Ke uliuli nei kāu keiki hānau hou? Ke pilikia nei ʻo ia i ka hānai waiū? Ke pilikia nei ʻo ia i ka hanu ʻana? I kekahi manawa, he mau hōʻailona kēia o kahi maʻi puʻuwai hānau. He mea maʻamau no ka makuahine a makua paha ke makaʻu loa i ka wā e ʻike ai lākou i kahi mea e like me kēia. I kēia lā, ke kamaʻilio nei mākou e pili ana i kahi maʻi puʻuwai kakaʻikahi loa, akā he mea nui loa e makaʻala. ʻO ia kahi maʻi i kapa ʻia ʻo Double Inlet Left Ventricle.
He aha ka Double Inlet Left Ventricle (DILV)?
I ka ʻōlelo maʻalahi, he kīnā puʻuwai hānau kakaikahi kēia i loaʻa i ka wā hānau. No ka hoʻomaopopo ʻana i kēia, e nānā mua kākou i ke ʻano o ka hana ʻana o kahi puʻuwai olakino.
E noʻonoʻo ʻoe ua like ko kākou naʻau me kahi hale me ʻehā lumi. ʻElua lumi ma luna a ʻelua lumi ma lalo.
- ʻO nā keʻena ʻelua o luna (Atria): ʻO kēia kahi e komo mai ai ke koko mai ke kino a hōʻiliʻili. Loaʻa i ke keʻena ʻākau (Atrium ʻĀkau) ke koko "lepo" i hoʻohana ʻia e ke kino a haʻahaʻa i ka oxygen. Loaʻa i ke keʻena hema (Atrium Hema) ke koko "maʻemaʻe" i piha i ka oxygen mai nā māmā.
- ʻO nā keʻena haʻahaʻa ʻelua (ventricles): ʻO kēia nā pamu nui ʻelua i loko o ka puʻuwai. Hoʻopuka ka ventricle ʻākau i ke koko nele i ka oxygen i nā māmā e kiʻi i ka oxygen. Hoʻopuka ka ventricle hema i ke koko waiwai i ka oxygen mai nā māmā a i ke koena o ke kino.
I kēia manawa, e nānā i ka mea e hana ana i loko o ka puʻuwai o kahi pēpē me ka Double Inlet Left Ventricle (DILV). I loko o kēia mau pēpē, hoʻokahi wale nō keʻena haʻahaʻa (ventricle) e hana maikaʻi ana. ʻO ia ka ventricle hema . ʻO ka ventricle ʻākau pinepine he liʻiliʻi loa ia e hana.
ʻO ka pilikia nui loa, ʻo ia ke koko mai nā keʻena ʻelua o luna (ʻākau a me ka hema o ka atria) e hui pū ana i loko o ke keʻena haʻahaʻa wale nō e hana ana (Left Ventricle). ʻO ia hoʻi, ua hui pū ke koko nele i ka oxygen a me ke koko waiwai i ka oxygen . A laila, ua hoʻokuʻu ʻia kēia koko i hui pū ʻia i ke kino a me nā māmā o ka pēpē. ʻO ia ke kumu e nui ai nā pilikia.
He aha nā hōʻailona o kēia maʻi?
ʻIke pinepine ʻia kēia mau hōʻailona i loko o kekahi mau lā a i ʻole mau pule ma hope o ka hānau ʻana o ke pēpē. Ma ke ʻano he makuahine, he mea nui loa iā ʻoe ke makaʻala i kēia mau mea.
| Hōʻailona | He wehewehe maʻalahi |
|---|---|
| Polū o ka ʻili a me nā lehelehe (Cyanosis) | ʻIke ʻia ka ʻili, nā lehelehe, a me nā kui o ka pēpē he uliuli ma muli o ke koko nele i ka oxygen e kaʻapuni ana ma ke kino holoʻokoʻa. ʻO kēia kekahi o nā hōʻailona nui. Ua kapa pū kekahi poʻe iā ia he "pēpē uliuli." |
| Ka hānai maikaʻi ʻole | Ke omo ka pēpē i ka waiū, luhi ʻo ia a paʻakikī ke hanu. No laila, inu iki ʻo ia a hoʻōki. |
| Paʻakikī ka hanu ʻana | Ke hanu wikiwiki nei ke pēpē. Me he mea lā e komo ana i loko o ka umauma. |
| ʻAʻole e piʻi ke kaumaha | ʻAʻole e piʻi pono ke kaumaha o ke pēpē no ka mea, ʻaʻole hiki iā ia ke loaʻa ka ikehu lawa mai ka inu ʻana i ka waiū lawa. |
| Ka hou ʻana | ʻOiai hoʻi i ka wā e inu ana i ka waiū, hou ʻoe a puni kou lae no ka mea pono e hana ikaika kou puʻuwai. |
| Puʻuwai puʻuwai maʻamau ʻole | Hiki i ka puʻuwai ke kuʻi wikiwiki a i ʻole he ʻano kuʻuna ʻole. Hiki i ke kauka ke lohe i kahi leo keu (heart murmur) i ka wā o ka hoʻokolokolo ʻana. |
No ke aha lā kēia e hana nei? He aha ke kumu o kēia?
ʻAʻole ʻike ʻia ke kumu kikoʻī o kēia . Hana pinepine ia i nā pule mua o ka hāpai ʻana, i ka wā e ulu ana ka puʻuwai o ka pēpē. ʻAʻole ia he hewa o ka makuahine a i ʻole ka makuakāne.
I ka manawa pinepine, hele pū ʻia ʻo DILV me kekahi mau pilikia puʻuwai ʻē aʻe, kahi e hoʻopilikia hou aku ai i ke kahe o ke koko.
- ʻO ka coarctation aortic: Ka hoʻohaiki ʻana o ke kīʻaha koko nui (aorta) e lawe ana i ke koko mai ka puʻuwai a i ke kino.
- ʻO ka atresia pulmonary: ʻAʻole i hoʻokumu pono ʻia ka valve pulmonary e lawe ana i ke koko i nā māmā.
- ʻO ka stenosis valve pulmonary: Ka hoʻohaiki ʻana o ka valve e lawe ana i ke koko i nā māmā.
Pehea e ʻike ai nā kauka i kēia maʻi?
Aia kekahi mau ala e ʻike ai i kēia maʻi.
I ka wā hāpai
I kekahi manawa hiki ke ʻike ʻia kēia ʻoiai ke pēpē i loko o ka ʻōpū. Inā ʻike kāu kauka i kekahi mau ʻano ʻē i loko o ka puʻuwai i kāu scan maʻamau (ultrasound), e kuhikuhi ʻo ia iā ʻoe i kahi scan kūikawā. Ua kapa ʻia ʻo ia he Fetal Echocardiogram . Hiki i kēia ke hāʻawi i kahi nānā kikoʻī i ke ʻano a me ka hana o ka puʻuwai o ka pēpē.
Ma hope o ka hānau ʻana o ke pēpē
I ka manawa pinepine, ʻike ʻia ka maʻi ma hope o ka hānau ʻana o ke pēpē.
- Hoʻāʻo Pulse Oximetry: Hana ʻia kēia hoʻāʻo i nā pēpē hānau hou ma aneane nā haukapila āpau ma Sri Lanka. Ana kēia i ka pae oxygen i loko o ke koko o ka pēpē. Inā haʻahaʻa ka pae oxygen, he hōʻailona paha ia o kahi maʻi puʻuwai.
- Ka hoʻokolokolo lapaʻau: Inā lohe ke kauka i kahi leo ʻē (murmur) i ka wā e hoʻolohe ana i ka puʻuwai o ka pēpē, a inā he uliuli ka ʻili, kānalua paha ʻo ia.
A laila, hana ʻia kekahi mau hoʻokolohua hou e hōʻoia i ka maʻi.
| Hoʻāʻo | He aha kāu e hana ai me kēia? |
|---|---|
| X-ray umauma | He kiʻi X-ray o ka umauma. Hiki i kēia ke hāʻawi i kahi manaʻo o ka nui o ka puʻuwai a me ka nui o ke koko e kahe ana i nā māmā. |
| ʻElectrocardiogram (ECG) | He hoʻāʻo e ana i ka hana uila o ka puʻuwai. Hiki iā ia ke ʻike i nā pilikia me ka ʻōʻō o ka puʻuwai. |
| ʻEchocardiogram (Echo) | ʻO kēia ka hoʻāʻo koʻikoʻi loa. Ua like ia me kahi scan. Hiki iā ia ke ʻike maopopo i nā keʻena o ka puʻuwai, nā valves, a me ke ʻano o ke kahe ʻana o ke koko. |
| Ka hoʻokomo ʻana o ka catheter cardiac | He hoʻāʻo paʻakikī e ana i ke kaomi a me nā pae oxygen i loko o ka puʻuwai ma ke kaomi ʻana i kahi ʻōmole lahilahi loa ma o kahi kīʻaha koko ma ka ʻūhā o ke pēpē. |
He aha nā lāʻau lapaʻau no kēia?
ʻAʻole hiki ke ho'ōla loa ʻia kēia maʻi me ka lāʻau lapaʻau. ʻO ka lāʻau lapaʻau nui he mau ʻoki kino.ʻAʻole kēia e hana i ʻelua keʻena i loko o ka puʻuwai o ka pēpē. Akā, hoʻohana ia i ka ventricle hoʻokahi e kū nei e hoʻokahe i ke koko ma ke kino me ka maikaʻi loa.
ʻAʻole kēia he ʻoki kino hoʻokahi manawa. Hana ʻia kēia mau ʻoki kino ma nā pae he nui, ma muli o ka makahiki o ke pēpē.
| Nā pae o ke ʻoki kino | Makahiki (kokoke) | He aha ka mea e hana ʻia ma hope o ke ʻoki kino? |
|---|---|---|
| Kahua mua: Blalock-Taussig (BT) Shunt | I loko o nā lā mua a i ʻole nā hebedoma ma hope o ka hānau ʻana | Inā ʻaʻole lawa ke kahe o ke koko i nā māmā, pili kēia ʻoki kino i ka hoʻokomo ʻana i kahi ʻōmole liʻiliʻi (shunt) e ʻae i ke kahe koko kūpono i nā māmā. |
| Ka lua o ka pae: Ke Kaʻina Hana ʻo Glenn Shunt | 4 - 6 mahina | ʻO ke koko nele i ka oxygen e hele mai ana mai ke kino o luna o ke pēpē e kuhikuhi pololei ʻia i nā māmā, e kāpae ana i ka puʻuwai. Hoʻemi kēia i ka ukana hana ma ka puʻuwai. |
| Kahua ʻEkolu: Kaʻina Hana Fontan | 2 - 3 mau makahiki | ʻO kēia ke ʻoki hope loa. Hoʻopili pololei ʻia ke koko deoxygenated mai ke kino haʻahaʻa i nā māmā. Ma hope o kēia ʻoki kino, ua aneane pau loa ka hui ʻana o ke koko deoxygenated a me ke koko oxygenated. |
Ma waho aʻe o ke ʻoki kino, hiki i ke kauka ke kuhikuhi i kekahi mau lāʻau lapaʻau no ka pēpē.
- Digoxin: Hoʻoikaika i ka ʻūlū ʻana o ka puʻuwai.
- Nā mea hoʻopaʻa ACE: Hoʻohaʻahaʻa i ke koko.
- Nā Anticoagulants: Kāohi i ke kāpili ʻana o ke koko.
- Diuretics: Wehe i ka wai pono ʻole mai ke kino.
I nā hihia koʻikoʻi loa, inā ʻaʻole hiki kēia mau ʻokiʻoki, hiki ke noʻonoʻo ʻia kahi hoʻoili puʻuwai .
He aha ka mea e hiki mai ana? Hiki iā kākou ke loaʻa ka manaʻolana?
He mea maopopo ke makaʻu i ka lohe ʻana i kahi maʻi e like me kēia. Akā ʻo ka mea nui e pono ai ʻoe e ʻike, ʻo ia me ka holomua o ka ʻepekema lapaʻau, loaʻa i kēia mau pēpē ka manawa kūpono e ola ai i kahi ola maikaʻi loa . ʻO ka nui o ke ola ʻana he 10 mau makahiki ma hope o ke ʻoki ʻana he 70% - 80%. He nui nā keiki e hiki i ka wā makua.
Akā, pono kēia mau keiki i ka nānā ʻana no ke ola holoʻokoʻa e ke kauka puʻuwai . He mea nui e hele i nā keʻena lapaʻau a hana i nā hoʻokolohua i ka manawa kūpono. Hiki ke loaʻa kekahi mau pilikia.
- Nā kui kuʻekuʻe wāwae i ulu i loko a me nā manamana lima i hoʻonui ʻia (clubbing).
- Nā maʻi pinepine o ka māmā e like me ka maʻi pneumonia.
- Nā pilikia o ka puʻuwai.
Eia kekahi, hiki i kēia mau keiki ke loaʻa kekahi mau palena i ka holo ʻana, ka lele ʻana, a me ka pāʻani ʻana e like me nā keiki ʻē aʻe. Pono e kūkākūkā ʻia kēia mau mea a pau a hoʻoholo ʻia me kāu kauka.
Leka Lawe-Home
- He maʻi puʻuwai hānau hānau kaʻawale, koʻikoʻi, akā hiki ke mālama ʻia.
- Inā polū ka ʻili o kahi pēpē hānau hou (cyanosis), paʻakikī i ka hānai ʻana, a paʻakikī paha i ka hanu ʻana, mai hoʻokaʻulua a lawe koke iā ia i ke kauka.
- ʻO ka lāʻau lapaʻau e pili ana i kahi moʻo o nā ʻokiʻoki paʻakikī i hana ʻia i kekahi mau pae ma muli o ka makahiki o ke pēpē.
- ʻOiai he mea paʻakikī kēia huakaʻi, mahalo i ka holomua o ka ʻepekema lapaʻau, hiki i ka nui o kēia mau keiki ke ola a hiki i ka wā makua.
- He mea nui loa ka mālama pono ʻana a me ke aʻo ʻana e ke kauka naʻau i ke ola holoʻokoʻa.
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