Ingaba intliziyo yasekhohlo yomntwana wakho ayikhuli kakuhle? (Hypoplastic Left Heart Syndrome - HLHS) Makhe sithethe ngale nto!

Namhlanje siza kuthetha ngesimo sentliziyo esinzima kancinci, nesingaqhelekanga, kodwa esibaluleke kakhulu. Esi sisifo esibizwa ngokuba yiHypoplastic Left Heart Syndrome , okanye (HLHS) ngamafutshane. Usenokuziva usoyika xa usiva oku, kodwa ungakhathazeki. Masithethe ngale nto ngokulula, ngendlela onokuyiqonda. Kuba, kubaluleke kakhulu ukuqaphela izinto ezinje.

Yintoni i-hypoplastic left heart syndrome (HLHS)?

Ngamafutshane, (i-HLHS) sisifo sentliziyo esizalwa naso esibakho xa umntwana ezalwa . Kule meko, ezinye iindawo zecala lasekhohlo lentliziyo yomntwana azikhuli kakuhle. Cinga ngaso njengepompo. Icala lasekhohlo lale mpompi yilo elithumela igazi elineoksijini emzimbeni wonke. Ngoko ke, xa eli cala lasekhohlo lingakhuli kakuhle, kuvela ingxaki. Kule meko, ezi ndawo zecala lasekhohlo lentliziyo zichaphazeleka kakhulu:

• I-ventricle yasekhohlo : Le yigumbi eliphambili kwicala lasekhohlo elisezantsi lentliziyo. Ipompa igazi elineoksijini kwi-aorta . Kwi-HLHS, idla ngokuba ncinci kakhulu ukuba isebenze kakuhle.
• I-Aorta : Lo ngumthambo wegazi omkhulu emzimbeni wethu. Uthwala igazi lisuka entliziyweni liye emzimbeni wonke. Kwi-(HLHS), nalo lisenokungaphuhli kakuhle.
• Iivalvu zeMitral nezeAortic: Ezi zifana neengcango. Zivumela igazi ukuba lihambe kwicala elinye kuphela. Ivalvu yeAortic ivumela igazi ukuba lihambe ukusuka kwiVenkile yasekhohlo ukuya kwiAorta. Ivalvu yeMitral ivumela igazi ukuba lihambe ukusuka kwigumbi eliphezulu (atrium) kwicala lasekhohlo lentliziyo ukuya kwigumbi elisezantsi (mitral valve). KwiHLHS, le valve isenokungasebenzi kakuhle okanye isenokuba ncinci kakhulu.

Ngamanye amaxesha, iintsana ezine-HLHS zinokuba nomngxuma omncinci eludongeni phakathi kwamagumbi amabini aphezulu entliziyo (i-atrial septal defect) . Ngokwesiqhelo, olu ludonga oluqinileyo.

Yintoni umahluko phakathi kwentliziyo eqhelekileyo kunye nentliziyo ene (HLHS)?

Intliziyo yethu eqhelekileyo inamacala amabini. Icala lasekunene limpompa igazi elingenayo ioksijini ukusuka emzimbeni ukuya emiphungeni ukuze liqokelele ioksijini. Emva koko, igazi elineoksijini liya kwicala lasekhohlo lize limpompelwe kulo lonke umzimba. Nangona kunjalo, kumntwana one-HLHS, icala lasekhohlo lentliziyo lincinci kangangokuba alikwazi ukumpompa igazi elaneleyo kulo lonke umzimba. Emva koko, i-ventricle yasekunene iyathatha indawo. Izama ukupompa igazi ukuya emiphungeni nakuwo wonke umzimba. Oku kwenziwa nge-ductus arteriosus.Ngomthambo wegazi okhethekileyo obizwa ngokuba yi-ductus arteriosus. Lo (ductus arteriosus) ngumthambo wegazi ozalwa ngumntwana ngamnye esibelekweni. Uhlala uvaleka emva kweentsuku ezimbalwa emva kokuzalwa. Kodwa kumntwana one-(HLHS), ukuba lo mthambo wegazi uyavaleka, ukuba awunyangwa, unokuba yingozi ebomini. Ngenxa yokuba icala lasekhohlo alisebenzi, indlela ekuphela kwayo yokuba igazi lifike emzimbeni iyavalwa.

Ixhaphake kangakanani (i-HLHS)?

(HLHS) sisifo esingaqhelekanga kakhulu. Ngokwezibalo eMelika, sichaphazela malunga nesinye kwiintsana ezingama-3,800 ezizalwa minyaka le. (HLHS) ibandakanya phakathi kwe-2% kunye ne-3% yazo zonke izifo zentliziyo ezizalwa nazo (CHD). Sixhaphake kakhulu kubafana kunamantombazana.

Zithini iimpawu zomntwana one-(HLHS)?

Iimpawu zomntwana one-(HLHS) zisenokungabonakali kwangoko. Zisenokubonakala kwiiyure okanye iintsuku ezimbalwa emva kokuzalwa. Iimpawu eziphambili zezi:

• I-Cyanosis : Umbala oluhlaza okwesibhakabhaka-ngwevu wolusu, imilebe, kunye neenzipho. Oku kunokubonakala njengombala ongwevu kwiintsana ezinesikhumba esimnyama. Kunokubonakala njengombala oluhlaza okwesibhakabhaka kwiintsana ezinesikhumba esikhanyayo. Oku kubangelwa kukungafumani ioksijini eyaneleyo emzimbeni.
• Ubunzima bokuphefumla : Umntwana usenokuziva ngathi kunzima ukuphefumla.
• Ubunzima bokusela ubisi: Usana lusenokungafuni ukusela ubisi, okanye lunokutshica xa lusela ubisi.
• Ukudinwa : Umntwana usenokubonakala elele kwaye ediniwe.
• Ukubetha kwentliziyo ngokukhawuleza.
• Ukubila , ulusu oluthambileyo, okanye ukuziva ubanda.
• Ukubetha kwentliziyo okubuthathaka.

Ukuba ubona naziphi na kwezi mpawu, kubalulekile ukufuna ingcebiso kagqirha ngoko nangoko. Musa ukoyika, kodwa thabatha amanyathelo ngokukhawuleza.

Zithini izizathu ze (HLHS)?

Kwiimeko ezininzi, akukho sizathu sithile se-HLHS. Ngamanye amaxesha isenokuba ngenxa yezinto ezibangela imfuza . Iintsana ezineenguqu ezithile zemfuza, ezifana ne -GJA1 okanye i-NKX2-5, zisengozini enkulu yokuphuhlisa i-HLHS. Kwakhona, iintsana ezineemeko ezithile zemfuza, ezifana ne -Turner syndrome okanye i-Trisomy 18, zinokuba ne-HLHS.

Ifunyaniswa njani i-HLHS?

Oogqirha banokuxilonga i-HLHS ngovavanyo lwemifanekiso olungangenisi ntsholongwane. Oku kungenziwa ngexesha lokukhulelwa okanye emva kokuba umntwana ezelwe. Ngexesha lokukhulelwa:

• I-ultrasound yangaphambi kokuzalwa: Olu luvavanyo oludla ngokwenziwa ngexesha lokukhulelwa.
• I-echocardiogram yosana olusesiswini: Olu luvavanyo olukhethekileyo olunokujonga iingxaki zentliziyo ngelixa umntwana esesesibelekweni.

Emva kokuba umntwana ezelwe: Oogqirha baxilonga imeko ngokujonga iimpawu nokujonga iziphumo zovavanyo. Ngamanye amaxesha, xa umamele intliziyo yomntwana nge-stethoscope, unokuva ukugquma kwentliziyo . Oku kuthetha ukuba igazi alihambi kakuhle.

Zeziphi iimvavanyo ezisetyenziselwa ukuxilonga (i-HLHS)?

• I-X-reyi yesifuba: Oku kunokujonga ubungakanani kunye nokuma kwentliziyo kunye nemiphunga yomntwana.
• I-Echocardiogram: Olu luvavanyo lwe-ultrasound. Izakhiwo zangaphakathi zentliziyo zinokubonakala ngokucacileyo.
• I-Electrocardiogram (EKG/ ECG ): Le isetyenziselwa ukulinganisa utshintsho lombane olwenzeka ngexesha lokubetha kwentliziyo.
• Uvavanyo lwe-pulse oximetry: Oku kunokumisela ubungakanani beoksijini egazini lomntwana.

Ngaba kukho unyango lwe-(HLHS)?

Ewe, kukho unyango. Okokuqala, umntwana kufuneka anikwe iyeza elibizwa ngokuba yi-prostaglandin . Oku kugcina i-ductus arteriosus ivulekile. Oku kudala indlela yokuba igazi lihambe emzimbeni. Ukongeza, amanye amayeza anokunikwa ukunceda intliziyo yomntwana isebenze kakuhle. Umntwana usenokufuna ukuncedwa ukuba aphefumle. Emva koko, kunokwenziwa utyando oluninzi. Olu tyando lusetyenziselwa ukuhambisa ukuhamba kwegazi emiphungeni nasemzimbeni. Emva kolu tyando, umsebenzi wentliziyo phantse udluliselwa kwi-ventricle yasekunene. Yiyo epompa igazi emzimbeni wonke.

Zeziphi iindlela zotyando ezenziwayo (i-HLHS)?

Oogqirha benza iinkqubo ezintathu eziphambili: inkqubo yaseNorwood , inkqubo yaseGlenn , kunye nenkqubo yaseFontan . Ezi nkqubo zenziwa ngokulandelelana. 1. Inkqubo yaseNorwood: Le nkqubo yenziwa kwiiveki ezimbini zokuqala zobomi beentsana ezine-HLHS. Kule nkqubo, oogqirha:

• I-aorta yomntwana engakhulanga kakuhle iphinda ibunjwe ukuze igazi lihambe liye emzimbeni.
• I -shunt , ityhubhu encinci, ifakwa ukuze iguqule igazi lisuke kwi-ventricle yasekunene, nokuba lisuka kwi-aorta, liye kwimithambo yemiphunga ekhokelela emiphungeni.
• Idala unxibelelwano phakathi kwamagumbi aphezulu (i-atria) entliziyo. Oku kunceda ukubonelela umzimba ngegazi elineoksijini eninzi elivela kwimiphunga.

2. Utyando lwe-shunt lweGlenn oluhamba ngeendlela ezimbini: LomntwanaUtyando lwesibini kufuneka lwenziwe malunga neenyanga ezi-4 ukuya kwezi-6. Ngexesha lotyando lukaGlenn:

• I-shunt endala iyasuswa.
• Kufakwa i-shunt entsha ize iqhagamshele i -vena cava (SVC) yomntwana (ethwala igazi elingenaoksijini ukusuka emzimbeni ongaphezulu ukuya entliziyweni) kwi-pulmonary artery.
• Le shunt iyanciphisa umthwalo kwi-ventricle yasekunene, kuba ivumela igazi ukuba liye ngqo emiphungeni.

3. Inkqubo yeFontan: Inkqubo yokugqibela yenziwa phakathi kweenyanga ezili- 18 kunye neminyaka emi-4 ubudala . Le nkqubo ihambisa lonke igazi elibuya emzimbeni liye emiphungeni, idlula intliziyo. Ngexesha lenkqubo yeFontan:

• I-vena cava (IVC) yomntwana engaphantsi (ethwala igazi elingenaoksijini ukusuka emzimbeni ongezantsi ukuya entliziyweni) iqhagamshela kumthambo wemiphunga.

Ngaba kukho naziphi na iingxaki kunyango?

Ewe, ezinye iintsana zinokulahlekelwa bubomi bazo ngelixa zisiya kolunye utyando. Kwakhona, kunokubakho iingxaki emva kotyando ngalunye. Umzekelo:

• Iingxaki ze-ventricle yasekunene engasebenzi kakuhle.
• Ukuvuza kwevalvu ye-aorta.
• Isifo sesibindi.
• Izingqisho zentliziyo ezingaqhelekanga.
• Amahlwili egazi.
• Usulelo.
• Ubunzima bokutya.
• Ukuxhuzula.
• Iingxaki zezintso.
• Ukubanjwa kwe-cardiac.

Ezi zizinto ezoyikisayo ukuziva, kodwa oogqirha baza kuthetha nawe ngezi ngozi baze bazame ukukunika unyango olungcono kangangoko.

Ngaba ukufakelwa intliziyo lunyango olulungileyo?

Ngamanye amaxesha, oogqirha botyando banokucebisa ukufakelwa intliziyo endaweni yokwenza olo tyando luthathu. Nangona kunjalo, iintsana eziye zafakelwa intliziyo kuya kufuneka zithathe amayeza (amayeza okunciphisa amasosha omzimba) ubomi bazo bonke.

Ngaba i-HLHS inganyangwa ngaphambi kokuzalwa?

Okwangoku akunakwenzeka ukunyanga ngokupheleleyo i-HLHS ngotyando ngexesha lokukhulelwa. Nangona kunjalo, oogqirha botyando abangekazalwa ngamanye amaxesha banokwenza amanyathelo athile okuthintela ezinye zeziphumo ezimbi ezinokwenzeka kumntwana osakhulayo one-HLHS (umz., ivalvu encinci ye-aortic). Kodwa oku kwenzeka kuphela kwiimeko ezikhethekileyo kakhulu.

Ngaba umngcipheko we-(HLHS) ungancitshiswa?

Ekubeni unobangela weemeko ezininzi ze-HLHS ungaziwa, kunzima ukutsho ukuba ungakuthintela njani ngokupheleleyo. Nangona kunjalo, kubalulekile ukulandela imikhwa enempilo ngexesha lokukhulelwa:

• Ukuphepha utywala nokutshaya.
• Ukulawula ezinye iimeko zempilo ezifana neDiabetes Mellitus .
• Ukutya ukutya okunempilo.
• Ukuthatha i-vitamin yokukhulelwa equlethe ubuncinane ii-microgram ezingama-400 (400 mcg) ze-folic acid imihla ngemihla .

Ukuba wena okanye iqabane lakho ninembali yentsapho ye-HLHS, kulungile ukuthetha nomcebisi wezemfuza ngaphambi kokuba nikhulelwe.

Ukuba umntwana wam unayo (i-HLHS), ndimele ndilindele ntoni?

Emva konyango lwe-HLHS, umntwana wakho kuya kufuneka abone ugqirha wentliziyo ubuncinane kanye ngonyaka ubomi bakhe bonke. Oku kuya kuqinisekisa ukuba intliziyo yakhe, imiphunga, kunye nezinye izitho zisebenza kakuhle. Njengoko umntwana wakho ekhula, kuya kufuneka abone ingcali kwisifo sentliziyo sokuzalwa sabantu abadala . Abantwana abaninzi abane-HLHS kuya kufuneka bathathe amayeza entliziyo ubomi babo bonke. Kuya kufuneka bathathe amayeza okubulala iintsholongwane ngaphambi kokuba benze naluphi na utyando, kuquka notyando lwamazinyo. Oku kunokunciphisa umngcipheko we -endocarditis (usulelo lwangaphakathi lwentliziyo).

Iyintoni imbonakalo yesimo (se-HLHS)?

Ukuba ayinyangwa, i-HLHS yimeko enokubangela ukufa kwiintsuku okanye iiveki emva kokuzalwa. Ngonyango, imeko ixhomekeke kubunzima besiphene sentliziyo kumntwana. Buza ugqirha womntwana wakho malunga neengozi zotyando ngalunye. Abanye abantwana banokuba nokukwazi ukuzilolonga ubomi babo bonke. Oogqirha badla ngokucebisa ukunciphisa imisebenzi enzima yomzimba, njengemidlalo yokhuphiswano.

Ingakanani izinga lokusinda kweentsana ezine-(HLHS)?

Le yinyaniso ebuhlungu kancinci, kodwa kubalulekile ukwazi. Phakathi kwama-20% nama-60% eentsana ezine-(HLHS) ziphila kunyaka wokuqala wobomi. Emva koko, izinga lokusinda kwiminyaka emihlanu, elishumi, kunye neshumi elinesihlanu ezayo limalunga nama-40%. Iintsana ezizelwe ngobunzima obuqhelekileyo bokuzalwa, ngaphandle kokuzalwa ngaphambi kwexesha, zineziphumo ezingcono kuneentsana ezizelwe zinobunzima obuphantsi. Olunye uphando lufumanise ukuba iintsana ezininzi eziphile kunyaka wokuqala zisaphila zineminyaka eli-18 ubudala.

Kunzima ukubona ezi zibalo. Kodwa khumbula, isayensi yezonyango iyaphucuka yonke imihla. Unyango olutsha, ubuchwepheshe obutsha buza. Ngoko ke kubalulekile ukugcina ithemba liphila.

Ndingamnyamekela njani umntwana wam?

Abantwana abazelwe benesi simo banokuphila ubomi obusempilweni ngononophelo lwexesha elide oluvela kugqirha wentliziyo. Ungamnyamekela umntwana wakho ngokuthi:

• Nika umntwana wakho isitofu somkhuhlane (umkhuhlane) kunye nesitofu se-COVID-19 minyaka le.
• Yisa umntwana wakho kugqirha wentliziyo rhoqo emva kweenyanga ezintandathu okanye kube kanye ngonyaka .
• Nika umntwana wakho amayeza anikwe ngugqirha ngexesha elifanelekileyo .
• Nciphisa umsebenzi womzimba onzima womntwana wakho , njengoko ugqirha wakho ekucebisile.
• Ukuba umntwana wakho uneengxaki zokufunda , mncede.

Ndingayibuza yiphi imibuzo ugqirha wam?

Ukuba umntwana wakho une-HLHS, ungambuza ugqirha imibuzo efana nale:

• Zithini iingozi zotyando?
• Ngaba kukho naziphi na iingxaki ekufuneka uziqaphele emva kotyando?
• Ngawaphi amayeza afunekayo kumntwana wam? Ngaba kukho naziphi na iziphumo ebezingalindelekanga kuloo mayeza?
• Le meko (i-HLHS) iza kuyichaphazela njani ubomi bomntwana wam?
• Luluphi unyango olufunekayo emva kotyando kumntwana wam?
• Ukuba ndinomnye umntwana, ngaba kukho umngcipheko wokuba loo mntwana naye abe nomntwana (HLHS)?

Ukuba nomntwana one-HLHS kunokuba ngamava adinisayo ngokweemvakalelo kwaye abe nesizungu . Xa ujongene noxinzelelo kunye nokungaqiniseki ngesifo sentliziyo somntwana wakho, kubalulekile ukufumana iqela lenkxaso okanye enye indawo yokufumana inkxaso ngokweemvakalelo. Ukuhlala uqinile ngokwengqondo kuya kukunceda ujongane nobunzima nempilo yomntwana wakho.

Umyalezo Wokuya Ekhaya

(HLHS) sisifo sentliziyo esinzulu, esizalwa naso. Nangona kunjalo, ukuba sifunyenwe kwangethuba kwaye sinyangwa ngokufanelekileyo, abantwana banethuba lokuphila ubomi obuhle. Oku kufuna utyando oluninzi olunzima kunye nolawulo lwezonyango olude. Eyona nto ibalulekileyo kukwazi ukuba awuwedwa. Oogqirha, abahlengikazi, kunye nabanye abasebenzi bezempilo bakhona ukunceda wena nomntwana wakho. Kubaluleke kakhulu ukufumana inkxaso kwabanye abazali abaye baba namava afanayo. Musa ukuphelelwa lithemba. Imibingelelo oyenzayo ngenxa yomntwana wakho ayinakuthelekiswa nanto.