I-Marfan Syndrome kunye neNtliziyo Yakho: Masithethe ngale nto!

Mhlawumbi ukhe wababona abantu abade kakhulu, abanemilenze emide, nababhityileyo kunabanye. Wonke umntu unale meko, kodwa ayisosifo. Kodwa ngamanye amaxesha, olu hlobo lomzimba lunokuba luphawu lwesifo semfuza esibizwa ngokuba yiMarfan Syndrome . Esi sisifo esenzeka xa izicubu ezidityanisiweyo emzimbeni wethu zingakhuli kakuhle. Ngamafutshane, esi zicubu ezidityanisiweyo yiyo ebamba amalungu ahlukeneyo omzimba wethu kunye kwaye iwanike amandla. Xa ezi zibuthathaka, amalungu amaninzi omzimba anokuchaphazeleka, ngakumbi intliziyo, amehlo, imithambo yegazi, kunye nenkqubo yamathambo . Namhlanje, siza kuthetha ngendlela esi sifo esibizwa ngokuba yiMarfan Syndrome esichaphazela ngayo intliziyo.

I-Marfan Syndrome iyichaphazela njani intliziyo?

Xa une-Marfan syndrome, iindawo ezimbini eziphambili zentliziyo zichaphazeleka kakhulu.

1. I-Aorta: Lo ngumthambo wegazi oyintloko, omkhulu nothwala igazi elineoksijini ukusuka entliziyweni yethu ukuya emzimbeni wonke. Ufana nombhobho oyintloko othwala amanzi ukusuka kwitanki yamanzi ukuya endlwini yakho.

2. Iivalvu zentliziyo: Ezi ziindawo ezisebenza njengeengcango phakathi kwamagumbi angaphakathi entliziyweni nakwimithambo ephambili ekhupha igazi entliziyweni. Ziqinisekisa ukuba igazi lihamba kwicala elinye kuphela.

Masijonge nganye kwezi ngokwahlukeneyo ukuze sibone ukuba kwenzeka ntoni.

Kwenzeka ntoni kwi-aorta?

I-Marfan syndrome ibangela ukuba izicubu ezidityanisiweyo ezindongeni zentliziyo zibe buthathaka. Njengetyhubhu yerabha endala, ilahlekelwa ngamandla ayo kunye nokuthamba kwayo. Oku kunokukhokelela kwiingxaki ezimbini eziphambili:

• Ukwanda kwe-aorta: I-aorta iqala ukwanda kancinci kancinci.
• I-Aortic aneurysm: Kwezinye iindawo, udonga lwentliziyo luba buthathaka kwaye lunokudumba ngaphandle njengebhaluni.

Khawuthelekelele, le aneurysm ifana netayara yebhayisekile evuthulukayo isuka kwenye indawo xa ityhubhu iba buthathaka.

Ngokukodwa, inxalenye yentliziyo ekufutshane nentliziyo, ebizwa ngokuba yi-'root aortic', yeyona ndawo idla ngokukhula okanye inwenwe ngale ndlela kubantu abane-Marfan syndrome. Le yimeko eyingozi kakhulu, kuba ukuba le 'aneurysm' ikhula ibe nkulu kwaye iqhume ('aortic dissection' okanye iqhume), inokuba yingozi ebomini.

Ezinye iimeko ezifana ne-Ehlers-Danlos syndrome, i-Loeys-Dietz syndrome, i-bicuspid aortic valve, kunye ne-Turner syndrome nazo zinokubangela ukwandiswa kwentliziyo, kodwa zinokwenzeka nakwezinye iindawo zentliziyo.

Kwenzeka ntoni kwiivalvu zentliziyo?

I-Marfan syndrome inokubangela iingxaki kwiivalvu zentliziyo. Ukuba ezi valve azisebenzi kakuhle, intliziyo kufuneka isebenze nzima ukupompa igazi. Ekuhambeni kwexesha, oku kunokukhokelela ekusileleni kwentliziyo.Ingahamba nangayiphi na indlela. Zimbini izifo eziphambili zevalvu ezibonwayo ezinxulumene neMarfan syndrome:

• Ukubuyela kwevalvu ye-Aortic: Xa ivalvu ephakathi kwe-aorta kunye negumbi elisezantsi lasekhohlo lentliziyo (i-ventricle yasekhohlo) ingavaleki kakuhle, inxalenye yegazi elimpompiweyo ivuza kwakhona entliziyweni. Kufana nombhobho ovuzayo.
• Ukuhla kwevalvu yeMitral: Ivalvu yemitral, ephakathi kwegumbi eliphezulu (i-atrium yasekhohlo) kunye negumbi elisezantsi (i-ventricle yasekhohlo) kwicala lasekhohlo lentliziyo, ayivali kakuhle, kwaye igumbi eliphezulu liphuma ngaphandle. Oku ngamanye amaxesha kunokubangela ukuba igazi livuza ngasemva (mitral regurgitation).

Isifo sentliziyo sixhaphake kangakanani kubantu abaneMarfan syndrome?

Enyanisweni, abantu abane-Marfan syndrome basengozini enkulu yokuba neengxaki zentliziyo. Uphando lubonise ukuba abantu abalithoba kwabalishumi abane-Marfan syndrome baya kuba nengxaki ethile kwi-valve yentliziyo okanye kwi-aorta yabo. Yiyo loo nto kubalulekile ukukwazi oku.

Ziziphi iimpawu zeMarfan syndrome ezichaphazela intliziyo?

Ukuba une-Marfan syndrome, efana ne-aortic aneurysm okanye isifo se-valve, unokufumana iimpawu ezithile. Nangona kunjalo, abanye abantu banokuba nezi meko ngaphandle kweempawu. Yiyo loo nto uvavanyo lwezonyango lubalulekile.

Ezi ziimpawu ezinokubonwa ngokubanzi:

• Intlungu yesifuba okanye intlungu engasentla yomqolo
• Ukukhohlela igazi (olu luphawu oluyingozi kancinci)
• Ubunzima bokuginya ukutya (ukuba intliziyo enkulu icinezela umphimbo )
• Ukuziva unesizungu okanye unentloko ebuhlungu
• Ndiziva ndidiniwe kakhulu okanye ndibuthathaka
• Ukuziva ukubetha kwentliziyo okungaqhelekanga (ukubetha kwentliziyo okungaqhelekanga)
• Ukurhawuzelela kwelizwi (ukuba intliziyo icinezela umthambo oqhagamshelwe kwiintambo zelizwi)
• Ubunzima bokuphefumla , ingakumbi xa udiniwe okanye ulele phantsi
• Ukudumba , ingakumbi emilenzeni nasemaqatheni
• Ukuphefumla (ukubetha umlozi) xa uphefumla

Khawuthelekelele ukuba unomhlobo omde kakhulu nobhityileyo. Uhlala ekhalaza ngeentlungu zesifuba kwaye enengxaki yokuhamba . Ukuba kunjalo, kungaba ngumbono olungileyo ukubona ugqirha ukuze ubone ukuba oko kunxulumene ne-Marfan syndrome.

Yintoni ebangela ukuba i-Marfan syndrome ichaphazele intliziyo?

Njengoko besitshilo ngaphambili, iMarfan syndrome sisifo esibangelwa kukunxibelelana kwezicubu zomzimba.Ayikakhiwa kakuhle. Izicwili ezidityanisiweyo ezisempilweni zifumaneka kuyo yonke indawo emizimbeni yethu. Zinika amandla, imo, kunye nokuguquguquka kwamalungu kunye nemithambo yegazi. Ezi zicwili ezidityanisiweyo zifumaneka eludongeni lwentliziyo kunye nemithambo yegazi, ngakumbi i-aorta, nakwiivalvu zentliziyo.

Xa ezi zicubu ezidityanisiweyo zibuthathaka ngenxa yeMarfan syndrome, zilahlekelwa ngamandla azo kunye nokuthamba kwazo. Oku kubangela ukuba intliziyo ikhule kwaye idumbe, ingakwazi ukumelana noxinzelelo lwegazi. Oku kubangela nokuba iivalvu zentliziyo zibe nesifo kwaye zingakwazi ukuvula okanye ukuvala kakuhle.

Ziziphi iimvavanyo zentliziyo ezinceda ekuxilongeni iMarfan syndrome?

I-Marfan syndrome ngamanye amaxesha inokuba nzima ukuyifumana, njengoko iimpawu zahlukile kumntu ngamnye kwaye zinokufana nezezinye iimeko. Uninzi lwabantu lufumanisa ukuba lunayo kuphela xa luseluncinci okanye lukhulile.

Ukuba ugqirha ukrokrela ukuba une-Marfan syndrome, baya kwenza izinto ezifana nezi:

• Kuza kwenziwa uvavanyo olupheleleyo lomzimba . Ukuphakama kwakho, ubunzima, ubude bengalo nomlenze, imo yesifuba, amehlo, kunye nolusu lwakho kuya kuhlolwa.
• Baza kubuza ngembali yempilo yosapho lwakho . Baza kujonga ukuba kukho umntu kusapho lwakho okhe waba neMarfan syndrome okanye iimpawu ezifanayo.
• Kuye kwenziwa iiskeni ezikhethekileyo (`iimvavanyo zomfanekiso`) ezihlola intliziyo . Ezona ziphambili zezi:
• I-Echocardiogram (Echo): Oku kukuvumela ukuba ubone ngokucacileyo ubungakanani, imilo, kunye nomsebenzi wentliziyo kunye nemithambo yegazi, njenge-ultrasound scan yentliziyo.
• I-Electrocardiogram (EKG): Le ilinganisa umsebenzi wombane wentliziyo, oko kukuthi, isingqisho sentliziyo.
• Uvavanyo lwemfuza: Olu vavanyo lungenziwa ukuqinisekisa ukuba unayo na inguqu yemfuza (kwi-FBN1 gene) ebangela i-Marfan syndrome.

Ziziphi iindlela zonyango zokulawula iingxaki zentliziyo ezibangelwa yiMarfan syndrome?

Nangona iMarfan syndrome ingenakunyangwa ngokupheleleyo, kukho unyango lokulawula iziphumo entliziyweni nokuthintela iingxaki ezinkulu. Ezi ziza ngeentlobo ezimbini: unyango olungelulo utyando kunye nonyango lotyando.

Ugqirha angacebisa utyando kwiimeko ezilandelayo:

• Ukuba ububanzi bomthambo wentliziyo yakho (i-aorta) buziisentimitha ezi-5 (malunga ne-1.97 intshi) okanye ngaphezulu.
• Ukuba izinga lokubetha kwentliziyo linyuka nge-0.5 centimeters (malunga ne-0.197 intshi) okanye ngaphezulu kunyaka (oku kubizwa ngokuba yi-'rapid expansion').
• Ukuba izalamane zakho zegazi (amalungu osapho lwakho) ziye zatyandwa ngolu hlobo (mhlawumbi ngenxa yempembelelo yemfuza, utyando lunokufuneka lwenziwe nokuba luncinci ububanzi).

Abantu abanemizimba emincinci badla ngokuba nemithambo yegazi emincinci, ngoko ke banokufuna utyando nokuba ububanzi bayo buncinci.

Unyango olungelulo utyando

La ngamanyathelo okuqala okulawula iMarfan syndrome.

• Utshintsho kwindlela yokuphila: Ngokuqinisekileyo kufuneka uziphephe izinto ezibangela uxinzelelo olukhulu entliziyweni.
• Akulunganga ukwenza izinto ezinje ngokuphakamisa iintsimbi okanye ukutyhala.
• Imidlalo enefuthe elikhulu efana nebhola ekhatywayo, iragbi, kunye nebhokisi ayifanelekanga.
• Thetha nogqirha wakho ukuze ufumane ukuba loluphi uhlobo lokuzivocavoca olukhuselekileyo nolufanelekileyo kuwe.
• Amayeza:
• Oogqirha banika amayeza abizwa ngokuba yi -`Angiotensin II receptor blockers (ARBs)` okanye i-`beta-blockers` . La mayeza anceda ekunciphiseni isantya sokwandiswa kwemithambo yentliziyo nokulawula uxinzelelo lwegazi.
• Uvavanyo oluqhelekileyo lwemifanekiso yentliziyo:
• Ubungakanani bentliziyo kunye nemeko yeevalvu kufuneka zihlolwe rhoqo ngugqirha. Oku kungenziwa kusetyenziswa iiskeni ezifana ne-`transthoracic echocardiography (Echo)`, `computerized tomographic angiography (CTA)`, okanye `magnetic resonance angiography (MRA)`.
• Ezi mvavanyo zinokubona utshintsho entliziyweni ngaphambi kokuba iqhekeke (iqhekeke).

Unyango lotyando

Ngamanye amaxesha, iingxaki zentliziyo azinakulawulwa ngamayeza kunye notshintsho kwindlela yokuphila kuphela. Kulapho ke kufuneka utyando.

Injongo ephambili yotyando lwentliziyo kwiMarfan syndrome kukulungisa inxalenye ebuthathaka yevalvu yentliziyo, ukuyisusa nokufaka ivalvu yokwenziwa, okanye ukulungisa ivalvu yentliziyo eyonakeleyo okanye ukuyitshintsha ngentsha.

Injongo ephambili yolu tyando kukuthintela iimeko ezingxamisekileyo ezisongela ubomi ezifana nokuqhekeka kwe-aorta.

Uninzi lwexesha, olu tyando lucwangciswa kwaye lwenziwa ngomhla omiselweyo (utyando olukhethiweyo). Nangona kunjalo, ukuba kuvela ukuqhekeka okanye ukuqhekeka kwemithambo yentliziyo ngequbuliso, kufuneka kwenziwe njengenkqubo engxamisekileyo.

Kukho iintlobo ezininzi eziphambili zotyando lwentliziyo olwenziwayo kwi-Marfan syndrome:

• Ukutshintshwa kweengcambu ze-Aortic: Olu lolona tyando luqhelekileyo kuba eli candelo lentliziyo lidla ngokudumba okanye livuleke.
• Ukulungiswa okanye ukutshintshwa kwevalvu ye-aorta.
• Ukulungiswa kokudumba kwimithambo yegazi kufutshane nentliziyo (`Ukulungiswa kwe-Ascending aortic aneurysm`).
• Ukulungiswa okanye ukutshintshwa kwevalvu yeMitral.

Ugqirha wentliziyo uza kuxoxa nawe aze agqibe ukuba loluphi utyando olulungele wena.

Ngaba isifo sentliziyo singathintelwa ukuba uneMarfan syndrome?

Ngelishwa, iMarfan syndrome ayinakuthintelwa, kuba ibangelwa lufuzo. Ngokufanayo, isifo sentliziyo esisibangelayo asinakuthintelwa ngokupheleleyo.

Nangona kunjalo, kukho izinto onokuzenza ukunciphisa umngcipheko weengxaki ezinkulu, ezifana nokuqhekeka kwe-aorta:

• Ukuphepha imisebenzi ebeka uxinzelelo entliziyweni (njengoko besitshilo ngaphambili, ukuphepha ukuphakamisa izinto ezisindayo kunye nemidlalo exinzelelekileyo).
• Ukuhlolwa kwemifanekiso yentliziyo kwenziwa ngexesha njengoko ugqirha emyalele.
• Ukuthatha onke amayeza anikwe ngugqirha ngokuchanekileyo nangexesha elifanelekileyo.

Into enye ekufuneka uyikhumbule ngakumbi kukuba ukuba ungumfazi one-Marfan syndrome kwaye ucinga ngokukhulelwa, ngokuqinisekileyo kufuneka uthethe nogqirha wakho ngayo. Izifundo zibonise ukuba ukuya kuthi ga kwi-40% yabasetyhini abane-Marfan syndrome banokufumana iingxaki ngexesha lokukhulelwa. Ke ngoko, kubaluleke kakhulu ukufuna iingcebiso zonyango ngaphambi kokuba ukhulelwe.

Iyintoni ikamva lomntu onesifo sentliziyo ngenxa yeMarfan syndrome?

Ukuba umntu onesifo seMarfan syndrome ulawulwa kakuhle, oko kukuthi, ulandela icebiso lezonyango aze afumane unyango olufunekayo, unokuphila ubomi obuqhelekileyo, mhlawumbi iminyaka engama-70 okanye engama-80.

Nangona kunjalo, iingxaki zentliziyo zezona zibangela ukufa phakathi kwabantu abaneMarfan syndrome, ngoko ke kubalulekile ukuhlala unxibelelana rhoqo nogqirha wakho wentliziyo ukuze ajonge imeko yakho.

Ndifanele ndimbone nini ugqirha? Zithini iimeko ezingxamisekileyo?

Ukuba ufumana naziphi na kwezi mpawu, isenokuba luphawu lokuqhekeka kwe-aortic aneurysm. Kufuneka ufune unyango olungxamisekileyo ngoko nangoko:

• Ukulahlekelwa yingqondo ngequbuliso.
• Isicaphucaphu nokuhlanza.
• Ukungabi nangqondo naphi na emzimbeni.
• Ukukhubazeka (ukungakwazi ukushukumisa ezinye iindawo zomzimba).
• Ubunzima obukhulu bokuphefumla.
• Intlungu engathethekiyo nenganyamezelekiyo - esifubeni, emqolo ongasentla, okanye esiswini.
• Ukubila okugqithisileyo.
• Ulusu luba mhlophe okanye lubanda ngendlela engaqhelekanga.
• Ukubetha kwentliziyo okubuthathaka kakhulu.

Ukuba ubona uphawu olufana nolu, ungalibazisi nomzuzu omnye.

Okokugqibela, yintoni ekufuneka uyikhumbule (Umyalezo Wokuya Ekhaya)

I-Marfan syndrome yimeko yokuzalwa echaphazela izicubu zomzimba ezinxibeleleneyo. Ingayichaphazela kakhulu intliziyo. Kodwa ungakhathazeki. Ukuba le meko ifunyaniswa kwangethuba kwaye ilawulwa ngokufanelekileyo, ungaphila ubomi obuqhelekileyo.

Eyona nto ibalulekileyo yile:

• Bona ugqirha wakho rhoqo.Emva koko unokujonga imeko yakho aze akunike uvavanyo olufunekayo kunye nonyango ngexesha elifanelekileyo.
• Yenza utshintsho olufunekayo kwindlela ophila ngayo. Ziphephe izinto ezikukhathazayo.
• Thatha iyeza kanye njengoko kuchaziwe.
• Ukuba ugqirha ucebisa utyando, thetha ngalo ngononophelo uze ukhethe okona kukulungeleyo.

Okukhona usazi ngakumbi ngemeko yakho, kokukhona kuya kuba lula kuwe ukuyilawula. Ke ngoko, sinethemba lokuba olu lwazi luya kuba luncedo kuwe. Ukuba unemibuzo, ungathandabuzi ukubuza ugqirha wakho.

I- Marfan Syndrome, isifo sentliziyo, inkqubo yentliziyo, iivalvu zentliziyo, izicwili ezidityanisiweyo, izifo zemfuza, utyando lwentliziyo