I-Marfan Syndrome Nenhliziyo Yakho: Ake Sixoxe Ngalokhu!

Cishe uke wababona abantu abade kakhulu, abanemilenze emide, futhi abancane kunabanye. Wonke umuntu unalesi simo, kodwa akusona isifo. Kodwa ngezinye izikhathi, lolu hlobo lwesimo somzimba lungaba uphawu lwesifo sofuzo esibizwa ngokuthi i-Marfan Syndrome . Lesi yisimo esenzeka lapho izicubu ezixhumene emzimbeni wethu zingakhuli kahle. Kalula nje, lezi zicubu ezixhumene yizo ezibamba izingxenye ezahlukene zomzimba wethu ndawonye futhi zizinike amandla. Uma lezi zibuthakathaka, izingxenye eziningi zomzimba zingathinteka, ikakhulukazi inhliziyo, amehlo, imithambo yegazi, kanye nesistimu yamathambo . Namuhla, sizoxoxa ngendlela lesi simo esibizwa ngokuthi i-Marfan Syndrome esithinta ngayo inhliziyo.

I-Marfan Syndrome iyithinta kanjani inhliziyo?

Uma une-Marfan syndrome, izingxenye ezimbili eziyinhloko zenhliziyo zithinteka kakhulu.

1. I-Aorta: Lona umthambo wegazi oyinhloko, omkhulu kunayo yonke othwala igazi elinomoya-mpilo lisuka enhliziyweni yethu liye emzimbeni wonke. Kufana nepayipi eliyinhloko elithwala amanzi lisuka ethangini lamanzi liye endlini yakho.

2. Ama-valve enhliziyo: Lezi yizingxenye ezisebenza njengeminyango phakathi kwamakamelo angaphakathi kwenhliziyo kanye nasemithanjeni eyinhloko ekhipha igazi enhliziyweni. Ziqinisekisa ukuthi igazi ligeleza ohlangothini olulodwa kuphela.

Manje ake sibheke ngayinye yalezi ngokwehlukana ukuze sibone ukuthi kwenzekani.

Kwenzekani ku-aorta?

I-Marfan syndrome ibangela ukuba izicubu ezixhumeneyo ezindongeni zenhliziyo zibe buthaka. Njengepayipi elidala lerabha, ilahlekelwa amandla ayo kanye nokunwebeka kwayo. Lokhu kungaholela ezinkingeni ezimbili eziyinhloko:

• Ukwanda kwe-aorta: I-aorta iqala ukuvuleka kancane kancane futhi ikhule.
• I-Aortic aneurysm: Kwezinye izindawo, udonga lwenhliziyo luba buthakathaka futhi lungavuvukala ngaphandle njengebhaluni.

Cabanga nje, lokhu kuphazamiseka kwemithambo yegazi kufana nethayi lebhayisikili eliqhuma endaweni eyodwa lapho ipayipi liba buthakathaka.

Ikakhulukazi, ingxenye yenhliziyo eseduze nenhliziyo, ebizwa ngokuthi `impande ye-aorta`, yingxenye evame ukwandiswa noma ukunwetshwa ngale ndlela kubantu abane-Marfan syndrome. Lesi yisimo esiyingozi kakhulu, ngoba uma le `aneurysm` ikhula ibe nkulu futhi iqhume (`ukuqhekeka kwe-aorta` noma ukuqhekeka), ingaba yingozi empilweni.

Ezinye izimo ezifana ne-Ehlers-Danlos syndrome, i-Loeys-Dietz syndrome, i-bicuspid aortic valve, kanye ne-Turner syndrome nazo zingabangela ukwandiswa kwenhliziyo, kodwa zingase zenzeke kwezinye izingxenye zenhliziyo.

Kwenzekani kuma-valve enhliziyo?

I-Marfan syndrome ingabangela nezinkinga ngama-valve enhliziyo. Uma la ma-valve engasebenzi kahle, inhliziyo kufanele isebenze kanzima ukupompa igazi. Ngokuhamba kwesikhathi, lokhu kungaholela ekuhlulekeni kwenhliziyo.Kungahamba noma yikuphi. Izifo ezimbili ezinkulu ze-valve zibonakala zihambisana ne-Marfan syndrome:

• Ukubuyela kwe-valve ye-aorta: Uma i-valve ephakathi kwe-aorta negumbi elingezansi kwesobunxele senhliziyo (i-ventricle yesobunxele) ingavali kahle, elinye igazi elipompiwe liphinde lingene enhliziyweni. Kufana nompompi ovuzayo.
• Ukwehla kwevalvu ye-mitral: Ivalvu ye-mitral, etholakala phakathi kwegumbi eliphezulu (i-atrium yangakwesobunxele) kanye negumbi elingezansi (i-ventricle yangakwesobunxele) ohlangothini lwesobunxele lwenhliziyo, ayivaleki kahle, futhi igumbi eliphezulu liphuma ngaphandle. Lokhu ngezinye izikhathi kungabangela ukuthi igazi livuza emuva (mitral regurgitation).

Isifo senhliziyo sivame kangakanani kubantu abane-Marfan syndrome?

Eqinisweni, abantu abane-Marfan syndrome basengozini enkulu kakhulu yokuba nezinkinga zenhliziyo. Ucwaningo lukhombisile ukuthi abantu abayisishiyagalolunye kwabayishumi abane-Marfan syndrome bazoba nenkinga ethile nge-valve yenhliziyo noma i-aorta yabo. Yingakho kubalulekile ukuqaphela lokhu.

Yiziphi izimpawu ze-Marfan syndrome ezithinta inhliziyo?

Uma une-Marfan syndrome, njenge-aortic aneurysm noma isifo se-valve, ungase ube nezimpawu ezithile. Kodwa-ke, abanye abantu bangase babe nalezi zimo ngaphandle kwezimpawu. Yingakho ukuhlolwa kwezokwelapha kubalulekile.

Lezi yizimpawu ezingabonakala ngokujwayelekile:

• Ubuhlungu besifuba noma ubuhlungu obungaphezulu komhlane
• Ukukhwehlela igazi (lokhu kuyisibonakaliso esiyingozi kancane)
• Ubunzima bokugwinya ukudla (uma inhliziyo ekhulisiwe icindezela umphimbo )
• Ukuzizwa unesizungu noma unekhanda elibuhlungu
• Ukuzizwa ukhathele kakhulu noma ubuthakathaka
• Ukuzwa kwesilinganiso senhliziyo esingavamile (ukushaya kwenhliziyo okungavamile)
• Ukuhosha kwezwi (uma inhliziyo icindezela imithambo exhunywe ezintanjeni zezwi)
• Ubunzima bokuphefumula , ikakhulukazi uma ukhathele noma ulele phansi
• Ukuvuvukala , ikakhulukazi emilenzeni nasemaqakaleni
• Ukushaya umoya (ukushaya ikhwela) lapho uphefumula

Cabanga ukuthi unomngane omude kakhulu futhi omncane. Uvame ukukhala ngobuhlungu besifuba futhi unenkinga yokuhamba . Uma kunjalo, kungaba ngumqondo omuhle ukubona udokotela ukuze ubone ukuthi kuhlobene yini nesifo iMarfan syndrome.

Yini ebangela ukuthi i-Marfan syndrome ithinte inhliziyo?

Njengoba sishilo ngaphambili, i-Marfan syndrome iyisifo esithinta izicubu ezixhumeneyo.Akwakhiwa kahle. Izicubu ezixhumeneyo ezinempilo zitholakala yonke indawo emizimbeni yethu. Zinikeza amandla, ukuma, kanye nokuguquguquka ezithweni nasemithanjeni yegazi. Lezi zicubu ezixhumeneyo zitholakala ezindongeni zenhliziyo nemithambo yegazi, ikakhulukazi i-aorta, kanye nasemavalufini enhliziyo.

Uma lezi zicubu ezixhumeneyo ziba buthaka ngenxa ye-Marfan syndrome, zilahlekelwa amandla azo kanye nokunwebeka kwazo. Lokhu kubangela ukuthi inhliziyo ivuleke futhi ivuvuke, ingakwazi ukumelana nokucindezeleka kwegazi. Lokhu futhi kubangela ukuthi ama-valve enhliziyo abe nesifo futhi angakwazi ukuvula noma ukuvala kahle.

Yiziphi izivivinyo zenhliziyo ezisiza ekuxilongeni i-Marfan syndrome?

I-Marfan syndrome ngezinye izikhathi ingaba nzima ukuyixilonga, njengoba izimpawu ziyahlukahluka kumuntu nomuntu futhi zingafana nezezinye izimo. Abantu abaningi bathola ukuthi banayo lapho besebancane noma bekhulile.

Uma udokotela esola ukuthi une-Marfan syndrome, uzokwenza izinto ezifana nalezi:

• Kuzohlolwa umzimba wakho ngokuphelele . Ukuphakama kwakho, isisindo, ubude bengalo nomlenze, ukuma kwesifuba, amehlo, kanye nesikhumba kuzohlolwa.
• Bazobuza ngomlando wezokwelapha womndeni wakho . Bazohlola ukuthi ukhona yini emndenini wakho oke waba ne-Marfan syndrome noma izimpawu ezifanayo.
• Kuhlelwe ukuskena okukhethekile okuningana (`ukuhlolwa kwezithombe`) okuhlola inhliziyo . Okuyinhloko yile:
• I-Echocardiogram (Echo): Lokhu kukuvumela ukuthi ubone kahle usayizi, ukuma, kanye nokusebenza kwenhliziyo nemithambo yegazi, njengokuskena kwenhliziyo nge-ultrasound.
• I-Electrocardiogram (EKG): Lokhu kulinganisa umsebenzi kagesi wenhliziyo, okungukuthi, isigqi sokushaya kwenhliziyo.
• Ukuhlolwa kofuzo: Lokhu kuhlolwa kungenziwa ukuqinisekisa ukuthi une-mutation yofuzo (ku-gene ye-FBN1) ebangela i-Marfan syndrome.

Yiziphi izindlela zokwelapha izinkinga zenhliziyo ezibangelwa yi-Marfan syndrome?

Nakuba i-Marfan syndrome ingelapheki ngokuphelele, kunezindlela zokwelapha ezilawula imiphumela enhliziyweni futhi zivimbele izinkinga ezinkulu. Lezi ziza ngezinhlobo ezimbili: ukwelashwa okungekona ukuhlinzwa kanye nokwelashwa ngokuhlinzwa.

Udokotela angase ancome ukuhlinzwa ezimweni ezilandelayo:

• Uma ububanzi bomthambo wakho wenhliziyo (i-aorta) bungamasentimitha angu-5 (cishe amasentimitha angu-1.97) noma ngaphezulu.
• Uma izinga lokushaya kwenhliziyo likhuphuka ngamasentimitha angu-0.5 (cishe amasentimitha angu-0.197) noma ngaphezulu phakathi nonyaka (lokhu kubizwa ngokuthi 'ukwanda okusheshayo').
• Uma izihlobo zakho zegazi (amalungu omndeni wakho wemvelo) sezike zahlinzwa ngalolu hlobo (mhlawumbe ngenxa yethonya lezakhi zofuzo, kungase kudingeke ukuthi kwenziwe ukuhlinzwa ngisho nangobubanzi obuncane).

Abantu abanemizimba emincane ngokuvamile banemithambo yegazi emincane, ngakho-ke bangadinga ukuhlinzwa noma ngabe ububanzi bayo buncane.

Ukwelashwa okungekona ukuhlinzwa

Lezi yizinyathelo zokuqala zokuphatha i-Marfan syndrome.

• Ukushintsha indlela yokuphila: Kufanele nakanjani ugweme imisebenzi ebeka ukucindezeleka okwengeziwe enhliziyweni.
• Akukuhle ukwenza izinto ezifana nokuphakamisa izinsimbi noma ukusunduza.
• Imidlalo enomthelela omkhulu njengebhola, i-rugby, kanye ne-boxing ayifaneleki.
• Khuluma nodokotela wakho ukuze uthole ukuthi yikuphi ukuvivinya umzimba okuphephile nokufanelekile kuwe.
• Imithi:
• Odokotela banikeza imithi ebizwa ngokuthi `Angiotensin II receptor blockers (ARBs)` noma `beta-blockers` . Lokhu kusiza ekunciphiseni izinga lapho imithambo yenhliziyo ivuleka khona futhi ilawule umfutho wegazi.
• Ukuhlolwa kwezithombe zenhliziyo okuvamile :
• Ubukhulu benhliziyo kanye nesimo sama-valve kufanele kuhlolwe njalo ngudokotela. Lokhu kungenziwa kusetshenziswa ama-scan anjenge-`transthoracic echocardiography (Echo)`, `computerized tomographic angiography (CTA)`, noma `magnetic resonance angiography (MRA)`.
• Lezi zivivinyo zingabona izinguquko enhliziyweni ngaphambi kokuba iqhume (ihlukanise).

Ukwelashwa ngokuhlinzwa

Ngezinye izikhathi, izinkinga zenhliziyo nemithambo yegazi azikwazi ukulawulwa ngemithi kanye nokushintsha indlela yokuphila kuphela. Yilapho-ke kudingeka khona ukuhlinzwa.

Umgomo oyinhloko wokuhlinzwa kwenhliziyo kwe-Marfan syndrome ukulungisa ingxenye ebuthaka yevalvu yenhliziyo, ukuyisusa bese ufaka ivalvu yokwenziwa, noma ukulungisa ivalvu yenhliziyo eyonakele noma ukuyishintsha ngentsha.

Umgomo oyinhloko walokhu kuhlinzwa ukuvimbela izimo eziphuthumayo ezisongela ukuphila njengokusikwa kwe-aorta.

Esikhathini esiningi, lokhu kuhlinzwa kuhlelwa futhi kwenziwa ngosuku olunqunyiwe kusengaphambili (ukuhlinzwa okukhethayo). Kodwa-ke, uma kwenzeka ukuqhekeka noma ukuqhekeka komthambo wenhliziyo ngokuzumayo, kufanele kwenziwe njengenqubo ephuthumayo.

Kunezinhlobo eziningana eziyinhloko zokuhlinzwa kwenhliziyo ezenzelwa i-Marfan syndrome:

• Ukufakwa esikhundleni kwezimpande ze-aorta: Lokhu kuyindlela yokuhlinzwa evame kakhulu ngoba le ngxenye yenhliziyo ivame ukuvuvukala noma ukuvuleka.
• Ukulungiswa noma ukushintshwa kwevalvu ye-aorta.
• Ukulungiswa kokuvuvukala emthanjeni eduze kwenhliziyo (`Ukulungiswa kwe-Ascending aortic aneurysm`).
• Ukulungiswa noma ukushintshwa kwevalvu ye-mitral.

Udokotela ohlinza inhliziyo uzoxoxa nawe futhi anqume ukuthi yikuphi ukuhlinzwa okukulungele.

Ingabe isifo senhliziyo singavinjelwa uma une-Marfan syndrome?

Ngeshwa, i-Marfan syndrome ayinakuvinjelwa, njengoba iwufuzo. Ngokufanayo, isifo senhliziyo esisibangelayo asinakuvinjelwa ngokuphelele.

Kodwa-ke, kunezinto ongazenza ukuze unciphise ingozi yezinkinga ezinkulu, njengokusikwa kwe-aorta:

• Ukugwema imisebenzi ebeka ukucindezeleka enhliziyweni (njengoba sixoxile ngaphambili, ukugwema ukuphakamisa izinto ezisindayo kanye nemidlalo ecindezela kakhulu).
• Ukuhlolwa kwezithombe zenhliziyo kwenziwa ngesikhathi njengoba udokotela emyalile.
• Ukuthatha yonke imithi oyinikezwe udokotela ngendlela efanele nangesikhathi.

Into eyodwa okufanele uyikhumbule ikakhulukazi ukuthi uma ungowesifazane one-Marfan syndrome futhi ucabanga ngokukhulelwa, kufanele nakanjani ukhulume nodokotela wakho ngakho. Ucwaningo lubonise ukuthi abesifazane abangafika ku-40% abane-Marfan syndrome bangase babe nezinkinga ngesikhathi sokukhulelwa. Ngakho-ke, kubaluleke kakhulu ukufuna iseluleko sezokwelapha ngaphambi kokukhulelwa.

Iyini ikusasa lomuntu onesifo senhliziyo ngenxa ye-Marfan syndrome?

Uma umuntu ene-Marfan syndrome ephathwa kahle, okungukuthi, elandela iseluleko sezokwelapha futhi ethola ukwelashwa okudingekayo, angaphila isikhathi esivamile sokuphila, mhlawumbe iminyaka engu-70 noma engu-80.

Kodwa-ke, izinkinga zenhliziyo ziyimbangela ehamba phambili yokufa kubantu abane-Marfan syndrome, ngakho-ke kubalulekile ukuhlala uxhumana nodokotela wakho wenhliziyo njalo ukuze aqaphe isimo sakho.

Kufanele ngimbone nini udokotela? Ziyini izimo eziphuthumayo?

Uma uhlangabezana nanoma yiziphi zalezi zimpawu, kungaba uphawu lokuqhuma kwe-aortic aneurysm. Kufanele ufune usizo lwezokwelapha oluphuthumayo ngokushesha:

• Ukulahlekelwa ingqondo ngokuzumayo.
• Isicanucanu nokuhlanza.
• Ukuba ndikindiki noma kuphi emzimbeni.
• Ukukhubazeka (ukungakwazi ukunyakaza ezinye izingxenye zomzimba).
• Ubunzima obukhulu bokuphefumula.
• Ubuhlungu obungazelelwe, obungabekezeleleki - esifubeni, emhlane ongaphezulu, noma esiswini.
• Ukujuluka ngokweqile.
• Isikhumba siba mhlophe noma sibanda ngendlela engavamile.
• Ukushaya kwenhliziyo okubuthakathaka kakhulu.

Uma ubona uphawu olufana nalolu, ungalibali ngisho nomzuzu.

Okokugcina, okufanele ukukhumbule (Umyalezo Wokuya Ekhaya)

I-Marfan syndrome yisimo sokuzalwa esithinta izicubu zomzimba ezixhumeneyo. Singayithinta kakhulu inhliziyo. Kodwa ungakhathazeki. Uma lesi simo sitholakala kusenesikhathi futhi siphathwa kahle, ungaphila impilo evamile.

Into ebaluleke kakhulu yile:

• Bona udokotela wakho njalo.Ngemuva kwalokho angaqapha isimo sakho futhi anikeze ukuhlolwa okudingekayo kanye nokwelashwa ngesikhathi esifanele.
• Yenza izinguquko ezidingekayo endleleni yokuphila. Gwema izinto ezikukhathazayo.
• Thatha umuthi njengoba nje uyalelwe.
• Uma udokotela encoma ukuhlinzwa, xoxa ngakho ngokucophelela bese ukhetha okulungele wena.

Uma wazi okwengeziwe ngesimo sakho, kulapho kuzoba lula khona ukuthi usilawule. Ngakho-ke, sithemba ukuthi lolu lwazi luzoba wusizo kuwe. Uma unemibuzo, ungangabazi ukubuza udokotela wakho.

I- Marfan Syndrome, isifo senhliziyo, uhlelo lwenhliziyo, ama-valve enhliziyo, izicubu ezixhumeneyo, izifo zofuzo, ukuhlinzwa kwenhliziyo