Ingaba icala lasekunene lentliziyo yomntwana wakho alikhuli kakuhle? (Hypoplastic Right Heart Syndrome)

Kuthekani ukuba umntwana wakho omncinci wazalwa enengxaki yentliziyo? Khawuthelekelele indlela ebekuya kuba buhlungu ngayo oko. Abanye abantwana bazalwa benecala lasekunene lentliziyo yabo elingakhuli kakuhle. Oogqirha babiza le Hypoplastic Right Heart Syndrome. Oku kunciphisa ubungakanani beoksijini efunyanwa ngumntwana. Makhe sithethe ngale nto ngokweenkcukacha, akunjalo?

Yintoni iHypoplastic Right Heart Syndrome?

Ngamafutshane, iHypoplastic Right Heart Syndrome sisifo sentliziyo esingaqhelekanga esizalwa sinaso ezinye iintsana . Okwenzekayo koku kukuba ezinye iindawo zecala lasekunene lentliziyo yomntwana azikhuli ngokupheleleyo. Cinga ngayo njengepompo encinci. Icala lasekunene lentliziyo yilo elithatha igazi elingenaoksijini emzimbeni lize liyithumele emiphungeni ukuze ifumane ioksijini. Ngoko ke, ukuba eli cala lasekunene alikhuli kakuhle, aliyi kuwenza kakuhle umsebenzi walo.

Kule meko, ezi ndawo zilandelayo zentliziyo zinokuchaphazeleka:

• Ivalvu yeTricuspid: Oku kufana nesango eliphakathi kwe-atrium yasekunene kunye ne-ventricle yasekunene yentliziyo. Xa ingasebenzi kakuhle, iyayithintela indlela igazi elihamba ngayo.
• Ivalvu yemiphunga: Le yivalvu apho umthambo wemiphunga, othwala igazi ukusuka kwi-ventricle yasekunene ukuya emiphungeni, uqala khona.
• I-ventricle yasekunene: Le yindawo ephambili yokupompa igazi kwicala lasekunene lentliziyo. Ukuba iba ncinci, iya kupompa igazi elincinci.
• Umthambo wepulmonary: Lo ngumthambo omkhulu othwala igazi ukusuka kwi-ventricle yasekunene ukuya emiphungeni.

Kuxhomekeke ekubeni imeko inzima okanye incinci kangakanani na, ukusebenza kwezi ndawo zentliziyo kunokuncipha okanye kwandiswe.

Ngamanye amaxesha, iintsana ezine-HRHS zinokuba nezinye iingxaki zentliziyo. Umzekelo:

• I-Atrial septal defect (ASD): Umngxuma eludongeni phakathi kwamagumbi amabini aphezulu (atria) entliziyo.
• I-Ventricular septal defect (VSD): Umngxuma eludongeni phakathi kwamagumbi amabini asezantsi (ii-ventricles) entliziyo.
• Ingxaki yomjelo we-Atrioventricular: Umngxuma omkhulu embindini wentliziyo kunye nezinye iingxaki ezininzi.

Ibaluleke kangakanani le meko?

I-Hypoplastic right heart syndrome (HRHS) inokuba yingozi kakhulu kwabanye abantu kunabanye . Abantu abaneemeko ezinzima, ezifana namanqanaba aphantsi kakhulu eoksijini , bafuna unyango olukhawulezileyo . Nangona kunjalo, abanye abantu basenokungabi nayo ingxaki enkulu kangako, kodwa baneengxaki ezincinci. Abantu abanjalo basenokungafunyaniswa ukuba baneengxaki ezinjalo de babe badala.

Ixhaphake kangakanani le meko?

Le yimeko engaqhelekanga kakhulu .Abaphandi baqikelela ukuba bangaphantsi kwama-50,000 abantu eMelika abane-HRHS. Inqabile le nto kuneHypoplastic Left Heart Syndrome, imeko apho icala lasekhohlo lentliziyo lingakhuli kakuhle.

Zithini iimpawu ze-hypoplastic right heart syndrome (HRHS)?

Ezi zilandelayo ziimpawu zale meko:

• Ukutshintsha kombala oluhlaza okwesibhakabhaka kwesikhumba sezandla neenyawo. Oku kubizwa ngokuba yi -cyanosis . Oku kuthetha ukuba umzimba awufumani ioksijini eyaneleyo.
• Ubunzima bokuphefumla. Oku kubizwa ngokuba yi -dyspnea .
• Ukuphefumla ngokukhawuleza. Oku kubizwa ngokuba yi -tachypnea .
• Usana aluseli ubisi ngendlela efanelekileyo.
• Ukudinwa ngokukhawuleza, ukuziva ulele ngalo lonke ixesha.

Ukuba umntwana wakho unenye okanye ngaphezulu kwezi mpawu, kungcono ukubona ugqirha ngokukhawuleza.

Yintoni ebangela oku?

Enyanisweni, unobangela ochanekileyo we-hypoplastic right heart syndrome (HRHS) awukafunyanwa .

Nangona kunjalo, abaphandi bakholelwa ukuba i-genetics isenokuba negalelo , njengoko amalungu aliqela osapho olunye exelwe ukuba ane-HRHS ekuzalweni. Ke ngoko, kufuneka uphando olongezelelekileyo.

Zeziphi ezinye iingxaki ezinokubangela oku?

I-Hypoplastic Right Heart Syndrome (HRHS) inokubangela iingxaki ezifana nezi:

• Amanqanaba e-oksijini aphantsi emzimbeni. Oku kubizwa ngokuba yi -hypoxemia .
• Ukusilela kwentliziyo kwicala lasekunene.
• Ukubetha kwentliziyo okungaqhelekanga. Oku kubizwa ngokuba yi-arrhythmia .

Ebancinci, kwanakwabo bangachaphazeleki kakhulu yi-HRHS, ukuphuphuma kwesisu kunokwenzeka ngenxa yamanqanaba aphantsi eoksijini emzimbeni.

Sifunyaniswa njani esi sifo?

Ngamanye amaxesha, umboneleli wezempilo unokubona i-HRHS ngelixa umntwana esesesibelekweni (uphuhliso lwe-fetal) . Basebenzisa i-ultrasound scan ukubona intliziyo yomntwana. Ukuba ufuna ukujonga kufutshane, ungenza ne-echocardiogram ye-fetal . Zombini ezi mvavanyo azingenisi ntsholongwane.

Zeziphi iimvavanyo ezenziwa emva kokuba umntwana ezelwe?

Emva kokuba umntwana ezelwe, ezi mvavanyo zingangenisi gazi zingenziwa ukuze kufunyaniswe ukuba umntwana une-HRHS na:

• I-pulse oximeter (i-pulse ox): Esi sisixhobo esincinci esifana ne-clip esilinganisa amanqanaba eoksijini ngokusincamathisela emnweni okanye endlebeni yakho.
• I-Electrocardiogram (EKG/ECG): Uvavanyo olulinganisa umsebenzi wombane wentliziyo.
• I-Echocardiogram (i-Echocardiogram - i-echo): Olu luvavanyo olusebenzisa amaza esandi, afana ne-ultrasound, ukuthatha imifanekiso yentliziyo. Lungabonisa ulwakhiwo lwentliziyo, indlela esebenza ngayo iivalvu, kunye nendlela igazi elihamba ngayo.
• I-MRI yentliziyo (iMagnetic Resonance Imaging): Isebenzisa amasimi emagnethi kunye namaza erediyo ukuvelisa imifanekiso eneenkcukacha zentliziyo.

Zithini iindlela zonyango zoku?

I-Hypoplastic Right Heart Syndrome (HRHS) inokunyangeka ngamayeza, iinkqubo ezingangenisi kakhulu, okanye utyando . Injongo ephambili yazo zonke ezi ndlela zonyango kukunceda imiphunga yomntwana ifumane igazi neoksijini eyaneleyo .

Iyeza

• I-Prostaglandin isetyenziselwa ukunceda ukugcina i-ductus arteriosus yomntwana ivulekile. Le ductus arteriosus yityhubhu esetyenziswa ngumntwana ukufumana igazi elineoksijini kumama esesibelekweni. Njengoko umntwana eqala ukuphefumla ngemiphunga emva kokuzalwa, le tyhubhu idla ngokuvaleka kwiintsuku ezimbalwa. Nangona kunjalo, kumntwana one-HRHS, ngenxa yokuba icala lasekunene lentliziyo alisebenzi kakuhle, ukugcina le tyhubhu ivulekile kunceda ukuhamba kwegazi ukuya emiphungeni.

Iinkqubo ezingafunekiyo kakhulu

• Ugqirha angasebenzisa ityhubhu encinci ebizwa ngokuba yi-catheter ukufaka i-stent (ityhubhu encinci efana ne-mesh yesinyithi) kwi-ductus arteriosus yomntwana. Oku kugcina i-duct ivulekile kwaye kuvumela igazi ukuba lihambe kakuhle ukuya emiphungeni.
• Enye into kukugqobhoza kwevalvu yemiphunga . Kule nto, i-catheter idluliselwa ngemithambo yegazi iye kwivalvu yemiphunga engavuleki kakuhle, nto leyo eyenza umngxuma kwivalvu. Oku kuvumela igazi ukuba lingene kwimithambo yemiphunga. Abanye oogqirha basebenzisa inkqubo ebizwa ngokuba yi-ablation kule nto.

Utyando

Amatyala anzima e-HRHS afuna utyando ukuze anyangwe. La maxesha adla ngokwenziwa ngamanqanaba ahlukeneyo.

• I-Blalock-Taussig-Thomas (BTT) shunt: Le isetyenziselwa ukubuyisela ukuhamba kwegazi emiphungeni yomntwana one-HRHS. Isebenzisa ilaphu elenziweyo ukwenza unxibelelwano phakathi kwe-subclavian artery (umthambo okwi-collarbone) kunye ne-pulmonary artery.
• Emva kweenyanga okanye iminyaka embalwa, ugqirha otyandayo angenza inkqubo kaGlenn kunye/okanye inkqubo kaFontan . Ngayinye kwezi nkqubo idala indlela entsha yokuba igazi lijikeleze i-ventricle yasekunene engasebenzi kakuhle. Ngokwesiqhelo, i-ventricle yasekunene ipompa igazi kwi-pulmonary artery.

Iinkqubo zikaGlenn noFontan zivumela igazi ukuba lihambe ngqo lisuka kumthambo omkhulu liye kumthambo wemiphunga, apho lisuka khona liye emiphungeni ukuze lifumane ioksijini.

Ngaba kukho naziphi na iingxaki kunyango?

Njengakwezinye iindlela zotyando, kukho iingxaki ezithile ezinokubakho ngolu nyango. Iingxaki zinokwahluka ngokuxhomekeke kunyango. Umzekelo:

• I-stent isenokusuka kwindawo yayo yokuqala.
• Utyando lwe-BTT shunt lunokubangela ukuba imithambo yegazi inciphe okanye yonakalise umthambo.
• Ukugqobhoka kwevalvu yepulmonary kunokubangela ukubetha kwentliziyo okungahambelaniyo (i-arrhythmia) okanye konakalise umthambo wegazi.
• Utyando olukhulu lunokubangela uxinzelelo lwegazi oluphezulu, ukuphuma kwegazi kwi-pleural, ukuminyana kwentliziyo, isifo sezintso okanye sesibindi, okanye ukubetha kwentliziyo okungaqhelekanga.

Kodwa ungakhathazeki. Oogqirha baza kukwazisa ngako konke oku baze bathathe amanyathelo afunekayo.

Kuthatha ixesha elingakanani ukuphiliswa?

Ixesha lokuchacha liyahluka ngokuxhomekeke kunyango. Utyando olukhulu luthatha ixesha elide. Umntwana wakho usenokuhlala esibhedlele iintsuku ezininzi, mhlawumbi ngaphezulu kweveki. Kuya kufuneka ajongwe ngokusondeleyo emva kokuba ebuyele ekhaya.

Ndingalindela ntoni ukuba umntwana wam ukule meko?

Ngenxa yokuba amanqanaba e-oksijini aphantsi kwasebuntwaneni, abantu abane-hypoplastic right heart syndrome (HRHS) banokujamelana nemingeni efana nale:

• Iingxaki zolwimi.
• Iingxaki zememori.
• Umngcipheko okhulayo wesifo sentliziyo, sesibindi, okanye sezintso xa sele ukhulile.
• Iingxaki zeemvakalelo okanye iingxaki zokuxhalaba.
• Ubunzima bokucwangcisa into ethile okanye ukusombulula ingxaki.

Kodwa, kukho iingcali ezinokunceda kuyo yonke le nto. Ngoko ke ungakhathazeki.

Ithini ingxelo yokubikezela ukuba le meko iza kwenzeka?

Kunqabile kakhulu ukuba abantu abane-hypoplastic right heart syndrome (HRHS) baphile baze babe ngabantu abadala ngaphandle kotyando . Nangona kunjalo, abantu abane-HRHS engembi kangako basenokungazi nokuba banale meko bade babe ngabantu abadala.

Umnyamekela njani umntwana one-HRHS?

Unyango umntwana wakho aludingayo luxhomekeke ekubeni imeko yakhe imbi kangakanani na . Ukuba wenze utyando emva nje kokuzalwa, kuya kufuneka ajongwe ngononophelo emva kokuba efikile ekhaya. Ugqirha wakho okanye umbelekisi wakho uya kukunika iingcebiso ezilungileyo malunga nendlela yokunyamekela umntwana wakho.

Udinga nini ukubona ugqirha?

Usana oluye lwenziwa utyando kuya kufuneka lubone ugqirha ukuze lufumane amadinga okulandela . Baza kukuxelela into omawuyilumkele nento omawuyixelele ugqirha ngokukhawuleza ukuba ubona nantoni na. Ukuba unemibuzo okanye iinkxalabo malunga nemeko yomntwana wakho, fowunela ugqirha ngokukhawuleza.

Ukuba umntwana wakho uneengxaki zokufunda okanye zokulawula iimvakalelo zakhe, unokufumana nezinye iingcali ezinokunceda kwezo ndawo. Ugqirha wakho uya kukunika ulwalathiso olufunekayo.

Ndingayibuza yiphi imibuzo ugqirha?

Nantsi eminye imibuzo onokuyibuza ugqirha womntwana wakho:

• Ngaba umntwana wam udinga utyando?
• Kufuneka wenze utyando ngokukhawuleza kangakanani?
• Zingaphi utyando oluza kufuneka, kwaye luza kwenziwa nini?
• Injani imeko yomntwana wam xa enale meko?

Ndiyaqonda ukuba kunzima kangakanani ukufumanisa ukuba umntwana wakho unesifo sentliziyo. Nangona kunjalo, abantwana abazelwe namhlanje banonyango olwalungafumaneki kwizizukulwana zangaphambili. Ungumntu onamandla onokuba nguye kumntwana wakho ngeli xesha. Funda nge-HRHS. Buza ugqirha wakho ngayo nayiphi na into engacacanga. Thetha nogqirha wakho ukuze agqibe ukuba yintoni elungele umntwana wakho.

Okokugqibela, khumbula oku (Umyalezo Wokuya Ekhaya)

I-Hypoplastic Right Heart Syndrome (HRHS) yimeko engaqhelekanga ezalwa nayo iintsana, apho icala lasekunene lentliziyo lingakhuli kakuhle. Oku kunciphisa umlinganiselo weoksijini efunyanwa ngumzimba. Ukuxilongwa kwangoko kunye nonyango olufanelekileyo (amayeza, utyando oluncinci, okanye utyando olukhulu) kunokuphucula ubomi bomntwana . Kwimeko enjalo, kubaluleke kakhulu kuwe njengomzali ukuba waziswe kwaye usebenzisane noogqirha bakho. Khumbula, awuwedwa. Ungafumana uncedo olufunayo.

Isifo sentliziyo esibizwa ngokuba yiHypoplastic Right Heart Syndrome, iHRHS, isifo sentliziyo sabantwana abancinci, iziphene zentliziyo ezizalwa nazo, iCyanosis, ubunzima bokuphefumla, utyando lwentliziyo