Tetralogy o Fallot - aha i keia? ʻike, a me ka lapaʻau

Tetralogy o Fallot - he congenital naau maʻi, a ua wehewehe aku ma kekahi mau abnormalities. Ma ke alo o keia maʻi EUIAaIN ka functioning o ka mea'ōnaehana mānowai koko nenoaiu, i mea fraught i oi nui hopena. No ka laʻana, i na kamalii uuku keia pathology ua pinepine pu ma ka cyanosis, pokole o ka hanu a me ka loa developmental ke kali ole. I mea no ke aha na makua i ai mea e hopohopo nei i na ninau e pili ana i ka mea he maʻi ma lalo o ka aoao o ka mea Makaʻu mea e hoʻomōhala ai, i ka pu ma symptoms. Ma hope o nā mea a pau, iʻike, a me ka kūpono lapaau - ka mea wale i ke hōʻoia i ke keiki i ka maʻamau ke ola i loko o ka wā e hiki mai.

He aha ka tetralogy o Fallot?

Tetralogy o Fallot (IBC) - ia mea innate, he luna 'naau kina. Morphological kumu o ka pathology ninoieo o eha hoailona (a, ma ka mea, paipai i ka manawa "puke noke"). Ka mea, e:

• iaʻi o ka akau ventricular outflow 'Oihana;
• pohupani hale o ka aorta;
• pono ventricular hypertrophy ;
• nui ventricular septal kina.

No ka mea manawa mua i ho'ākāka 'ia i kēia pathology ma 1888 ma ka Farani pathologist Fallot, nona ka inoa hope i ka inoa. E like me ka 'ikepili helu, ka maʻi mea e pili ana 7-10% o congenital malformations. Ma ke ala, tetralogy o Fallot kekahi manawa hui a me nā anomalies, no kekahi laʻana, iaoaioa ductus arteriosus, stenosis o na lala o ka pulmonary artery, aortic e pio mai a me ka pravoraspolozhennoy 'ole. D.

I ka papa kuhikuhiE kumu o ka maʻi

Tetralogy o Fallot - he pathology, a ua hanaʻia e like me ka hopena o ka 'aʻe' ana o ke kaʻina hana o ka hoomoe ana a me ka? Acaeoey o ka mea'ōnaehana mānowai koko'ōnaehana ma ka hola 2-8 pule o embryonic? Acaeoey. Ua Ua olelo mai i na kumu o keia anomaly ka mea, i ike maopopo. Ma ka kekahi lima, kekahi o ka kamepiula kumumea, ua hiki ke loaʻa mai i ka noiʻi.

No ka laʻana, ka mea pathology kānāwai i loko o ka hihia i loko o nā kahua o ka hoʻonaʻauao kakahiaka o ka hāpai keiki, he wahine i ae maʻiʻaʻai (rubella,ʻo Waimānalo,ʻulaʻula kuni, a me kekahi mau mea e ae). Kue ana i ka lepe puke a me nā loko o ka fetus hiki e pili ana i kekahi ai ', a me hormonal ai', hypnotic a me ka kiaha dala ai '. Ka loaa ana ai ', ka waiʻona kekahi e loli ai i ke kahua o ka congenital naau maʻi (CHD).

Tetralogy o Fallot ua i kekahi manawa diagnosed i loko o nā keiki a me nā abnormalities, e like me ka ai-kapaia Part I dwarfism. He He maʻi nui i ka pu ma kāna hana lehulehu, a me deformation auricles, hypertrichosis, myopia, optic nā aʻalolo atrophy, spinal deformation a me ka e like. D.

E like me ka rula, o ka hooulu ana o ka naau maʻi hoʻomaka i ka manawa ia, no kekahi kumu a me kekahi infundibulum ke ka ao ka counterclockwise. E like me ka hopena o kūpono kuapo aortic kiwikā o ua 'oni iki, a me ka aorta iho ua aia ma luna o ka interventricular septum. Me ka hooulu ana o ka fetal pulmonary kumu e hooloihi aku i, narrows a me ka hoʻololi ai ma muli o ke kūpono wahi o ka aorta.

Mea Maluna o ka maʻi

Tetralogy o Fallot i keiki ke hoʻokū 'ia e eha hoailona ia mea, ke kaumaha ma muli o ka iaʻi o ka akau ventricular outflow'āpana o ka auoiaea.

• Embryological, a me ka mea mua 'ano o ka tetralogy o Fallot - he' ano o ka pathology, ma a ka conical baffle pohupani hale ia lŘlŘ i ka hema a me ka mua. Fibrinous ke komo lima o ua i ino, a me ka mea i kā mākou wahi o ka stenosis kahi i loko o ke apo o ke Muscle.
• Ka luaʻano, a me hypertrophic tetralogy o Fallot - he ano o ka pathology, ma i he mea ole wale ka pohupani hale o ka paku (e like me ia i ho'ākāka 'ia ma ka mua hihia), akā, ia ia hypertrophy. i kā mākou? acaeoey āpana o ke stenosis ike i ka nui o ka akau ventricular outflow Oihana.
• Ke kolu o kaʻano o ka pathology ma ka lapaau haawiia i like omo, wehewehe aku ma PilikiaʻAʻole i mahele mau arterial kumu. E like me ka hopena o ia i ka 'aʻe' ana o ka mea maʻamau 'ole, he loli ma pulmonaryʻohana - ka mea, lilo hou a hoʻopōkole. Nō hoʻi, keʻano o ka maʻi wehewehe aku ma ka pulmonary stenosis a me ka hypoplasia fibrinous apo.
• Multicomponent, ha o ka palapala o ka tetralogy o Fallot ana pu ia e ka nui mailaila aku, hoʻopuʻupuʻu pā.

Features hemodynamics i loko o kēia maʻi

He aha nā loli i pu ma ka naau maʻi? Tetralogy o Fallot, ma kekahi hihia entails hemodynamic kāna hana i ke oolea o ka i me ka nānā 'ana i ka palapala o ke iaʻi o ka ventricle akau o ka auoiaea leki uea, e like me ke alo o nā loli i loko o ka interventricular septum.

Inā pulmonary stenosis nui, a he mea he nui septal kina, i ka nui dala o ke koko, mai nā ventricles kahe ana i loko o ka aorta. Akā, i loko o ka pulmonary artery ua hookauwa aku ka emi o ka maʻamau, i hoʻonāukiukiʻana mai o ka hooulu ana o ka arterial hypoxemia. Ma ka lōʻihi ano o keia maʻi i loko o ke koko i loko o ka pulmonary holo, mai ka aorta paha Via collaterals, a ma ka iaoaioa ductus arteriosus.

Inā iaʻi hoomanawanui lōpū, ka mea e hoʻomōhala ana i ka hema-pono shunt - oiai e haule ana atsianoticheskaya ano o ka maʻi. Aka hoi, i ka maʻi ua mau i holopono - mua ohi ana o ke koko i ke kea, a laila, pono-koe, a me ka maʻi ia iho mai ka keʻokeʻo kinona i loko o ka uliuli (cyanotic).

He aha i na symptoms o ka maʻi ua pu e?

Ka lapaʻau kiʻi o keia pathology i e okoa, e like me ka mea pili kēia kalele iho ma luna o ke kinona o ka maʻi. No ka laʻana, i kekahi manawa tetralogy o Fallot i loko o ke keiki ua pu e kaha cyanosis. Ma keia hihia, ke kamaiki i hana eha iaʻi mai ka pokole o ka hanu iloko, e ai ana. A me ka haahaa kaumaha i waiwai ai ike i loko o nā mahina mua o ke ola. Ma ka kekahi lima, ina ka pulmonary artery stenosis i loko o ke keiki i ka palena haʻahaʻa, a laila, i ke koena cyanosisʻano ua,ʻaʻole mālama, a no ia mea, e diagnose i ka maʻi ma ko lākouʻano i mea e mau loa ana e hiki koke.

Muli o kona kulana pinepine hypoxic ho'āhewa wale 'ana aku i ke kāʻei kua o physical haʻawina. Oia ka moku'āina ua pu ma ka walaau ole, a hele pinepine mai breaths. Ke kao keiki hoʻomaka e hea aku, lilo loa restless. Cyanosis i nā maoli. E like me ka rula, ia he kahua ua malama i na keiki opiopio, a me ka loa haʻi symptoms ikeia ma waena o nā makahiki o ka elua a me eha malama.

Inā tetralogy o Fallot i loko o ke keiki ua pu ma ka loa ho'āhewa wale, ka mea i alakai i ka mau lethargy a me ke kāʻili 'ana. I kekahi manawa, pathology hiki aku i ka make. Mua keiki kāohiʻeleu hula kekahi manawa iloko o instinctively squat i supposedly ho'ēmi i kā ka systemic venous hoʻi, a hoonui ae i ka oxygen saturation i loko o arterial koko.

XIX. Pathology symptoms - bouts o cyanosis a me ka dyspnea, pinepine pu ma ka poho o ka ike paka i. Ke degere o ka o oxygen deficiency a me ka oolea o ka ho'āhewa wale 'mea pololei e kaukaʻi ma ke degere o ka stenosis o ka pulmonary artery.

Ua E hoi e hoʻomaopopo i ka lapaʻau kiʻi, e like me ka rula, ma laila i kekahi mau kahua o ka hoʻonaʻauao i ke keiki ka maikaʻiʻana, e like me ka pono me kekahi oi loa kaumaha manawa:

• Mai hanau i 6 mahina. Ka mea o ia i loko o kēia wā nō i nā keiki,ʻaʻole loaʻeleu kāohi, a nolaila, cyanosis a me ka hypoxia hōʻike moderately. Maoli ai, keia mea,ʻaʻole e mau ana, na keiki kekahi e ae kokoke mai ka lā mua o ke ola.
• Ka loa paʻakikī ke kahua (mai ka 'inikua oiai ua) i ike i ka au mai ka eono mahina a hiki i elua makahiki. Ke kaua e hoʻomōhala ana i ka wā spasm o ka akau ventricular outflow apana. E like me ka hopena o spasm o ka mea a pau venous koko ua hookuu i loko o ka aorta, i hiki aku ai i ke kūpono 'ana o ka oi loa lolo hypoxia. I hookuu hoouka mai i ke keiki hoʻomaka koke - lilo restless. Mālama cyanosis a me ka dyspnea, kekahi manawa a hiki i ka lae o ka unconsciousness. Pinepine eia cramps. Kēia kaʻina hana, ma ka hapanui o na hihia ia lŘlŘ ma luna o ke kāʻei kua o ka mea naʻau a physical stress.

I ka wā e hiki mai, ka lapaʻau kiʻi hoʻololi iki. Ma mua makahiki, cyanosis mau ulu (kekahi manawa koke, kekahi manawa lohi), akā, ho'āhewa wale aneane loa nalo. Hoemiia tachycardia a me ka dyspnea a me ka pulmonary hana collateral holo puni.

Hoikehonua, ano o kaʻike,

Maoli ai, wale i ka palapala hōʻoia no kauka ke kau aku ia i kaʻike,. Tetralogy o Fallot - he pathology e pono ai i ka ohi ana o ka piha 'inikua mōʻaukala, a hoʻomaka i ka helu o nā haʻawina i i hoopaa ole wale i ke alo o ka congenital, akā, e kekahi kōkua ho oholo i ka kinona o ka maʻi, kona oolea, ka pono ola o ke keiki.

Inā symptoms ia noiʻi:

• Loiloi mua nā electrocardiogram. Iloko o ke kaʻina hana, ka mea, i ke kaulana na hoailona o ka overload o kekahi mau wahi o ka naʻau, e like me hypertrophy. No ka laʻana, nā hualoaʻa i hōʻike sinus maʻi pale a von hihia.
• Tetralogy o Fallot - he pathology ana e ike hiki ma umauma m-Ray. No ka laʻana, ua pu keia kina ma ka bulging o ka piʻo o ka hema a me ka akau ventricles, aia no he hiohiona depletion o pulmonary kumu. I keiki na kamalii uuku, o ka naau ma ka radiograph in shape pinepine kohu he slipper.
• Muli o kona kulana informative iaoia mea echocardiography. I loko o kēia like hiki pololei manaʻo i ka nui o ka ventricle akau, e like me ka hooholo i ke 'ano o ka pathology, e loiloi i ka degere o ka hou o ka auoiaea pele. E ho ohana i keia ano ki ina hana hookolokolo aku la ia naau kiwikā, pakahi aku la ia i ka aortic kiwikā ao ka mau ana o ka nohona kiwikā, e ana i ke anawaena o ka fibrous apo. Ka mea ka mea ina oe e e huai ae aortic stenosis, a kamaʻilio aorta, ma ka pau ana o ka iapaau ana i i complications, e like me pulmonary edema.

E like me ka rula, ua lawa nā haʻawina i ho'ākāka 'ia ma luna, e ike ai i ko ke keiki malformation mea i loaʻa. Tetralogy o Fallot, nae, - i kaʻike, e i kekahi manawa loa paʻakikī, a me ke koi i kekahi mau kaʻina hana.

No ka laʻana, ua i kekahi manawa lawe mai coronary angiography. Kēia ke kaʻina hana no ka pono e hooholo i ka kūlike loa wahi o ka coronary a me ka nui o na moku, kaha ina he mea kekahi hui pu anomalies, e like me ka loaʻa mai like ke 'ano o ka naʻau maʻi.

Catheterization - he oi huikau hana e komo ai e hou ana i ka naau cavities. Ua like i lawelawe i loko o ka hanana i au pono e ana i ka puʻe i loko o ka ventricle akau, ho oholo i ka degere o ka oxygenation o ke koko i loko o ka aorta.

Tetralogy o Fallot: iapaau ana

Ka iapaau ana regimen pili kēia hilinaʻi nui ma luna o ke kinona o ka maʻi a me kona oolea. Lāʻau lapaʻau kōkua e hoʻopau ho'āhewa wale a me ka pale aku i kekahi mau complications pili me ka o oxygen deprivation.

Nō kāu, nā pēpē i hoakaka prostaglandin infusion. Kēia ke kaʻina hana no kekahi manawa, e kokua i ka wehe hou arterial leki uea. Inā kekahi kamaiki i hiki na hoailona o ke kaua, ka mea, ua waiho no laila kaomi mea kuli i kou umauma. Mua na keiki hana squats - keia oweliweli kōkua pale hypoxemic hoouka.

Paipai intravenous fluids e hoopiha i ke koko ka leo. Ma kekahi mau hihia, opiopio e hoomanawanui i hoakaka Morphine. Inā pono, ua mahuahua ka ke koko, ma ka phenylephrine a me ka ketamine (ka waiwai i ka olelo, a me ka mai o kekahi kiaha dala hopena). Hōʻole 'relapses i kekahi manawa e kokua propranolol.

I kekahi manawa, cupping lawe o oxygen Inc, akā, e pono ke kūʻai 'ana i kumumanaʻo ana i ke kanawai o keia iapaau ana mea he mehana.

Palliativeʻoki kino

Maoli ai, likeʻole ai 'ke hooluolu aku i ka keiki ka ke ano. Eia naʻe, lapaʻauʻia hiki ole ke hoola i ka hoʻomanawanui mai ia i kekahi mea pepehi weliweli maʻi like tetralogy o Fallot. Hana mea ma kahi loihi ka wale ala, e hoʻopau i ka kina. Waleʻoki kino ke haawi aku i ke keiki i ka manawa no ka maʻamau e ulu ana mai a me ka hooko ana i ke ola.

I kēia lā, loaʻa nō nā papa kuhikuhi ano oʻoki lāʻau lapaʻau lapaau. Palliativeʻoki kino mea, e hoʻokō i acee? Iuo oeiia mezharterialnyh ioauoa e leie aku oe, e hoihoi i ka holo. No ka laʻana, ka loa hanohano kaʻina hana i keia la o ke kau o ka podklyuchichnolegochnogo anastomosis. Iloko o ke kaʻina hana, ka subclavian artery hoʻohana i ka kūikawā e kanu pūʻia hui me unilateral pulmonary artery.

'ano'ēʻoki kino me ka iapaau ana

Kauka paipai aku me ka hikiwawe e loaa, e lawe mai'ano'ēʻoki kino, e like me ka mea e hāʻawi mai ai mea maikaʻi i nā hualoaʻa. Nō kāu, e like me ka hana ua lawe mai ma waena o nā makahiki o 6 mahina i 3 makahiki - i loko o kēia wā, he mea ho'āno loa ka hoʻokō (ina ka hou kaʻina hana o ka maturation a me ka ulu ana o ke keiki, ua noonoo).

'ano'ē lapaʻau mea e hoʻokō 'ia i ka? Iaea o'āno'ē hale. E ho ohana i one surgeons pani i ka ventricular septal kina, pulmonary hoomaka stenotic hapa o ka artery. O ka holo ana, ke kaʻina hana ka huikau, akā, no ka mea,ʻaʻole e paʻa wale nō ka palapala hōʻoia no naʻau surgeon.

Tetralogy o Fallot: ka prognosis o nā mea maʻi

O ka papa, mua o nā mākua hoihoi ka manaʻolana 'ia me ia i ka maʻi. Tetralogy o Fallot i keiki - he loa mea pathology, kāpae'ōlelo ia i loko o kekahi hihia ole i ke Akua.

Ma ka hoʻomaopopo aku i ka papa o ka maʻi hilinaʻi nui ma luna o ka oolea o pulmonary stenosis. Inā mākou e kamaʻilio e pili ana i ka 'ikepili helu, laila, Eia naʻe, aneane he hapaha o na keiki e make i loko o kā lākou makahiki mua. Meʻoki kino, ka awelika makahiki o na keiki e noho ana me ka maʻi, he 12 makahiki. Wale ma kahi o 5% noho mai i ka 40 makahiki me diagnosed like tetralogy o Fallot. Kīnā o ke kino, developmental pilikia, ikaika hooponopono a me ka poe ilihune ma Hawaiʻi nei - keia mea i kekahi mea me ka i, Eia naʻe, i ka maka makua nā mea maʻi.

Ke 'ano like, o ka papa, he mea. Mai poina e pili ana i kaʻoki lāʻau lapaʻau lapaʻau, a ua pinepine hoakaka no na keiki me kaʻike, o "tetralogy o Fallot." Ma hope oʻoki kino, keiki pele ola koke ulu maʻamau, a hiki hiki ke pale aku i ka māluhiluhi hoounauna. I kekahi 'he kumu -ʻoki kino hope i loko o ke ola, aole ia e haawi i ka maikaʻi i nā hualoaʻa ma ka wā e hiki mai, e like me ka hana i hoʻokō iʻuʻuku mai.

Nā mea maʻi i kēia kāna hana, a ma hope o ka pau ana o Inc E i regular checkups ma ka naau surgeon a me ka cardiologist. Ma mua o kekahiʻoki lāʻau lapaʻau a me kahua niho ke kaʻina hana, e hoomanawanui i pono undergo kūikawā lapaʻau i mea e pale aku i ka pilikia o ka endocarditis. Maoli ai, ma laila nō nā koi, kapu i ke ahonui o ka ola, a ke kauka ua koi 'ia e hai.