Ebstein ka Anomaly: 'ehaʻeha ka, Symptoms,ʻike, lapaʻau

Kekahi o ka pohihihi keia naau mā o Ebstein ka anomaly. He He congenital maʻi i loko i ka tricuspid naau kiwikā o ua hoʻoneʻe i loko o ka ventricle akau, aole hoi ka mea pono ia ma luna o ka palena ma waena o ka atria a me na ventricles. He me ka nui loa haʻahaʻa koko.

anatomical hiʻona

Ebstein ka anomaly hiki aku ai i ka mea i ka lua o ka ventricle akau lilo uuku, a me ka atrium'ākau - oi ma mua o ke kūmau. Puapua kiwikā o kekahi oko. Ka mea, i ke improperly ulu a hoʻoneʻe. Ua Nyos i ke ana a me nā mea a pau chordal a me ka muscular nenoaia, i kipaku ka tricuspid i hoʻomākaukauʻia.

Aie i ka mea uuku ka nui o ka ventricle pono, mai ka mea kī ana emi nui o ke koko. A me ka mahuahua nui o ka atrium pono no i ka mea i ka mea loaʻa hapa o ka venous koko.

Ma na mea he nui nā mea maʻi me Ebstein ka anomaly a diagnosed me atrial septal kina a me ka iaoaioa foramen ovale. Kēia collateral hewa no ka nui loa o ke ola-olaʻi. Ma septal hewa, mai ka awa akau atrium shunt ia lŘlŘ. Oiaio, i ke venous koko i loko o ka lima hema atrium mea kawili pu ia me arterial. KŰia ke kumu no ka oxygen pololi o nā'aʻaʻa a me nā loko.

Nā kumu o ka maʻi

Emi iho malalo o 1% o kanaka me ka congenital naau maʻi diagnosed Ebstein ka anomaly. Kumu no kona ulu hiki ole ke manao pololei. E like me nā mea mahuʻi, eia kahi mea akaka ma muli o ke aaiaoe anomalies. Nō hoʻi, i loko o kekahi mana, no ka mea, he kūpono 'ana o ka naau Muscle hiki ka hopena i? Eaia i hāpai keiki hoomakaukau ana i loaʻa ka likiuma paakai.

Pu ma ka congenital maʻi i loko o 50% o ka hihia o atrial septal kina. Ma nā hihia, he mea kue o ka pāpana. E maopopo ke kumu malaila i kekahi hui malu pu ana o ka naau mā, kauka i ole i pomaikai ae la lakou.

Hiki Lolina o ka maʻi

Ebstein ka anomaly mea he pilikia congenital naʻau maʻi. Ua lŘlŘ i loko o nā keiki kāne a me nā kaikamāhine a pau. Maʻi papa a me ka prognosis e hilinaʻi ma ka degere o deformation o ka tricuspid kiwikā. I nui pathophysiological loli i i wahi ma ka atrium akau a me ka ventricle.

Ma kekahi mau hihia, ke kina ma ka mea naha o ka fetal holo. Aia ka naau e hemahema, hydrops e hoʻomōhala ai, a ma ka 27% o ka hihia ana i loko o utero ka make ana o ke keiki. Me ka hohonu pathology ma hope o ka hanau ana o ke keiki make i loko o ka malama mua o ke ola. Wahi a ka 'ikepili helu, keia mea ma kahi o 25% o nā keiki a me Ebstein ka anomaly i' akomi i. I kaʻike, a hāpai keiki hiki ke hoʻouka kekahi a hiki i ka hebedoma 20th.

About 68% o nā keiki i kēia kāna hana e make ai imua o na malama eono, a i ka 5 makahiki - 64% o nā keiki. Kēia pūʻulu nā keiki, ka poe i ka hana o ka tricuspid kiwikā a me ka ventricle'ākau mea kupono. Ka mea, e make i loko o ka hapanui manawa ma muli o ka holumua ai ka naʻau o ka hoʻokō 'ole a me ka arrhythmias.

Na Mea Maluna o ke ano o ka maʻi

Poe akamai i kuhikuhi i kekahi mau kuʻina o ka maʻi, i ike like Ebstein ka anomaly. Nā symptoms e hilinaʻi ma ka nui naʻau ole. Ka loa pohihihi o ka asymptomatic ke kahua. Ua hoʻomanawanui iki mai ke ole e manao ka pathology, a alakai i ka maʻamau ola. Ka mea, e lawe maikai hoounauna.

Ma ka lua o ke kahua lapaʻau hoike ana'ōlelo ai. Kēia anomaly ikeia ma kamalii mai, a lawe paakiki kupono. Kaawale loaa hou kapuwai o ka:

- II he - ka mea, ua wehewehe aku ma ka wa e kaawale aku o cardiac arrhythmias;

- II o ka e - ho'āhewa wale i pinepine.

Ke kolu o ke kahua ua kapaʻia ka wā o kona koʻikoʻi decompensation. Ua Ua diagnosed ka wā i ke kino, ua hiki ke compensate no ka ha awina o ka naʻau ma kekahi servo-ʻano papa hana.

Ka lapaʻau kiʻi o ka maʻi

I hanau, na keiki a me Ebstein ka anomaly wehewehe aku ma ka cyanosis. ua hoemiia Cyanosis hope 2-3 mahina o ke ola ma muli o ia ka mea i ua hoemiia ka pulmonary vascular e kū'ē'ē mai. Akā, i loko o na keiki me ka hōʻemi iki mā ma cardiac septum maoli i ka pilikia o ka make, mai complications o cyanosis a me ka holumua ai ka naʻau 'ole.

Ma na keiki me Ebstein ka anomaly mea pinepine malama i kēia mau hōʻailona:

- pokole o ka hanu, a hiki i hoʻomaha manawa;

- edema, i hiki ke ike ma ka laloʻaoʻao;

- luhi, o ka oi aku kekahi 'iloko o physical exertion;

- naau pāpana kāna hana;

- cyanosis o ka ili a me ka lehelehe.

Cyanosis i ke keiki mua symptoms o ka naʻau ole. Mua keiki e ohumu a recurrent bouts o tachycardia.

ʻike, no ka maʻi

Ma kekahi mau hihia, i ka maʻi me ka nānā 'i hāpai keiki a koke ma hope o ka hānau i loko o ka haukapila. Aia i kekahi mau ano helehelena, a i kou kauka i hāʻupu aʻe i ke keiki Ebstein ka anomaly. ʻike, nā auscultation, umauma radiography, echocardiography, EKG. A pau o kēia mau epekema ma ka hui pu ana, ka ho'ā pololeiʻike.

Auscultation kauka i lohe i ka kiʻiona pāpana, ka mea hiki ia he three- a eha-pa. He mokuhia mai i ka kiʻekiʻe noho malie systolic ohumu o tricuspid insufficiency. Eia hou kekahi, haʻi o ka splitting o ka lua o ka leo, ka mea, 'aʻole e hoʻololi i hanu. Ma ka luna o ka haʻalele kae o ka sternum E e lohe mezodiastolichesky malie squeaky leo.

Ma radiography visualized Hoʻonui i ke cardiac malu, keia mea aie a hiki i ka nui mahuahua ma ka atrium akau. Pulmonary vascular kumu nō kāu haikea. Heart pinepine i ke ano o ka poepoe.

Loli mālama i loko o ka ECG. Ma ka ECG hōʻike hoailona e hoike ana hypertrophy o ka atrium akau, ka PQ wā mawaena ua kikooia aku, a me he mea huina a me hapa blockade o ka pono laulau lālā aeie mea ulana lole.

Ma echocardiography ke ike i ke ākea puka o ka tricuspid kiwikā. Septal huʻa ua biased i ka luna.

ʻO nā kaʻa mokulele ma congenital anomalies

Ma hope o kaʻike, a me ka hooholo i ka degere o ka lukuʻia i koho lapaau. O ka holo ana, ua hoʻomanawanui iki mai ponoʻoki lāʻau lapaʻau 'uao. Ke wale, koe nae ka mea he uuku pūʻulu o nā mea maʻi me Ebstein ka anomaly mea asymptomatic.

Lapaʻau ua lawe mai ke kaumaha ma muli'ē aʻe nā nīnūnē. Inā he hoomanawanui maʻamau ola wā kamaliʻi, i ka hana hiki eʻula a hōʻea i ka manawa a ka mea i haʻi symptoms o ka naʻau ole.

Nā keiki a me kēia pilikia i ke kiai pediatric cardiologist e specializes i loko o congenital naʻau maʻi. A me kona kūlana E e ua hoʻonānā cardiac surgeons.

Inā na nā mea maʻi i hoikeia ka ho'ēmiʻana i loko o pulmonary koko, a me na hoailona o ka naau ka hoʻokō 'ole, ka infusion i'ōleloʻia ai ionotropic ai' prostogladinov pūʻulu E. nō hoʻi e hōʻike 'malama i ka iaea o metabolic acidosis. Oia lapaʻau hiki hoonui cardiac auaiaea, a emi hema ventricular mīkini'opihia kaʻaoʻao ÿö lima'ākau o.

Ma na wahi he mea he tachycardia, ia mea e pono ke lawe mai kūikawā kanaka antiarrhythmics.

Nāʻikepili no kaʻoki kino

Ma ka neonatal wā ho'āʻo mai e hana i ka hana. Akā, he mea pono i ka hihia ma ka akau ventricular dysplasia Ua haʻi, a ua ukali ma ka haunaele o antegrade koko pele i loko o ke akemāmā Kanal. I ka Ia manawa he mea heʻoi? Anoe o ka naau pono, a me ka i ka wā hoʻokahi mīkini'opihia o ka ventricle hema.

No ka pili contraindications nā makahiki o 4-5 makahiki. Akā, inā he pono, e ka hana a me nā pēpē. Ua 'ole' ia iʻoki kino a me ka poe i hoʻomaka 'ana ke aloha irreversible loli ma ka na nā loko.

E hoomanawanui i ka poe Ebstein ka anomaly i ike, aole e hana ke ola i ka awelika o ka 20 makahiki. Ko lakou make ka IeAUPIIe, IAa IOʻana. Ua lŘlŘ manawa e ventricular fibrillation o ka naau.

ka hana

Wale maʻoki kino hiki loa paʻipaʻi i ke kulana. Ma keia hihia, kauka paipai aku ina he mea hiki, e hana ia i ka mua makahiki no ka mea, o ka oi uuku naʻau nui ma kaʻuʻuku mai.

Ke hana ua hana hamama-naauʻoki kino, no kona nā papaha o e hoʻolālā i ke kaʻina hana o ka cardiopulmonary holo alaʻokoʻa. Oi sutured hapa o ka atrium akau, a me ka tricuspid kiwikā o na nakaka e pii ai a hiki i ka oihana i mea kokoke i ka maʻamau. Ma na wahi i kēia mea,ʻaʻole e hiki, ka mea, ua wehe 'a auou caiaiai me ka prosthesis. Ua apono 'oe, e hoʻopoina e pili ana i ka mea i ka hoʻomanawanui i Ebstein ka anomaly. I kanaka makua nā mea maʻi i hoʻomaka prosthetics, akā, na keiki ma lalo o 15 makahiki i ea kiwikā.

Make nei i hana, aole ia i oi aku mamua o 2-5%. Ka pahiki o ka make hilinaʻi nui ma luna o ka hana ana o ka naau surgeon, a mai ka hope umekaumaha. Kokoke 90% o nā mea maʻi i loko o ka papa o ka makahiki hiki hoʻi i ka maʻamau ola.

Mau noonoo E e haawiia i ka mea e hoomanawanui i anesthesiologists. Ma hope o nā mea a pau, e pono i ka hana e hoʻokokoke i nā mea maʻi i Ebstein ka anomaly. Hiʻona o ka anesthesia E e ike ai i ke kauka. It E e kaʻikaʻiʻia ma ka manao i puʻe i loko o kēia mau hoʻomanawanui iki mai i e lolelua. No laila, i ka anesthesiologist nā kahu nānā i kona hoʻomanawanuiʻana, a ma hopeʻoki kino.

'ano o nā dentures

Ma ka hapanui o na, e hooholo pehea ia e e lawe mai i ka hana, he mea hiki wale i ka wa a ka hoʻomanawanui ka ua ma luna o ka pae'ōnaehana papa. Inā he mea hiki, e hoopaa i ka nohona kiwikā plasty, ua haawiia mai i keia koho koho. Akā, he mau wahi, i hiki i ka wa a ka prosthesis ua koi 'ia i loko o nā mea maʻi i i Ebstein ka anomaly. No laila, i kapaʻiaʻimi hoʻopunipuni kiwikā, a nana e like me ka ke komo lima, i hoʻokū pono ia e ka kemikala uluna. Loko o ia mea heʻano papa hana i hiki ke wehe a pani i ka ai-kapaia'īpuka. Ua hiki e me mechanical paha liluwelo. Keʻano mua ua i o ka titanium oka a pau, a me ka lua o ke e i o ka nohona kiwikā o ka puaa, a me kanaka cardiac'aʻaʻa pālule,.

A hiki aku 'oe i kekahi me mechanical kiwikā hoomanawanui pono mau inu laau lapaau, koko thinners e hoʻouka, akā, adherence, e hana ka mea, hiki hope hou. Liluwelo lima kūlana kiʻekiʻe Ua emi mau nō.