Tetralogy na Fallot - menene wannan? Ganewar asali da kuma magani

Tetralogy na Fallot - wani nakasar zuciya cuta, wanda aka halin da dama munanan. A gaban wannan cuta ya canjãwa aiki na zuciya da jijiyoyin jini tsarin, wanda shi ne fraught tare da mafi tsanani sakamakon. Alal misali, a jarirai wannan Pathology ne sau da yawa tare da cyanosis, shortness na numfashi da kuma mai tsanani na cin gaba da bata lokaci ba. Wannan shi ne dalilin da ya sa iyaye ne don haka damuwa da tambayoyi game da abin da yake akwai wata cuta a ƙarƙashin rinjayar abin da abubuwan shi tasowa, abin yana tare da bayyanar cututtuka. Bayan duk, farkon ganewar asali da kuma dace magani - kadai abu da zai iya tabbatar da yaro da wata al'ada rayuwa a nan gaba.

Mene ne tetralogy na Fallot?

Tetralogy na Fallot (IBC) - shi ne m, wani hadadden zuciya lahani. Morphological tushen Pathology kunshi hudu ãyõyi (wanda, a gaskiya, ya nuna da kalmar "Mai kula da kawancen"). Su ne:

• toshewa da dama na ramin zuciya outflow sashen.
• hijirar na lakã.
• dama na ramin zuciya hypertrophy .
• m na ramin zuciya septal lahani.

A karo na farko da wannan Pathology aka bayyana a cikin 1888 da Faransa sani akan cututtuka Fallot, sunansa daga baya aka mai suna. Bisa kididdigar da, cutar ne game da 7-10% na nakasar malformations. Af, wani lokacin tetralogy na Fallot Associated tare da sauran anomalies kamar patent ductus arteriosus, stenosis na da rassan da na huhu jijiya, aortic baka da pravoraspolozhennoy t. D.

Babban Sanadin cutar

Tetralogy na Fallot - a Pathology, wanda aka kafa a sakamakon take hakkin da aiwatar da kwanciya da kuma ci gaban da zuciya da jijiyoyin jini tsarin da kimanin 2-8 makonni na da amfrayo ci gaba. An ce cewa Sanadin wannan anomaly ba su cikakken sani. A daya hannun, wasu daga cikin hadarin dalilai da masu bincike sun iya gano.

Alal misali, Pathology iya faruwa a cikin harka a farkon matakai na ciki, da wata mace sha wahala kamuwa da cuta (rubella, kyanda da mulufi zazzabi, da kuma wasu wasu). Take hakkin alamomin kuma gabobin da tayin za a iya alaka da wasu kwayoyi, ciki har da hormonal kwayoyi, hypnotic da magani mai kantad da hankali kwayoyi. Karbar kwayoyi, barasa kuma rinjayar da samuwar nakasar zuciya cuta (CHD).

Tetralogy na Fallot ne, wani lokacin kamu a yara da sauran munanan, kamar abin da ake kira Amsterdam wada. Wannan shi ne rashin lafiya mai tsanani da cewa yana tare da m cuta, ciki har da nakasawa auricles, hypertrichosis, myopia, na gani jijiya atrophy, kashin baya nakasawa da kuma kama. D.

Matsayin mai mulkin, ci gaban da cututtukan zuciya fara a lokacin da, daya dalili ko wata infundibulum rotates counterclockwise. A sakamakon rashin iya juyawa aortic bawul aka canja, da kuma lakã kanta ne located a kan interventricular septum. Tare da ci gaban da fetal na huhu akwati lengthens, Narrows kuma canjawa saboda rashin wurin da lakã.

rarrabuwa na cututtuka

Tetralogy na Fallot a yara za a iya wakilta hudu iri, dangane da toshewa da dama na ramin zuciya outflow fili na fitarwa:

• Jujjuyawar, ko na farko da irin tetralogy na Fallot - wani nau'i na Pathology, a cikin abin da conical gagara kawar da faruwa zuwa hagu da kuma gaba. Fibrinous zobe ba maras kyau, da kuma iyakar yankin na stenosis aka located in wani zobe na tsoka.
• Nau'i na biyu, ko hypertrophic tetralogy na Fallot - wani irin Pathology, a cikin abin da akwai ba kawai da korar bangare (kamar yadda aka bayyana a baya hali), amma shi alama hypertrophy. matsakaicin ci gaban yankin na stenosis lura a cikin matakin da dama na ramin zuciya outflow sashen.
• The uku irin Pathology a magani kira a matsayin tubular, halin da sakon division janar na jijiya akwati. A sakamakon irin wannan take hakkin da al'ada tsari, wata canji a huhu mazugi - shi wãyi quntata kuma taqaitaccen. Har ila yau, iri-iri na cuta halin da na huhu stenosis da hypoplasia fibrinous zobe.
• Multicomponent, hudu nau'i na tetralogy na Fallot yawanci tare da wani gagarumin tsawo mazugi katangu.

Features hemodynamics a wannan cuta

Abin da canje-canje suna tare da cututtukan zuciya? Tetralogy na Fallot, a wani hali entails hemodynamic cuta mai tsanani daga abin da aka ƙaddara da nau'i na toshewa da dama ventricle na fitarwa bututu, kazalika gaban canje-canje a cikin interventricular septum.

Idan na huhu stenosis gagarumin kuma akwai manyan septal aibi, ya fi girma adadin jini daga duka ventricles gudana a cikin lakã. Amma a cikin huhu jijiya ne bauta yawa kasa da al'ada, wanda tsokani ci gaban da jijiya hypoxemia. A wani matsananci nau'i na wannan cuta a cikin jini shiga cikin huhu wurare dabam dabam daga lakã ko via collaterals, ko ta hanyar da lamban kira ductus arteriosus.

Idan toshewa haƙuri matsakaiciya, shi tasowa a hagu-dama shunt - yayin da akwai atsianoticheskaya nau'i na cutar. Duk da haka, cutar ne kullum ci gaba - na farko tarin jini zama giciye, sa'an nan dama-bar, da kuma cutar da ake canza kama daga fari siffar a cikin blue (cyanotic).

Abin da ake da bayyanar cututtuka da cutar yana tare da?

The asibiti hoto na wannan Pathology iya zama daban-daban, kamar yadda shi ne sun fi mayar dogara a kan siffar da cutar. Alal misali, wani lokacin tetralogy na Fallot a cikin jariri yana tare da alama cyanosis. A wannan yanayin, sai jaririn ya iya shan wahala daga shortness na numfashi a lokacin feedings. Kuma low nauyi riba lura a farkon watanni na rayuwa. A daya hannun, idan na huhu jijiya stenosis a wani yaro ne kadan, sa'an nan sauran cyanosis yanayin da aka ba a lura, sabili da haka don gane asali cutar da alãmarsu, ba kullum zai yiwu da sauri.

Popular sau da yawa hypoxic harin faruwa da wani bango na jiki aiki. Irin wannan jiha yana tare da bayyanawa da kuma sosai m breaths. The yaro yana farawa kuka, zama sosai m. Cyanosis hankali qara. Kamar yadda mai mulkin, irin wannan halin da ake ciki an lura a matasa da yara, tare da mafi furta bayyanar cututtuka bayyana tsakanin zamanai na biyu da wata hudu.

Idan tetralogy na Fallot a cikin jariri yana tare da mai tsanani, hare haren da shi na iya kai wa ga general lethargy da seizures. Wani lokaci Pathology take kaiwa zuwa mutuwa. Mazan yara wani lokacin a lokacin jiki aiki play instinctively squat cewa wai rage tsari venous samu da kuma inganta oxygen jikewa a jijiya da jini.

Basic Pathology cututtuka - bouts na cyanosis da dyspnea, sau da yawa tare da asarar sani. A mataki na oxygen rashi da tsananin hare-haren ne kai tsaye dogara a kan mataki na stenosis na huhu jijiya.

Ya kamata kuma a lura da cewa na asibiti hoto, kamar yadda mai mulkin, akwai dama, saukarwa ga yaro ta alheri, kazalika da wasu musamman m lokaci:

• Daga haihuwa zuwa watanni 6. Gaskiyar ita ce, a wannan lokaci da yara ba sosai aiki a jiki, sabili da haka cyanosis da hypoxia bayyana moderately. Babu shakka, wannan ba ko da yaushe, wasu yara wahala kusan daga na farko kwanaki na rayuwa.
• A mafi wuya mataki (daga likita bisa) aka lura a lokacin daga watanni shida zuwa shekaru biyu. A hari tasowa a lokacin da spasm da dama na ramin zuciya outflow yankuna. A sakamakon spasm na dukan venous jini da aka dakatar a cikin lakã, wadda take kaiwa zuwa cin gaban mafi tsanani kwakwalwa hypoxia. Yawancin lokaci hari da yaro zai fara kwatsam - ya zama m. Lura cyanosis da dyspnea, wani lokacin ga zance na yin suma. Sau da yawa akwai cramps. Wannan tsari, a mafi yawan lokuta ya auku a kan wani bango na tunanin ko ta jiki da danniya.

A nan gaba, da na asibiti hoto canjãwa dan kadan. A mazan zamanai, cyanosis kullum girma (wani lokacin da sauri, wani lokacin sannu a hankali), amma hare-hare kusan gaba daya bace. Rage tachycardia da dyspnea da na huhu kafa na biyu dabam dabam kewaye.

Hanyoyin zamani na ganewar asali

Da sauƙi, kawai wani m likita zai iya sa irin wannan ganewar asali. Tetralogy na Fallot - a Pathology cewa bukatar tarin cikakken likita tarihi da kuma gudanar da wani yawan nazarin cewa ba kawai ya tabbatar da kasancewar nakasar, amma kuma za ta taimaka ƙayyade da siffar da cutar, ta mai tsanani, da sauran harkokin kiwon lafiya na yaro.

Idan bayyanar cututtuka irin bincike:

• Harufan kimantawa hada electrocardiogram. A lokacin hanya, shi iya lura ãyõyin obalodi wasu sassa na zuciya, kazalika da hypertrophy. Alal misali, sakamakon iya nuna sinus ciwo ko AV block.
• Tetralogy na Fallot - a Pathology cewa za a iya gani a lokacin kirji x-ray. Alal misali, wannan aibi ne tare da bulging na baka na hagu da kuma dama ventricles, akwai wani alama depletion na huhu juna. A jariri jarirai, da zuciya a kan radiograph a siffar sau da yawa kama da sifila.
• Quite m Hanyar ne echocardiography. A wannan binciken zai iya fitowa sosai kimanta girman da dama ventricle, kazalika domin sanin irin Pathology, don kimanta mataki na takaita na fitarwa kwarara. Amfani da wannan hanyar nazari zuciya bawuloli, musamman aortic bawul nazari da janar jihar na bawuloli, auna diamita na fibrous zobba. Gaskiyar ita ce, idan ba ka gane aortic stenosis kuma ku bi da lakã, a karshen jiyya na iya haifar da rikitarwa, kamar na huhu edema.

Matsayin mai mulkin, da nazarin aka bayyana a sama shine isa domin sanin ko yaro malformation ne ba. Tetralogy na Fallot, duk da haka, - wani bincike da cewa sanya wani lokacin wuya sosai, kuma na iya bukatar wasu ƙarin hanyoyin.

Alal misali, jijiyoyin zuciya angiography ne wani lokacin da za'ayi. Wannan hanya wajibi ne sanin wuri da jijiyoyin zuciya da kuma manyan tasoshin, duba idan akwai wata dangantaka anomalies, kazalika da wa gano daidai da irin cutar zuciya.

Catheterization - a mafi rikitarwa hanya da ya shafi sokin da zuciya cavities. Wannan binciken da aka gudanar a yayin da ba ka bukatar ka gwada da matsa lamba a dama ventricle, ƙayyade mataki na oxygenation na jini a cikin lakã.

Tetralogy na Fallot: Jiyya

A lura regimen sun fi mayar dogara a kan siffar da cutar da kuma ta mai tsanani. Drug magani taimaka wajen kawar da kai hare-hare da kuma hana wasu rikitarwa hade tare da oxygen rashi.

Yawanci, jarirai wajabta prostaglandin jiko. Wannan hanya wani lokacin taimaka wajen bude jijiya bututu sake. Idan wani jariri ya iya gani ãyõyin hari, shi ne shawarar zuwa manema gwiwoyinsa ya kirji. Mazan yara yi squats - wannan matsayi taimaka kauce wa hypoxemic hari.

Shawarar igiyar jini ruwaye zuwa replenish jini girma. A wasu lokuta, matasa marasa lafiya wajabta morphine. Idan ya cancanta, da jini ne ya karu da phenylephrine da ketamine (a abu da magana, kuma yana da magani mai kantad da hankali sakamako). A hana yawan komawan wani lokacin taimaka propranolol.

Wani lokaci cupping kwashe oxygen far, amma shi ne ya kamata a lura da cewa sakamakon wannan magani ne quite m.

Palliative tiyata

Babu shakka, daban-daban kwayoyi za su sassauta yaro ta yanayin. Duk da haka, magunguna ba zai iya ajiye haƙuri daga irin wannan hadari cuta kamar yadda tetralogy na Fallot. Operation ne da nisa da kawai hanyar kawar da lahani. Kawai tiyata iya ba yaro damar domin wata al'ada girma da kuma cika rayuwa.

Yau, akwai biyu main hanyoyin da m magani. Palliative tiyata ne don gabatar daban-daban iri mezharterialnyh shafukan da cewa ba ka damar mayar da wurare dabam dabam. Alal misali, mafi m hanya a yau ne danniyan daga podklyuchichnolegochnogo anastomosis. A lokacin da hanya, da subclavian jijiya ta amfani da musamman implant guda biyu tare da unilateral na huhu jijiya.

M tiyata a matsayin magani

Doctors bayar da shawarar maza maza don gudanar da wani m tiyata, kamar yadda ya bada sakamako mai kyau. Yawanci, irin wannan hanya ne da za'ayi tsakanin zamanai na 6 months zuwa shekaru 3 - a lokacin da wannan lokaci, shi ne mafi inganci (idan mun yi la'akari da kara aiwatar da maturation da kuma ci gaban da yaro).

M magani ne don kammala gyara da mahaukaci Tsarin. Amfani da faci Surgeons rufe wani na ramin zuciya septal aibi, na huhu kumbura stenotic rabo daga jijiya. Hakika, da hanya ne rikitarwa, amma saboda shi zai iya rike kawai m zuciya likita.

Tetralogy na Fallot: hangen nesa da marasa lafiya

Hakika, na farko na dukkan iyaye sha'awar al'amurra da irin wannan cuta. Tetralogy na Fallot a yara - wani musamman tsanani Pathology, watsi da shi a cikin wani hali ba zai yiwu ba.

An fahimci cewa hanya da cutar dogara da tsanani daga huhu stenosis. Idan muna magana ne game statistics, sa'an nan, da rashin alheri, kusan kwata na yara mutu a cikin shekarar farko. Ba tare da tiyata, da talakawan shekaru na yara da suke zaune tare da cuta shi ne shekaru 12 da. Kawai game da 5% rayu har zuwa shekaru 40 da suka kamu da yadda tetralogy na Fallot. Tawaya, na cin gaba da matsaloli, m iko da matalauta ingancin rayuwa - Wannan shi ne wani abu da wanda, da rashin alheri, su hadu da adult marasa lafiya.

A madadin, ba shakka, akwai. Kada ka manta game da m magani, wanda sau da yawa wajabta yara da ganewar asali "tetralogy na Fallot." Bayan tiyata, yara yawanci warke da sauri ci gaba kullum, ko iya yin tsayayya da m motsa jiki. Lura da wani abin kwaikwaya - tiyata daga baya a rayuwa ba ba da irin wannan sakamako mai kyau a nan gaba, kamar yadda da aiki yi a cikin jariri.

Marasa lafiya tare da wannan cuta, ko da bayan kammala far ya kamata sha yau da kullum rajistan shiga-rubucen a zuciya likita da kuma likitan zuciyar. Kafin wani m, kuma hakori hanyoyin, marasa lafiya dole ne sha musamman magani domin kauce wa hadarin endocarditis. Babu shakka, akwai sauran bukatun, hani a cikin haƙuri na rayuwa, wanda likitan ake bukata gaya.