Ngaba ukuhamba kwegazi kwentliziyo yakho kuye kwaphambukiswa? Masifunde nge-PAPVR (Partial Anomalous Pulmonary Venous Return)!

Ngaba ngamanye amaxesha uziva udiniwe? Ngaba ukufumanisa kunzima ukuphefumla xa unyuka izinyuko? Okanye ngaba unamathandabuzo malunga nokukhula komntwana wakho? Kunokubakho izizathu ezininzi zezi zinto. Kodwa ngamanye amaxesha unobangela woku unokuba lutshintsho oluncinci entliziyweni esizalwa sinalo. Namhlanje siza kuthetha ngemeko enjalo, enxulumene nokujikeleza kwegazi entliziyweni, kodwa ayiqhelekanga kangako. Oku kubizwa ngokuba yi-PAPVR, emele (Partial Anomalous Pulmonary Venous Return).

Ngaba uyazi ukuba yintoni i-PAPVR (Partial Anomalous Pulmonary Venous Return)?

Ngamafutshane, i-PAPVR sisifo sentliziyo esizalwa naso . Okwenzekayo koku kukuba kukho ingxaki encinci ngendlela igazi elicociweyo, elineoksijini elivela emiphungeni yethu elibuyela ngayo entliziyweni. Ngokwesiqhelo, lonke eli gazi lineoksijini liya kwicala lasekhohlo lentliziyo. Emva koko, icala lasekhohlo lentliziyo limpompa elo gazi liye kuwo wonke umzimba.

Kodwa kumntu one-PAPVR, lincinci kakhulu eli gazi liphuma emiphungeni eliya kwicala lasekunene lentliziyo . Uyazi, icala lasekunene lentliziyo lihlala lifumana igazi elisetyenziswe ngumzimba, elinomoya-mpilo omncinci. Ngoko ke xa elinye igazi elinomoya-mpilo lihamba ngendlela engalunganga, inani legazi eliza kwicala lasekunene lentliziyo liyanda. Kwenzeka ntoni ke? Icala lasekunene lentliziyo kufuneka lisebenze neli gazi lingaphezulu, oko kuthetha ukuba kufuneka lisebenze nzima . Lo mthwalo ungaphezulu unokuzalisa intliziyo ngokuhamba kwexesha, kwaye unokuchaphazela nempilo yethu iyonke.

Kodwa i-PAPVR ayichaphazeli wonke umntu ngendlela efanayo. Kuxhomekeke kubungakanani begazi eliphambukisiweyo. Abanye abantu banokuba nesifo esincinci kakhulu, kwaye basenokungabi nazimpawu ubomi babo bonke. Kodwa ukuba kuvela iimpawu okanye kuvela ezinye iingxaki, oogqirha bethu banokuyilawula kakuhle le meko. Kwiimeko ezininzi, utyando lunokuyinyanga ngempumelelo i-PAPVR kwaye lukuncede uphile ubomi obude nobunempilo.

Le yindlela eqhelekileyo yokuhamba kwegazi ezintliziyweni zethu, kwaye itshintsha njani xa sine-PAPVR.

Ukuze siqonde impembelelo ye-PAPVR ekuhambeni kwegazi, masiqale sijonge indlela igazi elihamba ngayo rhoqo phakathi kwentliziyo nemiphunga yethu. Cinga ngayo njengohambo olufutshane.

1. Okokuqala, igazi elisetyenzisiweyo nelingenaoksijini emzimbeni lihamba ngemithambo emibini emikhulu (i-vena cava ephezulu kunye ne-vena cava engaphantsi) liye kwigumbi eliphezulu (i-atrium yasekunene) kwicala lasekunene lentliziyo.

2. Emva koko, eli gazi lingenawo umoya-mpilo lihamba lisuka kwigumbi eliphezulu lasekunene liye kwigumbi elisezantsi lasekunene (`(i-ventricle yasekunene)`).

3. Eli gazi limpontshwa lisuka kwigumbi elisezantsi lasekunene liye emiphungeni , nto leyo encediswa yimithambo yemiphunga. Emiphungeni, eli gazi liphinda lifakwe ioksijini, ngokungathi linikwe ubomi obutsha.

4. Igazi elitsha, elineoksijini ngoku libuyela entliziyweni ngemithambo emininzi (imithambo yemiphunga) ukusuka emiphungeni, lisiya ngqo kwigumbi eliphezulu lasekhohlo lentliziyo.(`(i-atrium yasekhohlo)`).

5. Eli gazi lizele yioksijini elivela kwigumbi eliphezulu ngasekhohlo liya kwigumbi elisezantsi ngasekhohlo (`(i-ventricle yasekhohlo)`).

6. Okokugqibela, eli gazi lizele yioksijini elivela kwigumbi elisezantsi kwicala lasekhohlo lisasazwa emzimbeni wonke ngomthambo wegazi omkhulu (i-aorta).

Uyabona, ngesiqhelo igazi elineoksijini encinci likwiigumbi ezingasekunene, kwaye igazi elineoksijini eninzi likwiigumbi ezingasekhohlo. Azidibani.

Kodwa nge-PAPVR, le ndlela ayisebenzi kakuhle. I-PAPVR ibangela indlela engaqhelekanga yokuhamba kwegazi entliziyweni, ebizwa ngokuba yi-cardiac shunt esuka ekhohlo ukuya ekunene. "Into engaqhelekanga" ithetha ukuba yinxalenye yegazi elineoksijini elivela emiphungeni kuphela elingafikeleliyo entliziyweni ngendlela eqhelekileyo.

Oku kungenxa yokuba omnye okanye ngaphezulu kwemithambo yakho yemiphunga ipompa igazi elineoksijini kwigumbi eliphezulu ngasekunene, endaweni yokupompa kwigumbi eliphezulu ngasekhohlo. Kwenzeka ntoni emva koko? Igazi elingenaoksijini kwigumbi lakho eliphezulu ngasekunene lixutywa negazi elineoksijini eninzi. Ngoko ke, eli gazi lixutyiweyo lipompelwa kwigumbi elisezantsi ngasekunene, emiphungeni.

Oku kunemiphumo emibi emibini:

• Eyokuqala kukuba elinye igazi elinomoya-mpilo omninzi ngokungeyomfuneko libuya ngendlela efanayo nelafika ngayo, oko kukuthi, emiphungeni.
• Okwesibini, ubungakanani begazi elisetyenziswa kwigumbi lakho lasekunene elisezantsi ukuze ulipompe liye emiphungeni yakho buyanda . Lo msebenzi wongezelelekileyo unokubangela uxinzelelo entliziyweni yakho ngokuhamba kwexesha.

Ngaba ezinye izifo zentliziyo zingenzeka nge-PAPVR?

Abanye abantu banokuba ne-PAPVR yodwa, ngaphandle kwesinye isifo sentliziyo. Oku kubizwa ngokuba yi-"isolated PAPVR". Nangona kunjalo, abantu abaninzi abane-PAPVR banokuba nezinye iingxaki, njengomngxuma ophakathi kwamagumbi amabini aphezulu entliziyo . Oku kubizwa ngokuba yi-Atrial Septal Defect (ASD).

Kunqabile kakhulu, i-PAPVR inokunxulunyaniswa nokukhubazeka komphunga wasekunene kunye nomthambo othwala igazi ukuya emphungeni (`(Scimitar syndrome)`).

Inqabile kangakanani i-PAPVR?

Abaphandi baqikelela ukuba malunga nabantu abayi-4 ukuya kwabayi-7 kwabayi-1,000 bane-PAPVR. Nangona kunjalo, eli nani linokuba phezulu kakhulu, njengoko abanye abantu besenokungaze babe nazo naziphi na iimpawu kwaye basenokungaze baxilongwe ukuba banale meko.

Zithini iimpawu ze-PAPVR?

Ukuba umlinganiselo wegazi oya kwicala elingalunganga (elibizwa ngokuba yi-'shunting') uphantsi, i-PAPVR ayisoloko ibangela iimpawu. Nangona kunjalo, xa oku 'shunting' kusanda, unokubona iimpawu. Abantwana abancinci banokuba neempawu ukuba banale meko kunye nezinye izifo zentliziyo. Nangona kunjalo, uninzi lwabantu abane-PAPVR luqala ukuba neempawu xa beneminyaka engama-20 okanye engama-30 .

Iimpawu kubantwana abancinci:

• Ukulibaziseka kophuhliso.
• Ukudinwa ngokukhawuleza xa usenza umthambo okanye udlala.
• Usulelo oluqhelekileyo lokuphefumla (umz., ukuxinana kwesifuba).
• Ukuqhawukelwa ngumphefumlo.
• Ubunzima bokufumana ubunzima.

Iimpawu kubantu abadala:

• Ubunzima bokuphefumla.
• Ukuziva udiniwe ngalo lonke ixesha (`(Ukudinwa)`).
• Ukuva ukubetha kwentliziyo okungaqhelekanga (`(Intliziyo ibetha ngamandla)`).
• Intlungu yesifuba (`(Intlungu yesifuba)`).
• Isantya sentliziyo siyanda.
• Iintsholongwane zokuphefumla ezixhaphakileyo.

Kutheni le PAPVR isenzeka?

Njengezinye izifo zentliziyo ezizalwa nazo, i-PAPVR yenzeka xa intliziyo ingakheki kakuhle ngexesha lokukhula komntwana ongekazalwa . Ngokwesiqhelo, kwiiveki ezimbalwa zokuqala zokukhulelwa, imithambo emine yemiphunga iyavela kumthambo oqhelekileyo wemiphunga. Le mithambo iqhagamshela kwigumbi eliphezulu lasekhohlo lentliziyo yomntwana ongekazalwa. Nangona kunjalo, ukuba kukho naluphi na utshintsho kule nkqubo, umthambo wemiphunga unokunxibelelana negumbi eliphezulu lasekunene endaweni yegumbi eliphezulu lasekhohlo, okanye neminye imithambo ekufutshane (njenge-vena cava ephezulu okanye umthambo we-brachiocephalic wasekhohlo). Kulapho i-PAPVR isenzeka khona.

Ziziphi iingxaki ezinokubakho ze-PAPVR?

Ii-shunts ezisuka ekhohlo ukuya ekunene ezingakhange zinyangwe ixesha elide zinokubuyela umva kwaye zikhokelele kwimeko embi ebizwa ngokuba yi-Eisenmenger syndrome. Ke ngoko, kubalulekile ukufuna ingcebiso kagqirha ngokukhawuleza ukuba uneempawu.

Uyazi njani ukuba une-PAPVR?

Oogqirha baxilonga i-PAPVR ngovavanyo lomzimba kunye nezinye iimvavanyo ezikhethekileyo . Ngelixa bekuhlola, ugqirha wakho uza kumamela intliziyo yakho nge-stethoscope. Abanye abantu abane-PAPVR banokuva isandi sentliziyo esingaqhelekanga (ukugquma kwentliziyo). Ukuba oku kurhanelwa, ugqirha wakho uza kuyalela iimvavanyo ezongezelelweyo.

Ubudala bokuxilongwa kwe-PAPVR buyahluka kumntu nomntu. Ingafunyanwa isengumntwana, okanye ingafunyanwa sele ikhulile. Ungaba ne-PAPVR, kodwa ungabi nazimpawu, ngoko usenokungazi ubomi bakho bonke. Ngamanye amaxesha, le meko ifunyanwa ngengozi ngexesha lovavanyo olwenziwe kwenye into.

Zeziphi iimvavanyo ezenziwayo?

Uvavanyo olunceda ekuxilongeni i-PAPVR zezi:

• I-Echocardiogram (Echocardiogram) - ngakumbi i-transesophageal echocardiogram (TEE). Oku kuthatha umfanekiso we-ultrasound wentliziyo.
• I-MRI yentliziyo (i-MRI yentliziyo).
• I-cardiac computed tomography (CT) scan.

Ngaba i-PAPVR ifuna utyando?

Abanye abantwana nabantu abadala kufuneka benze utyando lokulungisa i-PAPVR ukuze kulungiswe indlela yokuhamba kwegazi entliziyweni. Abantwana abafunyaniswe bene-PAPVR banamathuba amaninzi okufuna utyando kunabantu abadala. Oku kungenxa yokuba badla ngokuba ne-PAPVR kunye nezinye izifo zentliziyo ezifuna utyando.

Oogqirha bacebisa utyando.Kuphela xa kuyimfuneko ngokwenene ukunciphisa iimpawu okanye ukuthintela iingxaki ezizayo. Wena okanye umntwana wakho unokufuna utyando ukuba i-PAPVR ibangela ukushukuma okukhulu ukusuka ekhohlo ukuya ekunene. Oku kuthetha ukuba inani elikhulu legazi ekufuneka liye emzimbeni libuyela kwicala lasekunene lentliziyo nemiphunga. Oku kunokubangela iimpawu ezingathandekiyo kunye neengxaki ezifana noxinzelelo lwegazi oluphezulu lwemiphunga (i-PAH) okanye ukusilela kwentliziyo. Ugqirha wakho uza kusebenzisa iimvavanyo zokujonga imifanekiso ukuze abone ukuba olu shukuma lungakanani.

Utyando lunokunyangeka ngempumelelo i-PAPVR. Kubalulekile ukuthetha nogqirha wakho malunga neenkcukacha zotyando lwakho kunye noko ungakulindela.

Indlela yotyando ingahluka ngokuxhomekeke kwimilo yentliziyo yakho kunye nemithambo yemiphunga. Ngokubanzi, oogqirha bajonge ukutshintsha indlela igazi elihamba ngayo ukuze igazi elineoksijini lihambe liye kumagumbi asekhohlo entliziyo.

Nangona kunjalo, uninzi lwabantu aluyi kufuna utyando . Ukuba i-PAPVR yakho ayithambi, ugqirha wakho uya kuyijonga imeko yakho. Ukuba ubonakalisa iimpawu okanye ubonisa iimpawu zeengxaki, ugqirha wakho unokucebisa utyando ngelo xesha.

Ngaba i-PAPVR ingathintelwa?

Ngelishwa, akukho ndlela yokuthintela i-PAPVR. Yinto esizalwa nayo. Nangona kunjalo, ukuba uceba ukukhulelwa, thetha nogqirha wakho malunga neendlela zokunciphisa umngcipheko wokuba nomntwana onesiphene sentliziyo sokuzalwa. Nazi ezinye iingcebiso:

• Ziphephe ngokupheleleyo utywala, iziyobisi, iimveliso zecuba, kunye nomsi osetyenziswa ngabanye abantu.
• Yiya kuzo zonke iintlanganiso zokukhulelwa nogqirha wakho.
• Sebenzisana nogqirha wakho ukulawula naziphi na ezinye iimeko zempilo onokuba nazo (umz. isifo seswekile, uxinzelelo lwegazi oluphezulu).

Ngaba umntu one-PAPVR angaphila ubomi obuqhelekileyo?

Kunokwenzeka ngokupheleleyo ukuba uphile ubomi obude nobunempilo nge-PAPVR. Abantu abaninzi baphila iminyaka emininzi bengenazo iimpawu okanye iingxaki. Kwanabo bafuna utyando badla ngokuba neziphumo ezilungileyo. Nangona kunjalo, iziphumo zinokwahluka ngokuxhomekeke kwesinye isifo sentliziyo okanye iimeko ezingaphantsi komntu. Ngoko ke kungcono ukuthetha nogqirha wakho malunga nento omawuyilindele ngokwemeko yakho.

Ukuba une-PAPVR, uzinyamekela njani? / Umnyamekela njani umntwana wakho?

Ukuphila nesifo sentliziyo esizalwa naso kunokuba yingxaki ngamanye amaxesha. Kubalulekile ukukhumbula ukujongana nezinto ngosuku ngalunye. Nazi ezinye iingcebiso eziqhelekileyo onokuzilandela:

• Funa unyango kwingcali yentliziyo egxile kwizifo zentliziyo zabantu abadala. Uza kujonga imeko yakho ngokuhlolwa rhoqo nokuvavanywa.
• Buza ugqirha wakho ukuba zeziphi iintlobo zokuzilolonga ezifanelekileyo kuwe. Kunye, ningenza isicwangciso sokuzilolonga.
• Landela ukutya okunempilo kwentliziyo (umz., Ukutya kweMeditera).
• Sela onke amayeza njengoko ugqirha wakho ewayalele.
• Kuphephe ukutshaya kunye nazo zonke iimveliso zecuba. Buza ugqirha wakho ukuba ufuna uncedo lokuyeka ukutshaya.

Ukuba umntwana wakho une-PAPVR, buza ugqirha wakhe ukuba angamnyamekela njani ngcono. Umntwana wakho unokufuna ukulandela izikhokelo ezikhethekileyo zokuzilolonga okanye ezinye izinto azenzayo, ingakumbi ukuba unezinye iingxaki zentliziyo azalwe nazo.

Udinga nini ukubona ugqirha?

Bona ugqirha wakho njengoko kucwangcisiwe. Ukulandela rhoqo iintlanganiso kunye novavanyo lwemifanekiso kubalulekile ukujonga imeko ye-PAPVR yakho kunye nokulawula iingxaki.

Fowunela ugqirha wakho ngokukhawuleza ukuba wena okanye umntwana wakho nifumana nayiphi na kwezi zilandelayo:

• Ukuba kuvela iimpawu ezintsha okanye iimpawu ezikhoyo ziyatshintsha.
• Ukuba kukho iziphumo ebezingalindelekanga ezivela kwiyeza.
• Ukuba unemibuzo okanye iinkxalabo.

Kuphantsi kweziphi iimeko apho kufuneka uye kwiYunithi yoNyango oluNgxamisekileyo (i-ETU) ?

Ukuba wena okanye umntwana wakho nibonisa naziphi na kwezi mpawu, fowunela i-911 okanye inombolo yakho yongxamiseko yasekuhlaleni ngoko nangoko:

• Isantya sentliziyo esikhawulezayo kakhulu (ii-beats ezili-100-150 ngomzuzu) kwaye esinganciphisi isantya.
• Ukulahlekelwa zingqondo kunye nokuquleka.
• Ukuphefumla okugqithisileyo xa usenza umthambo okanye ungaphumli xa uphumla.
• Intlungu yesifuba ekhawulezileyo neqatha.
• Intloko ebuhlungu nengxamisekileyo.
• Ubuthathaka ngequbuliso okanye ukuba ndindisholo kwiingalo okanye emilenzeni.

Imibuzo ebalulekileyo yokubuza ugqirha wakho

Thetha nogqirha wakho ngemeko yakho kunye noko ungakulindela kwixesha elizayo. Nazi ezinye zemibuzo onokuyibuza:

Ukuba une-PAPVR:

• Ingxaki yam inzulu kangakanani?
• Zeziphi iinguqu zendlela yokuphila ekufuneka ndizenze?
• Ngaba ndiza kufuna utyando?
• Ziziphi iingenelo kunye neengozi zotyando?
• Liza kuba njani ikamva lam?

Ukuba umntwana wakho une-PAPVR, buza ugqirha wakhe:

• Ingxaki yomntwana wam inzulu kangakanani?
• Ngaba umntwana wam unezinye izifo zentliziyo azalwe nazo?
• Ngaba umntwana wam uza kufuna utyando okanye olunye unyango?
• Ndingamnyamekela njani umntwana wam ekhaya?
• Ngaba unayo nayiphi na imiqathango yemisebenzi yakhe?
• Ziziphi izixhobo ezikhoyo zokunceda umntwana wam aqonde oku ngendlela efanelekileyo kubudala bakhe?

Yintoni umahluko phakathi kwe-PAPVR kunye ne-TAPVR?

I-PAPVR yahlukile kwimeko ebizwa ngokuba yi-``Total Anomalous Pulmonary Venous Return (TAPVR)``. Kumntu one-TAPVR, yonke imithambo emiphungeni iqhagamshelwe entliziyweni ngendlela engaqhelekanga. I-TAPVR yimeko embi kakhulu kunezinye iintlobo ze-PAPVR, kwaye idla ngokufuna unyango olungxamisekileyo emva nje kokuzalwa. Nangona kunjalo, zombini i-PAPVR kunye ne-TAPVR ziyafana kuba ziphazamisa indlela eqhelekileyo yokuhamba kwegazi entliziyweni.

Okokugqibela, khumbula ezi zinto zilandelayo:

I-PAPVR isenokungachaphazeli ubomi bakho konke konke. Okanye, inokubangela iimpawu okanye iingxaki ezifuna unyango. Nangona kunjalo, kuqhelekile ukuba uzive uxinezelekile okanye uxhalabile nge-PAPVR. Ukuba umntwana wakho une-PAPVR, kuqhelekile ukuba ukhathazeke ngempilo-ntle yakhe. Ezi mvakalelo ziqhelekile, kwaye ungaziva kakubi ngazo.

Yabelana ngeengxaki zakho nogqirha wakho. Buza ngezixhobo ezinokukunceda wena nosapho lwakho. Ukufunda okungakumbi ngesifo sentliziyo esizalwa naso – nokuba siyakuchaphazela, umntwana wakho, okanye umntu omthandayo – kunokukunceda ulawule imeko yakho ngcono. Ukuthetha nomcebisi kunokukunceda ukhululeke. Ngeli xesha lohambo, ungaze ungathandabuzi ukubuza ugqirha wakho ngayo nayiphi na imibuzo okanye iinkxalabo onokuba nazo. Awuwedwa.

I- PAPVR, isifo sentliziyo, izifo zokuzalwa, imiphunga, ukujikeleza kwegazi, utyando lwentliziyo, isifo sentliziyo sokuzalwa, imithambo yemiphunga, utyando lwentliziyo