Ua ʻike anei ʻoe i kēia mau mea e pili ana i ka ventricle hema o kou puʻuwai? (Left Ventricular Non-Compaction - LVNC) E kamaʻilio kākou!

I kēia lā, e kamaʻilio mākou e pili ana i kahi ʻano paʻakikī iki, akā he mea nui loa paha o ka puʻuwai. ʻAʻole paha ʻoe i lohe i kēia ma mua. Ua kapa ʻia ʻo Left Ventricular Non-Compaction (LVNC) e like me ka kapa ʻana a nā kauka. He mea nui paha ka inoa, akā mai hopohopo, e mālama kākou i ka maʻalahi.

No laila he aha ka hemahema o ka puʻuwai noncongestive ventricular hema (LVNC)?

I ka ʻōlelo maʻalahi, he ʻano kēia e kū mai ana i ka wā e ulu pono ʻole ai ke keʻena haʻahaʻa ma ka ʻaoʻao hema o ko kākou puʻuwai, ʻo ia hoʻi ka Ventricle Hema . E noʻonoʻo, ʻo ka maʻamau, ʻo kēia ventricle hema o ko kākou puʻuwai he ʻāpana ʻiʻo ikaika a laumania hoʻi. ʻO ia ka ʻāpana nui e hoʻomoʻa i ke koko i ko kākou kino holoʻokoʻa. Akā i loko o kekahi me kēia (LVNC), ua like ka ventricle hema me ka huʻahuʻa, ʻoi aku ka mānoanoa, a hoʻonohonoho ʻia e like me ka ʻupena. He ʻano puʻu kona, e like me loko o kahi berena.

ʻO ka maʻamau, ua manaʻo ʻia kēia he maʻi puʻuwai hānau. ʻO ke ʻano kēia, aia paha kēia maʻi i loko o kou puʻuwai mai kou wā i hānau ʻia ai. Inā ʻaʻole mālama pono ʻia, hiki i kēia "(LVNC)" ke alakaʻi i nā pilikia puʻuwai koʻikoʻi, ʻo ia hoʻi, nā maʻi puʻuwai.

Pehea e pili ai kēia kūlana LVNC i ka hana o ka puʻuwai?

E nānā, he hana koʻikoʻi ko ka ventricle hema o ko kākou puʻuwai. ʻO ia ka mea e pamu i ke koko momona i ka oxygen mai nā māmā a i ke kino holoʻokoʻa. E like me ka motika wai. Eia naʻe, i ka wā e kū mai ai kēia kūlana (LVNC), ʻaʻole hiki i ka ventricle hema, e like me ka huʻa, ke ʻūlū a hoʻomaha pono. ʻO ia hoʻi, he paʻakikī iā ia ke hoʻopaʻa a hoʻokuke i ke koko i mua, a i ʻole e hoʻomaha a hoʻopiha hou iā ia me ke koko. No laila, ʻo ka hopena , ʻaʻole hana pono ka hana o ka pamu ʻana i ke koko i ke kino holoʻokoʻa . ʻO ia ke kumu e hoʻomaka ai nā pilikia like ʻole.

Pehea ka laha ʻole o kēia ʻano i kapa ʻia ʻo LVNC?

He ʻano maʻi laha ʻole kēia. Wahi a nā loea lapaʻau, he ʻumikūmālua wale nō kānaka i loko o hoʻokahi miliona i ʻike ʻia me kēia maʻi i kēlā me kēia makahiki. Eia nō naʻe, ʻoi aku paha ka nui o ka helu maoli . ʻO ke kumu, ʻaʻole hōʻike kekahi poʻe i kekahi mau hōʻailona. A laila, ʻaʻole lākou e hele i ke kauka, a no laila, ʻaʻole i loaʻa kahi hōʻailona.

He aha nā hōʻailona o LVNC?

ʻO kēia ka mea nui loa. ʻAʻole paha e hōʻike kekahi poʻe i kekahi mau hōʻailona . Eia nō naʻe, hiki i kekahi poʻe ke loaʻa nā pilikia puʻuwai koʻikoʻi loa. No ka laʻana, hiki ke make koke ka puʻuwai. Ma kahi o ʻelua hapakolu o ka poʻe me LVNC e pilikia ana i ka loaʻa ʻana o ka hāʻule ʻana o ka puʻuwai .

ʻAe, e nānā kākou i ke ʻano o nā hōʻailona e hōʻike ai kekahi kanaka me (LVNC):

Nā hōʻailona i ʻike pinepine ʻia:

• Hiki ke hana ʻia nā ʻūpē koko . He mea weliweli loa kēia, no ka mea, inā hele kēia mau ʻūpē i ka lolo a i ʻole nā ​​māmā, hiki ke lilo i pilikia nui.
• Hiki paha iā ʻoe ke pilikia i ka hanu ʻana i kou wā e moe ana ma kahi moe.
• Ka mae ʻana .
• Nā palpitations o ka puʻuwai - Hiki i kēia ke manaʻo e like me ka pahu e kuʻi ana i loko o ka umauma, a i ʻole e like me ka holo ʻana o ka puʻuwai.
• ʻAʻole hiki ke hoʻoikaika kino, pōkole ka hanu ʻoiai me ka hoʻoikaika iki ʻana (Dyspnea) .
• ʻO ke ʻano puʻuwai kūpono ʻole (Arrhythmia) .
• Ka pehu ʻana o nā wāwae (Lymphedema) - ʻoi aku hoʻi ma nā kuʻekuʻe wāwae.
• Pōkole o ka hanu / Dyspnea - Manaʻo e like me ka pau ʻana o ka hanu ʻoiai me kahi hoʻoikaika iki.

No ke aha e kū mai ai kēia kūlana LVNC? He aha nā kumu?

ʻO ka hapa nui o ka manawa, ʻo kēia kūlana (LVNC) i hoʻokumu ʻia e kahi loli genetic (Gene mutation) i hana ʻia ma mua o kou hānau ʻana. He mea paʻakikī loa kēia. Hiki ke hana maʻamau kēia loli genetic, ʻo ia hoʻi me ke kumu ʻole, a i ʻole hiki ke hoʻoilina ʻia mai kou mau mākua mai. ʻO ia hoʻi, hiki ke lilo i mea e holo i loko o nā ʻohana. E noʻonoʻo wale, he nui ka nui o kēia ʻano maʻi puʻuwai i kekahi mau ʻohana.

Eia kekahi, he hapa liʻiliʻi loa o nā wahine e loaʻa paha iā LVNC no ka manawa mua i ka wā hāpai me ke kumu ʻole. Manaʻo nā kauka he kumu paha kēia o ke kaumaha keu ma ka ʻiʻo puʻuwai i ka wā hāpai. No kekahi mau wahine, nalowale kēia mau hōʻailona ma hope o ka hānau ʻana. Akā no kekahi poʻe, hiki i ka LVNC ke loaʻa nā hopena lōʻihi.

Pehea e ʻike ai nā kauka i kēia kūlana LVNC?

E hana kekahi kauka i kekahi mau hoʻokolohua e hoʻoholo pono ai inā loaʻa iā ʻoe kēia kūlana "(LVNC)". E nānā kākou i ke ʻano o ia mau mea:

• Echocardiogram: Nānā kēia i ke ʻano o ka puʻuwai e pamu ana i ke koko, pehea ka maikaʻi o ka hana ʻana o nā valves, a inā he pilikia me nā valves. Ua like ia me kahi scan ultrasound o ka puʻuwai. ʻO kēia kahi āu e ʻike mua ai i ke ʻano spongy o ka ventricle hema.
• Electrocardiogram (ECG): Ke ana kēia i ka hana uila o kou puʻuwai. Hiki iā ia ke ʻike i nā pilikia me ke kuʻina o kou puʻuwai.
• Nā hoʻāʻo kiʻi kūikawā: ʻO kēia mau mea e komo pū me nā scan CT cardiac a me ke kiʻi resonance magnetic (MRI). Hiki i kēia mau mea ke hōʻike i ke ʻano o ka puʻuwai, e like me ke ʻano o ka hana ʻana o ka puʻuwai a me ka mānoanoa o kona mau paia. Ma LVNC, ʻike maopopo ʻia ke ʻano e like me ka huʻa ma kēia mau scan.
• ʻO ka angiogram coronary: Hoʻāʻo kēia i ke kahe ʻana o ke koko ma o nā aʻa coronary e hoʻolako ana i ke koko i ka puʻuwai, a inā he mau mea e ālai ana. I kekahi manawa pono paha kēia hoʻāʻo e kāpae i nā maʻi puʻuwai ʻē aʻe.

He aha nā lāʻau lapaʻau no kēia kūlana LVNC?

ʻOkoʻa ka lāʻau no ka LVNC ma muli o kāu mau hōʻailona. ʻAʻole like ka mālama ʻia ʻana o nā kānaka a pau. No ka laʻana, inā loaʻa iā ʻoe ka puʻuwai palupalu, hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau e like me:

• Nā mea hoʻopaʻa enzyme hoʻololi-angiotensin (ACE inhibitors): No ka laʻana, benazepril (Lotensin®) a i ʻole lisinopril (Prinivil®, Zestril®) a i ʻole sacubitril-valsartan (Entresto®) (nā mea hoʻopaʻa angiotensin-neprilysin). Hana kēia mau mea ma ka hoʻomaha ʻana a me ka wehe ʻana i kou mau kīʻaha koko (nā aʻa koko a me nā aʻa) e maʻalahi ai ke kahe ʻana o ke koko ma o lākou. Hoʻemi kēia i ka ukana hana ma ka puʻuwai.
• Nā mea pale Beta: No ka laʻana, metoprolol (Lopressor®, Toprol XL®) a i ʻole atenolol (Tenormin®). Hoʻopaʻa kēia i kekahi mau hormones (e.g. adrenaline), hoʻohaʻahaʻa i ke koko a me ka kaohi ʻana i ka helu o ka puʻuwai.
• Digoxin (Digoxin®, Lanoxin®): Hoʻomalu kēia i ka helu o kou puʻuwai, hoʻonui iki i ka ikaika o ka pumping o kou puʻuwai, a hoʻomaikaʻi i ke kahe koko.
• Nā lāʻau diuretic a i ʻole "nā pila wai": Hana kēia ma ke kumu ʻana i ke kino e hoʻokuʻu i ka paʻakai a me ka wai keu ma o ka mimi. Hoʻemi kēia i ka pehu a hoʻemi i ka ukana ma ka puʻuwai.

I kekahi manawa, inā he pilikia o ka hopu koke ʻana o ka puʻuwai, hiki i ke kauka ke ʻōlelo aku e hoʻokomo i kahi mea i kapa ʻia ʻo Implantable Cardioverter Defibrillator (ICD) ma lalo o ka ʻili o ka puʻuwai. He mea liʻiliʻi kēia e hoʻohana ʻia ana e ka pila. ʻIke ia i kahi mele wikiwiki a hoʻoweliweli i ke ola i loko o ka puʻuwai a hāʻawi i kahi haʻalulu uila i ka puʻuwai e hoʻihoʻi i ke mele maʻamau. I nā hihia koʻikoʻi loa, inā ʻaʻole holomua nā lāʻau ʻē aʻe, pono paha kahi hoʻoili puʻuwai .

Hiki ke ho'ōla loa ʻia kēia maʻi LVNC? Hiki ke hoʻohuli ʻia?

ʻAuē. ʻAʻohe lāʻau lapaʻau no LVNC i kēia manawa . ʻO ia hoʻi, ʻaʻole hiki ke hoʻohuli ʻia. Akā, mai hopohopo. Hiki i ka lāʻau lapaʻau ke hoʻomaikaʻi i kou ʻano ola a hōʻemi i nā pilikia e hiki mai ana ma muli o ka maʻi puʻuwai . A, e like me kaʻu i ʻōlelo ai ma mua, i nā hihia koʻikoʻi loa, pono paha kahi hoʻoili puʻuwai.

Pehea mākou e pale aku ai i kēia kūlana LVNC?

ʻAʻohe ala e pale aku ai i ka LVNC. ʻO kēia no ka mea ua hoʻokumu pinepine ʻia e nā kumu genetic. Eia nō naʻe, inā loaʻa iā ʻoe ka mutation gene e hoʻoulu ai i ke kūlana, a inā manaʻo ʻoe he loaʻa paha iā ʻoe (no ka laʻana, inā loaʻa i kekahi o kou ʻohana ke kūlana), hiki ke kōkua nui ka hoʻāʻo genetic.E wehewehe kekahi kākā'ōlelo genetic i kāu mau hopena ho'āʻo iā ʻoe a me kou ʻohana. E kōkua lākou iā ʻoe e hoʻomaopopo i ke ʻano o ka loaʻa ʻana o LVNC a me kou pilikia o ka hoʻoili ʻana i kāu mau keiki.

Aia kekahi mau pilikia olakino ʻē aʻe e pili ana i kēia kūlana LVNC?

ʻAe. I kekahi manawa hiki ke ʻike ʻia kēia kūlana i kapa ʻia ʻo (LVNC) ma ke ʻano he ʻāpana o nā kūlana genetic ʻē aʻe (syndromes). He ʻoi aku ka paʻakikī o kēia mau mea, akā noʻonoʻo pū nā kauka i kēia mau kūlana i ka wā e ʻike ai i ka (LVNC). Eia kekahi mau laʻana:

• ʻO ka maʻi Barth
• ʻO ka maʻi Noonan
• ʻO ka maʻi Roifman
• ʻO ka maʻi Toriello Carey

He aha ka wā e hiki mai ana o kekahi me LVNC? He aha ka manaʻolana e ola ai?

Hiki ke ʻokoʻa ka nānā ʻana no kekahi me LVNC. He ʻokoʻa ia mai kēlā kanaka a kēia kanaka. Me ka mālama maikaʻi ʻana, hiki ke kaohi ʻia nā hōʻailona , ​​a ke ola nei kekahi poʻe i kahi ola maʻamau.

He maʻi make paha ka LVNC, akā, ʻaʻole ia he mea mau . ʻAʻole make ka hapa nui o ka poʻe me LVNC i ka maʻi. ʻO ka mea nui loa, ʻo ia ke ʻike pinepine i kahi kauka puʻuwai a hoʻāʻo ʻia . Hiki iā ia ke kōkua iā ʻoe e mālama i ke olakino o kou puʻuwai a hōʻemi i kou pilikia o nā pilikia.

Inā loaʻa iaʻu a i ʻole kaʻu keiki ka LVNC, he aha nā nīnau e pono ai iaʻu e nīnau aku i ke kauka?

Inā loaʻa iā ʻoe a i ʻole kāu keiki (LVNC), a inā he mau hopohopo kāu, he mea maikaʻi ke nīnau i kāu kauka i nā nīnau e like me kēia:

• He aha ke kumu kūpono loa o ka maʻi LVNC o kaʻu/kaʻu keiki? (ʻo ia hoʻi, he genetic a i ʻole kekahi mea ʻē aʻe)
• Ke paipai nei ʻoe i ka hoʻāʻo ʻana i ka genetic no LVNC? Inā pēlā, he mea nui anei ia no nā lālā ʻē aʻe o ka ʻohana?
• He aha nā hoʻokolohua e pono ai iaʻu/kaʻu keiki e loaʻa ai no ka ʻike ʻana i ka LVNC? He aha kaʻu e manaʻo ai mai ia mau mea?
• He aha nā koho lapaʻau no LVNC? ʻO wai ka lāʻau lapaʻau maikaʻi loa noʻu/kaʻu keiki? Aia kekahi mau hopena ʻaoʻao i kēia mau lāʻau lapaʻau?
• Inā loaʻa koke iaʻu a i ʻole kaʻu keiki kahi hopu puʻuwai, he aha nā hōʻailona? He aha kaʻu e hana koke ai?

E kōkua kēia mau nīnau iā ʻoe e kūkākūkā me kāu kauka a loaʻa kahi ʻike maikaʻi aʻe i kou kūlana.

No laila, he aha nā mea nui e pono ai kākou e hoʻomanaʻo mai kēia moʻolelo?

ʻO ka puʻuwai hana ʻole o ka ventricle hema (LVNC) he maʻi puʻuwai laha ʻole, a pinepine i hānau ʻia. ʻO kēia ka wā ʻaʻole ulu pono ka ventricle hema o ka puʻuwai. Ma kahi o ka lilo ʻana i ʻāpana ʻiʻo laumania a ikaika, lilo ia i mānoanoa a huʻahuʻa. ʻO kēia ke kumu o ka puʻuwai ʻaʻole e pamu pono i ke koko.

Inā loaʻa iā ʻoe ka (LVNC),ʻO ka mea nui loa, ʻo ia ke ʻike pinepine ʻana i kahi kauka puʻuwai a hahai i kā lākou ʻōlelo aʻo. ʻOiai ʻaʻohe lāʻau lapaʻau no kēia, hiki i ka lāʻau a kāu kauka e hāʻawi ai iā ʻoe ke hōʻemi i kou pilikia o nā pilikia a hoʻomaikaʻi i kou ʻano ola. He mea nui e pale aku i ka hopohopo a loaʻa ka lāʻau kūpono . E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi, a aia nā kauka e kōkua iā ʻoe.

ʻO ka maʻi puʻuwai, ventricle hema, LVNC, nā hōʻailona maʻi puʻuwai, ka hāʻule ʻana o ka puʻuwai, nā maʻi genetic, ka mālama ʻana i ka maʻi puʻuwai