteri. Izvi zvinoreva kuti ropa shomanana rinosvika pamapapu uye rinova oksijeni; iyo inopararira ropa ikozvino okisijeni-murombo, chirwere chakaipa chinonzi cyanosis . Saka, mamiriro akaoma zvikuru e TOF ndeaya ayo mapirmita arteri stenosis ndiye mukuru.
Kana mishonga yemapurisa stenosis isiri yakaoma kwazvo, ipapo ropa rinonzwisisika rinotumirwa kumapapu uye rinova oxygenated. Vanhu ava vane zvishoma zvishoma zvekushandiswa kwe cyanosis, uye kuvapo kwe TOF kungave kusisipo pakuberekwa.
Chinyorwa chinotsanangurwa zvakanaka che TOF, chinowanikwa mune vana vakawanda vane chirwere ichi, ndechokuti chiyero chemapurmonary arteri stenosis chinogona kuchinja. Muzviitiko izvi, cyanosis inogona kuuya uye ichienda. Cyanotic episodes inogona kuitika, somuenzaniso, apo mwana ane TOF anogumburwa kana anotanga kuchema, kana kuti mwana akare anodzidzira TOF. Izvo "tsvina" ye cyanosis inowanzonzi "tet spells" kana hypercyanotic spell. Iyi tsvina ye cyanosis inogona kuva yakaoma kwazvo, uye inogona kudikanwa mune zvekurapa.
Zviratidzo zveTetralogy yeShema
Zviratidzo zvinowanzoenderana, sezvatakaona, pamwero wekudzivirira uripo mumapurmita arteri. Kana kugadziriswa kwemapirinari kuvharwa kwemishonga yakakomba, yakanyanya cyanosis inowanikwa muchecheche (mamiriro ayo aiwanzoti "bhuruu mwana"). Idzi idzi dzinotambudzika uye dzinoshungurudza zvakanyanya, uye dzinoda kurapwa nokukurumidza.
Vana vane kuvharidzirwa kwemaitiro emapurmita zvinowanzoonekwa kune imwe nguva. Chiremba anganzwa kunzwa mwoyo kugunun'una uye kurongedza kuongororwa; kana vabereki vangacherechedza hypercyanotic spells kana mwana achishungurudzika. Zvimwe zviratidzo zve TOF zvinogona kusanganisira kunetseka kudyisa, kusakwanisa kukura zvakakodzera, uye dyspnea .
Vana vakwegura vane TOF vanowanzodzidza kugadzira kuderedza zviratidzo zvavo. Kusvetwa kunowedzera kushorwa mumidziyo yeropa, iyo inokonzera kukonzera kubuda kweropa mu-aorta, nokudaro inotungamirira zvimwe ropa remwoyo mukati memapurmonary circulation. Izvi zvinoderedza cyanosis kune vanhu vane TOF. Dzimwe nguva vana vane TOF ndivo vanotanga kuongororwa apo vabereki vavo vanotaura kuti vanowanzotaurira chiremba.
Zviratidzo zve TOF-cyanosis, kushaya muviri kushivirira, kuneta, uye dyspnea-kunowedzera kuipa sezvo nguva inofamba. Muchizhinji chevanhu vane TOF, kuongororwa kunoitwa munguva yehuduku kunyange kana isingazivikanwi pakuberekwa.
Mune vanhu vane TOF vanobatanidza chete madhimoni emishonga inonzi stenosis, tsvina ye cyanosis haigone kuitika zvachose, uye makore angapfuura vasati vatsvaga. Dzimwe nguva, TOF inogona kunge isina kuonekwa kusvikira munhu mukuru. Pasinei nekushayikwa kwechianosis yakaoma, vanhu ava vachiri kudikanwa kurapwa, sezvo vachiwanzoita kukundikana kwemoyo nekukura.
Chii chinokonzera Tetralogy yeShema?
Sezvakangoita nemhando dzakawanda dzehutano hwepfungwa, chirwere che TOF hachizivikanwi. TOF inowanikwa nepamusoro-pfupi kuvana vane Down syndrome uye nedzimwewo maitiro ekusaremara. Zvisinei, kuonekwa kweUFF kunowanzowanzoitika zvisikwa uye hauna nhaka. TOF yave ichibatanidza nevana vemubereki rubella, chikafu chisina kunaka chekuberekwa kana kushandiswa kwewaini, uye makore ekuberekwa kwemakore makumi mana kana kupfuura. Kazhinji yenguva, zvisinei, hapana chimwe chezviitiko izvi zvinokonzera zviripo apo mwana akaberekwa neAIDS.
Kuongorora Tetralogy yeShema
Kana imwe nguva chirwere chepfungwa chikafungidzirwa, chirwere cheAIDS chinogona kuitwa ne echocardiogram kana MRI yemwoyo , iyo inogona kuratidzira kusagadzikana kwemoyo wematayidi. Chirwere chemwoyo chinowanzobatsira mukujekesa chimiro chemwoyo kusati kwagadzirwa.
Kurapa Tetralogy yeShema
Mushonga we TOF unoshandiswa. Ikozvino maitiro ndekuita chirongwa chekugadzirisa mukati megore rokutanga rehupenyu, zvakanaka pakati pemwedzi mitatu nematanhatu yemakore. "Kuvhiyiwa kwekugadzirisa" muAIDS kunoreva kupfigira chinonzi ventricular septal defect (nokudaro kuparadzanisa rutivi rworudyi rwemwoyo kubva kuruboshwe rwomwoyo) uye kubvisa mishonga inoputika. Kana zvinhu zviviri izvi zvichikwanisa kuitwa, ropa rinopinda mukati memwoyo rinogona kunyanya kushandiswa.
Mumamiriro ezvinhu apo kunetseka kwakanyanya kuripo pakuberekwa, chimiro chekuvhiya chirwere chinogona kudiwa, chinofanira kugadzirisa mwana kusvikira asimba zvakakwana pakugadzirisa chirwere. Kuvhiyiwa kwepfungwa kunowanzobatanidza kugadzira kuvhara pakati peimwe yemaitiro (systemic arterium) (kazhinji subterlavia arteri) uye imwe yemapuraneti yemapmonary, yekudzorera kumwe kweropa rekupararira kumapapu.
Kune vanhu vakwegura vanorwara neAIDS, chirongwa chekugadzirisa chinorudzirwawo, kunyange zvazvo ngozi yekuvhiya yakakwirira kune vakwegura kupfuura kuvana vaduku.
Nemaitiro emazuva ano, chirwere chekugadzirisa chirongwa che TOF chinogona kuitwa nehupenyu hwekufa kwe 0 kusvika ku3 muzana mucheche nevana. Kuparara kwehutano hwehutano hwe TOF kugadziriswa kune vanhu vakuru, zvisinei, inogona kuva yakakwirira kudarika 10 inotangira. Nenzira inonakidza zvikuru kuti TOF "irege kushaya" nhasi kuva munhu mukuru.
Chii Chichaguma Murefu weTetralogy yeShema?
Pasina kugadziriswa chirwere, inenge hafu yevanhu vane TOF vanofa mukati memakore mashomanana ekuberekwa, uye vashomanana (kunyange avo vane "mafungiro" maitiro echiremara) vanorarama makumi matatu.
Pashure pokuvhiyiwa kwokutanga, kurarama kwenguva refu ikozvino kwakanakisisa. Ruzhinji rwevanhu vakagadzirirwa TOF vanorarama kusvika pakuva vanhu vakuru. Nokuti maitiro ekugadzirwa kwemazuva ano mashoma mashoma emakore akare, isu tichiri tisingazivi kuti chiyero chekupedzisira chekupona chichave chei. Asi zvakave zvakajairika kuvadzidzi ve cardiologists kuti vaone varwere vane TOF mumakumi emakore nemakore makumi manomwe ehupenyu.
Kunyange zvakadaro, matambudziko emwoyo anowanzojairika kuvanhu vakuru vakagadzirirwa TOF. Vapulmonary valve kudzokerazve , mwoyo kusagadzikana, uye cardiac arrhythmias (zvikurukuru atrial tachycardia uye ventricular tachycardia ) ndiwo matambudziko anowanzoitika apo makore anofamba. Nokuda kwechikonzero ichi, ani zvake anenge agadzirisa TOF anoda kuva pasi pekutarisirwa kwechirwere chemapurisa anokwanisa kuaongorora nguva dzose, kuitira kuti chero matambudziko emwoyo anokurudzirwa anogona kugadziriswa nehasha.
Shoko Rinobva
Nekurapa kwemazuva ano, tetralogy yeEprilot yakashandurwa kubva pakuzvarwa kwemoyo wemamiriro ezvinhu iyo kazhinji yakazoguma nekufa muhuduku, kune dambudziko rakanyatsoruramisa, iro, nehutano hwakanaka uye rinopfurikidza kurapwa, rinoenderana nekurarama kusvika pakupera kwekukura. Nhasi, vabereki vemucheche vakaberekerwa vane chirwere ichi vane zvikonzero zvose zvekutarisira kuti vawane mhando dzakawanda dzomufaro nemoyo unogona kutarisira nemumwe mwana.
> Sources:
> Khairy P, Aboulhosn J, Gurvitz MZ, et al. Arrhythmia Burden muAdults neRurgly yakagadzirwa Tetralogy ye Fallot: Multi-institutional Study. Circulation 2010; 122: 868.
> Park CS, Lee JR, Lim HG, et al. Iyo Yenguva Yenguva Yakazara Yehuwandu Yakagadziriswa yeTetralogy ye Fallot. Eur J Cardiothorac Surg 2010; 38: 311.
> Anonyevera CA, Williams RG, Bashore TM, et al. ACC / AHA 2008 Mirayiridzo yekutungamirirwa kwevanhu vakuru neConital Heart Disease: Nyaya yeAmerican College of Cardiology / American Heart Association Task Force pamusoro pekudzidzisa mazano (Komiti Yokunyora Kukurudzira Mitemo Yekutungamirirwa Kwevanhu Vakuru neConital Heart Heart). Yakagadzirirwa Mukubatana NaAmerican Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, uye Society of Thoracic Surgeons. J Am Coll Cardiol 2008; 52: e143.