Ebstein kuti Anomaly: Zvinokonzera, Zviratidzo, nechirwere, Treatment

Rimwe kushoma mwoyo urema ndiro Ebstein kuti anomaly. Ihosha vachiberekwa umo tricuspid mwoyo vharuvhu ndanogara rworudyi ventricle, kunyange zvazvo zvinofanira kuva pamusoro muganhu pakati atria uye ventricles. It zviri kuderedza ropa riyerere.

anatomical zvinhu

Ebstein kwakaita anomaly kunotungamirira ndechokuti mhango rwerudyi ventricle kunova maduku, uye zvakarurama atrium - kupfuura chakaipa nazvo. Anopapama vharafu zvinosiyanawo. Vangave isina kukodzera yakatanga kana misha. It kwakakanganiswa kugadzira uye zvose chordal uye tsandanyama ezvinhu, iyo inobudisa tricuspid midziyo.

Nokuda duku kukura zvakanaka ventricle kubva inongorira shoma shoma ropa. Uye akawedzera ukuru rworudyi atrium nokuti rikawira chikamu venous ropa kuti.

Varwere vakawanda Ebstein kwakaita anomaly uye nechirwere atrial septal kuremara uye bvumidzwa foramen ovale. rubatso ichi kuremara vakawanda upenyu-okuponesa. Kuburikidza septal kuremara kubva ungana kurudyi atrium shunt kunoitika. Chokwadi, venous ropa iri kuruboshwe atrium riri yakavhenganiswa arterial. Izvi zvinokonzera kuti oxygen nenzara pamusoro nyama uye nhengo dzomuviri.

Zvinokonzera chirwere

Asingasviki 1% yevanhu vane vachiberekwa mwoyo chirwere akaonekwa Ebstein kuti anomaly. Zvikonzero kwayo kukura vasingagoni ngatitsunge zvakarurama. Maererano zvekungofungira, zvinoita sokuti nokuda magene anomalies. Uyewo, mumwe shanduro, chakadaro kukura mumwoyo tsandanyama kunogona womuchato panguva nhumbu kugadzirira ane lithium munyu.

Achiperekedzwa imwe vachiberekwa chirwere muna 50% kuti zviitiko atrial septal urema. Mune zvimwe zviitiko, pane kutyora mutinhimira. Kuti tinzwisise nei pane dzimwe mubatanidzwa mwoyo urema, vanachiremba havasati kubudirira.

Possible dzakasiyana chirwere

Ebstein kwakaita anomaly chinhu dzinonetsa chirwere vachiberekwa mwoyo. It unowanikwa vakomana nevasikana zvakafanana. Chirwere kosi uye fungidziro achavaponesa ukuru deformation pamusoro tricuspid vharuvhu. Uyewo pathophysiological kuchinja kunokosha kwakaitika zvakarurama atrium uye ventricle.

Mune zvimwe zviitiko, akaremara zvinhu pakakuvara ari fetus riparadzirwe. Pane mwoyo kutadza, hydrops zvikamuka, uye muna 27% nyaya rwakavapo utero rufu mwana. With yakadzika zvokurwara azvarwa vacheche vanofa mukati rokutanga mwedzi upenyu. Maererano nenhamba, izvi vanenge 25% vana vose Ebstein kuti anomaly akanga dzakaonekwa. The chirwere pamuviri anogona kuiswa kunyange vhiki rechi20.

About 68% kwevacheche vane chirwere ichi kufa pamberi mwedzi mitanhatu, uye kusvika 5 makore - 64% vana. boka iri zvinosanganisira vana vane kushanda tricuspid vharuvhu uye yakarurama ventricle ndiyo inogutsa. Vanofa kakawanda nokuda mberi mwoyo nokukundikana uye arrhythmias.

The kupatsanura pakati zvirwere

Nyanzvi anozivisa nzendo dzakawanda chirwere, rinozivikanwa Ebstein kuti anomaly. Zviratidzo ruchabudirira mwoyo huru sei kukundikana. Chinonyanya kushoma ndiyo asymptomatic pachikuva. Vanaamai vakadaro vanogona kunyange vanofungidzira kuti zvokurwara uye kurarama sezvinongoita. Vanotakura zvakanaka kurovedza muviri.

In yechipiri Danho kunotoda kuratidzwa kutaurwa. anomaly ichi rinowanikwa pauduku uye anotora zvakaoma zvakakwana. Yoga akapora nhanho:

- II mumwe - nayo kunoratidzwa kusavapo cardiac arrhythmias;

- II reruoko - kurwisa zviri kakawanda.

Wechitatu Danho inonzi nguva kuramba decompensation. Zvinonzi chirwere kana muviri haakwanisi nekure basa kwomwoyo chero servo-mashandiro.

The kunotoda Mufananidzo chirwere

Pakuzvarwa, vana vane Ebstein kwakaita anomaly hwokuratidza cyanosis. Cyanosis pashure 2-3 mwedzi upenyu ipfupi nokuti chokuti pulmonary vascular nemishonga ipfupi. Asi vana vane zvishomanana kuremara mu cardiac septum kunowedzera ngozi norufu yematambudziko ose cyanosis uye mberi mwoyo nokukundikana.

In vana vane Ebstein kuti anomaly kazhinji vakacherechedza zviratidzo izvi:

- kupfupika mweya kunyange zororo nguva;

- edema, izvo zvinogona kuoneka rezasi migumo;

- kuneta, kunyanya kuoneka panguva panyama nesimba;

- mwoyo mutinhimira nyonganyonga;

- cyanosis ganda uye miromo.

Cyanosis anooneka vana vasati zviratidzo mwoyo kukundikana. Vana vakura vanogona chokunyunyuta pamusoro idzokororo tichibatwa tachycardia.

chirwere chirwere

Mune zvimwe zviitiko, chirwere akatsunga panguva pamuviri kana nguva pfupi vazvarwa muchipatara. Kune akawanda hunhu features, iyo chiremba wako anogona vanofungidzira kuti mwana Ebstein kuti anomaly. Chirwere zvinosanganisira auscultation, muchipfuva radiography, echocardiography, EKG. Nzira dzose idzi pamwe chete kunoita kuongororwa zvakarurama.

Auscultation chiremba anogona kunzwa nevamwe mutinhimira, inogona mashanu kana mana mudanga. Iye drowns kunze wakanyarara mukuru systolic kutsutsumwa pamusoro tricuspid insufficiency. Uyezve, haana ndiyo kukamurwa rechipiri matauriro, hazvina haachinji panguva kufema. At pamusoro kuruboshwe pamucheto sternum anofanira kunzwika mezodiastolichesky rakanyarara squeaky ruzha.

On radiography mufanidziro kunowedzerwa cardiac mumvuri, ichi imhaka kuwedzera zvakarurama atrium. Pulmonary vascular muenzaniso yemanyorero zvacheneruka. Mwoyo kazhinji anotora chimiro sebhora.

Kuchinja akaona ECG. On the ECG anoratidza zviratidzo kuratidza hypertrophy rworudyi atrium, kuti PQ penguva chinoshanda, uye hapana zvachose kana kukwana blockade rworudyi tsapo yebazi nechigumbuso danda.

On echocardiography anogona kuona rakafara pakuvhurwa tricuspid vharuvhu. Septal kurova-rova iri nekambani kurutivi pamusoro.

The nzira iri vachiberekwa anomalies

Mushure nechirwere uye kuona ukuru kuparadzwa anosarudzwa kurapwa. Zvechokwadi, varwere vakadaro vanoda kuvhiya kupindira. The chete Kunze ndiko kuti boka duku varwere Ebstein kuti anomaly ndiyo asymptomatic.

Treatment rinoitwa zvichienderana dzimwe nyaya inoenderana. Kana murwere kazhinji akapukunyuka paucheche, kuvhiyiwa inogona kumbosiyiwa kusvikira nguva yakadaro kusvikira pane raidudzwa zviratidzo mwoyo kukundikana.

vana vose dambudziko iri kurinda chiremba cardiologist uyo ane unyanzvi mu vachiberekwa mwoyo chirwere. Uye ayo chinzvimbo zvinofanira kuongororwa cardiac vanovhiya.

Kana varwere akaratidza zvinoderedza ropa kuyerera pulmonary uye pane zviratidzo mwoyo kukundikana, asi aizomupa yakatarwa ionotropic zvinodhaka prostogladinov mapoka E. Also kuratidzwa wakabata rakasungwa metabolic acidosis. kurapwa kwakadaro kunogona kuwedzera cardiac goho uye kuderedza rworuboshwe ventricular compression of akatambanudzirwa divi rworudyi.

Muzviitiko apo pane tachycardia, zvinokosha kuita vavashandire antiarrhythmics.

Zvairatidza kunorapwa

In iri neonatal nguva edza kuita oparesheni. Asi zvinokosha zviitiko apo kodzero ventricular dysplasia anotaurwa uye achabatsirwa vachivavhiringidza rimwe antegrade ropa riyerere ari zvemapapu chiteshi. Panguva iyo pane yakapinza rokuwedzera rworudyi mwoyo uye mawa compression pakati ventricle kuruboshwe.

Nokuti hama contraindications zvinosanganisira makore 4-5. Asi, kana zvakakodzera, kuti kuvhiyiwa uye vachangoberekwa. Zvinonzi zvakare haana akaita oparesheni uye vaya vakatanga Organic kusingarapiki kuchinja zvomukati.

Varwere Ebstein kuti anomaly wawanikwa, hapana akavhiyiwa anogona kurarama paavhareji yemakore 20. rufu rwavo Kazhinji kamwe. It kunoitika nokuda ventricular fibrillation mwoyo.

The oparesheni

Chete kuburikidza Kuvhiya anogona kugadzirisa mamiriro ezvinhu zvachose. Muchiitiko chino, vanachiremba vanoti kana zvichibvira kuzviita nenguva mukuru pazera nokuti mwoyo yesaizi achiri mucheche.

The oparesheni rinoitwa Kuvhiya mwoyo, nokuti ayo yaibvira pakuronga muitiro Moyo yokunzvenga. Zvakawandisa mugove sutured rwerudyi atrium, uye tricuspid vharafu vazvo achisimuka chinzvimbo chiri pedyo dzose. Muzviitiko apo zvisingaiti, izvozvo chinobviswa uye vakatsiviwa prosthesis. Izvi zvinobvumira kuti kukanganwa nezvokuti murwere aiva Ebstein kuti anomaly. Varwere vakura vanogona kuitisa prosthetics, asi makore vana pasi 15 okuberekwa akasarudzei epurasitiki vharafu.

Rufu panguva akavhiyiwa asingaiti kupfuudza 2-5%. Mukana rufu kunoenderana zvakaitika mwoyo chiremba, uye kubva mutevedzeri negiravhiti. Vanenge 90% wevarwere mukufamba kwegore anogona kudzokera dzose upenyu.

Pfungwa dzinofanira kupiwa vakadaro varwere Anesthesiologists. Handiti, zvinoda chaizvo nzira varwere Ebstein kuti anomaly. Zvinoumba yokutindivadza zvinofanira kuzivikanwa kuti chiremba. Zvinofanira kurangarirwa kuti kumanikidza Varwere izvi zvingava kugadzikana. Naizvozvo, kuti anesthesiologist monitors varwere vake uye pashure kuvhiyiwa.

marudzi dentures

Kazhinji, kusarudza sei chichaitwa pokuvhiyiwa, zvinokwanisika chete kana murwere ari kare patafura uchishandisa. Kana zvichibvira kubata vharuvhu plasty, zvatinofarira rakapiwa nezvechisarudzo ichi. Asi pane zvinhu kana prosthesis chinodiwa varwere vane Ebstein kuti anomaly. Saka akadana chakagadzirwa vharafu, rinotarira sezvinoita mhete, yakanyatsoiswa kupfurikidza chakaita zvokugadzirwa piro. Inside chiri mashandiro kuti anogona kuzarura uye kuvhara Yaunoti suo. Zvinogona kuva nomuviri kana zvipenyu. Musimboti yokutanga wakaitwa titanium chiwanikwa, uye wechipiri vangava wakaitwa vharafu dzenguruve kana munhu cardiac tishu Shirts.

Kana iwe hwokugadza zvokuimba vharafu murwere unofanira kugara kunwa mishonga, ropa thinners, asi kuomerera ishande chinogona kwenguva yakareba. Biological mudziyo iri shoma yakasimba.