Kodi Cardiac Amyloidosis Ndi Chiyani?

Amyloidosis ndi banja la mavuto omwe mapuloteni osadziwika, otchedwa amyloid mapuloteni, amakaikidwa m'magulu osiyanasiyana. Zosungiramo mavitaminiwa zimatha kusokoneza kwambiri ntchito za thupi.

Mu mtima wamyloidosis, mapuloteni osungunuka amakhala mu minofu ya mtima. Pulogalamu ya amyloid imapangitsa kuti mpanda wolimba wa mtima ukhale wolimba , umene umapangitsa kuti matenda a diastolic asamayende bwino .

Kulephera kwa diastolic, mtima sungathe kupumula bwino pakati pa kugunda kwa mtima, kotero umadzaza ndi magazi mochepa. Kuonjezera apo, amyloid deposits amachepetsa mphamvu ya minofu ya mtima kuti igwirizane bwinobwino.

Choncho ndi mtima wamyloidosis, ntchito ya mtima imakhudzidwa panthawi ya diastole (gawo lokhazika mtima pansi kwa mtima) ndi systole (chigawo cha kupweteka kwa mtima) -momwe mtima ukugunda .

Chifukwa cha mavutowa ndi diastole ndi systole, mtima wodwala umakhala wofala ndi mtima wamyloidosis. Kuonjezera apo, anthu omwe ali ndi vutoli amayamba kukula kwambiri. Cardiac amyloidosis ndi vuto lalikulu lomwe nthawi zambiri limachepetsa moyo wautali kwambiri.

Kodi Zimayambitsa Amyloidosis?

Zambiri zingayambitse mapuloteni a amyloid kuti asungunuke m'matumbo. Izi zikuphatikizapo:

• Primary amyloidosis. Dzina lakuti "primary amyloidosis" poyamba linkatchulidwa mtundu wa amyloidosis momwe palibe chifukwa chomwe chingadziwike. Masiku ano, primary amyloidosis imadziwika kuti imachokera ku matenda a maselo a plasma (maselo a Plasma ndiwo maselo oyera omwe amachititsa ma antibodies ). , ndi mawonekedwe a myeloma angapo . Amayi ambiri omwe ali ndi mtima wamyloidosis amakhala ndi amyloidosis, omwe ali ndi zilembo za amyloid. ndi mtundu uwu wa amyloidosis wamtima kawirikawiri amakhalanso ndi mapulogalamu a amyloid mu impso zawo, chiwindi ndi matumbo.

• Sekondi ya amyloidosis Secondary amyloidosis imachitika mwa anthu omwe ali ndi mtundu wina wa matenda opweteka aakulu, makamaka lupus , kutupa kwa m'mimba , kapena matenda a nyamakazi . Chifukwa cha kutupa kosatha, mavitamini a mtundu wa "amyloid type A" (omwe amatchedwanso AA protein) angakhale owonjezera. Chiwerengero cha amyloidosis chachiwiri chimakhala ndi impso, chiwindi, nthenda, ndi ma lymph. Sekondale amyloidosis sizimakhudza mtima, komabe. Pafupifupi 5 peresenti ya amyloidosis ya mtima imayambitsidwa ndi mapulogalamu a AA mapuloteni.

• Senile amyloidosis Senile amyloidosis amachokera ku mfundo yakuti nthawi zambiri amawoneka mwa amuna achikulire, kawirikawiri ndi amuna oposa zaka makumi asanu ndi awiri. Pachikhalidwe ichi, mavitamini ochulukirapo amapezeka ndi mapuloteni ena omwe amatha kutchedwa, omwe amapangidwa m'chiwindi. Mu senile amyloidosis, mapuloteni a TTR amapezeka nthawi zambiri pamtima. Nthenda yotchedwa senile amyloidosis imapezeka pafupifupi 45 peresenti ya matenda a mtima amyloidosis.

Zizindikiro za mtima wa Amyloidosis

Monga tafotokozera, mtima wamtima wa amyloidosis umakhudzanso kukhuta kwa mtima ndi kupopera kwa mtima, choncho siziyenera kudabwitsa kuti thupi lonse limakhala loipa kwambiri.

Chotsatira kwambiri cha mtima wamyloidosis ndi mtima wosalimba. Ndipotu, zizindikilo za mtima kulephera-makamaka ndi dyspnea komanso zofunikira za edema (kutupa) -zimene zimayambitsa matenda a amyloidosis.

M'thupi la amyloidosis lopangidwa ndi mapuloteni a AL (primary amyloidosis), ziwalo za m'mimba zimakhudzidwa kuwonjezera pa mtima. Choncho anthuwa amakhala ndi zizindikiro za m'mimba, monga kusowa kwa kudya, kusamvana msanga, ndi kutaya thupi. Kuonjezera apo, mapuloteni a AL amaphatikizapo mitsempha yaing'ono yamagazi, yomwe ingayambitse kuvulaza, angina , kapena kumwa mankhwala (minofu ikuphwanyidwa ndi mphamvu).

Anthu omwe ali ndi mtima wamyloidosis amakhala okonzeka ku syncope (zigawo za kutaya mtima). Ndi amyloidosis, syncope ikhoza kukhala chizindikiro choopsa, chifukwa chimasonyeza kuti mtima wamagetsi watambasulidwa pafupi ndi malire ake. Makamaka, anthu omwe ali ndi amyloidosis omwe amakhudza mitima yawo ndi mitsempha ya magazi sangathe kubwezeretsa china chilichonse chimene chimayambitsa mavuto a mtima, ngakhale pang'ono. Chochitika choterocho chingaphatikizepo chiwonetsero cha vasovagal chomwe chingangopangitsa kanthawi kozunguza mwa munthu wina.

Kotero, pamene imfa yadzidzidzi imapezeka mwa anthu omwe ali ndi mtima wamyloidosis, kugwa kwa mtima mwadzidzidzi ndiko chifukwa.

Izi zikusiyana kwambiri ndi anthu omwe amafa imfa mwadzidzidzi kuchokera ku matenda ena amtima, omwe mtima wamaganizo (makamaka ventricular tachycardia kapena ventricular fibrillation ) nthawi zambiri ndi chifukwa chake. Chifukwa chake, kuika munthu wodzitetezera m'thupi mwa anthu omwe ali ndi mtima wamyloidosis nthawi zambiri samapitiriza kupulumuka. Pamene anthu omwe ali ndi mtima wamyloidosis amakhala ndi syncope, chiopsezo cha imfa mwadzidzidzi mkati mwa miyezi yotsatira ikukwera.

Ndi mtima wa amyloidosis, ma deposy amyloid amapezeka kawirikawiri pamtundu wa magetsi. ( Fufuzani za kayendedwe kabwino ka mankhwala ). Mu senile amyloidosis, mtundu wa TTR wa mapuloteni amachititsa kuti munthu akhale ndi mtima wolimba kwambiri, ndipo amafunika kukhazikika kwa pacemaker yosatha. Komabe, ndi mtundu wa AL-amyloidosis bradycardia siwodziwika, ndipo kawirikawiri siwatsogolera pacemaker.

Anthu omwe ali ndi mtima wa amyloidosis nthawi zambiri amawombera magazi mosavuta, m'mitsempha ya mitsempha ndi m'mtima, zomwe zimapangitsa kuti chiopsezo chachikulu cha thromboembolism chiwonjezeke .

Kuwonongeka kwa ubongo ndi vuto la anthu omwe ali ndi AL amyloidosis.

Kodi Cardiac Amyloidosis Amadziwika Bwanji?

Madokotala ayenera kulingalira kuti angathe kukhala ndi mtima wamtima wamtima pamene munthu ali ndi zifukwa zosadziwika, makamaka ngati dyspnea ndi edema ndizozizindikiro kwambiri.

Munthu yemwe ali ndi vuto la mtima watsopano, kukhalapo kwa kuthamanga kwa magazi, chiwindi chofutukuka, phokoso la m'mimba, kapena puloteni mu mkodzo, ziyenera kukumbukiranso momwe mungathere mtima wa amyloidosis.

Kachipangizo ka electrocardiogram mu mtima wamyloidosis angasonyeze kutsika kwa mphamvu (ndiko kuti, chizindikiro cha magetsi ndi chachichepere kuposa masiku onse), koma kawirikawiri chimakhala chodziwika bwino chomwe chimapereka chitsimikizo chabwino ku matenda oyenerera.

Kafukufukuyu amasonyezeratu kuwonjezeka kwa minofu ya mtima ya zonsezi. Kuonjezera apo, ma-amyloid amadzipangika nthawi zambiri amapanga pachithunzi chomwe chilipo "mawonekedwe" omwe amachokera mkati mwa minofu ya mtima. Magazi amafukula mumtima amapezedwanso mwachilungamo.

Ngakhale kuti kafukufuku wamakono amatsogolera njira yopita kuchipatala, kupatsirana ndi matenda a amyloidosis kumafuna chiwonetsero cha minofu chomwe chimasonyeza ma depositi amyloid. Kwa anthu okhala ndi AL amyloidosis, kachilombo kawirikawiri kamatha kupezeka kuchokera ku mimba ya m'mimba kapena ku mafupa a mafupa. Komabe, vuto la mtima ndilofunika ndi TTR amyloidosis (ndipo nthawi zina ngakhale AL amyloidosis). Mtima wonyansa umachita kawirikawiri, osati pogwiritsa ntchito njira ya catheter.

Kodi Cardiac Amyloidosis Amachita Motani?

Kawirikawiri, mtima wamyloidosis uli ndi vuto loipa. Komabe, m'zaka zaposachedwa njira zothandizira zatsopano zakhazikitsidwa chifukwa cha mtima wa amyloidosis, ndipo anthu omwe ali ndi vutoli ali ndi chifukwa chokhala ndi chiyembekezo choposa momwe akadakhalira zaka zingapo zapitazo.

Mankhwala a mtima amyloidosis angaganizidwe m'magulu awiri: kuchiza mtima kulephera, ndi kuchiza matenda omwe amachititsa kuti amyloid apange.

Kuchiza kwa Mtima Kusalephereka

Kuchiza mtima kusokonekera chifukwa cha amyloidosis ya mtima kumasiyana kwambiri ndi kuchiza mtima kusokonekera chifukwa cha zinthu zina. Ngakhale kuti beta-blockers ndi ACE inhibitors ndizofunikira kwambiri pochiza mitundu yosiyanasiyana ya mtima, mankhwalawa (komanso calcium-channel blockers ) angapangitse mtima waumtima kukhala wovuta kwambiri. Zolephera izi zimapangitsa kuchipatala kwa vuto la mtima kukhala vuto lalikulu mu amyloidosis.

Kugwiritsiridwa ntchito kwa diuretics yozungulira , monga furosemide (Lasix), ndiyo njira yaikulu ya mankhwala opatsirana mu mtima wa amyloidosis. Mankhwalawa nthawi zambiri amathandiza kuchepetsa mphuno yowopsya yomwe imayendetsa matendawa, komanso imatha kuthetsa dyspnea (chizindikiro china chofala). Mankhwala otsegula m'mimba amagwiritsidwa ntchito moyenera, ndipo amatha kuperekedwa mwachangu ngati akufunikira.

Ophwanya Beta sayenera kugwiritsidwa ntchito mu mtima wamyloidosis. Kutha kwa mtima kuli kochepa mu chikhalidwe ichi, ndipo panthawi imodzimodzi mtima sungakhoze kudzaza ndi magazi mwachangu kwambiri. Chotsatira chake, kuwonjezeka kwa mtima kumakhala kofunikira kuti mukhale ndi mtima wabwino wokwanira mu mtima wamyloidosis. Izi zikutanthawuza kuti beta blockers, pochepetsa kuchepa kwa mtima, zingachititse kuti anthuwa asokonezeke mwadzidzidzi. Calcium blockers akhoza kuchepetsa kuthamanga kwa mtima, komanso ayenera kupeŵa.

Kwa anthu okhala ndi AL-amyloidosis, ACE inhibitors angapangitse kuchepa kwakukulu (ndipo mwina kupha) m'magazi-mwinamwake chifukwa chakuti mavitamini a m'mitsempha ya mthupi amachititsa kuti mitsempha yowonongeka ikwaniritse zovuta zomwe ACE zimayambitsa kawirikawiri. Kutaya kwakukulu kwa magazi sikukuwoneka mwa anthu omwe ali ndi TTR amyloidosis, ndipo mwa anthuwa ACE inhibitors akhoza kuyesedwa mosamala.

Kuwombera mtima sikungatheke kwa anthu okhala ndi AL-amyloidosis, chifukwa ali ndi matenda akuluakulu mu ziwalo zina zingapo. Ngakhale anthu omwe ali ndi TTR-mtundu amyloidosis ali ndi matenda okhazikika pamtima, amakhala okalamba kwambiri kuti asamayesedwe kuti ndi oyenerera kuika mtima kwa mtima. Kusambala kungakhale njira yowonjezeka yachinyamata yemwe ali ndi TTR-mtundu wa mtima amyloidosis.

Kuchiza kwa Matendawa Kumayambitsa Amyloidosis

Primary, AL-mtundu wa Amyloidosis. Mtundu uwu wa amyloidosis umayamba chifukwa cha maselo osadziwika a maselo a plasma omwe amapanga zilembo zambiri za AL-type amyloid. Chifukwa chake, m'zaka zaposachedwapa mankhwala a chemotherapeutic akhazikitsidwa kuti ayese kupha maselo osadziwika. Mankhwala a melphalan omwe amatsatiridwa ndi mafupa a mafupa ndi mankhwala omwe amalangizidwa. Tsoka ilo, anthu okhala ndi AL amyloidosis omwe ali ndi mtima wokhudzana ndi mtima nthawi zambiri sali otheka kuti athe kulekerera mtundu uwu wa mankhwala opweteka. Mankhwala ena a chemotherapy angagwiritsidwe ntchito mwa anthuwa, komabe, komanso yankho laling'ono likuwonekera ambiri mwa iwo. Ngati AL amyloidosis ingapezedwe ndi kuchiritsidwa isanakhale yowonjezereka, zotsatira zowonjezeka ndizovuta.

Secondary Amyloidosis. Ndi ochepa chabe a anthu omwe ali ndi mtima wamyloidosis ali ndi vutoli. Komabe, kupsa mtima kwa matenda opweteka kwambiri kumachepetsa kukula kwa amyloidosis.

Senile Amyloidosis. Kwa anthu omwe ali ndi mtima wamyloidosis womwe umayambitsa TTR amyloid, mapuloteni owonjezera amapangidwa m'chiwindi. Zikuoneka kuti TTR-mtundu amyloidosis ndi mitundu iwiri. Mu imodzi mwa mitundu iyi, mitundu yosawerengeka yomwe imawoneka kwa achinyamata, kuikidwa kwa chiwindi kumachotsa puloteni ya TRY-type amyloid ndipo imayimitsa kukula kwa amyloidosis. Tsoka ilo, kwa anthu achikulire omwe ali ndi zambiri, tyala la mtundu wa amyloidosis, chiwindikiro cha chiwindi sichimakhudza kupitirira kwa matenda.

Mankhwalawa akufufuzidwa kuti athe "kukhazikitsa" puloteni ya TTR kotero kuti sichidzakhalanso ngati ma deposy amyloid. Komabe, ndikumayambiriro kwambiri kuti mudziwe ngati mankhwalawa akhoza kusintha kwambiri zotsatira za TTR-mtundu wa cardiac amyloidosis.

Pansi

Cardiac amyloidosis ndi vuto lalikulu lomwe limayambitsa zizindikiro zazikulu, ndipo zimachepetsa moyo wautali. Mavuto angapo angapangitse amyloidosis, komanso mankhwala abwino kwambiri-ndipo pamlingo winawake umatanthawuza kuti mankhwalawa ndi osiyana siyana - amasiyana ndi mtundu wa mapuloteni amyloid omwe akuikidwa m'matumbo.

Ngakhale zili zovuta izi, pakupita patsogolo kumvetsetsa mitundu yambiri ya mtima wamyloidosis, komanso poyesa njira zabwino zothandizira aliyense payekha.

> Zotsatira:

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> Dubrey SW, Hawkins PN, Falk RH. Matenda a Amyloid a Mtima: Kufufuza, Kuzindikira, ndi Kutumiza. Mtima wa 2011; 97:75.

> Kristen AV, Dengler TJ, Hegenbart U, ndi al. Kupanga Mavitamini a Odwala Magetsi Odwala Magetsi Odwala Amtima Odwala Amtima Odwala Amtima Ndiponso Amphawi Ambiri Akufa Mwadzidzidzi. Mtima; 5: 235.

> Wechalekar AD, Zambezi SO, Kastritis E, et al. Phunziro la Ogwirizanitsa la European Treatment Outcomes mu Odwala 346 Omwe Ali Ndi Maganizo a Mtima Wachikondi Wachilendo Wowonjezera Wachitatu AL Amyloidosis. Magazini 2013; 121: 3420.