Hypoplastic kuruboshwe moyo chirwere

Hypoplastic left moyo syndrome inoitika kana mativi erutivi rweruboshwe rwemoyo (mitral vharafu, kuruboshwe ventricle, aortic vharuvhu, uye aorta) hazvikure zvakakwana. Mamiriro acho aripo pakuzvarwa (kuberekwa).

Hypoplastic kuruboshwe moyo ndiyo isingawanzo mhando yechirwere chepfungwa yemoyo. Izvo zvakajairika muvanhurume kupfuura vakadzi.

Sezvo nekuwanda kwekuzvarwa kwemoyo hurema, hapana chinozivikanwa chikonzero. Inenge gumi muzana yevana vane hypoplastic vakasara moyo syndrome vane zvakare zvimwe zvinokanganisa kuberekwa. Iyo inosanganiswazve nezvimwe zvirwere zvemajini zvakaita seTurner syndrome, Jacobsen syndrome, trisomy 13 na18.

Dambudziko rinokura usati wazvarwa apo ventricle yekuruboshwe nezvimwe zvivakwa zvikasakura zvakanaka, kusanganisira izvo:

• Aorta (iro reropa rinotakura ropa-rakazara ropa kubva kune yekuruboshwe ventricle kuenda kumuviri wese)
• Kupinda uye kubuda kweiyo ventricle
• Mitral uye aortic mavharuvhu

Izvi zvinokonzeresa ventricle yekuruboshwe uye aorta kuti inyatso kukura, kana hypoplastic. Muzviitiko zvakawanda, kuruboshwe ventricle uye aorta zvidiki zvakanyanya pane zvakajairwa.

Muvacheche vane chirwere ichi, kuruboshwe kwemoyo hakugone kutumira ropa rakaringana kumuviri. Nekuda kweizvozvo, kurudyi kwemoyo kunofanirwa kuchengetedza kutenderera kwemapapu nemuviri. Iyo ventricle chaiyo inogona kutsigira kutenderera kune ese mapapu nemuviri kwechinguva, asi iri rekuwedzera basa rekupedzisira rinozokonzeresa kurudyi kwemoyo kutadza.

Iyo chete mukana wekupona kubatana pakati pekurudyi nerutivi rweruboshwe rwemoyo, kana pakati petsinga uye mapurmonary arteri (tsinga dzeropa dzinotakura ropa kuenda kumapapu). Vacheche vanowanzozvarwa vaine maviri eaya kubatana.

• Foramen ovale (gomba pakati peiyo kurudyi uye kuruboshwe atrium)
• Ductus arteriosus (shoma reropa rinobatanidza iyo aorta kune pulmonary arteri)

Ose akabatana aya anowanzozvivharira ega mazuva mashoma mushure mekuzvarwa.

Muvacheche vane hypoplastic left moyo syndrome, ropa rinosiya kurudyi kwemoyo kuburikidza nepurmonary arteri inofamba nepaductus arteriosus kuenda kuorta. Iyi ndiyo chete nzira yekuti ropa risvike kumuviri. Kana iyo ductus arteriosus ichibvumidzwa kuvhara mumucheche ane hypoplastic left moyo chirwere, mwana anogona kukurumidza kufa nekuti hapana ropa richaputirwa kumuviri. Vacheche vane inozivikanwa hypoplastic yakasara yemoyo syndrome inowanzo kutanga pamushonga wekuchengetedza iyo ductus arteriosus yakavhurika.

Nekuti pane kuyerera kushoma kana kusabuda kubva pamoyo wekuruboshwe, ropa rinodzokera kumoyo kubva mumapapu rinoda kupfuura nepamuforamu ovale kana atrial septal defect (gomba rinobatanidza dzimba dzekuunganidza kuruboshwe uye kurudyi mativi emoyo) dzokera kurudyi kwemoyo. Kana pasina foramen ovale, kana kana iri diki kwazvo, mwana anogona kufa. Vacheche vane dambudziko iri vane buri pakati peatria yavo yakavhurwa, kungave nekuvhiyiwa kana kushandisa chubhu, inoshanduka chubhu (moyo catheterization).

Pakutanga, mwana achangozvarwa ane hypoplastic reruboshwe moyo anogona kunge akajairika. Zviratidzo zvinogona kuitika mumaawa mashoma ekutanga ehupenyu, kunyangwe zvingangotora kusvika kumazuva mashoma kuti uve nezviratidzo. Zviratidzo izvi zvinogona kusanganisira:

• Bluish (cyanosis) kana isina ruvara rweganda
• Maoko anotonhora uye tsoka (magumo)
• Lethargy
• Kupomba kushoma
• Varombo kuyamwa uye kufudza
• Inorova moyo
• Kukurumidza kufema
• Kufema kwekufema

Mune vacheche vane hutano, ruvara rwebhuruu mumawoko nemakumbo ndiko kupindura kunotonhora (izvi zvinodaidzwa kunzi peripheral cyanosis).

Ruvara rwebhuruu mubhokisi kana mudumbu, miromo, uye rurimi hazvina kujairika (zvinonzi central cyanosis). Icho chiratidzo chekuti hapana oxygen yakakwana muropa. Central cyanosis inowanzo kuwedzera nekuchema.

Kuongorora kwemuviri kunogona kuratidza zviratidzo zvekutadza kwemoyo:

• Inokurumidza kupfuura yakajairwa kurova kwemoyo
• Lethargy
• Kuwedzera kwechiropa
• Kukurumidza kufema

Zvakare, kupisa munzvimbo dzakasiyana siyana (ruoko, kugomera, nevamwe) zvinogona kunge zvisina kusimba. Iko kune kazhinji (asi kwete nguva dzose) moyo usina kujairika unonzwika kana uchiteerera chipfuva.

Miedzo inogona kusanganisira:

• Catheterization yemwoyo
• ECG (magetsi emagetsi)
• Echocardiogram
• X-ray yechipfuva

Kamwe kuongororwa kwehypoplastic moyo wekuruboshwe kwaitwa, mwana anozotambirwa kuchipatara chekubereka kwakanyanya. Muchina wekufema (mweya wekufema) ungangodiwa kubatsira mwana kufema. Mushonga unonzi prostaglandin E1 unoshandiswa kuchengetedza ropa kutenderera mumuviri nekuchengeta iyo ductus arteriosus yakavhurika.

Aya matanho haagadzirise dambudziko. Mamiriro acho nguva dzose anoda kuvhiyiwa.

Kuvhiya kwekutanga, kunonzi oparesheni yeNorwood, kunoitika mukati memazuva ekutanga emwana ekuzvarwa. Iyo nzira yeNorwood inosanganisira kuvaka aorta nyowani na:

• Uchishandisa iyo pulmonary vharafu uye arteri
• Kubatanidza iyo hypoplastic yekare aorta uye coronary arteri kune nyowani aorta
• Kubvisa rusvingo pakati peatria (atrial septum)
• Kugadzira chinongedzo chekugadzira kubva kune chaicho ventricle kana wemuviri arteri kune pulmonary arteri kuchengetedza kweropa kuyerera kumapapu (inonzi shunt)

Musiyano wemaitiro eNorwood, unonzi maitiro eSano, unogona kushandiswa. Maitiro aya anogadzira kurudyi ventricle kune pulmonary artery kubatana.

Mushure meizvozvo, mwana anoenda kumba kazhinji. Mwana anofanirwa kutora mishonga yezuva nezuva uye kunyatsoteverwa nachiremba wevana, anozoona kuti danho rechipiri rekuvhiya rinofanira kuitwa riini.

Stage II yekuvhiya inonzi iyo Glenn shunt kana hemi-Fontan maitiro. Inotumidzwawo seyevapopmonmon shunt. Maitiro aya anobatanidza tsinga huru inotakura ropa rebhuruu kubva pahafu yepamusoro yemuviri (iyo yepamusoro vena cava) yakanangana nemitsipa yeropa kumapapu (pulmonary arteri) kuti uwane oxygen. Kuvhiyiwa kunowanzoitwa kana mwana aine mwedzi mina kusvika kumatanhatu ekuberekwa.

Pakati pematanho I ne II, mwana anogona kunge achiratidzika kunge bhuruu (cyanotic).

Stage III, yekupedzisira nhanho, inonzi maitiro ePontan. Dzimwe tsinga dzinotakura ropa rebhuruu kubva mumuviri (iyo yakaderera vena cava) dzakabatana zvakanangana nemidziyo yeropa kumapapu. Iyo chaiyo ventricle ikozvino inoshanda chete sekamuri yekupomba yemuviri (haisisiri mapapu nemuviri). Kuvhiya uku kunowanzoitwa kana mwana aine mwedzi gumi nemasere kusvika pamakore mana ekuberekwa. Mushure meiyi nhanho yekupedzisira, mwana haasisina cyanotic uye ane yakajairwa okisijeni muropa.

Vamwe vanhu vangangoda kuvhiiwa kwakawanda muma20s kana makumi matatu emakore kana vakakura zvakaoma kudzora arrhythmias kana mamwe matambudziko eiyo nzira yePontan.

Vamwe vanachiremba vanoona kuisirwa pamoyo imwe nzira yekuvhiya nhanho nhatu. Asi pane mashoma akapihwa moyo anowanikwa kune vacheche vadiki.

Kana ikasiiwa isina kurapwa, hypoplastic yakasara moyo syndrome inouraya. Kupona kwehuwandu hwegadziriro yakagadziriswa inoenderera ichikwira sezvo hunyanzvi hwekuvhiya uye kutarisirwa mushure mekuvhiyiwa kuri nani. Kupona mushure mekutanga nhanho inopfuura 75%. Vana vanopona gore ravo rekutanga vane mukana wakanaka kwazvo wekupona kwenguva refu.

Mhedzisiro yemwana mushure mekuvhiyiwa inoenderana nesaizi uye mashandiro eiyo ventricle chaiyo.

Matambudziko anosanganisira:

• Kuvharirwa kweiyo shunt yekugadzira
• Ropa clots iro rinogona kutungamira kune sitiroko kana pulmonary embolism
• Yenguva refu (isingaperi) manyoka (kubva kuchirwere chinonzi protein-kurasikirwa enteropathy)
• Fluid mudumbu (ascites) uye mumapapu (pleural effusion)
• Kutadza kwemoyo
• Zvisina kujairika, zvinomhanya pamoyo mutinhimira (arrhythmias)
• Kurohwa uye kumwe kushushikana kweshungu
• Kukanganiswa kwehutano
• Kufa kamwe kamwe

Bata wako mupi wezvehutano pakarepo kana mwana wako:

• Anodya zvishoma (kuderera kudya)
• Ine bhuruu (cyanotic) ganda
• Iine shanduko nyowani mukufema maitiro

Iko hakuna kuzivikanwa kwekudzivirira kwe hypoplastic left moyo syndrome. Sezvo nezvakawanda zvirwere zvekuzvarwa, izvo zvinokonzeresa hypoplastic left moyo syndrome hazvina chokwadi uye hazvina kubatana nechirwere chaamai kana maitiro.

HLHS; Mwoyo wekuzvarwa - hypoplastic kuruboshwe moyo; Cyanotic moyo chirwere - hypoplastic kuruboshwe moyo

• Mwoyo - chikamu kuburikidza nepakati
• Moyo - pamberi pekutarisa
• Hypoplastic yakasara yemoyo syndrome

Fraser CD, Kane LC. Congenital chirwere chemoyo. Mu: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Chinyorwa Chekuvhiya: Iyo Biological Hwaro hweMazuva Ano Kuvhiya Maitiro. 20th ed. Philadelphia, PA: Elsevier; 2017: chitsiko 58.

Webb GD, Smallhorn JF, Therrien J, Redington AN.Congenital chirwere chemoyo mune mukuru uye wevarwere murwere. Mune: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Chirwere Chemwoyo cheBraunwald: A Chinyorwa cheCardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019: chits. 75.