Marfan Syndrome leh I Thinlung: Hemi chungchang hi i sawiho ang u!

Midang aiin a sang zawk, ke ruh sei zawk, leh a hring zawk i hmu tawh ngei ang. Mi zawng zawng hian hetiang dinhmun hi kan nei vek a, mahse natna a ni lo. Mahse, a châng chuan, he taksa pianzia chi hi Marfan Syndrome tih hming pu genetic condition symptom a ni thei. Hei hi kan taksaa connective tissue a lo thang tha lo a nih chuan a lo awm thin a ni. A awlsam zawngin sawi ila, he connective tissue hi kan taksa peng hrang hrangte inzawmkhawmtu leh chakna petu a ni. Chûngte chu a chak loh chuan taksa pêng tam tak a nghawng thei a, a bîk takin thinlung, mit, thisen kalna kawng, leh ruhro . Vawiin hian he Marfan Syndrome an tih hian thinlung a nghawng dan kan sawi dawn a ni.

Marfan Syndrome hian engtin nge thinlung a nghawng?

Marfan syndrome i neih hian thinlung hmunpui pahnih a nghawng nasa ber.

1. Aorta: Hei hi kan thinlung atanga kan taksa pum puia oxygen tamna thisen phurtu thisen kalna kawng lian ber, lian ber a ni. Tui dahna atanga i in thlenga tui phurtu main pipe ang mai a ni.

2. Heart valves: Hengte hi thinlung chhunga chamber inkar leh thinlung atanga thisen chhuaktu main vein-a kawngkhar ang maia che thinte an ni. Thisen chu kawng khatah chauh a kal theih nan an enkawl a ni.

Tunah chuan heng zawng zawng hi a hranin en ila, eng nge thleng tih kan hre ang.

Aorta hi engtin nge a awm?

Marfan syndrome hian thinlung bang chhunga connective tissue te chu a tichak lo hle. Rubber tube hlui ang maiin a chakna leh a elasticity a hloh thin. Hei hian harsatna lian tak pahnih a thlen thei a ni:

• Aortic dilation: Aorta hi zawi zawiin a zau chho tan a, a lian chho tan bawk.
• Aortic aneurysm: Hmun thenkhatah chuan thinlung bang chu a chak lo va, balloon ang maiin pawn lamah a bul chhuak thei a ni.

Ngaihtuah teh, he aneurysm hi bicycle tire, tube a chak loh chuan hmun khat atanga bulge chhuak ang mai a ni.

A bik takin, thinlung hnaih ber, `aortic root` tia koh hi Marfan syndrome vei zingah hetiang hian a lian emaw, a zau emaw tam ber a ni. Hei hi thil hlauhawm deuh a ni a, a chhan chu he `aneurysm` hi a lian a, a puak (`aortic dissection` emaw rupture) a nih chuan nunna atana hlauhawm tak a ni thei a ni.

Thil dang Ehlers-Danlos syndrome, Loeys-Dietz syndrome, bicuspid aortic valve, Turner syndrome te pawhin thinlung hi a tizau thei a, mahse thinlung hmun dangah pawh a awm thei bawk.

Heart valve te hi engtin nge an awm?

Marfan syndrome hian heart valve-ah harsatna a thlen thei bawk. Heng valve te hian hna a thawk tha lo a nih chuan thisen pump turin thinlung hian nasa zawkin hna a thawh a ngai a ni. Hun a kal zel a, hei hian heart failure a thlen thei a ni.Kawng lehlamah a kal thei. Valve natna lian ber pahnih chu Marfan syndrome nena inzawm hmuh a ni a, chungte chu:

• Aortic valve regurgitation: Aorta leh thinlung veilam chamber hnuai lam (left ventricle) inkar valve chu a khar tha loh chuan thisen pump chhuah thenkhat chu thinlungah a leak leh thin. Faucet leak ang mai a ni.
• Mitral valve prolapse: Mitral valve, thinlung veilam chamber chunglam (left atrium) leh lower chamber (left ventricle) inkar a awm chu a khar tha lo va, upper chamber chu pawn lam hawiin a rawn chhuak a ni. Hei hian a chang chuan thisen hnunglam (mitral regurgitation) a tichhuak thei.

Marfan syndrome vei zingah hian engtiang chiahin nge thinlung natna hi a hluar?

Dik tak chuan Marfan syndrome vei te hi thinlung lam harsatna an neih theihna chance a sang zawk hle . Research atanga a lan dan chuan Marfan syndrome vei mi sawm zinga 9 chuan an heart valve emaw aorta emaw ah harsatna chi khat emaw an nei dawn a ni. Chuvang chuan hei hi hriat chian a pawimawh hle.

Marfan syndrome hian thinlung a nghawng dan engte nge ni?

Marfan syndrome, aortic aneurysm emaw valve natna emaw i neih chuan symptom thenkhat i nei thei. Mahse, mi thenkhat chuan heng natna hi an nei thei a, a lan chhuah dan erawh a awm lo. Chuvang chuan medical test neih a pawimawh.

Hengte hi a tlangpuiin a lan chhuah dan chu a ni:

• Chest na emaw upper back na emaw a awm thei
• Thisen khuh (hei hi chhinchhiahna hlauhawm deuh a ni) .
• Chaw ei harsa (thinlung lian zawkin chaw kawng a press a nih chuan ) .
• Luak chhuak emaw, lu hah emaw tih hriat
• Chau lutuk emaw, chak lo emaw anga inhriatna
• Lung chak lo (heart palpitations) tih hriatna .
• Aw ri bengchheng (thinlung hian vocal cords nena inzawm nerve a press chuan) .
• Thâwk harsa , a bik takin i chau emaw, i mut emaw hian
• Swelling , a bik takin ke leh ke ruh ah
• Thâwk chhuah laiin thawk hah (whistling).

I thianpa sang tak leh cher tak i nei ang tih han ngaihtuah teh. A rilru a na tih a complain fo thin a , kal pawh a harsat phah thin . Chutiang a nih chuan Marfan syndrome nen a inzawm em tih doctor pan a tha ang.

Eng thilin nge Marfan syndrome hian thinlung a nghawng?

Kan sawi tawh ang khan Marfan syndrome hi connective tissue disorder a ni.A insiam tha tawk lo. Kan taksa hmun tinah connective tissue hrisel tak tak a awm a. Organ leh thisen kalna kawngte chu chakna, pianzia leh inthlak danglam theihna a pe a ni. He connective tissue hi thinlung leh thisen kalna bang, a bik takin aorta leh heart valve-ah te a awm a.

Marfan syndrome avanga heng connective tissue te hi an chak loh chuan an chakna leh an elasticity an hloh thin. Hei hian thinlung chu a tizau a, a ti hring a, thisen pressure a tuar thei lo. Hei hian heart valve te pawh a ti na a, a inhawng tha thei lo va, a khar tha thei lo bawk.

Eng heart test nge Marfan syndrome hriat theihna tura puitu?

Marfan syndrome hi a chang chuan hriat harsa tak a ni thei a, a lan dan hi mi hrang hrangah a inang lo va, natna dang ang bawkin a awm thei bawk. Mi tam zawk chuan an naupan lai emaw, an upa zawk emaw chauhvin an nei tih an hmuchhuak thin.

Doctor-in Marfan syndrome i vei nia a rinhlelh chuan hetiang hi an ti ang:

• Taksa exam kimchang neih a ni ang. I san zawng, i rih zawng, i kut leh ke sei zawng, i rilru pianzia, i mit leh vun rawng te chu enfiah a ni ang.
• I chhungkaw medical history an zawt ang . I chhungkuaah hian Marfan syndrome emaw, chutiang chi natna emaw nei an awm em tih an enfiah ang.
• Thinlung enfiahna tur special scan (`imaging test`) engemawzat tih a ni. A bulpui berte chu:
• Echocardiogram (Echo): Hei hian thinlung leh thisen kalna kawng lian leh te, a pianzia leh hnathawh dan chiang takin a hmu thei a, chu chu thinlung ultrasound scan ang maiin a ni.
• Electrocardiogram (EKG): Hei hian thinlung electrical activity a teh a, chu chu heartbeat rhythm a ni.
• Genetic testing: He test hi Marfan syndrome thlentu genetic mutation (FBN1 gene-a) i neih leh neih loh finfiah nan tih theih a ni.

Marfan syndrome vanga thinlung lama harsatna awmte enkawl dan tur engte nge ni?

Marfan syndrome hi tihdam kim theih ni lo mah se, thinlunga nghawng a neih dan thunun thei tur leh harsatna lian tham lo thleng tur venna tur enkawlna a awm. Hengte hi chi hnih a awm a, chungte chu non-surgical treatment leh surgical treatment te an ni.

Doctor chuan a hnuaia thil thlengah hian operation neih a rawt thei ang:

• I thinlung kawng (aorta) diameter chu centimetre 5 (inches 1.97 vel) emaw a aia lian emaw a nih chuan.
• Kum khat chhunga heart rate chu centimetre 0.5 (inches 0.197 vel) emaw a aia tam emaw a san chuan (hei hi `rapid expansion` an ti).
• I thisen chhungte (biological family member) te hian hetiang surgery hi an lo nei tawh a nih chuan (genetic influence vang pawh a ni mai thei, diameter te zawk pawha operation tih a ngai mai thei).

Taksa te zawk nei te hian thisen kalna kawng te tak te an nei tlangpui a, chuvangin an diameter te zawk pawh nise operation an mamawh mai thei.

Surgery hmanga enkawlna ni lo

Hengte hi Marfan syndrome enkawlna atana hmalakna hmasa berte an ni.

• Nunphung tihdanglam: Thinlung tichhe lutuk thei thil tih hi i pumpelh ngei tur a ni.
• Thil rit phurh emaw, nawr emaw ang chi thil tih hi a tha lo.
• High-impact sport football, rugby, boxing te hi a tha lo hle.
• Eng exercise nge i tana him leh tha ber tih hriat tumin i doctor nen inbia ang che.
• Damdawi hman tur: 1.1.
• Doctor-te chuan damdawi `Angiotensin II receptor blockers (ARBs)` emaw `beta-blockers` emaw an pe thin. Chungte chuan heart vessels dilate rate a ti slow a, thisen sang a control bawk.
• Cardiac imaging exam neih fo thin :
• Thinlung lian leh te leh valve dinhmun te hi doctor-in a enfiah fo tur a ni. Hei hi `transthoracic echocardiography (Echo)`, `computerized tomographic angiography (CTA)`, emaw `magnetic resonance angiography (MRA)` ang chi scan hmanga tih theih a ni.
• Heng test te hian thinlung a rupture (dissection) hmain a danglamna a hmuchhuak thei a ni.

Surgery hmanga enkawlna te

A châng chuan rilru lam harsatna chu damdawi leh nunphung tihdanglam ringawt pawhin a thunun theih loh. Chutih lai chuan surgery a ngai ta a ni.

Marfan syndrome natna avanga heart surgery neih tum ber chu heart valve chak lo zawk siamthat a, lakchhuah a, artificial valve dah emaw, heart valve chhia siamthat emaw, a thara thlak emaw a ni.

Heng surgery te hian a tum ber chu nunna atana hlauhawm emergency, aortic dissection ang chi te ven hi a ni.

A tam zawkah chuan heng surgery te hi ruahman lawk leh hun ruat lawk (elective surgery)-a tih thin a ni. Mahse, coronary artery a rupture emaw rupture emaw a awm chuan emergency procedure angin tih a ngai a ni.

Marfan syndrome natna hi heart surgery chi hrang hrang tih a ni a, chungte chu:

• Aortic root replacement: Hei hi surgery neih tam ber a ni a, a chhan chu thinlung hmun hi a hring emaw a zau emaw fo thin vang a ni.
• Aortic valve siamthat emaw thlak emaw.
• Thinlung bula artery-a bulge awm siamthat (`Ascending aortic aneurysm repair`).
• Mitral valve siamthat emaw thlak emaw.

Cardiac surgeon chuan a sawipui ang che a, eng surgery nge i tan a tha ber tih a rel ang.

Marfan syndrome i neih chuan thinlung natna hi i veng thei ang em?

Vanduaithlak takin Marfan syndrome hi a veng thei lo a, genetic a nih avangin. Chutiang bawkin thinlung natna a thlen hi a pumpelh kim thei lo.

Mahse, harsatna lian tham i neih theihna tur tihtlem nan thil i tih theih aortic dissection te hi a awm a:

• Thinlung tichhe thei thil tih loh (kan sawi tawh ang khan heavy lifting leh high-stress sports tih loh).
• Doctor-in a tih angin a hun taka cardiac imaging exam neih tir.
• Damdawi pek zawng zawng dik tak leh a hun taka ei.

A bik takin thil pakhat hriat reng tur chu Marfan syndrome nei hmeichhia i nih a, naupai tum i nih chuan i doctor nen inbiakna neihpui ngei tur a ni. Zirna hrang hrangah Marfan syndrome vei hmeichhia 40% thlengin naupai laiin harsatna an tawk thei tih hmuhchhuah a ni. Chuvang chuan naupai hma hian damdawi lam thurawn lak hmasak a pawimawh hle.

Marfan syndrome vanga thinlung natna vei tan hmalam hun eng nge ni ang?

Enkawlna \ha tak, chu chu damdawi lam thurawn zawm leh enkawlna \ul tak dawng a nih chuan Marfan syndrome vei chu a dam chhung pangngaiin a nung thei a, kum 70 emaw, kum 80 emaw pawh a ni thei.

Mahse, Marfan syndrome vei zingah hian thinlung lam harsatna hi thihna thlentu ber a nih avangin i dinhmun enfiah turin i cardiologist nen inbiak reng a pawimawh hle.

Engtikah nge Doctor ka hmuh ang? Emergency chu eng nge ni?

Heng symptoms te hi i neih chuan ruptured aortic aneurysm chhinchhiahna a ni thei. Emergency medical treatment i zawng nghal tur a ni:

• Thil hriatna hloh nghal mai.
• Nausea leh luak chhuak.
• Taksa khawi hmunah pawh rilru hahna.
• Paralysis (taksa hmun thenkhat che thei lo).
• Thâwk harsa na tak.
• Natna rang tak, tuar theih loh - rilru, hnungzang chunglam, emaw, pum chhungah emaw.
• Thaw hah lutuk.
• Vun chu a danglam bik lo va, a vawt emaw a vawt emaw.
• Pulse chak lo tak a ni.

Hetiang chhinchhiahna i hmuh chuan minute khat pawh tikhawtlai suh.

A tawp berah chuan hriat reng tur (Take-Home Message) .

Marfan syndrome hi pianpui natna a ni a, taksa inzawmkhawmna (connective tissue) te a nghawng a ni. A bik takin thinlung a nghawng thei a ni. Mahse, lungngai suh. He natna hi a hmaa hmuhchhuah a nih a, uluk taka enkawl a nih chuan nun pangngai tak i hmang thei a ni.

A pawimawh ber chu:

• Doctor hmu fo ang che.Tichuan i dinhmun chu a enfiah thei ang a, a hun takah test leh enkawlna tul tak tak a pe thei ang.
• Nunphung tihdanglamna tul tak tak siam rawh. I thinlung tizau thei thil lakah inthiarfihlim rawh.
• Damdawi pek ang chiah chiah ei tur a ni.
• Doctor-in operation a rawt chuan uluk takin sawiho la, i tana ṭha ber tur chu thlang rawh.

I dinhmun i hriat tam poh leh i enkawl a awlsam zawk ang. Chuvangin he thu hi i tan a tangkai ngei kan beisei. Zawhna i neih chuan doctor zawt hreh suh.

` Marfan Syndrome, thinlung natna, rilru lam natna, thinlung valve, connective tissue, genetic natna, thinlung surgery