Ingaba umntwana wakho omncinci unale ngxaki yentliziyo? Masifunde nge-Pulmonary Artery Stenosis!

Ngaba umntwana wakho ngamanye amaxesha uziva ephelelwa ngumphefumlo? Okanye udinwa ngokukhawuleza nangona edlale okwethutyana? Abanye abantwana abancinci banokuba nezifo zentliziyo zokuzalwa. Namhlanje siza kuthetha ngesifo sentliziyo esinjalo, esingaqhelekanga, kodwa esibaluleke kakhulu ekufuneka sazi ngaso. Esi sibizwa ngokuba yi-`(Pulmonary Artery Stenosis)`. Ngamafutshane, kukuvaleka komthambo oyintloko othwala igazi ukuya emiphungeni.

Yintoni i-Pulmonary Artery Stenosis?

Ngamafutshane, kukho umthambo omkhulu wegazi othwala igazi ukusuka kwicala lasekunene lentliziyo yethu (esilibiza ngokuba yi-"right ventricle"`) ukuya emiphungeni. Oku kubizwa ngokuba yi-"pulmonary artery". Xa lifika emiphungeni, eli gazi lifakwa ioksijini, kwaye emva koko elo gazi lifakwe ioksijini lithwalwa kuwo wonke umzimba.

Ngoku, kule meko ibizwa ngokuba yi-`(Pulmonary Artery Stenosis)`, umthambo wegazi othwala igazi emaphashini uyavalwa okanye uncitshiswe kwenye indawo okanye kwelinye lamasebe awo. Kufana nombhobho wamanzi ovalwa phakathi. Kwenzeka ntoni ke emva koko? Kuba nzima ukuba igazi lihambe liye emaphashini. Ukuba igazi alihambi kakuhle, umzimba awufumani umlinganiselo weoksijini owufunayo. Ngenxa yesi sizathu, intliziyo yomntwana kunye nomzimba wonke awukwazi ukusebenza kakuhle.

Olu xinaniso lunokwenzeka kumthambo oyintloko wemiphunga (`(umthambo oyintloko wemiphunga)`), okanye kumasebe awo asekhohlo okanye asekunene (`(amasebe asekhohlo okanye asekunene)`). Xa olu xinaniso lusenzeka, icala lasekunene lentliziyo (`(i-ventricle yasekunene)`) kufuneka lisebenze nzima kakhulu ukupompa igazi lidlule kuloo xinaniso. Kufana nokusebenzisa amandla amaninzi ukutyhala amanzi kumbhobho ovalekileyo. Ekuhambeni kwexesha, lo mzamo uqhubekayo unokonakalisa izihlunu zentliziyo.

Zeziphi iingxaki ezinokuvela kule meko?

Ukuba oku akuphathwa kakuhle, ngokuhamba kwexesha icala lasekunene lentliziyo linokuba lingasebenzi kakuhle. Oko kukuthi, kukho ithuba lokufumana imeko ebizwa ngokuba yi-"Right-sided heart failure". Kungoko ke, kubaluleke kakhulu ukuyinyamekela le nto.

Ngubani ochaphazeleka kakhulu yile meko?

Le meko ibizwa ngokuba yi-`(Pulmonary Artery Stenosis)` ayiqhelekanga . Oko kuthetha ukuba ayenzeki kuwo wonke umntu. Abanye abantwana banokuyihlakulela kunye nezinye izifo zentliziyo (`(isifo sentliziyo esizalwa naso)` - oko kukuthi, izifo zentliziyo ezikhoyo ekuzalweni). Ngamanye amaxesha inokuba yodwa engenazo ezinye iingxaki zentliziyo. Kwakhona, le meko inokwenzeka emva kotyando lwentliziyo.

Zithini iimpawu zoku? Jonga ukuba umntwana wakho unazo na ezi.

Ezi mpawu zixhomekeke ekubeni umbhobho unciphile kangakanani. Ngamanye amaxesha, ukuba i-stenosis incinci kakhulu (`i-stenosis ethambileyo`), kusenokungabikho zimpawu konke konke . Kodwa njengoko i-stenosis isiba mandundu, umntwana wakho unokuzibonakalisa izinto ezifana nezi:

• Ukuziva ngathi kunzima ukuphefumla (`(Ukuphefumla kancinci)`).
• Ukuziva udiniwe ngalo lonke ixesha (`(Ukudinwa)`).
• Kuvakala ngathi uphefumla ngokukhawuleza kakhulu , okanye mhlawumbi uphefumlela phezulu.Ingaba ijongeka ngolu hlobo? (`(Ukuphefumla okunzima okanye okukhawulezayo)`).
• Ngaba isantya sentliziyo yakho sivakala ngathi sibetha ngokukhawuleza kakhulu ? (Isantya sentliziyo esikhawulezayo).
• Ngaba kukho ukudumba emilenzeni, emaqatheni, ebusweni, kwiinkophe, okanye esiswini sakho? (`(Ukudumba)`).
• Ngaba ngamanye amaxesha uziva unesizungu okanye uphelelwe ngamandla ? (`(Unesizungu okanye uphelelwe ngamandla)`).
• Ngaba imilebe yakho, iminwe, neenzwane zakho zijika zibe luhlaza okwesibhakabhaka ? Oku sikubiza ngokwezonyango ngokuba yi-"Cyanosis".
• Ngaba uyadinwa ngokukhawuleza xa udlala nabanye abantwana? Ngaba awukwazi ukubaleka nokutsiba kakuhle njengabanye abantwana? (`(Ukunyamezela umthambo okunciphileyo)`).

Khumbula, ukuba nenye okanye ezimbini kwezi mpawu akuthethi ukuba unesifo. Kodwa ukuba umntwana wakho unenye yezi zinto, kungcono ukufuna ingcebiso kagqirha.

Kutheni le nto umthambo wemiphunga uvalekile? Zithini izizathu?

Abanye abantwana bazalwa benale meko (i-Pulmonary Artery Stenosis), kwaye banokuba neengxaki ngeevalvu, iindonga, okanye ezinye iindawo zentliziyo. Abanye banokuzalwa benale meko kodwa abanazo ezinye iingxaki zentliziyo. Ukongeza, le meko ingenzeka emva kotyando lwentliziyo okanye ngenxa yezinye iimeko ezingaqhelekanga.

Izizathu zokuzalwa

Lilonke, malunga nama-40% abantu bazalwa benesi sifo (i-Pulmonary Artery Stenosis), kodwa abanazo ezinye iingxaki zentliziyo.

Nangona kunjalo, phakathi kwe-2% kunye ne-3% yabantu abanezinye iziphene zentliziyo abazalwa nazo banokuba nale meko (i-Pulmonary Artery Stenosis). Nazi ezinye zeziphene zentliziyo eziqhelekileyo:

• (Tetralogy of Fallot): Kule meko, umntwana uneengxaki ezine zentliziyo, eziphazamisa ukuhamba kwegazi.
• (Pulmonary atresia): Kule meko, ivalvu (pulmonary valve) ephakathi kwe-ventricle yasekunene yentliziyo kunye nomthambo we-pulmonary (pulmonary artery) ayiveli. Ngenxa yoko, igazi alihambi liye emiphungeni.
• (Truncus arteriosus): Umthambo wegazi omnye omkhulu endaweni yezimbini eziqhelekileyo. Oku kubangela ukuba igazi elineoksijini lixubane negazi elingenaoksijini.
• I-Aortic valve stenosis: Ingxaki yevalvu ekwi-main artery ethwala igazi lisuka entliziyweni ukuya emzimbeni, nto leyo enciphisa inani legazi elihamba liye emzimbeni.
• I-Atrial septal defect: Umngxuma eludongeni phakathi kwamagumbi amabini aphezulu entliziyo (i-atria). Oku kuvumela igazi elineoksijini nelingenaoksijini ukuba lixubane.
• Ingxaki ye-Ventricular septal: Umngxuma eludongeni owahlula amagumbi amabini asezantsi (ii-ventricles) entliziyo. Oku kunokubangela ukuba igazi elininzi lingene emiphungeni.
• (Ukutshintshwa kwemithambo emikhulu): Imithambo yegazi emibini ephambili ethwala igazi entliziyweni ijike yajika. Oku kuphazamisa ukuhamba kwegazi okuqhelekileyo kwaye kunciphisa ubungakanani beoksijini efikelela kwiiseli zomzimba.
• (I-Patent ductus arteriosus):Olu lunxibelelwano phakathi komthambo wemiphunga womntwana kunye nomthambo oyintloko othwala igazi emzimbeni (i-aorta). Oku kufanele kuvalwe emva kokuzalwa, kodwa ukuba akwenzeki njalo, igazi elininzi liya kuphuma liye emiphungeni.

Ezinye izizathu

Ngaphandle koku, kunokubakho ezinye izizathu ezininzi:

• Isifo seRubella: Ukuba ufumana iRubella ngexesha lokukhulelwa, umntwana wakho usenokuba neengxaki zentliziyo nezinye izifo.
• (i-Williams syndrome): Oku kubangela iingxaki ezahlukeneyo entliziyweni yomntwana nakwezinye izitho zomzimba.
• (Isifo se-Alagille): Oku kubangela iingxaki zesibindi kunye nesifo sentliziyo.
• I-arteritis kaTakayasu: Oku kukudumba okonakalisa imithambo yegazi emikhulu.
• Ezinye iingxaki malunga nendlela umthambo wemiphunga womntwana otyhalelwa ngayo ngaphandle.

Izizathu zotyando

Abanye abantu banokuba nale meko emva kotyando. Umzekelo:

• Ukufakelwa imiphunga.
• Utyando lokulungisa isiphene sentliziyo yokuzalwa kumntwana, okanye ukuphucula ukuhamba kwegazi entliziyweni.
• Utyando olubizwa ngokuba yi-"Pulmonary artery banding". Kule meko, umthambo uncitshiswa ngabom ukuze kuncitshiswe inani legazi eliya emiphungeni.

Indlela yokuxilonga esi sifo?

Xa umntwana wakho ehlolwa ngugqirha, usenokuva isandi sentliziyo esingaqhelekanga ("ukumbombozela"). Ukuba kunjalo, baya kuyalela ezinye iimvavanyo ezininzi. Ezinye zezi ziquka:

• I-Electrocardiogram - ECG okanye EKG: Le irekhoda utshintsho lombane olwenzeka xa intliziyo ibetha. Iyakwazi ukubona ukubetha kwentliziyo okungaqhelekanga (i-arrhythmias) kunye noxinzelelo kwimisipha yentliziyo.
• I-X-reyi yesifuba: Oku kungabonisa ubungakanani kunye nokuma kwentliziyo, imiphunga, kunye nemithambo yemiphunga.
• I-Echocardiogram: Oku kufana nomfanekiso oshukumayo wentliziyo. Isebenzisa amaza esandi ukujonga izihlunu zentliziyo kunye neevalvu.
• I-Cardiac magnetic resonance imaging (MRI): Le yindlela yokufota enemilinganiselo emithathu (3D) enokubona indlela igazi elihamba ngayo entliziyweni yomntwana wakho nakwimithambo yegazi.
• (I-CT scan): Olu luvavanyo lwe-`(X-ray)`. Ikhompyutha isebenzisa uthotho lwemifanekiso ye-`(X-ray)` yentliziyo yomntwana ukuze idale imibono enqamlezileyo. Idayi ekhethekileyo (`(IV contrast)`) ifakwa entliziyweni ukuze ijonge ulwakhiwo kunye nokuhamba kwegazi kwentliziyo.
• Ukufakwa kwe-catheter kwintliziyo: Kule nkqubo, kufakwa ityhubhu encinci (i-catheter) kumthambo okanye kumthambo wemithambo yegazi ize ikhokelwe entliziyweni. Oku kuvumela ugqirha ukuba ajonge amanqanaba eoksijini, utshintsho kuxinzelelo, aze athathe ii-X-ray zentliziyo.
• I-angiography yemiphunga: I-X-ray yemithambo yemiphunga kunye nemithambo yentliziyo ithathwa kusetyenziswa idayi.
• (Iskeni yokuHamba kwegazi): Kule meko, kufakwa inani elincinci lezinto ezinemisebe. Emva koko, kusetyenziswa umatshini okhethekileyo ukujonga ukuba igazi lihamba kakuhle kangakanani na kwimiphunga nganye.

Ukuba ugqirha wakho ufumanisa ukuba umntwana wakho une-Pulmonary Artery Stenosis, uza kukuthumela kwingcali yentliziyo yokuzalwa. Olu hlobo logqirha lunezakhono noqeqesho lokuchonga ngokuchanekileyo ingxaki yentliziyo yomntwana wakho, ukwenza naluphi na uvavanyo olufunekayo, ukunika unyango, ukwenza utyando lwentliziyo, kunye nokubonelela ngovavanyo olulandelayo . Banokwenza novavanyo olongezelelweyo ukuba kuyimfuneko.

Xa ethetha ngale meko, ugqirha wakho unokubiza ngokuba yi-Type I, II, III, okanye IV. Olu luhlulo olusekelwe ekubeni zeziphi iindawo zomthambo ezivalekileyo kunye nokuba zingakanani.

Zithini iindlela zokunyanga oku? (Unyango)

Unyango olungcono luxhomekeke kwiimpawu zomntwana wakho kunye nezinye izinto. Ukuba kukho ukuncitshiswa okuncinci ukuya kokuphakathi kwesebe elinye okanye ngaphezulu lomthambo wemiphunga, unyango lusenokungabi yimfuneko . Nangona kunjalo, ukuba ukuncitshiswa kukhulu, unyango luyimfuneko .

Unyango lwe-`(Pulmonary Artery Stenosis)` zezi:

Ukwandiswa kwebhaluni / i-Angioplasty

Kule meko, ugqirha wenza oku:

• I-catheter efana nebhaluni (i-catheter yokwandisa ibhaluni) ifakwa kumjelo ovalekileyo.
• Emva koko, ngononophelo olukhulu, ibhaluni iyavuthelwa ukusuka kuxinzelelo oluphantsi ukuya kuxinzelelo oluphezulu.
• Emva koko loo mbhobho uvaliweyo uyakhula.
• Emva koko umoya uyasuswa kwibhaluni uze ukhutshwe.

Ukwandiswa kwebhaluni kunye nokubekwa kwe-stent (oku kwenziwa rhoqo)

Kule meko, ugqirha wenza oku:

• I-stent enokwandiswa yibhaluni (i-stent yityhubhu encinci ye-mesh) isetyenziselwa ukuvula umthambo ovalekileyo.
• Le stent ibekwa phezu kwe-catheter yebhaluni ye-angioplasty ize igqunywe ngeshelufu.
• I-stent ifuduselwa kwindawo efanelekileyo.
• Isigqubuthelo esikwibhaluni ye-stent siyasuswa.
• Emva koko ibhaluni ivuthelwa ngamandla ukuya kuxinzelelo olufanelekileyo, yandisa i-stent kwaye ibangele ukuba ihlale inamathele apho.

Ibhaluni ekhethekileyo ebizwa ngokuba yi-``(The Cutting Balloon™)``

Le bhaluni ifana nje nebhaluni eqhelekileyo. Nangona kunjalo, inamabala amancinci amaninzi kubude bayo. Xa ugqirha efutha ibhaluni, la mabhaluni ayasebenza aze asike indawo evalekileyo. Oku kwenza kube lula ukwandisa umbhobho, kwenze kube lula ukuvula umngxuma omkhulu.

Uninzi lwabantu abangenazo ezinye izifo zentliziyo abazalwa nazo bafumana iziphumo ezilungileyo ngale nkqubo. Nangona kunjalo, malunga ne-21% yabantu banokuvaleka kwakhona umthambo wabo emva kweenyanga ezimbalwa.

Utyando

Oogqirha basebenzisa iindlela ezahlukeneyo zokunyanga i-pulmonary artery stenosis. Ukhetho luxhomekeke kuhlobo lokuvaleka, imithambo yegazi ejikelezileyo, kunye nezinye izakhiwo.

Ngaba kukho naziphi na iingxaki kunyango?

Ukwandiswa kweebhaluni kunciphisa ukuvaleka kwabantu abaninzi. Nangona kunjalo, ngokuhamba kwexesha, umthambo ungavaleka kwakhona nge-15% ukuya kwi-20%, kwaye le nkqubo kufuneka iphindwe. Abaphandi okwangoku baphanda iintlobo ezahlukeneyo zeebhaluni ezinokubonelela ngeziphumo ezingcono nezihlala ixesha elide.

Iingxaki ezinokwenzeka ngexesha lokuvulwa kwebhaluni:

• I-aneurysm yemithambo yemiphunga.
• Ukuqhaqha umthambo wepulmonary (Ukuqhaqha umthambo wepulmonary).
• Ukuqhekeka komthambo wemiphunga (`(Ukuqhekeka komthambo wemiphunga)`).
• Ukudumba kwemithambo yegazi (`(Ukudumba kwemithambo yegazi yegazi)`).
• Kunqabile kakhulu ukuba kubekho ukufa.

Iingxaki ezinokubakho zokusetyenziswa kwe-stent:

• Amahlwili egazi.
• Ukungahambi kakuhle kwesingqisho sentliziyo (`(Ventricular arrhythmias)`).
• I-stent isenokungafakwanga okanye ikhutshwe.
• Ukuphinda kufuneke ukuba ndivule umthambo kwakhona (oku kwenzeka rhoqo).

Zithini iingenelo zonyango?

Oogqirha bakhetha ukubeka ii-stents kwi-Pulmonary Artery Stenosis ngenxa yezi zizathu:

• Zisebenza malunga ne-96% ngoko nangoko .
• Kuthatha ixesha elide ukugcina umthambo uvulekile.
• Indawo encitshisiweyo ingandiswa ngokuphindwe kabini .
• Ayibizi kakhulu kwaye iyasebenza ngakumbi kunokufaka iibhaluni okanye ukwenza utyando.
• Iphumelele ngakumbi kune-balloon angioplasty.

Oogqirha banokukhetha i-balloon angioplasty endaweni ye-stent kwiimeko ezilandelayo:

• Ukuba imeko yomntwana imbi kakhulu .
• Ukuba isakhiwo sangaphakathi somzimba womntwana siyinkimbinkimbi (`(i-anatomy eyinkimbinkimbi)`).
• Ukuba umntwana mncinci kakhulu .

Kwenzeka ntoni emva kotyando?

Ukuba umntwana wakho une-stent, kuya kufuneka athathe amayeza okubulala iintsholongwane kwaye mhlawumbi amayeza okunciphisa igazi okwethutyana. La mayeza anikwa ukuthintela usulelo kunye nokuthintela ukujiya kwegazi.

Umntwana uza kuziva ngcono nini emva konyango?

Inkqubo yokwandisa umthambo wemiphunga womntwana wakho isebenza ngoko nangoko. Oku kuthetha ukuba kuba lula kakhulu kumntwana wakho ukufaka igazi emiphungeni yakhe aze afumane ioksijini.

Ukuba umntwana wam unale meko, liza kuba njani ikamva? (Ingqikelelo)

Ngenxa yonyango oluphucukileyo lwanamhlanje, abantu abaninzi abanale meko baphila baze babe ngabantu abadala. Nangona kunjalo, abantu abaneengxaki ezifana ne-"Williams syndrome" kunye ne-"Alagille syndrome" basenokungasabeli kakuhle kunyango.

Ufanele umse nini umntwana wakho kugqirha?

Kuya kufuneka umse umntwana wakho rhoqo ukuze ahlolwe ukuba unjani na. Kuya kufuneka wenze nee-echocardiogram rhoqo ukuze ubone ukuba umthambo kufuneka uvulwe kwakhona na kwixesha elizayo.

Ndingayibuza ntoni ugqirha?

Ungabuza ugqirha imibuzo efana nale:

• Kutheni umntwana wam waphuhliswa yi-`(Pulmonary Artery Stenosis)`?
• Yeyiphi indlela engcono yonyango kumntwana wam?
• Ngawaphi amathuba okuba umntwana wam aphinde anyangwe ngenxa yoku?

Ukuba umntwana wakho ufuna unyango lwe-Pulmonary Artery Stenosis, ugqirha uya kuqwalasela unyango olufanelekileyo kwimeko yomntwana wakho. Musa ukuthandabuza ukubuza imibuzo malunga nantoni na ongayiqondiyo malunga nenkqubo yomntwana wakho.

Umyalezo Wokuya Ekhaya

Xa kufikwa kule meko ibizwa ngokuba yi-`(Pulmonary Artery Stenosis)`, eyona nto ibalulekileyo kukubona iimpawu kwangethuba uze ufumane ingcebiso kagqirha . Kubalulekile ukufuna ingcebiso kwi-`(ingcali yentliziyo ezalwa nayo)`, ingakumbi ukuba abantwana banale meko.

Ungakhathazeki, kukho unyango loku . Le meko ingalawulwa ngezinto ezifana nokwandiswa kwebhaluni, ukufakwa kwe-stent, kwaye, ukuba kuyimfuneko, utyando. Kwanasemva konyango, kubaluleke kakhulu kwimpilo yomntwana ukuya kuhlolwa rhoqo kwezonyango kwaye alandele imiyalelo kagqirha . Thetha nogqirha ngayo nayiphi na imibuzo okanye iinkxalabo onokuba nazo.

I- Pulmonary Artery Stenosis, Isifo seNtliziyo sabantwana, Iziphene zeNtliziyo ezizalwa nazo, Ubunzima bokuphefumla, Utyando lweNtliziyo, I-Stent