Marfan Syndrome thiab Koj Lub Plawv: Cia Peb Tham Txog Qhov No!

Tej zaum koj twb tau pom cov neeg uas siab dua, muaj ceg ntev dua, thiab nyias dua li lwm tus. Txhua tus neeg muaj tus mob no, tab sis nws tsis yog kab mob. Tab sis qee zaum, hom lub cev no tuaj yeem yog ib qho tsos mob ntawm tus mob caj ces hu ua Marfan Syndrome . Qhov no yog ib qho mob uas tshwm sim thaum cov ntaub so ntswg sib txuas hauv peb lub cev tsis loj hlob zoo. Hais yooj yim, cov ntaub so ntswg sib txuas no yog qhov uas tuav cov feem sib txawv ntawm peb lub cev ua ke thiab muab zog rau lawv. Thaum cov no tsis muaj zog, ntau qhov ntawm lub cev tuaj yeem raug cuam tshuam, tshwj xeeb tshaj yog lub plawv, qhov muag, cov hlab ntsha, thiab cov pob txha . Hnub no, peb yuav tham txog seb tus mob no hu ua Marfan Syndrome cuam tshuam rau lub plawv li cas.

Marfan Syndrome cuam tshuam li cas rau lub plawv?

Thaum koj muaj Marfan syndrome, ob feem tseem ceeb ntawm lub plawv raug cuam tshuam ntau tshaj plaws.

1. Aorta: Qhov no yog lub hlab ntsha loj tshaj plaws uas nqa cov ntshav uas muaj oxygen ntau los ntawm peb lub plawv mus rau tag nrho lub cev. Nws zoo li lub yeeb nkab loj uas nqa dej los ntawm lub taub dej mus rau koj lub tsev.

2. Cov qhov ntshav hauv lub plawv: Cov no yog cov khoom uas ua haujlwm zoo li lub qhov rooj ntawm cov chav hauv lub plawv thiab hauv cov hlab ntsha loj uas nqa ntshav tawm ntawm lub plawv. Lawv ua kom cov ntshav ntws mus rau ib qho kev taw qhia xwb.

Tam sim no cia peb saib txhua yam no sib cais kom pom tias muaj dab tsi tshwm sim.

Yuav ua li cas rau lub aorta?

Marfan syndrome ua rau cov nqaij sib txuas hauv phab ntsa ntawm lub plawv tsis muaj zog. Ib yam li lub raj roj hmab qub, nws poob nws lub zog thiab elasticity. Qhov no tuaj yeem ua rau muaj ob qho teeb meem tseem ceeb:

• Aortic dilation: Lub aorta maj mam pib dav thiab loj dua.
• Aortic aneurysm: Hauv qee qhov chaw, phab ntsa ntawm lub plawv yuav tsis muaj zog thiab tuaj yeem nthuav tawm zoo li lub npas.

Xav txog seb, qhov aneurysm no zoo li lub log tsheb kauj vab uas tawm los ntawm ib qho chaw thaum lub raj tsis muaj zog.

Tshwj xeeb, qhov ntawm lub plawv uas nyob ze tshaj plaws rau lub plawv, hu ua 'aortic root', yog qhov uas feem ntau loj dua lossis dav dua li no rau cov neeg uas muaj Marfan syndrome. Qhov no yog ib qho mob txaus ntshai heev, vim tias yog tias 'aneurysm' no loj thiab tawg ('aortic dissection' lossis rupture), nws tuaj yeem ua rau tuag taus.

Lwm yam mob xws li Ehlers-Danlos syndrome, Loeys-Dietz syndrome, bicuspid aortic valve, thiab Turner syndrome kuj tuaj yeem ua rau lub plawv nthuav dav, tab sis lawv tuaj yeem tshwm sim rau lwm qhov ntawm lub plawv.

Ua li cas rau cov hlab ntsha hauv lub plawv?

Kab mob Marfan kuj tseem ua rau muaj teeb meem nrog cov hlab ntsha hauv lub plawv. Yog tias cov hlab ntsha no tsis ua haujlwm zoo, lub plawv yuav tsum ua haujlwm hnyav dua los tso ntshav. Raws sijhawm mus, qhov no tuaj yeem ua rau lub plawv tsis ua haujlwm.Nws mus tau ob txoj kev. Muaj ob hom kab mob tseem ceeb uas pom tau tias muaj feem cuam tshuam nrog Marfan syndrome:

• Kev rov qab qhib lub plawv aortic valve: Thaum lub valve ntawm aorta thiab lub plawv sab laug qis (lub plawv sab laug) tsis kaw zoo, qee cov ntshav uas tau nqus tawm yuav ntws rov qab rau hauv plawv. Nws zoo li lub qhov dej xau.
• Qhov mitral valve prolapse: Qhov mitral valve, nyob nruab nrab ntawm lub plawv sab saud (lub plawv sab laug) thiab lub plawv sab qis (lub plawv sab laug) ntawm sab laug ntawm lub plawv, tsis kaw zoo, thiab lub plawv sab saud tawm mus sab nraud. Qhov no qee zaum ua rau ntshav ntws rov qab (mitral regurgitation).

Kab mob plawv feem ntau tshwm sim rau cov neeg uas muaj tus mob Marfan syndrome li cas?

Qhov tseeb, cov neeg uas muaj tus kab mob Marfan syndrome muaj feem yuav muaj teeb meem plawv ntau dua . Kev tshawb fawb tau qhia tias cuaj ntawm kaum tus neeg uas muaj tus kab mob Marfan syndrome yuav muaj teeb meem nrog lawv lub plawv valve lossis aorta. Yog vim li ntawd nws thiaj li tseem ceeb uas yuav tsum paub txog qhov no.

Cov tsos mob ntawm Marfan syndrome uas cuam tshuam rau lub plawv yog dab tsi?

Yog tias koj muaj Marfan syndrome, xws li aortic aneurysm lossis kab mob valve, koj yuav muaj qee cov tsos mob. Txawm li cas los xij, qee tus neeg yuav muaj cov mob no yam tsis muaj tsos mob. Yog vim li cas kev kuaj mob thiaj li tseem ceeb.

Cov no yog cov tsos mob uas pom tau feem ntau:

• Mob hauv siab los yog mob nraub qaum sab saud
• Hnoos ntshav (qhov no yog ib qho cim qhia txog kev phom sij me ntsis)
• Nyuaj nqos zaub mov (yog tias lub plawv loj nias rau ntawm txoj hlab pas )
• Hnov ​​​​mob taub hau los yog lightheaded
• Xav tias nkees heev los yog tsis muaj zog
• Kev xav tias lub plawv dhia tsis zoo (lub plawv dhia tsis xwm yeem)
• Lub suab nrov qis (yog tias lub plawv nias rau ntawm cov hlab ntsha uas txuas nrog cov hlab ntsha suab)
• Ua pa nyuaj , tshwj xeeb tshaj yog thaum koj nkees lossis pw
• O , tshwj xeeb tshaj yog nyob rau hauv ob txhais ceg thiab pob luj taws
• Ua pa nrov nrov (whistling) thaum ua pa

Xav txog koj muaj ib tug phooj ywg uas siab thiab nyias heev. Nws hais tias nws feem ntau mob hauv siab thiab nyuaj rau taug kev. Yog tias muaj, nws yuav yog ib lub tswv yim zoo kom mus ntsib kws kho mob kom paub seb nws puas muaj feem cuam tshuam nrog Marfan syndrome.

Ua cas ho ua rau muaj tus mob Marfan syndrome no cuam tshuam rau lub plawv?

Raws li peb tau hais ua ntej, Marfan syndrome yog ib qho teeb meem ntawm cov ntaub so ntswg sib txuas.Nws tsis tau tsim kom zoo. Cov nqaij sib txuas zoo nyob txhua qhov chaw hauv peb lub cev. Nws muab lub zog, cov duab, thiab kev ywj pheej rau cov kabmob thiab cov hlab ntsha. Cov nqaij sib txuas no pom muaj nyob rau hauv phab ntsa ntawm lub plawv thiab cov hlab ntsha, tshwj xeeb tshaj yog aorta, thiab hauv cov hlab ntsha plawv.

Thaum cov nqaij sib txuas no qaug zog vim yog Marfan syndrome, lawv poob lawv lub zog thiab elasticity. Qhov no ua rau lub plawv nthuav dav thiab o, tsis tuaj yeem tiv taus qhov siab ntawm cov ntshav. Qhov no kuj ua rau cov qhov dej ntawm lub plawv ua mob thiab tsis tuaj yeem qhib lossis kaw kom raug.

Cov kev kuaj plawv twg pab kuaj mob Marfan syndrome?

Qee zaum mob Marfan syndrome nyuaj rau kuaj mob vim tias cov tsos mob txawv ntawm ib tus neeg mus rau ib tus neeg thiab tuaj yeem zoo ib yam li lwm yam mob. Cov neeg feem coob tsuas pom tias lawv muaj tus mob no thaum lawv tseem hluas lossis laus dua.

Yog tias tus kws kho mob xav tias koj muaj Marfan syndrome, lawv yuav ua tej yam xws li:

• Yuav kuaj xyuas koj lub cev tag nrho . Koj qhov siab, qhov hnyav, koj txhais caj npab thiab ceg ntev npaum li cas, koj lub hauv siab zoo li cas, koj ob lub qhov muag, thiab koj daim tawv nqaij yuav raug kuaj xyuas.
• Lawv yuav nug txog koj tsev neeg keeb kwm kev mob nkeeg . Lawv yuav xyuas seb puas muaj leej twg hauv koj tsev neeg tau muaj tus mob Marfan syndrome lossis cov tsos mob zoo sib xws.
• Muaj ntau qhov kev tshuaj xyuas tshwj xeeb ('kev kuaj duab') uas kuaj lub plawv . Cov tseem ceeb yog:
• Echocardiogram (Echo): Qhov no tso cai rau koj pom meej meej qhov loj me, cov duab, thiab kev ua haujlwm ntawm lub plawv thiab cov hlab ntsha, zoo ib yam li kev ntsuas ultrasound ntawm lub plawv.
• Electrocardiogram (EKG): Qhov no ntsuas qhov ua haujlwm hluav taws xob ntawm lub plawv, uas yog, lub suab ntawm lub plawv dhia.
• Kev kuaj mob caj ces: Qhov kev kuaj no tuaj yeem ua tiav los xyuas kom meej tias koj puas muaj kev hloov pauv caj ces (hauv FBN1 gene) uas ua rau muaj Marfan syndrome.

Cov kev kho mob dab tsi los tswj cov teeb meem plawv uas tshwm sim los ntawm Marfan syndrome?

Txawm hais tias tus mob Marfan syndrome no kho tsis tau tag nrho los xij, muaj cov kev kho mob los tswj cov teebmeem ntawm lub plawv thiab tiv thaiv cov teeb meem loj. Cov no muaj ob hom: kev kho mob tsis phais thiab kev kho mob phais.

Tus kws kho mob yuav pom zoo kom phais mob rau cov xwm txheej hauv qab no:

• Yog tias lub plawv txoj kab uas hla (aorta) yog 5 centimeters (kwv yees li 1.97 nti) lossis loj dua.
• Yog tias lub plawv dhia nce ntxiv 0.5 centimeters (kwv yees li 0.197 nti) lossis ntau dua hauv ib xyoos (qhov no hu ua 'kev nthuav dav sai').
• Yog tias koj cov txheeb ze ntshav (cov tswv cuab ntawm tsev neeg) tau ua hom kev phais no (tej zaum vim muaj kev cuam tshuam los ntawm noob caj noob ces, kev phais yuav tsum tau ua txawm tias ntawm qhov me dua).

Cov neeg uas muaj lub cev me dua feem ntau muaj cov hlab ntsha me dua, yog li ntawd lawv yuav tsum tau phais txawm tias lub cev me dua los xij.

Kev kho mob uas tsis yog phais

Nov yog thawj kauj ruam hauv kev tswj hwm Marfan syndrome.

• Kev hloov pauv txoj kev ua neej: Koj yuav tsum zam cov haujlwm uas ua rau lub plawv ntxhov siab ntxiv.
• Nws tsis zoo rau ua tej yam xws li tsa cov hnyav lossis thawb.
• Cov kev ua si uas muaj kev cuam tshuam loj xws li football, rugby, thiab boxing tsis haum.
• Tham nrog koj tus kws kho mob kom paub seb cov kev tawm dag zog twg muaj kev nyab xeeb thiab haum rau koj.
• Cov Tshuaj:
• Cov kws kho mob sau ntawv kom siv cov tshuaj hu ua 'Angiotensin II receptor blockers (ARBs)' lossis 'beta-blockers' . Cov no pab qeeb qhov ceev ntawm cov hlab ntsha hauv lub plawv thiab tswj cov ntshav siab.
• Kev kuaj mob plawv tsis tu ncua :
• Tus kws kho mob yuav tsum tau kuaj xyuas qhov loj ntawm lub plawv thiab qhov mob ntawm cov valve. Qhov no tuaj yeem ua tiav los ntawm kev siv cov scans xws li 'transthoracic echocardiography (Echo)', 'computerized tomographic angiography (CTA)', lossis 'magnetic resonance angiography (MRA)'.
• Cov kev kuaj no tuaj yeem ntes tau cov kev hloov pauv hauv lub plawv ua ntej nws tawg (kev txiav).

Kev phais mob

Qee zaum, cov teeb meem ntawm lub plawv thiab cov hlab ntsha tsis tuaj yeem tswj tau los ntawm kev siv tshuaj thiab kev hloov pauv hauv lub neej xwb. Yog thaum ntawd yuav tsum tau phais.

Lub hom phiaj tseem ceeb ntawm kev phais plawv rau Marfan syndrome yog kho ib feem ntawm lub plawv valve uas tsis muaj zog, tshem nws tawm thiab muab ib lub plawv valve cuav tso rau hauv, lossis kho lub plawv valve uas puas lawm lossis hloov nws nrog ib lub tshiab.

Lub hom phiaj tseem ceeb ntawm cov kev phais no yog los tiv thaiv cov xwm txheej ceev uas hem txoj sia xws li kev txiav cov hlab ntsha hauv lub plawv (aorta dissection).

Feem ntau, cov kev phais no yog npaj thiab ua rau hnub uas tau teem tseg ua ntej lawm (kev phais xaiv tau). Txawm li cas los xij, yog tias cov hlab ntsha hauv lub plawv tawg lossis tawg tam sim ntawd, nws yuav tsum tau ua raws li kev phais mob thaum muaj xwm txheej ceev.

Muaj ntau hom kev phais plawv tseem ceeb uas ua rau Marfan syndrome:

• Kev hloov cov hauv paus plawv (Aortic root replacement): Qhov no yog kev phais mob uas feem ntau ua vim tias qhov no ntawm lub plawv feem ntau o lossis dav dua.
• Kho lossis hloov lub plawv valve.
• Kho qhov o ntawm cov hlab ntsha ze ntawm lub plawv ('Kev kho aneurysm aortic nce mus txog').
• Kev kho lossis hloov pauv ntawm lub mitral valve.

Tus kws phais plawv yuav nrog koj tham thiab txiav txim siab seb qhov kev phais twg zoo tshaj plaws rau koj.

Yog koj muaj tus mob Marfan syndrome no, puas yuav tiv thaiv tau kab mob plawv?

Tu siab kawg li, Marfan syndrome no tsis tuaj yeem tiv thaiv tau, vim nws yog los ntawm noob caj noob ces. Ib yam li ntawd, kab mob plawv uas nws ua rau tsis tuaj yeem tiv thaiv tau tag nrho.

Txawm li cas los xij, muaj tej yam uas koj ua tau los txo koj txoj kev pheej hmoo ntawm cov teeb meem loj, xws li aortic dissection:

• Tsis txhob ua tej yam uas ua rau lub plawv ntxhov siab (zoo li peb tau tham ua ntej lawm, tsis txhob nqa khoom hnyav thiab ua si nawv uas ntxhov siab heev).
• Tau txais kev kuaj mob plawv raws sijhawm raws li tus kws kho mob tau hais.
• Noj tag nrho cov tshuaj uas tau sau tseg kom raug thiab raws sijhawm.

Muaj ib qho uas yuav tsum nco ntsoov tshwj xeeb yog tias yog koj yog ib tug poj niam uas muaj tus kab mob Marfan thiab xav txog kev xeeb tub, koj yuav tsum nrog koj tus kws kho mob tham txog qhov no. Cov kev tshawb fawb tau qhia tias txog li 40% ntawm cov poj niam uas muaj tus kab mob Marfan yuav muaj teeb meem thaum cev xeeb tub. Yog li ntawd, nws yog ib qho tseem ceeb heev uas yuav tsum nrhiav kev pab tswv yim los ntawm kws kho mob ua ntej cev xeeb tub.

Yav tom ntej tus neeg uas muaj kab mob plawv vim yog Marfan syndrome yuav zoo li cas?

Yog tias koj tswj tau zoo, uas yog ua raws li cov lus qhia ntawm kws kho mob thiab tau txais kev kho mob tsim nyog, tus neeg uas muaj tus kab mob Marfan syndrome no yuav muaj sia nyob tau li qub, tej zaum yuav txog li 70 lossis 80 xyoo.

Txawm li cas los xij, cov teeb meem plawv yog qhov ua rau neeg tuag ntau tshaj plaws ntawm cov neeg uas muaj Marfan syndrome, yog li nws yog ib qho tseem ceeb uas yuav tsum sib cuag nrog koj tus kws kho mob plawv tas li kom saib xyuas koj tus mob.

Thaum twg kuv yuav tsum mus ntsib kws kho mob? Tej yam xwm txheej ceev yog dab tsi?

Yog tias koj muaj cov tsos mob no, nws yuav yog ib qho cim qhia tias lub plawv aortic aneurysm tawg. Koj yuav tsum nrhiav kev kho mob thaum muaj xwm txheej ceev tam sim ntawd:

• Tsis nco qab kiag li.
• Xeev siab thiab ntuav.
• Kev tsis muaj zog nyob rau txhua qhov chaw ntawm lub cev.
• Tuag tes tuag taw (tsis muaj peev xwm txav qee qhov ntawm lub cev).
• Ua pa nyuaj heev.
• Mob tam sim ntawd, mob tsis taus - hauv siab, nraub qaum sab saud, lossis plab.
• Tawm hws ntau dhau.
• Tawv nqaij daj ntseg lossis txias txawv txawv.
• Lub plawv dhia tsis muaj zog heev.

Yog koj pom ib lub cim zoo li no, tsis txhob ncua ib pliag li.

Thaum kawg, yuav tsum nco ntsoov li cas (Cov Lus Coj Mus Tsev)

Marfan syndrome yog ib yam mob uas muaj txij thaum yug los uas cuam tshuam rau cov nqaij sib txuas ntawm lub cev. Nws tuaj yeem cuam tshuam rau lub plawv. Tab sis tsis txhob txhawj. Yog tias tus mob no raug kuaj pom thaum ntxov thiab tswj hwm kom zoo, koj tuaj yeem ua neej nyob zoo li qub.

Qhov tseem ceeb tshaj plaws yog:

• Mus ntsib koj tus kws kho mob tsis tu ncua.Ces nws tuaj yeem saib xyuas koj tus mob thiab muab cov kev kuaj mob thiab kev kho mob tsim nyog raws sijhawm.
• Hloov koj txoj kev ua neej kom haum koj siab. Tsis txhob ua tej yam uas ua rau koj ntxhov siab.
• Noj tshuaj raws nraim li tau qhia.
• Yog tus kws kho mob pom zoo kom phais, tham kom zoo zoo thiab xaiv qhov zoo tshaj plaws rau koj.

Koj paub ntau ntxiv txog koj tus mob, nws yuav yooj yim dua rau koj los tswj nws. Yog li ntawd, peb vam tias cov ntaub ntawv no yuav pab tau koj. Yog tias koj muaj lus nug, tsis txhob yig nug koj tus kws kho mob.

Marfan Syndrome, kab mob plawv, lub plawv dhia, cov qhov (valves) ntawm lub plawv, cov nqaij sib txuas, cov kab mob caj ces, kev phais plawv