Marfan Syndrome neMwoyo Wako: Ngatikurukurei Izvi!

Pamwe wakamboona vanhu vakareba zvikuru, vane makumbo marefu, uye vakatetepa kupfuura vamwe. Munhu wese ane chirwere ichi, asi hachisi chirwere. Asi dzimwe nguva, rudzi urwu rwemuviri runogona kuva chiratidzo chechirwere chemajini chinonzi Marfan Syndrome . Ichi chirwere chinoitika kana tambo dzemuviri wedu dzisingakure zvakanaka. Zvichitaurwa zviri nyore, tambo dzemuviri idzi ndidzo dzinobata nhengo dzakasiyana dzemuviri wedu pamwe chete uye dzinodzipa simba. Kana idzi dzisisina simba, nhengo dzakawanda dzemuviri dzinogona kukanganiswa, kunyanya moyo, maziso, mitsipa yeropa, uye mapfupa . Nhasi, tichataura nezvekuti chirwere ichi chinonzi Marfan Syndrome chinokanganisa sei moyo.

Marfan Syndrome inokanganisa sei mwoyo?

Kana uine Marfan syndrome, zvikamu zviviri zvikuru zvemoyo ndizvo zvinonyanya kukanganiswa.

1. Aorta: Iyi ndiyo tsinga huru, yakakura kwazvo inotakura ropa rakawanda neokisijeni kubva pamwoyo wedu kuenda kumuviri wese. Yakafanana nepombi huru inotakura mvura kubva mutangi remvura kuenda kumba kwako.

2. Mavharuvhu emwoyo: Aya ndiwo magonhi anoshanda semasuo ari pakati pemakamuri ari mukati memoyo nemutsinga huru dzinotakura ropa kubva mumoyo. Anoita kuti ropa rifambe nenzira imwe chete.

Ngatitarisei chimwe nechimwe cheizvi zvakasiyana kuti tione zvinoitika.

Chii chinoitika ku aorta?

Marfan syndrome inoita kuti nhengo dzemuviri dziri mumadziro emwoyo dzishaye simba. Kufanana nechubhu yekare yerabha, inorasikirwa nesimba rayo uye kutwasuka kwayo. Izvi zvinogona kukonzera matambudziko maviri makuru:

• Kukura kwetsinga dzeropa: Mtsinga dzeropa dzinotanga kukura zvishoma nezvishoma.
• Aortic aneurysm: Mune dzimwe nzvimbo, madziro emwoyo anoshaya simba uye anogona kuputira kunze sebharumu.

Fungidzira, aneurysm iyi yakafanana netayi rebhasikoro rinobuda panzvimbo imwe chete kana chubhu yacho yapera simba.

Zvikuru sei, chikamu chemwoyo chiri pedyo nemoyo, chinonzi `mudzi weaortic`, ndicho chikamu chinowanzo wedzera kukura kana kukura nenzira iyi muvanhu vane Marfan syndrome. Ichi chirwere chine njodzi, nekuti kana `aneurysm` iyi ikakura uye ikaputika (`aortic dissection` kana kuputika), inogona kuisa hupenyu panjodzi.

Zvimwe zvirwere zvakaita seEhlers-Danlos syndrome, Loeys-Dietz syndrome, bicuspid aortic valve, uye Turner syndrome zvinogonawo kukonzera kukura kwemoyo, asi zvinogona kuitika mune dzimwe nzvimbo dzemoyo.

Chii chinoitika kumavharuvhu emwoyo?

Marfan syndrome inogonawo kukonzera matambudziko emavharuvhu emwoyo. Kana mavharuvhu aya asingashande zvakanaka, moyo unofanirwa kushanda nesimba kuti upombe ropa. Nekufamba kwenguva, izvi zvinogona kukonzera kutadza kushanda kwemoyo.Zvinogona kuenda chero kupi zvako. Zvirwere zviviri zvikuru zvevharuvhu zvinoonekwa pamwe chete neMarfan syndrome:

• Kudzoreredza vharuvhu yeAortic: Kana vharuvhu iri pakati peaorta nekamuri rezasi rekuruboshwe remoyo (ventricle yekuruboshwe) ikasavhara zvakanaka, rimwe ropa rinopombwa rinodzokera mumoyo. Rinoita sepombi inodonhedza mvura.
• Kudonha kwevharuvhu yeMitral: Vharuvhu yemitral, iri pakati pekamuri repamusoro (atrium yekuruboshwe) nekamuri repasi (ventricle yekuruboshwe) kuruboshwe rwemoyo, haivhari zvakanaka, uye kamuri repamusoro rinobuda kunze. Izvi zvinogona dzimwe nguva kukonzera kuti ropa ribude kumashure (mitral regurgitation).

Chirwere chemwoyo chakajairika sei kuvanhu vane Marfan syndrome?

Kutaura zvazviri, vanhu vane Marfan syndrome vari panjodzi huru yekuve nematambudziko emwoyo. Tsvagiridzo yakaratidza kuti vanhu vapfumbamwe pavanhu gumi vane Marfan syndrome vachave nedambudziko remwoyo wavo kana kuti aorta. Ndosaka zvichikosha kuziva izvi.

Ndezvipi zviratidzo zveMarfan syndrome zvinokanganisa mwoyo?

Kana uine Marfan syndrome, yakadai se aortic aneurysm kana chirwere chevharuvhu, unogona kuona zvimwe zviratidzo. Zvisinei, vamwe vanhu vanogona kunge vaine zvirwere izvi vasina zviratidzo. Ndosaka kuongororwa kwechiremba kuchikosha.

Izvi ndizvo zviratidzo zvinogona kuonekwa kazhinji:

• Kurwadziwa kwechipfuva kana kurwadziwa kwemusana wekumusoro
• Kukosora ropa (ichi chiratidzo chine njodzi zvishoma)
• Kuoma kumedza chikafu (kana moyo wakakura uchidzvanya pagurokuro )
• Kunzwa dzungu kana kuti musoro wakaneta
• Kunzwa kuneta zvakanyanya kana kushaya simba
• Kunzwa kurova kwemoyo kusingawanzoitiki (kurova kwemoyo)
• Kunzwa kurira kwenzwi (kana moyo uchimanikidza tsinga yakabatana netambo dzenzwi)
• Kuoma kufema , kunyanya kana wakaneta kana kurara pasi
• Kuzvimba , kunyanya mumakumbo nemuzvitsitsinho
• Kufema (kuridza muridzo) kana uchifema

Fungidzira uine shamwari yakareba uye yakaonda. Inowanzo nyunyuta nezvekurwadziwa pachipfuva uye inonetseka nekufamba . Kana zvakadaro, zvingava zvakanaka kuona chiremba kuti uone kana zvine chekuita neMarfan syndrome.

Chii chinoita kuti Marfan syndrome ibate mwoyo?

Sezvatakambotaura, Marfan syndrome chirwere chinokonzerwa nekubatana kwenhengo dzemuviri.Hazvina kuumbwa zvakanaka. Nyama ine hutano inowanikwa kwese kwese mumuviri wedu. Inopa simba, chimiro, uye kuchinjika kunhengo dzemuviri netsinga dzeropa. Nyama iyi inowanikwa mumadziro emwoyo netsinga dzeropa, kunyanya aorta, uye mumavharuvhu emwoyo.

Kana nhengo idzi dzinobatana dzisisina simba nekuda kweMarfan syndrome, dzinorasikirwa nesimba radzo uye kusimba kwadzo. Izvi zvinoita kuti mwoyo uwedzere kuzvimba, usingakwanise kumira kumanikidzwa kweropa. Izvi zvinoitawo kuti mavharuvhu emwoyo ave nechirwere uye asakwanise kuvhura kana kuvhara zvakanaka.

Ndezvipi zviedzo zvemoyo zvinobatsira pakuongorora Marfan syndrome?

Dzimwe nguva chirwere cheMarfan chinogona kuoma kuona, sezvo zviratidzo zvichisiyana kubva pamunhu kuenda kune mumwe uye zvinogona kutevedzera zvezvimwe zvirwere. Vanhu vazhinji vanongoona kuti vane chirwere ichi pavanenge vachiri vadiki kana vakuru.

Kana chiremba akafungidzira kuti une Marfan syndrome, achaita zvinhu zvakaita seizvi:

• Kuongororwa kwemuviri wako kwakazara kuchaitwa. Kureba kwako, huremu, kureba kweruoko negumbo, chimiro chechipfuva, maziso, uye ganda rako zvichaongororwa.
• Vachakubvunza nezvenhoroondo yezveutano hwemhuri yako . Vachatarisa kana paine munhu mumhuri yako akambova neMarfan syndrome kana zviratidzo zvakafanana.
• Kuongororwa kwakakosha kwakasiyana-siyana ('miedzo yekufungidzira') kunoongorora moyo kwakaitwa. Mimwe mikuru ndeiyi:
• Echocardiogram (Echo): Izvi zvinokubatsira kuona zvakajeka saizi, chimiro, uye mashandiro emwoyo netsinga dzeropa, sezvinoita ultrasound scan yemoyo.
• Electrocardiogram (EKG): Iyi inoyera mashandiro emagetsi emoyo, kureva kuti, mutinhimira wekurova kwemoyo.
• Kuongororwa kwemajini: Kuongororwa uku kunogona kuitwa kuti usimbise kana uine kushanduka kwemajini (mujini reFBN1) kunokonzera Marfan syndrome.

Ndeipi mishonga yekurapa matambudziko emwoyo anokonzerwa neMarfan syndrome?

Kunyange hazvo Marfan syndrome isingarapike zvachose, kune nzira dzekurapa dzinodzora migumisiro pamwoyo nekudzivirira matambudziko akakomba. Idzi dzinouya mumhando mbiri: nzira dzekurapa dzisingashandiswi uye nzira dzekurapa dzinoshandiswa pakuvhiya.

Chiremba anogona kukurudzira kuvhiyiwa mune zvinotevera:

• Kana dhayamita yetsinga yemoyo wako (aorta) iri 5 centimeters (inenge 1.97 inches) kana kupfuura.
• Kana kurova kwemoyo kukawedzera ne 0.5 centimeters (inenge 0.197 inches) kana kupfuura mukati megore (izvi zvinonzi `rapid expansion`).
• Kana hama dzako dzeropa (nhengo dzemhuri yako) dzakambovhiyiwa nenzira iyi (zvichida nekuda kwezvikonzero zvemajini, kuvhiyiwa kungafanira kuitwa kunyangwe padhayamita diki).

Vanhu vane miviri midiki vanowanzova netsinga dzeropa diki, saka vangangoda kuvhiyiwa kunyangwe dhayamita yacho iri diki.

Kurapa kusiri kwekuvhiya

Aya ndiwo matanho ekutanga ekurapa Marfan syndrome.

• Kuchinja mararamiro: Unofanira kudzivisa mabasa anoremedza mwoyo zvakanyanya.
• Hazvina kunaka kuita zvinhu zvakaita sekusimudza zvinorema kana kusunda.
• Mitambo ine simba guru yakaita senhabvu, rugby, uye boxing haina kukodzera.
• Taura nachiremba wako kuti uwane kuti ndeapi maekisesaizi akachengeteka uye akakodzera iwe.
• Mishonga:
• Vanachiremba vanonyora mishonga inonzi `Angiotensin II receptor blockers (ARBs)` kana `beta-blockers` . Iyi mishonga inobatsira kuderedza kumhanya kwetsinga dzemwoyo uye kudzora BP.
• Kuongororwa kwemifananidzo yemwoyo nguva dzose :
• Saizi yemoyo uye mamiriro emavharuvhu zvinofanirwa kuongororwa nguva nenguva nachiremba. Izvi zvinogona kuitwa uchishandisa maskani akadai se `transthoracic echocardiography (Echo)`, `computerized tomographic angiography (CTA)`, kana `magnetic resonance angiography (MRA)`.
• Miedzo iyi inogona kuona shanduko dziri mumoyo usati watsemuka (kupatsanurwa).

Kurapa kwekuvhiya

Dzimwe nguva, matambudziko emwoyo haagone kugadziriswa nemishonga uye kuchinja mararamiro chete. Ndipo panodiwa kuvhiyiwa.

Chinangwa chikuru chekuvhiyiwa kwemoyo weMarfan syndrome ndechekugadzirisa chikamu chevharuvhu yemwoyo chisina kusimba, kuibvisa uye kuisa vharuvhu yekugadzira, kana kugadzirisa vharuvhu yemwoyo yakakuvara kana kuitsiva neimwe itsva.

Chinangwa chikuru chekuvhiyiwa uku ndechekudzivirira njodzi dzinogona kutyisidzira hupenyu dzakadai sekutsemuka kwetsinga yeropa.

Kazhinji, kuvhiyiwa uku kunorongwa uye kunoitwa pazuva rakatarwa (elective surgery). Zvisinei, kana tsinga yemoyo ikaputika kamwe kamwe, inofanira kuitwa senzira yekukurumidzira.

Kune mhando dzakasiyana-siyana dzekuvhiyiwa kwemoyo dzinoitirwa Marfan syndrome:

• Kutsiva midzi yemoyo: Uku ndiko kuvhiyiwa kunowanzoitwa nekuti chikamu ichi chemoyo chinowanzozvimba kana kukura.
• Kugadziriswa kana kutsiviwa kwevharuvhu yeAortic.
• Kugadziriswa kwebundu mutsinga iri pedyo nemoyo (`Kugadziriswa kweaneurysm yeaorta inokwira`).
• Kugadziriswa kana kutsiva vharuvhu yeMitral.

Chiremba wemwoyo achakurukura newe osarudza kuti ndeipi nzira yakakunakira.

Chirwere chemwoyo chinogona kudzivirirwa here kana uine Marfan syndrome?

Zvinosuwisa kuti Marfan syndrome haigone kudzivirirwa, sezvo iri majini. Saizvozvowo, chirwere chemwoyo chainokonzera hachigone kudzivirirwa zvizere.

Zvisinei, pane zvinhu zvaunogona kuita kuti uderedze njodzi yematambudziko akakomba, akadai sekupatsanurwa kwetsinga yeropa:

• Kudzivisa mabasa anoisa kushushikana pamoyo (sezvatakambokurukura, kudzivisa mitambo inorema uye mitambo inoshungurudza zvakanyanya).
• Kuongororwa kwemifananidzo yemwoyo panguva yakatarwa nachiremba.
• Kutora mishonga yese yakanyorerwa nemazvo uye nenguva.

Chimwe chinhu chekurangarira zvikuru ndechekuti kana uri mukadzi ane Marfan syndrome uye uri kufunga nezvekuva nepamuviri, unofanira kutaura nachiremba wako nezvazvo. Ongororo dzakaratidza kuti vakadzi vanosvika 40% vane Marfan syndrome vanogona kusangana nematambudziko panguva yekuzvitakura. Saka, zvakakosha kutsvaga mazano echiremba usati wava nepamuviri.

Ndeipi tarisiro yeramangwana yemunhu ane chirwere chemwoyo nekuda kweMarfan syndrome?

Kana munhu ane Marfan syndrome akatarisirwa zvakanaka, kureva kuti, achitevera mazano echiremba uye achiwana kurapwa kwakakodzera, anogona kurarama hupenyu hwakafanana, zvichida makore 70 kana 80.

Zvisinei, matambudziko emwoyo ndiwo anonyanya kukonzera rufu pakati pevanhu vane Marfan syndrome, saka zvakakosha kugara uchitaura nachiremba wemwoyo kuti akuongorore.

Ndinofanira kuona chiremba riini? Chii chinonzi emergency?

Kana ukaona chimwe chezviratidzo izvi, chingave chiratidzo chekuputika kweaneurysm yeropa. Unofanira kutsvaga rubatsiro rwechiremba nekukasika:

• Kurasikirwa nepfungwa kamwe kamwe.
• Kusvotwa nekurutsa.
• Kushaya simba kwese kwese mumuviri.
• Kuoma mitezo (kusakwanisa kufambisa dzimwe nhengo dzemuviri).
• Kunetseka zvakanyanya kufema.
• Kurwadziwa kusingatarisirwi kamwe kamwe - muchipfuva, kumusana wepamusoro, kana mudumbu.
• Kudikitira zvakanyanya.
• Ganda rinova rakachenuruka kana kutonhora zvisingawanzoitiki.
• Kurova kwemwoyo kusina simba.

Kana ukaona chiratidzo chakadai, usanonoka kana miniti imwe chete.

Pakupedzisira, zvekurangarira (Mharidzo Yekuenda Kumba)

Marfan syndrome chirwere chekuzvarwa nacho chinokanganisa nhengo dzemuviri. Chinogona kukanganisa mwoyo zvakanyanya. Asi musazvidya mwoyo. Kana chirwere ichi chikaonekwa nekukurumidza uye chikarapwa nemazvo, munogona kurarama hupenyu hwakajairika.

Chinhu chinonyanya kukosha ndechekuti:

• Ona chiremba wako nguva nenguva.Ipapo anogona kutarisa mamiriro ako ezvinhu uye kukupa bvunzo dzinodiwa uye kurapwa panguva yakakodzera.
• Shandura mararamiro ako. Rega zvinhu zvinokunetsa.
• Inwa mushonga wacho sezvawakarairwa.
• Kana chiremba achikurudzira kuvhiyiwa, taura nezvazvo nokungwarira uye sarudza zvakakunakira.

Kana uchiziva zvakawanda nezvechirwere chako, zvichava nyore kuti uchigadzirise. Saka, tinovimba kuti ruzivo urwu ruchakubatsira. Kana uine mibvunzo, usazeza kubvunza chiremba wako.

Marfan Syndrome, chirwere chemwoyo, hurongwa hwemwoyo, mavharuvhu emwoyo, nhengo dzinobatana, zvirwere zvemajini, kuvhiyiwa kwemoyo