Isifo sentliziyo esekho Hypoplastic

Isifo sentliziyo yasekhohlo sehypoplastic senzeka xa iinxalenye zecala lasekhohlo lentliziyo (i-mitral valve, i-ventricle yasekhohlo, i-aortic valve, kunye ne-aorta) zingakhuli ngokupheleleyo. Le meko ikhoyo ekuzalweni (kokuzalwa).

Intliziyo yasekhohlo e-Hypoplastic luhlobo olunqabileyo lwesifo sentliziyo esibelekweni. Kuqheleke kakhulu emadodeni kunabafazi.

Njengazo zonke iziphene zentliziyo yokuzalwa, akukho sizathu saziwayo. Phantse iipesenti ezili-10 zeentsana ezine-hypoplastic heart syndrome nazo zinezinye iziphene zokuzalwa. Ikwanxulunyaniswa nezinye izifo zemfuza ezinje nge-Turner syndrome, i-Jacobsen syndrome, i-trisomy 13 kunye ne-18.

Ingxaki ikhula ngaphambi kokuzalwa xa i-ventricle yasekhohlo kunye nezinye izinto zingakhuli kakuhle, kubandakanya:

• I-Aorta (isitya segazi esiphethe igazi eline-oxygen eninzi ukusuka kwi-ventricle yasekhohlo ukuya kuwo wonke umzimba)
• Ukungena kunye nokuphuma kwe-ventricle
• Mitral kunye nevalve aortic

Oku kubangela ukuba i-ventricle yasekhohlo kunye ne-aorta zikhule kakubi, okanye i-hypoplastic. Kwiimeko ezininzi, i-ventricle yasekhohlo kunye ne-aorta zincinci kakhulu kunesiqhelo.

Kwiintsana ezinale meko, icala lasekhohlo lentliziyo alikwazi ukuthumela igazi elaneleyo emzimbeni. Ngenxa yoko, icala lasekunene lentliziyo kufuneka ligcine ukujikeleza kwemiphunga kunye nomzimba. I-ventricle elungileyo inokuxhasa ukujikeleza kwimiphunga nasemzimbeni okwethutyana, kodwa lo msebenzi ungaphezulu ekugqibeleni ubangela icala lasekunene lentliziyo lisilele.

Elinye kuphela ithuba lokuphila kukunxibelelana phakathi kwecala lasekunene nelasekhohlo lentliziyo, okanye phakathi kwemithambo kunye nemithambo yemiphunga (imithambo yegazi ethwala igazi liye emiphungeni). Iintsana zihlala zizalwa zinokunxibelelana ezimbini:

• I-Foramen ovale (umngxuma phakathi kwe-atrium yasekunene neyasekhohlo)
• I-Ductus arteriosus (isitya esincinci segazi esidibanisa i-aorta kwi-pulmonary artery)

Zombini ezi zinto ziqhagamshelekayo zihlala zivala ngokwazo kwiintsuku ezimbalwa emva kokuzalwa.

Kwiintsana ezine-hypoplastic heart syndrome, igazi elishiya icala lasekunene lentliziyo ngokusebenzisa i-pulmonary artery ihamba nge-ductus arteriosus iye kwi-aorta. Le kuphela kwendlela yegazi yokufika emzimbeni. Ukuba i-ductus arteriosus ivumelekile ukuvala umntwana one-hypoplastic left heart syndrome, umntwana usenokufa ngokukhawuleza kuba akukho gazi liya kutofelwa emzimbeni. Iintsana ezine-hypoplastic syndrome yentliziyo yasekhohlo zihlala ziqala amayeza ukugcina i-ductus arteriosus ivulekile.

Kuba akukho kuncinci ukuphuma okanye ukuphuma kwentliziyo yasekhohlo, igazi elibuyela entliziyweni lisuka kwimiphunga kufuneka lidlule kwi-foramen ovale okanye isiphene se-atrial septal (umngxunya odibanisa amagumbi okuqokelela kwicala lasekhohlo nasekunene kwentliziyo) buyela kwicala lasekunene lentliziyo. Ukuba akukho foramen ovale, okanye ukuba incinci kakhulu, umntwana angafa. Iintsana ezinale ngxaki zinomngxunya phakathi kwe-atria yazo evuliweyo, nokuba kungokuhlinzwa okanye kusetyenziswe ityhubhu ecekeceke, ebhetyebhetye (intliziyo yokubulala iintlungu).

Ekuqaleni, umntwana osandul 'ukuzalwa onentliziyo ekhohlo yokulala unokubonakala eqhelekileyo. Iimpawu zinokwenzeka kwiiyure zokuqala ezimbalwa zobomi, nangona kunokuthatha ukuya kwiintsuku ezimbalwa ukukhula kweempawu. Ezi mpawu zinokubandakanya:

• I-Bluish (cyanosis) okanye umbala ombi wesikhumba
• Izandla kunye neenyawo ezibandayo (iziphelo)
• Ukuhamba
• Ukubetha okungalunganga
• Ukuhlupheka ukuncancisa kunye nokondla
• Intliziyo ebethayo
• Ukuphefumla ngokukhawuleza
• Ukuqhawukelwa ngumphefumlo

Kwiintsana ezisandula ukuzalwa ezisempilweni, umbala oluhlaza okwesibhakabhaka ezandleni nasezinyaweni yindlela yokuphendula ingqele (le mpendulo ibizwa ngokuba yipheripheral cyanosis).

Umbala oluhlaza okwesifuba okanye isisu, imilebe, kunye nolwimi akuqhelekanga (okubizwa ngokuba yi-cyanosis esembindini). Luphawu lokuba akukho oksijini yaneleyo egazini. I-cyanosis esembindini inyuka ngokukhala.

Uvavanyo lomzimba lunokubonisa iimpawu zokwehluleka kwentliziyo:

• Ngokukhawuleza kunokubetha kwentliziyo eqhelekileyo
• Ukuhamba
• Ukwandiswa kwesibindi
• Ukuphefumla ngokukhawuleza

Kwakhona ukubetha kwiindawo ezahlukeneyo (esihlahleni, kwi-groin, nakwezinye) kunokuba buthathaka kakhulu. Kukho rhoqo (kodwa hayi rhoqo) izandi zentliziyo engaqhelekanga xa umamele isifuba.

Uvavanyo lunokubandakanya:

• Ukucinywa kwentliziyo
• I-ECG (i-electrocardiogram)
• Echocardiogram
• X-ray esifubeni

Nje ukuba kufunyaniswe isifo se-hypoplastic heart, umntwana uyakwamkelwa kwicandelo lokhathalelo olunzulu lwe-neonatal. Kusenokufuneka umatshini wokuphefumla (ukuphefumla) ukunceda umntwana aphefumle. Iyeza elibizwa ngokuba yi-prostaglandin E1 lisetyenziselwa ukugcina igazi lijikeleza emzimbeni ngokugcina i-ductus arteriosus ivulekile.

La manyathelo awasombululi ingxaki. Imeko ihlala ifuna utyando.

Utyando lokuqala, olubizwa ngokuba ngumsebenzi weNorwood, lwenzeka kwiintsuku zokuqala ezimbalwa zobomi bosana. Inkqubo yeNorwood iquka ukwakha i-aorta entsha ngu:

• Sebenzisa ivelufa yemiphunga kunye nomthambo
• Ukudibanisa i-aorta endala ye-hypoplastic kunye nemithambo yegazi kwi-aorta entsha
• Ukususa udonga phakathi kwe-atria (septum atrial)
• Ukwenza unxibelelwano olungelulo olwenziweyo ukusuka kwi-ventricle elungileyo okanye umthambo womzimba wonke kwimithambo ye-pulmonary ukugcina ukuhamba kwegazi kwimiphunga (ebizwa ngokuba yi-shunt)

Umahluko wenkqubo yeNorwood, ebizwa ngokuba yinkqubo yeSano, inokusetyenziswa. Le nkqubo yenza i-ventricle efanelekileyo kwi-pulmonary artery connection.

Emva koko, umntwana uye ekhaya amaxesha amaninzi. Umntwana kuya kufuneka athathe amayeza emihla ngemihla kwaye alandelwe ngokusondeleyo ngugqirha wezifo zentliziyo yabantwana, oya kugqiba ukuba inqanaba lesibini lotyando kufuneka lenziwe nini.

Isigaba II sokusebenza sibizwa ngokuba yiGlenn shunt okanye inkqubo ye-hemi-Fontan. Ikwabizwa ngokuba yi-cavopulmonary shunt. Le nkqubo idibanisa umthambo omkhulu ophethe igazi eliluhlaza okwesibhakabhaka ukusuka kwisiqingatha esiphezulu somzimba (i-vena cava ephezulu) ngqo kwimithambo yegazi emiphungeni (kwimithambo ye-pulmonary) yokufumana ioksijini. Olu tyando luhlala lusenziwa xa umntwana eneenyanga ezi-4 ukuya kwezi-6 ubudala.

Ngexesha lesigaba I no-II, umntwana usenokubonakala ngathi ublue (cyanotic).

Isigaba III, inyathelo lokugqibela, libizwa ngokuba yinkqubo yePontian. Eminye imithambo ephethe igazi eliluhlaza emzimbeni (i-vena cava engaphantsi) idityaniswe ngqo kwimithambo yegazi ukuya emiphungeni. I-ventricle elungileyo ngoku isebenza njengegumbi lokumpompa umzimba (ayisenguye imiphunga nomzimba). Olu tyando luhlala lwenziwa xa umntwana eneenyanga ezili-18 ukuya kwiminyaka emi-4 ubudala. Emva kweli nyathelo lokugqibela, umntwana akasenayo i-cyanotic kwaye unenqanaba eliqhelekileyo leoksijini egazini.

Abanye abantu banokufuna uqhaqho oluninzi kwii-20s zabo okanye ii-30s ukuba bakhula nzima ukulawula ii-arrhythmias okanye ezinye iingxaki zenkqubo yePontian.

Abanye oogqirha bacinga ukuba ukufakelwa kwentliziyo kungenye indlela yotyando lwamanqanaba ama-3. Kodwa zimbalwa iintliziyo ezinikelweyo ezifumanekayo kwiintsana ezincinci.

Ukuba ishiywe inganyangwa, isifo sentliziyo esekhohlo siyabulala. Amaxabiso okusinda kulungiso oluhleliweyo aqhubeka nokunyuka njengoko iindlela zotyando kunye nokhathalelo emva kotyando luphucuka. Ukusinda emva kwenqanaba lokuqala kungaphezulu kwama-75%. Abantwana abasinda kunyaka wabo wokuqala banethuba elihle kakhulu lokusinda ixesha elide.

Iziphumo zomntwana emva kotyando zixhomekeke kubungakanani kunye nokusebenza kwe-ventricle efanelekileyo.

Iingxaki zibandakanya:

• Ukuvaleka kwe-shunt eyenziweyo
• Amagqabantshintshi egazi anokukhokelela ekubethekeni okanye kwi-pulmonary embolism
• Ixesha elide (elingapheliyo) isifo sohudo (kwisifo esibizwa ngokuba yi-protein-losing enteropathy)
• Ulwelo esiswini (ascites) nasemiphungeni (ukungxola)
• Ukumelwa yintliziyo
• Ngokungaqhelekanga, ngokukhawuleza kwentliziyo (arrhythmias)
• Ukubetha kunye nezinye iingxaki zenkqubo ye-nervous
• Ukuphazamiseka kwimithambo-luvo
• Ukufa ngesiquphe

Nxibelelana nomboneleli wakho wezempilo kwangoko ukuba usana lwakho:

• Itya kancinci (ukunciphisa ukondla)
• Inesikhumba esiluhlaza (cyanotic)
• Ineenguqulelo ezintsha kwiipateni zokuphefumla

Akukho sithintelo saziwayo sehypoplastic yentliziyo yesifo sentliziyo. Njengokuba kunjalo ngezifo ezininzi zokuzalwa, oonobangela besifo sentliziyo esekho hypoplastic abaqinisekanga kwaye khange banxulunyaniswe nesifo sikamama okanye isimilo.

HLHS; Intliziyo yengqondo - i-hypoplastic left heart; Isifo sentliziyo seCyanotic- intliziyo esekho hypoplastic

• Icandelo lentliziyo phakathi embindini
• Intliziyo - umbono wangaphambili
• Isifo sentliziyo esekho Hypoplastic

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IWebb GD, i-Smallhorn JF, iTherrien J, iRedington AN.Isifo sentliziyo esibelekwe kumntu omkhulu nakwisigulana sabantwana. Ku: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, ii-eds. Isifo sentliziyo seBraunwald: Incwadi yesifundo seMpilo yeNtliziyo. Ngomhla we-11. IPhiladelphia, PA: Elsevier; I-2019: isahluko 75.