Isifo senhliziyo esine-Hypoplastic

I-Hypoplastic left heart syndrome yenzeka lapho izingxenye zohlangothi lwesobunxele lwenhliziyo (i-mitral valve, i-ventricle engakwesokunxele, i-aortic valve, ne-aorta) zingakhuli ngokuphelele. Isimo sikhona ngesikhathi sokuzalwa (esibelethweni).

Inhliziyo yesobunxele ye-Hypoplastic uhlobo olungavamile lwesifo senhliziyo esizalwe naso. Kuvame kakhulu kwabesilisa kunabesifazane.

Njengokukhubazeka kwenhliziyo okuningi, asikho isizathu esaziwayo. Cishe i-10% yezingane ezine-hypoplastic left heart syndrome nazo zinokunye ukukhubazeka kokuzalwa. Iphinde ihlotshaniswe nezinye izifo zofuzo ezifana ne-Turner syndrome, i-Jacobsen syndrome, i-trisomy 13 ne-18.

Inkinga iba ngaphambi kokuzalwa lapho i-ventricle yangakwesobunxele nezinye izakhiwo zingakhuli kahle, kufaka phakathi:

• I-aorta (isitsha segazi esithwala igazi elicebile oksijini lisuka ku-ventricle engakwesokunxele liye emzimbeni wonke)
• Ukungena nokuphuma kwe-ventricle
• Ama-valve we-Mitral ne-aortic

Lokhu kubangela ukuthi i-ventricle yangakwesobunxele ne-aorta ingakhuli kahle, noma i-hypoplastic. Ezimweni eziningi, i-ventricle engakwesokunxele ne-aorta zincane kakhulu kunokujwayelekile.

Ezinganeni ezinalesi simo, uhlangothi lwangakwesokunxele lwenhliziyo alukwazi ukuthumela igazi elanele emzimbeni. Ngenxa yalokhu, uhlangothi lwangakwesokudla lwenhliziyo kufanele lugcine ukusakazeka kwamaphaphu nomzimba. I-ventricle efanele ingakusekela ukusakazeka kwamaphaphu nasemzimbeni okwesikhashana, kepha lo msebenzi owengeziwe ekugcineni ubangela uhlangothi lwangakwesokudla lwenhliziyo ukuthi lwehluleke.

Okuwukuphela kwendlela yokusinda ukuxhumana phakathi kwesokudla nesinxele senhliziyo, noma phakathi kwemithambo nemithambo yamaphaphu (imithambo yegazi ehambisa igazi emaphashini). Izingane zivame ukuzalwa zinokubili kwalokhu kuxhuma:

• I-Foramen ovale (imbobo phakathi kwe-atrium yangakwesokudla nangakwesobunxele)
• I-Ductus arteriosus (isitsha segazi esincane esixhumanisa i-aorta nomthambo we-pulmonary)

Kokubili lokhu kuxhumana kuvamise ukuvala ngokwako ezinsukwini ezimbalwa ngemuva kokuzalwa.

Ezinganeni ezine-hypoplastic heart syndrome, igazi elishiya uhlangothi olungakwesokudla senhliziyo ngomthambo wamaphaphu lihamba nge-ductus arteriosus liye e-aorta. Le ukuphela kwendlela igazi elingafika ngayo emzimbeni. Uma i-ductus arteriosus ivunyelwe ukuvala enganeni ene-hypoplastic left heart syndrome, ingane ingafa ngokushesha ngoba alikho igazi elizofakwa emzimbeni. Izingane ezine-hypoplastic left heart syndrome ezaziwayo zivame ukwenziwa kumuthi wokugcina i-ductus arteriosus ivulekile.

Ngoba kunokugeleza okuncane noma akukho okuphuma enhliziyweni yangakwesobunxele, igazi elibuyela enhliziyweni lisuka emaphashini lidinga ukudlula kwi-foramen ovale noma i-atrial septal defect (imbobo exhuma amakamelo okuqoqa ngakwesokunxele nangakwesokudla senhliziyo) buyela ohlangothini lokunene lwenhliziyo. Uma ingekho i-foramen ovale, noma uma incane kakhulu, ingane ingafa. Izingane ezinale nkinga zivuleke imbobo phakathi kwe-atria yazo, kungaba ngokuhlinzwa noma ngokusebenzisa ithubhu elincane, eliguquguqukayo (i-heart catheterization).

Ekuqaleni, umntwana osanda kuzalwa onenhliziyo yesobunxele engabonakali angabonakala ejwayelekile. Izimpawu zingavela emahoreni ambalwa okuqala okuphila, noma kungathatha izinsuku ezimbalwa ukuthuthukisa izimpawu. Lezi zimpawu zingafaka:

• I-Bluish (cyanosis) noma umbala wesikhumba ompofu
• Izandla nezinyawo ezibandayo (imikhawulo)
• Imikhuba emibi
• Ukushaya ngamandla
• Abancela abancelayo nabondlayo
• Inhliziyo eshayayo
• Ukuphefumula ngokushesha
• Ukuphelelwa umoya

Ezinganeni ezisanda kuzalwa ezinempilo, umbala oluhlaza okwesibhakabhaka ezandleni nasezinyaweni kuyimpendulo kumakhaza (lokhu kusabela kubizwa ngokuthi yi-peripheral cyanosis).

Umbala oluhlaza okwesibhakabhaka esifubeni noma esiswini, ezindebeni, nasolimini akuvamile (okubizwa nge-cyanosis emaphakathi). Kuwuphawu lokuthi ayikho i-oxygen eyanele egazini. I-cyanosis emaphakathi ivame ukwanda ngokukhala.

Ukuhlolwa komzimba kungakhombisa izimpawu zokwehluleka kwenhliziyo:

• Ishesha kunokushaya kwenhliziyo okuvamile
• Imikhuba emibi
• Ukwandiswa kwesibindi
• Ukuphefumula ngokushesha

Futhi, ukushaya kwezindawo ezahlukahlukene (isihlakala, ukububula, nokunye) kungahle kube buthakathaka kakhulu. Kukhona imisindo yenhliziyo engajwayelekile (kepha hhayi njalo) lapho ulalele isifuba.

Ukuhlolwa kungafaka:

• I-catheterization yenhliziyo
• I-ECG (electrocardiogram)
• I-Echocardiogram
• I-X-ray yesifuba

Lapho kutholakala ukuxilongwa kwenhliziyo engakwesobunxele ye-hypoplastic, ingane izongeniswa egumbini lokunakekelwa kwabagula kakhulu. Kungadingeka umshini wokuphefumula (i-ventilator) ukusiza ingane ukuthi iphefumule. Umuthi obizwa nge-prostaglandin E1 usetshenziselwa ukugcina igazi ligijimela emzimbeni ngokugcina i-ductus arteriosus ivulekile.

Lezi zinyathelo azixazululi inkinga. Isimo ngaso sonke isikhathi sidinga ukuhlinzwa.

Ukuhlinzwa kokuqala, okubizwa ngokuthi yi-Norwood operation, kwenzeka ngaphakathi kwezinsuku zokuqala ezimbalwa zokuphila kwengane. Inqubo yeNorwood yakha ukwakha i-aorta entsha ngo:

• Kusetshenziswa i-valve yamaphaphu nomthambo
• Ukuxhuma i-aorta endala ene-hypoplastic kanye nemithambo yegazi e-coronary ku-aorta entsha
• Ukususa udonga phakathi kwe-atria (atrial septum)
• Ukwenza uxhumano lokufakelwa kusuka ku-ventricle elungile noma umthambo womzimba wonke emthanjeni we-pulmonary ukugcina ukugeleza kwegazi kuya emaphashini (okuthiwa i-shunt)

Ukwehluka kwenqubo yeNorwood, ebizwa ngenqubo yeSano, kungasetshenziswa. Le nqubo idala i-ventricle efanele ekuxhumaneni komthambo wamaphaphu.

Ngemuva kwalokho, ingane iya ekhaya ezimweni eziningi. Ingane izodinga ukuthatha imithi yansuku zonke futhi ilandelwe eduze nodokotela wenhliziyo wezingane, ozonquma ukuthi isigaba sesibili sokuhlinzwa kufanele senziwe nini.

Isigaba II sokusebenza sibizwa ngokuthi inqubo kaGlenn shunt noma inqubo ye-hemi-Fontan. Kubizwa nangokuthi i-cavopulmonary shunt. Le nqubo ixhuma umthambo omkhulu ophethe igazi eliluhlaza okwesibhakabhaka ovela engxenyeni ephezulu yomzimba (i-vena cava ephakeme) ngqo emithanjeni yegazi eya emaphashini (imithambo yamaphaphu) ukuthola i-oxygen. Ukuhlinzwa kuvame ukwenziwa lapho ingane inezinyanga ezine kuya kwezingu-6 ubudala.

Ngesikhathi sezigaba I no-II, ingane isengabonakala ngathi iluhlaza okwesibhakabhaka (cyanotic).

Isigaba III, isinyathelo sokugcina, sibizwa ngokuthi inqubo ye-Fontan. Eminye imithambo ethwala igazi eliluhlaza okwesibhakabhaka emzimbeni (i-inferior vena cava) ixhunywe ngqo emithanjeni yegazi eya emaphashini. I-ventricle efanele manje isebenza njengendawo yokupompa umzimba kuphela (akusekho amaphaphu nomzimba). Lokhu kuhlinzwa kuvame ukwenziwa lapho ingane inezinyanga eziyi-18 kuya kwengu-4 ubudala. Ngemuva kwalesi sinyathelo sokugcina, ingane ayisasebenzi i-cyanotic futhi inezinga le-oxygen ejwayelekile egazini.

Abanye abantu bangadinga ukuhlinzwa okuningi kuma-20s abo noma ama-30s uma bekhula kanzima ukulawula ama-arrhythmias noma ezinye izinkinga zenqubo ye-Fontan.

Abanye odokotela babheka ukufakelwa inhliziyo njengenye indlela yokuhlinzwa okuyizinyathelo ezintathu. Kepha kunezinhliziyo ezimbalwa ezinikelwe ezitholakalayo zezinsana ezincane.

Uma ishiywe ingalashwa, i-hypoplastic left heart syndrome iyabulala. Izinga lokusinda ngokulungiswa okuhleliwe liyaqhubeka nokukhuphuka njengoba amasu okuhlinza nokunakekelwa ngemuva kokuhlinzwa kuthuthuka. Ukusinda ngemuva kwesigaba sokuqala kungaphezu kwama-75%. Izingane ezisinda onyakeni wazo wokuqala zinethuba elihle kakhulu lokusinda isikhathi eside.

Umphumela wengane ngemuva kokuhlinzwa uncike ebungako nasekusebenzeni kwe-ventricle efanele.

Izinkinga zifaka:

• Ukuvalwa kwe-shunt yokufakelwa
• Amahlule egazi angaholela ekushayweni unhlangothi noma embolism yamaphaphu
• Isifo sohudo sesikhathi eside (esingapheli) (esivela kwisifo esibizwa nge-protein-losing enteropathy)
• Uketshezi esiswini (ascites) nasemaphashini (i-pleural effusion)
• Ukwehluleka kwenhliziyo
• Isigqi senhliziyo esingajwayelekile, esisheshayo (arrhythmias)
• Ukushaywa unhlangothi nezinye izinkinga zesistimu yezinzwa
• Ukukhubazeka kwezinzwa
• Ukufa okungazelelwe

Xhumana nomhlinzeki wakho wezempilo ngokushesha uma usana lwakho:

• Idla kancane (ukuncipha kokudla)
• Inesikhumba esiluhlaza okwesibhakabhaka (cyanotic)
• Inezinguquko ezintsha emaphethini okuphefumula

Akukho ukuvimbela okwaziwayo kwe-hypoplastic left heart syndrome. Njengezifo eziningi zokuzalwa, izimbangela ze-hypoplastic left heart syndrome aziqinisekile futhi azixhunywanga nesifo somama noma isimilo sakhe.

HLHS; Inhliziyo ezelwe - inhliziyo engakwesobunxele ene-hypoplastic; Isifo senhliziyo seCyanotic - inhliziyo engakwesobunxele ye-hypoplastic

• Inhliziyo - isigaba phakathi nendawo
• Inhliziyo - ukubuka kwangaphambili
• Isifo senhliziyo esine-Hypoplastic

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I-Webb GD, iSmallhorn JF, uTherrien J, uRedington AN.Isifo senhliziyo esizalwa naso esigulini esidala nesingane. Ku: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, ama-eds. Isifo Senhliziyo SikaBraunwald: Incwadi Yemithi Yezinhliziyo Nemithambo. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2019: isahluko 75.