I-Endocardial cushion defect

I-Endocardial cushion defect (ECD) yisimo senhliziyo esingajwayelekile. Izindonga ezihlukanisa wonke amakamelo amane enhliziyo zakhiwe kabi noma azikho. Futhi, ama-valve ahlukanisa amakamelo aphezulu naphansi enhliziyo anokukhubazeka ngesikhathi sokwakheka. I-ECD yisifo senhliziyo esizalwe naso, okusho ukuthi sikhona kusukela ekuzalweni.

I-ECD yenzeka ngenkathi ingane isakhula esibelethweni. Ama-cushion e-endocardial yizindawo ezimbili ezijiyile ezikhula zibe izindonga (septum) ezihlukanisa amakamelo amane enhliziyo. Futhi zakha ama-mitral kanye ne-tricuspid valve. Lawa ngama-valve ahlukanisa i-atria (amakamelo aphezulu okuqoqela) kuma-ventricles (amakamelo okupompa phansi).

Ukuntuleka kokuhlukaniswa phakathi kwezinhlangothi zombili zenhliziyo kudala izinkinga eziningana:

• Ukunyuka kwegazi okuya emaphashini. Lokhu kubangela ukukhuphuka kwengcindezi emaphashini. Ku-ECD, igazi ligeleza ngokuvuleka okungavamile ukusuka kwesobunxele kuye kwesokudla senhliziyo, bese kuya emaphashini. Ukugeleza kwegazi okwengeziwe emaphashini kwenza umfutho wegazi emaphashini ukhuphuke.
• Ukwehluleka kwenhliziyo. Umzamo owengeziwe odingekayo wokumpompa wenza inhliziyo isebenze kanzima kunokujwayelekile. Imisipha yenhliziyo ingakhulisa futhi inciphise. Lokhu kungadala ukuvuvukala enganeni, izinkinga ekuphefumuleni, nobunzima ekondleni nasekukhuleni.
• ICyanosis. Njengoba umfutho wegazi ukhuphuka emaphashini, igazi liqala ukugeleza lisuke ngakwesokunene senhliziyo liye kwesobunxele. Igazi elingenawo umoya-mpilo lihlangana negazi elinothile we-oxygen. Ngenxa yalokho, igazi elinomoya-mpilo omncane kunokujwayelekile likhiphelwa emzimbeni. Lokhu kubangela i-cyanosis, noma isikhumba esiluhlaza okwesibhakabhaka.

Kunezinhlobo ezimbili ze-ECD:

• Qedela i-ECD. Lesi simo sifaka i-atrial septal defect (ASD) kanye ne-ventricular septal defect (VSD). Abantu abane-ECD ephelele bane-valve eyodwa enkulu kuphela yenhliziyo (i-AV evamile) esikhundleni sama-valve amabili ahlukile (i-mitral ne-tricuspid).
• I-ECD engaphelele (noma engaphelele). Kulesi simo, kukhona i-ASD, noma i-ASD ne-VSD kuphela. Kunama-valve amabili ahlukile, kepha eyodwa yawo (i-mitral valve) ivame ukungajwayelekile ngokuvuleka ("cleft") kuyo. Lokhu kukhubazeka kungavuza igazi emuva nge-valve.

I-ECD ixhunyaniswe kakhulu ne-Down syndrome. Izinguquko eziningana zofuzo zixhunyaniswa ne-ECD. Kodwa-ke, ayaziwa imbangela ngqo ye-ECD.

I-ECD ingahle ihlotshaniswe nokunye ukukhubazeka kwenhliziyo okuzalwa, njenge:

• I-ventricle yesokudla ephindwe kabili
• I-ventricle eyodwa
• Ukuguqulwa kwemikhumbi emikhulu
• I-Tetralogy of Fallot

Izimpawu ze-ECD zingafaka:

• Amathayi ezingane kalula
• Umbala wesikhumba o-Bluish, owaziwa nangokuthi i-cyanosis (izindebe nazo zingaba luhlaza okwesibhakabhaka)
• Ukondla ubunzima
• Ukwehluleka ukukhuluphala nokukhula
• I-pneumonia ejwayelekile noma izifo
• Isikhumba esiphaphathekile (i-pallor)
• Ukuphefumula ngokushesha
• Ukushaya kwenhliziyo okusheshayo
• Ukujuluka
• Imilenze evuvukele noma isisu (ezingavamile ezinganeni)
• Ukuphefumula okunenkinga, ikakhulukazi ngesikhathi sokudla

Ngesikhathi sokuhlolwa, umhlinzeki wezokunakekelwa kwempilo cishe uzothola izimpawu ze-ECD, kufaka phakathi:

• I-electrocardiogram engavamile (ECG)
• Inhliziyo ekhulisiwe
• Inhliziyo ibubula

Izingane ezine-ECD engaphelele kungenzeka zingabi nazo izimpawu noma izimpawu zesifo ebuntwaneni.

Ukuhlolwa kokuthola i-ECD kufaka:

• I-Echocardiogram, eyi-ultrasound ebuka ukwakheka kwenhliziyo nokugeleza kwegazi ngaphakathi kwenhliziyo
• I-ECG, elinganisa umsebenzi kagesi wenhliziyo
• I-x-ray yesifuba
• I-MRI, enikeza isithombe esiningiliziwe senhliziyo
• I-catheterization yenhliziyo, inqubo lapho kufakwa khona ithubhu elincane (i-catheter) enhliziyweni ukubona ukugeleza kwegazi nokuthatha izilinganiso ezinembile zomfutho wegazi namazinga e-oxygen

Ukuhlinzwa kuyadingeka ukuvala izimbobo eziphakathi kwamagumbi enhliziyo, nokwakha ama-valic tricuspid ahlukile kanye nama-mitral valves. Isikhathi sokuhlinzwa sincike esimweni sengane nobukhulu be-ECD. Kungenziwa kaningi lapho ingane inezinyanga ezi-3 kuya kwezi-6 ubudala. Ukulungisa i-ECD kungadinga ukuhlinzwa okungaphezu kokukodwa.

Udokotela wengane yakho angakunikeza umuthi:

• Ukwelapha izimpawu zokwehluleka kwenhliziyo
• Ngaphambi kokuhlinzwa uma i-ECD igulise ingane yakho kakhulu

Imithi izosiza ingane yakho ukuthi ikhuluphale futhi ibe namandla ngaphambi kokuhlinzwa. Imithi evame ukusetshenziswa ifaka phakathi:

• I-diuretics (amaphilisi wamanzi)
• Izidakamizwa ezenza inhliziyo ivumelane ngamandla, njenge-digoxin

Ukuhlinzwa kwe-ECD ephelele kufanele kwenziwe onyakeni wokuqala wokuphila kwengane. Ngaphandle kwalokho, ukulimala kwamaphaphu okungenzeka kungakwazi ukuhlehliswa kungenzeka. Izingane ezine-Down syndrome zivame ukuthuthukisa isifo samaphaphu ngaphambi kwesikhathi. Ngakho-ke, ukuhlinzwa ngaphambi kwesikhathi kubaluleke kakhulu kulezi zingane.

Ukuthi ingane yakho yenza kahle kangakanani kuya ngokuthi:

• Ubunzima be-ECD
• Impilo ephelele yengane
• Ukuthi isifo samaphaphu sesivele sivele yini

Izingane eziningi ziphila impilo ejwayelekile, esebenzayo ngemuva kokulungiswa kwe-ECD.

Izinkinga ezivela ku-ECD zingafaka:

• Ukwehluleka kwenhliziyo yokubopha
• Ukufa
• Isifo se-Eisenmenger
• Umfutho wegazi ophezulu emaphashini
• Ukulimala okungenakulungiseka emaphashini

Izinkinga ezithile zokuhlinzwa kwe-ECD kungenzeka zingaveli ingane ize ikhule. Lokhu kufaka phakathi izinkinga zesigqi senhliziyo kanye ne-mitral valve evuzayo.

Izingane ezine-ECD zingaba sengozini yokutheleleka kwenhliziyo (i-endocarditis) ngaphambi nangemva kokuhlinzwa. Buza udokotela wengane yakho ukuthi ngabe ingane yakho idinga ukuthatha ama-antibiotic ngaphambi kwezinqubo ezithile zamazinyo.

Shayela umhlinzeki wengane yakho uma ingane yakho:

• Amathayi kalula
• Unenkinga yokuphefumula
• Inesikhumba noma izindebe eziluhlaza okwesibhakabhaka

Futhi khuluma nomhlinzeki uma ingane yakho ingakhuli noma izuza isisindo.

I-ECD ixhunyaniswe nokungajwayelekile kwezakhi zofuzo. Izithandani ezinomlando womndeni we-ECD zingafisa ukuthola ukwelulekwa ngezakhi zofuzo ngaphambi kokukhulelwa.

Ukukhubazeka komsele we-Atrioventricular (AV); Isici se-septal se-atrioventricular; I-AVSD; I-AV orifice ejwayelekile; I-Ostium primum atrial septal defect; Ukukhubazeka kwenhliziyo okuzalwa - i-ECD; Ukukhubazeka kokuzalwa - i-ECD; Isifo seCyanotic - ECD

• Isici se-septal se-Ventricular
• Isici se-atrial septal
• Umsele we-Atrioventricular (i-endocardial cushion defect)

I-Basu SK, iDobrolet NC. Ukukhubazeka kokuzalwa kwesistimu yenhliziyo. Ku: Martin RJ, Fanaroff AA, Walsh MC, ama-eds. UFanaroff kanye neMithi kaMartin's Neonatal-Perinatal Medicine. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2020: isahluko 75.

I-Ebels T, iTretter JT, i-Spicer DE, no-Anderson RH. Amaphutha we-septal antroventricular. Ku: Wernovsky G, Anderson RH, Kumar K, et al. I-Anderson's Pediatric Cardiology. Umhla wesi-4. IPhiladelphia, PA: Elsevier; 2020: isahluko 31.

IKliegman RM, iSt. Geme JW, iBlum NJ, i-Shah SS, iTasker RC, iWilson KM. Isifo senhliziyo esizelwe yi-Acyanotic: izilonda ezisuka kwesokunxele ziye kwesokudla. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 453.