Te Mate Marfan me tō Manawa: Me Kōrero Tātou mō tēnei!

Kua kite pea koe i ngā tāngata he roa ake, he roa ake ō rātou waewae, ā, he kikokore ake i ētahi atu. He mate tēnei mō te katoa, engari ehara i te mate. Engari i ētahi wā, ko tēnei momo āhua tinana he tohu pea nō tētahi mate ira e kiia nei ko te Marfan Syndrome . He mate tēnei e puta ana ina kore e whanake tika te kiko honohono o roto i tō tātou tinana. He māmā noa iho, ko tēnei kiko honohono te mea e pupuri ana i ngā wāhanga rerekē o tō tātou tinana, e whakakaha ana hoki i a rātou. Ina ngoikore ēnei, ka pāngia te nuinga o ngā wāhanga o te tinana, inā koa ko te ngākau, ngā kanohi, ngā uaua toto, me te pūnaha angaiwi . I tēnei rā, ka kōrero tātou mō te pānga o tēnei mate e kiia nei ko te Marfan Syndrome ki te ngākau.

He pēhea te pānga o te Marfan Syndrome ki te ngākau?

Ina pāngia koe e te Marfan syndrome, e rua ngā wāhanga matua o te ngākau ka tino pāngia.

1. Aorta: Koinei te oko toto matua, nui rawa atu e kawe ana i te toto whai hāora mai i te ngākau ki te tinana katoa. He rite ki te paipa matua e kawe ana i te wai mai i te tāke wai ki tō whare.

2. Ngā takirere o te ngākau: Koinei ngā wāhanga e mahi ana pērā i ngā tatau i waenganui i ngā rūma o roto i te ngākau me ngā uaua matua e kawe ana i te toto i waho o te ngākau. Mā ēnei ka rere te toto i te taha kotahi anake.

Kia tirohia takitahitia ēnei kia kite ai tātou i te aha ka tupu.

He aha te mea ka tupu ki te aorta?

Ka ngoikore te kiko honohono i roto i ngā pakitara o te ngākau nā te mate Marfan. Pērā i te ngongo rapa tawhito, ka ngaro tōna kaha me tōna ngāwari. Ka taea e tēnei te arahi ki ngā raruraru matua e rua:

• Te whānui haere o te aorta: Ka tīmata te whānui haere me te whakanui haere o te aorta.
• Te aneurysm o te aorta: I ētahi wāhi, ka ngoikore te pakitara o te ngākau, ā, ka pupuhi ki waho pērā i te poihau.

Whakaarohia, he rite tēnei aneurysm ki tētahi potae pahikara e pupuhi ana i tētahi wāhi ina ngoikore te ngongo.

Ina koa, ko te wāhanga o te ngākau e tata ana ki te ngākau, e kiia nei ko te 'pūtake aortic', koinei te wāhanga e whakanuia ana, e whānuitia ana rānei i roto i te hunga whai Marfan syndrome. He mate tino morearea tēnei, nā te mea ki te nui haere tēnei 'aneurysm' ka pakū ('aortic dissection', ka pakaru rānei), ka mate pea.

Ka taea hoki e ētahi atu āhuatanga pēnei i te mate Ehlers-Danlos, te mate Loeys-Dietz, te takirere aorta bicuspid, me te mate Turner te whakawhanui i te ngākau, engari ka puta pea ēnei i ētahi atu wāhanga o te ngākau.

He aha te mea ka pā ki ngā takirere o te ngākau?

Ka pā mai hoki ngā raruraru ki ngā takirere o te ngākau i te mate Marfan. Ki te kore e mahi tika ēnei takirere, me kaha ake te mahi a te ngākau ki te pupuhi toto. I roto i te wā, ka arahi pea tēnei ki te ngoikore o te ngākau.Ka taea te haere i ngā taha e rua. E rua ngā mate takirere matua e kitea ana e hono ana ki te Marfan syndrome:

• Te ruakitanga o te takirere aorta: Ina kore e kati tika te takirere i waenganui i te aorta me te rūma maui o raro o te ngākau (te ventricle maui), ka turuturu ētahi o te toto kua pupuhihia ki roto i te ngākau. He rite ki te waikeri e turuturu ana.
• Te hekenga o te takirere mitral: Ko te takirere mitral, kei waenganui i te ruma o runga (atrium maui) me te ruma o raro (ventricle maui) i te taha maui o te ngākau, kāore e kati tika, ā, ka puta te ruma o runga ki waho. I ētahi wā ka turuturu te toto ki muri (mitral regurgitation).

He pēhea te noa o te mate manawa i roto i te hunga whai Marfan syndrome?

Ina hoki, he nui ake te tūponotanga o te hunga whai Marfan syndrome ki te pāngia e ngā raruraru o te ngākau. Kua whakaatuhia e ngā rangahau e iwa i roto i te tekau o ngā tāngata whai Marfan syndrome ka pāngia e tētahi momo raruraru o te takirere ngākau, o te aorta rānei. Koia te mea nui kia mōhio ki tēnei.

He aha ngā tohu o te Marfan syndrome e pā ana ki te ngākau?

Mena kei a koe te Marfan syndrome, pērā i te aneurysm aortic, te mate takirere rānei, tera pea ka pāngia koe e ētahi tohu. Heoi, tera pea ētahi tāngata e pāngia ana e ēnei āhuatanga, kāore he tohu. Koia te hiranga o ngā whakamātautau hauora.

Koinei ngā tohu ka kitea whānuitia:

• Mamae o te uma, mamae rānei o te tuara o runga
• Te maremare toto (he tohu mōrearea ake tēnei)
• Te uaua ki te horomi kai (mēnā kei te pēhi te ngākau nui ki te esophagus )
• Te pōuri, te māhunga rānei
• Te tino ngenge, te ngoikore rānei
• Te ahua o te tere o te ngakau (ngā palpitations o te ngakau)
• Te pāorooro o te reo (mēnā ka pēhia te uaua e hono ana ki ngā taura reo e te ngākau)
• Te uaua ki te manawa , ina koa ka ngenge koe, ka takoto rānei
• Te pupuhi , inā koa i ngā waewae me ngā pona
• Te wheehe (te whiowhio) i te wā e manawa ana

Whakaarohia he hoa tōu he tino roa, he kikokore. He maha ana amuamu mō te mamae o te uma, ā, he uaua ki te hikoi . Mena koinā, he mea pai kia toro atu ki te tākuta kia kite ai mēnā he hononga ki te Marfan syndrome.

He aha te pānga o te Marfan syndrome ki te ngākau?

E ai ki tā mātou i kōrero ai i mua, ko te Marfan syndrome he mate hononga hononga.Kāore i te tika te hanganga. Kei ngā wāhi katoa o ō tātou tinana te kiko honohono hauora. Ka homai he kaha, he āhua, he ngāwari hoki ki ngā okana me ngā oko toto. Kei roto i ngā pakitara o te ngākau me ngā oko toto, inā koa te aorta, me ngā takirere ngākau tēnei kiko honohono.

Ina ngoikore ēnei kiko honohono nā te mate Marfan, ka ngaro te kaha me te ngāwari. Ka whānui, ka pupuhi te ngākau, ā, ka kore e taea te tu atu i te pēhanga o te toto. Ka mate hoki ngā takirere ngākau, ā, kāore e taea te whakatuwhera, te kati rānei i te tika.

He aha ngā whakamātautau ngakau e āwhina ana ki te tautuhi i te Marfan syndrome?

He uaua te tautuhi i te mate Marfan i ētahi wā, nā te mea he rerekē ngā tohu i ia tangata, i ia tangata, ā, ka taea te rite ki ngā tohu o ētahi atu mate. Ko te nuinga o te tangata ka kitea kua pāngia rātou i te wā e tamariki ana, e pakeke haere ana rānei.

Mena kei te whakapae te tākuta kei a koe te Marfan syndrome, ka mahi rātou i ngā mea penei:

• Ka whakahaerehia he whakamātautau tinana katoa . Ka tirohia tō teitei, tō taumaha, tō roa o te ringa me te waewae, tō āhua o te uma, ō kanohi, me tō tae kiri.
• Ka pātai atu rātou mō tō hītori hauora whānau . Ka tirohia e rātou mēnā kua pāngia tētahi o tō whānau e te Marfan syndrome, e ngā tohumate rite rānei.
• He maha ngā matawai motuhake ('whakamātautau whakaahua') ka whakaritea hei tirotiro i te ngākau . Ko ngā mea matua ko:
• Echocardiogram (Echo): Mā tēnei ka taea e koe te kite mārama i te rahi, te āhua, me te mahi a te ngākau me ngā oko toto, he rite tonu ki te matawai ultrasound o te ngākau.
• Electrocardiogram (EKG): Mā tēnei ka ine i te ngohe hiko o te ngākau, arā, te manawataki o te pao o te ngakau.
• Whakamātautau ira: Ka taea tēnei whakamātautau hei whakaū mēnā he whakarerekētanga ira tōu (i roto i te ira FBN1) e hua ai te mate Marfan.

He aha ngā maimoatanga hei whakahaere i ngā raruraru ngākau e puta mai ana i te Marfan syndrome?

Ahakoa kāore e taea te whakaora katoa i te Marfan syndrome, kei reira ngā maimoatanga hei whakahaere i ngā pānga ki te ngākau me te ārai i ngā raruraru nui. E rua ngā momo o ēnei: ​​ko ngā maimoatanga kore-pokanga me ngā maimoatanga pokanga.

Ka tūtohu pea te tākuta i te pokanga i roto i ngā take e whai ake nei:

• Mena he 5 henimita (tata ki te 1.97 inihi) te whānui o te oko toto o tō ngākau (aorta), he nui ake rānei.
• Ki te piki ake te tere o te manawa mā te 0.5 henimita (tata ki te 0.197 inihi) neke atu rānei i roto i te tau (ka kiia tēnei ko te 'whanui tere').
• Mena kua pāngia ō whanaunga toto (ngā mema o te whānau tūturu) i tēnei momo pokanga (nā ngā awe ira pea, me mahi pea te pokanga ahakoa he iti ake te diameter).

Ko te hunga he iti ake te tinana, he iti ake ō rātou uaua toto, nō reira, me pokanga pea rātou ahakoa he iti ake te whānui.

Ngā maimoatanga kore-pokanga

Koinei ngā taahiraa tuatahi ki te whakahaere i te Marfan syndrome.

• Ngā whakarerekētanga o te momo noho: Me karo rawa koe i ngā mahi e tino taumaha ai te ngākau.
• Kāore i te pai te mahi i ngā mea pēnei i te hiki taumaha, te pana rānei.
• Kāore i te tika ngā hākinakina whai pānga nui pēnei i te whutupaoro, te whutupōro, me te mekemeke.
• Kōrero ki tō tākuta kia mōhio ai koe ki ngā mahi whakakori tinana e haumaru ana, e tika ana hoki māu.
• Ngā rongoā:
• Ka tohua e ngā tākuta ngā rongoā e kiia nei ko ngā 'Angiotensin II receptor blockers (ARBs)' me ngā 'beta-blockers' . Ka āwhina ēnei ki te whakaroa i te tere o te whānui o ngā oko toto o te ngākau, me te whakahaere i te toto toto tiketike.
• Ngā whakamātautau whakaahua o te ngākau i ia wā :
• Me tirotiro tonu te tākuta i te rahi o te ngākau me te āhua o ngā takirere. Ka taea tēnei mā te whakamahi i ngā matawai pēnei i te `transthoracic echocardiography (Echo)`, `computerized tomographic angiography (CTA)`, `magnetic resonance angiography (MRA)` rānei.
• Ka taea e ēnei whakamātautau te kite i ngā huringa o te ngākau i mua i te pakaru (te tapahi).

Ngā maimoatanga pokanga

I ētahi wā, kāore e taea te whakahaere i ngā raruraru o te ngākau me te ngākau mā te rongoā me ngā huringa āhua noho anake. Koinei te wā e tika ai te pokanga.

Ko te whāinga matua o te pokanga ngakau mō te mate Marfan he whakatika i tētahi wāhanga ngoikore o te takirere ngakau, he tango atu, he whakato i tētahi takirere horihori, he whakatika rānei i tētahi takirere ngakau kua pakaru, he whakakapi rānei ki tētahi mea hou.

Ko te whāinga matua o ēnei pokanga he ārai i ngā āhuatanga ohorere e morearea ana ki te ora pērā i te tapahi o te aorta.

I te nuinga o te wā, ka whakaritea, ka mahia hoki ēnei pokanga i tētahi rā kua whakaritea i mua (pokanga whiriwhiri). Heoi, ki te puta he pakaru ohorere, he pakaru rānei o tētahi uaua o te ngākau, me mahi hei tikanga ohorere.

He maha ngā momo pokanga ngakau matua e mahia ana mō te Marfan syndrome:

• Te whakakapinga o te pakiaka o te aorta: Koinei te pokanga tino noa nā te mea ka pupuhi, ka whānui rānei tēnei wāhanga o te ngākau.
• Te whakatikatika, te whakakapi rānei i te takirere aorta.
• Te whakatikatika i te pupuhi i roto i te uaua tata ki te ngākau ('Te whakatikatika i te aneurysm aortic piki').
• Te whakatikatika, te whakakapi rānei i te takirere mitral.

Ka kōrero tahi te taote ngakau me koe, ā, ka whakatau ko tēhea pokanga te mea pai mōu.

Ka taea te ārai i te mate manawa mēnā kei a koe te Marfan syndrome?

Engari, kāore e taea te ārai i te Marfan syndrome, nā te mea he ira tuku iho. Waihoki, kāore e taea te ārai katoa i te mate ngakau e puta mai ana.

Heoi anō, kei reira ētahi mea ka taea e koe te mahi hei whakaiti i tō tupono ki ngā raruraru nui, pērā i te wehenga o te aorta:

• Te karo i ngā mahi e whakararuraru ana i te ngākau (e ai ki tā tātou i kōrero ai i mua ake nei, te karo i te hiki taumaha me ngā hākinakina e tino taumaha ana).
• Te whakahaere i ngā whakamātautau whakaahua o te ngākau i te wā tika e ai ki te whakahau a te tākuta.
• Te tango i ngā rongoā katoa kua tohua i roto i te tikanga tika, me te wā tika hoki.

Kotahi te mea hei maumahara, mēnā he wahine koe e pāngia ana e te Marfan syndrome, ā, kei te whakaaro koe kia hapu, me kōrero koe ki tō tākuta mō tēnei mea. Kua whakaatuhia e ngā rangahau tae atu ki te 40% o ngā wāhine e pāngia ana e te Marfan syndrome ka pāngia pea e ngā raruraru i te wā e hapu ana. Nō reira, he mea tino nui kia rapu tohutohu hauora i mua i te haputanga.

He aha te tirohanga ā muri ake nei mō te tangata e pāngia ana e te mate manawa nā te Marfan syndrome?

Mā te whakahaere pai, arā, te whai i ngā tohutohu hauora me te whiwhi i te maimoatanga e tika ana, ka taea e te tangata whai Marfan syndrome te ora i tētahi roanga o te oranga noa, tae atu ki te 70, 80 rānei tau.

Heoi, ko ngā raruraru o te ngākau te tino take o te mate i waenga i te hunga e pāngia ana e te Marfan syndrome, nō reira he mea nui kia noho tonu koe ki te toro atu ki tō tākuta ngākau kia aroturukitia ai tō āhua.

Āhea ahau me toro atu ki te tākuta? He aha ngā āhuatanga ohorere?

Ki te pāngia koe e tētahi o ēnei tohu, he tohu pea tēnei nō te pakaru o te aneurysm aortic. Me rapu tonu koe i te maimoatanga hauora ohorere:

• Te ngaronga ohorere o te māramatanga.
• Whakapairuaki me te ruaki.
• Te ngenge i ngā wāhi katoa o te tinana.
• Te pararutiki (te kore e taea te neke i ētahi wāhanga o te tinana).
• Te uaua nui ki te manawa.
• Te mamae ohorere, e kore e taea te whakamanawanui - i te uma, i te tuara o runga, i te puku rānei.
• Te werawera nui.
• Ka māwhero haere te kiri, ka makariri rānei, he rerekē te āhua.
• Tino ngoikore te pā o te manawa.

Ki te kite koe i tētahi tohu pēnei, kaua e whakaroa ahakoa mō tētahi meneti.

Hei whakamutunga, ngā mea hei maumahara (Karere hei tango ki te kāinga)

He mate whānau te Marfan syndrome e pā ana ki ngā kiko honohono o te tinana. Tērā pea ka pā ki te ngākau. Kaua e manukanuka. Mēnā ka kitea wawetia tēnei mate, ā, ka whakahaerea tika, ka taea e koe te noho i te oranga noa.

Ko te mea tino nui ko:

• Tirohia tō tākuta i ia wā, i ia wā.Kātahi ka taea e ia te aroturuki i tō āhua me te whakarato i ngā whakamātautau me te maimoatanga e tika ana i te wā tika.
• Me whakarerekē i ngā āhuatanga o tō oranga. Me noho tawhiti atu i ngā mea e taumaha ana ki tō ngākau.
• Tangohia te rongoā kia rite ki te mea i whakaritea.
• Ki te tūtohu te tākuta kia pokanga koe, kōrerohia marietia, ā, whiriwhiria te mea e pai ana māu.

Mā te nui ake o tō mōhiotanga mō tō mate, ka māmā ake te whakahaere i a ia. Nō reira, ko te tumanako ka whai hua ēnei mōhiohio ki a koe. Mena he pātai āu, kaua e mangere ki te pātai ki tō tākuta.

` Te Mate Marfan, te mate manawa, te pūnaha manawa, ngā takirere manawa, te kiko honohono, ngā mate ira, te pokanga manawa