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October 10, 2025
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ʻO ka transposition o nā arteries nui kahi kūlana pilikia o ka naʻau kahi i hoʻololi ʻia ai nā kīʻaha koko nui ʻelua e haʻalele ana i ka naʻau. ʻO ia hoʻi, ʻaʻole i hiki i ke koko momona i ka oxygen ke hiki pono i ke kino, a ʻaʻole i hiki i ke koko haʻahaʻa i ka oxygen ke hiki i nā māmā e like me ka mea e pono ai.
Hana ʻia kēia kūlana i ka wā hāpai ke hana nei ka naʻau o ke pēpē. Hoʻopili ia i kahi 1 i 4,000 mau pēpē i hānau ʻia i kēlā me kēia makahiki. ʻOiai ke kani nui nei kēia, loaʻa i ka lāʻau lapaʻau hou nā lapaʻau maikaʻi e kōkua ai i nā keiki me kēia kūlana e ola i nā ola piha a olakino.
I loko o ka naʻau maʻamau, lawe ʻia nā kīʻaha nui ʻelua i ke koko mai ka naʻau. Lawe ka aorta i ke koko momona i ka oxygen i ke kino, ʻoiai lawe ka pulmonary artery i ke koko haʻahaʻa i ka oxygen i nā māmā. I ka transposition o nā arteries nui, hoʻololi ʻia kēia mau kīʻaha ʻelua a i ʻole "transposed."
E noʻonoʻo iā ia e like me ʻelua mau alanui nui i hui pū ʻia kā lākou mau ala hele. Ke kahe nei ke koko, akā ke hele nei i nā wahi hewa. Ke pai ke ʻaoʻao ʻākau o ka naʻau i ke koko i ke kino ma kahi o nā māmā, ʻoiai ke pai ke ʻaoʻao hema i ke koko i nā māmā ma kahi o ke kino.
Hoʻokumu kēia i ʻelua mau pōʻaiapuni kahe koko kaʻawale ʻaʻole i pili pono. Me ka ʻole o ka lapaʻau, ʻaʻole hiki i nā pēpē ke loaʻa ka oxygen lawa i kā lākou mau kino a me nā pūnaewele. ʻO ka ʻoiaʻiʻo maikaʻi, hiki i nā kauka ke hoʻoponopono i kēia kūlana me ka ʻoki kino.
ʻElua mau ʻano nui o kēia kūlana, a ke hoʻomaopopo ʻana i ke ʻano o kāu pēpē e kōkua ai i nā kauka e hoʻolālā i ke ala lapaʻau maikaʻi loa.
Transposition maʻalahi (D-TGA): ʻO kēia ka ʻano maʻamau loa, kahi i hoʻololi ʻia ai nā arteries nui wale nō. Hana maʻamau nā keʻena pai ʻana o ka naʻau a me nā valves. Ma kahi o 70% o nā hihia, ʻo kēia ke ʻano.
Transposition paʻakikī (L-TGA): I kēia ʻano ʻoi aku ka liʻiliʻi, hoʻololi ʻia nā arteries nui a me nā keʻena haʻahaʻa o ka naʻau. Hoʻopili pinepine ʻia kēia ʻano me nā hemahema naʻau ʻē aʻe a ʻaʻole paha e hōʻike i nā hōʻailona no nā makahiki.
E hoʻohana kāu kauka i nā hoʻokolohua kūikawā e hoʻoholo ai i ke ʻano o kāu keiki. Kōkua kēia ʻike iā lākou e hana i ka papahana lapaʻau maikaʻi loa no kāu kūlana kūikawā.
Hōʻike ka hapa nui o nā pēpē me kēia kūlana i nā hōʻailona i loko o nā lā mua a i ʻole nā pule o ke ola. Hana nā hōʻailona no ka mea ʻaʻole lawa ka oxygen i kā lākou kino.
Eia nā hōʻailona āu e ʻike ai i kāu pēpē hou:
Hiki i kekahi mau pēpē ke ʻano maikaʻi i ka wā hānau akā e hoʻomohala i nā hōʻailona i nā lā mua ke pani nā pilina naʻau maʻamau. Inā ʻike ʻoe i kekahi o kēia mau hōʻailona, e kelepona koke i kāu kauka.
I nā hihia liʻiliʻi me ka transposition paʻakikī, ʻaʻole paha e ʻike ʻia nā hōʻailona a hiki i ka wā ma hope o ke kamaliʻi a i ʻole ke ola makua. Hiki i kēia mau keiki ke ʻike i ka luhi, ka pōkole o ka hanu i ka wā hana, a i ʻole nā pilikia o ka naʻau.
Hoʻomohala ʻia kēia kūlana i loko o nā hebedoma 8 mua o ka hāpai ke hana nei ka naʻau o kāu pēpē. ʻAʻole maopopo loa ke kumu pololei, akā hana ia ke ʻole nā arteries nui i kā lākou mau kūlana maʻamau.
I ka wā hoʻomohala naʻau maʻamau, hoʻomaka ka naʻau e like me kahi paipu maʻalahi e huli a māhele ʻia i nā keʻena. I kekahi manawa, ʻaʻole hana pololei kēia kaʻina hana paʻakikī, e alakaʻi ana i ka hoʻopili ʻia ʻana o nā arteries i nā keʻena hewa.
Hana ka hapa nui o nā hihia me ka ʻole o ke kumu kūikawā. ʻAʻole ia he mea āu i hana ai a i ʻole ʻaʻole i hana i ka wā hāpai. ʻAʻole hoʻoilina pinepine ʻia kēia kūlana, ʻoiai ke loaʻa ʻana o hoʻokahi keiki me ka hemahema naʻau e hoʻonui iki i ka manawa no nā hāpai e hiki mai ana.
ʻO kekahi mau mea e hoʻonui iki ai i ka pilikia, ʻo ia ka maʻi diabetes o ka makuahine, kekahi mau lāʻau lapaʻau i ka wā hāpai, a i ʻole ka makahiki makua nui. Eia naʻe, hānau ʻia ka hapa nui o nā pēpē me kēia kūlana i nā makuahine me ka ʻole o nā mea pilikia.
E kelepona koke i kāu kauka inā ʻike ʻoe i kekahi kala uliuli i ka ʻili, nā lehelehe, a i ʻole nā manamana lima o kāu pēpē hou. ʻO kēia kala uliuli, i kapa ʻia ʻo cyanosis, ʻo ia hoʻi ʻaʻole lawa ka oxygen o kāu pēpē a pono i ka mālama pilikia.
Pono nō hoʻi ʻoe e kelepona koke i kāu kauka inā pilikia kāu pēpē i ka hanu, hānai maikaʻi ʻole, a i ʻole ke ʻano luhi a huhū paha. Hiki i kēia mau hōʻailona ke hoʻomohala wikiwiki i nā pēpē hou me nā kūlana naʻau.
Inā ʻike ʻia kāu pēpē me kēia kūlana ma mua o ka hānau ʻana, e hōʻoia e hānau ʻoe ma kahi haukapila me kahi kikowaena naʻau pediatric. Hiki i ka loaʻa ʻana o nā loea mai ka manawa hānau ke hana i kahi ʻokoʻa nui i ka mālama ʻana i kāu pēpē.
No nā keiki nui me ka transposition paʻakikī, e nānā i nā hōʻailona e like me ka luhi maʻamau ʻole i ka wā pāʻani, ka pōkole o ka hanu, a i ʻole nā hoʻopiʻi o ka ʻeha umauma. Hiki i kēia mau hōʻailona ke hoʻomohala mālie a pono i ka loiloi lapaʻau.
ʻAʻole loaʻa i ka hapa nui o nā pēpē me kēia kūlana nā mea pilikia kūikawā, akā hiki i kekahi mau kūlana ke hoʻonui iki i nā manawa. Hiki i ka hoʻomaopopo ʻana i kēia mau mea ke kōkua i ka ʻike mua a me ka hoʻolālā mālama.
Eia nā mea e hoʻonui ai i ka pilikia:
E hoʻomanaʻo, ʻaʻole ʻo ka loaʻa ʻana o nā mea pilikia e ʻo ia hoʻi e loaʻa ana i kāu pēpē kēia kūlana. Hānau ʻia ka nui o nā pēpē me kēia mau mea pilikia me nā naʻau olakino loa. Ma ke ʻano ʻē, ʻaʻohe mea pilikia i ʻike ʻia i ka hapa nui o nā pēpē me ka transposition.
Me ka ʻole o ka lapaʻau, hiki i kēia kūlana ke alakaʻi i nā pilikia koʻikoʻi no ka mea ʻaʻole lawa ka oxygen i ke kino. Ke hoʻomaopopo nei i kēia mau pilikia e kōkua i ka wehewehe ʻana i ke kumu he mea nui loa ka lapaʻau koke.
ʻO nā pilikia koke loa:
Me ka lāʻau ʻoki hou, pale ka hapa nui o nā keiki i kēia mau pilikia koʻikoʻi. Eia naʻe, ʻoiai ma hope o ka ʻoki kino holomua, aia kekahi mau manaʻo lōʻihi. Hiki i kēia ke komo pū me ka pono no nā kaʻina hana hou, ka nānā ʻana i ka naʻau, a i ʻole nā palena hana.
ʻO ka ʻoiaʻiʻo maikaʻi, ulu ka hapa nui o nā keiki e loaʻa ana i ka lapaʻau kūpono e ola i nā ola maʻamau a hana nui. Kōkua ka nānā mau ʻana me kahi cardiologist pediatric e hopu a hoʻoponopono i nā pilikia i ka wā mua.
Hiki i nā kauka ke ʻike i kēia kūlana ma mua o ka hānau ʻana, ma hope koke o ka hānau ʻana, a i ʻole ma hope inā hoʻomohala mālie nā hōʻailona. Hoʻomaka pinepine ka kaʻina hana diagnosis ke ʻike kekahi i nā hōʻailona pilikia a i ʻole i ka wā nānā prenatal maʻamau.
Ma mua o ka hānau ʻana, hiki i kahi ultrasound i kapa ʻia ʻo fetal echocardiogram ke hōʻike i ke ʻano o ka naʻau. Hāʻawi kēia ultrasound kūikawā i nā kiʻi kikoʻī o ke ʻano o ke kahe koko ma ka naʻau o kāu pēpē. Inā ʻike ʻia ma mua o ka hānau ʻana, hiki i nā kauka ke hoʻolālā no ka mālama koke ma hope o ka hānau ʻana.
Ma hope o ka hānau ʻana, hoʻomaka pinepine ka diagnosis me kahi hoʻokolohua kino kahi e hoʻolohe ai ke kauka i ka naʻau a nānā i ka kala uliuli. Hiki iā lākou ke kauoha i kekahi mau hoʻokolohua e komo pū ana i kahi X-ray umauma, electrocardiogram (EKG), a me kahi echocardiogram e ʻike i ke ʻano a me ka hana o ka naʻau.
I kekahi manawa pono nā kauka i nā hoʻokolohua hou aku e like me ka cardiac catheterization, kahi e hoʻokomo ai lākou i kahi paipu lahilahi i nā kīʻaha koko e loaʻa ai nā kiʻi kikoʻī hou aku. Kōkua kēia mau hoʻokolohua i nā kauka e hoʻomaopopo pono i ke ʻano o ka naʻau o kāu keiki a hoʻolālā i ke ala lapaʻau maikaʻi loa.
ʻO ka lapaʻau no kēia kūlana e komo pinepine ana i ka ʻoki kino e hoʻopili hou i nā arteries i nā keʻena kūpono. ʻO ka ʻoiaʻiʻo maikaʻi, ua hoʻomaikaʻi nui nā ʻenehana ʻoki kino, a hana maikaʻi loa ka hapa nui o nā keiki ma hope o ka lapaʻau.
I nā lā mua o ke ola, pono paha nā kauka e hana i kahi kaʻina hana i kapa ʻia ʻo balloon atrial septostomy. Hoʻokumu kēia i kahi wehe manawa ma waena o nā keʻena kiʻekiʻe o ka naʻau, e ʻae ana i ke koko momona i ka oxygen a me ke koko haʻahaʻa i ka oxygen e hui maikaʻi a hiki ke hana ʻia ka ʻoki kino.
ʻO ka lapaʻau ʻoki kino nui ʻo ia ka arterial switch operation. Hoʻokaʻawale nā kauka i nā arteries nui a hoʻopili hou iā lākou i nā keʻena kūpono. Hoʻoneʻe lākou i nā arteries coronary, kahi e hoʻolako ai i ke koko i ka ʻiʻo naʻau ponoʻī. Hana pinepine ʻia kēia ʻoki kino i loko o nā pule mua o ke ola.
No nā hihia paʻakikī, hiki i nā kauka ke ʻōlelo i kahi ala ʻokoʻa i kapa ʻia ʻo ka Mustard a i ʻole Senning procedure, kahi e hoʻokele hou ai lākou i ke kahe koko me nā ʻāpana a i ʻole ka ʻiʻo naʻau ponoʻī. Ke hilinaʻi nei ke koho i ke ʻano kūikawā o kāu keiki a me nā mea ʻē aʻe.
Ma hope o ka ʻoki kino, pono ka hapa nui o nā keiki i ka mālama nānā mau me kahi cardiologist pediatric. Kōkua kēia e hōʻoia i ka hana mau ʻana o ka naʻau ke ulu a hoʻomohala kāu keiki.
ʻO ka mālama ʻana i kāu keiki ma ka home ma hope o ka ʻoki kino naʻau e pono ai ka nānā ʻana i kekahi mau wahi nui. E hāʻawi kāu hui lapaʻau iā ʻoe i nā ʻōlelo aʻoaʻo kūikawā, akā eia nā kuhikuhi maʻamau e kōkua ai i ka hoʻōla.
Mua, e nānā i ka meaʻai a me ka hānai. Hiki i kāu pēpē ke luhi maʻalahi i ka wā hānai, no laila e hāʻawi i nā meaʻai liʻiliʻi a pinepine. Inā paʻakikī ka hānai ʻana, mai kānalua e noi aku i ke kōkua mai kahi mea kūkākūkā lactation e hoʻomaopopo ana i nā kūlana naʻau.
E nānā i nā hōʻailona o ka maʻi a puni ka wahi ʻoki kino, e komo pū ana ka ʻulaʻula nui, ka pehu, a i ʻole ka hoʻokahe. E mālama i ka ʻoki maʻemaʻe a maloʻo e like me nā ʻōlelo a kāu hui ʻoki kino. Hiki i ka hapa nui o nā pēpē ke ʻauʻau mālie ke ʻae ke kauka.
E nānā i kāu keiki no kekahi mau hōʻailona pilikia e like me ka nui ʻana o ka kala uliuli, ka pilikia hanu, ka hānai maikaʻi ʻole, a i ʻole ka huhū maʻamau ʻole. Ke loaʻa iā ʻoe kahi papa inoa kākau o ka wā e kelepona ai i ke kauka e kōkua iā ʻoe e manaʻo hilinaʻi hou aku e pili ana i ka mālama ʻana i kāu keiki.
Ke ulu kāu keiki, pono paha lākou i kekahi mau palena hana i ka wā mua, akā hiki i ka hapa nui o nā keiki ke komo i nā hana kamaliʻi maʻamau. E alakaʻi kāu cardiologist iā ʻoe i ka mea kūpono i kēlā me kēia pae o ka hoʻōla.
ʻO ka hoʻomākaukau ʻana no nā hālāwai me ka hui naʻau o kāu keiki e kōkua iā ʻoe e hana i ka hapa nui o kāu manawa pū a hōʻoia iā ʻoe e loaʻa nā pane i kāu mau nīnau nui loa.
Ma mua o kēlā me kēia kipa, e kākau i nā hōʻailona āu i ʻike ai, nā nīnau e pili ana i ka mālama ʻana i kāu keiki, a me nā hopohopo e pili ana i ka hoʻomohala ʻana a i ʻole nā hana. E lawe mai i kahi papa inoa o nā lāʻau a pau āu keiki e lawe nei, e komo pū ana nā dosages a me ka manawa.
E mālama i ka moʻolelo o ka ulu ʻana o kāu keiki, nā ʻano hānai, a me nā pae hana ma waena o nā kipa. Kōkua kēia ʻike i nā kauka e hoʻomaopopo i ka maikaʻi o kāu keiki. Hiki ke kōkua nā kiʻi a i ʻole nā wikiō o nā hōʻailona pilikia.
E lawe mai i kahi lālā ʻohana a hoaaloha paha i nā hālāwai nui. Ke kōkua nei ke kākoʻo iā ʻoe e hoʻomaopopo i ka ʻike a hoʻomanaʻo i nā kikoʻī nui. Mai kānalua e noi i nā kauka e wehewehe i nā mea ma nā ʻano ʻē aʻe inā ʻaʻole maopopo kekahi mea.
E hoʻomākaukau i kāu keiki nui no nā hālāwai ma ka wehewehe ʻana i ka mea e hana ʻia ma nā huaʻōlelo kūpono i ka makahiki. Hiki i ka lawe ʻana mai i nā mea hōʻoluʻolu e like me kahi pāʻani punahele ke kōkua i ka hoʻemi ʻana i ka hopohopo no ʻoukou ʻelua.
ʻO ka mea nui loa e hoʻomanaʻo ai, ʻo ia kahi kūlana koʻikoʻi ka transposition o nā arteries nui e pono ai ka mālama lapaʻau koke, akā hiki ke mālama ʻia me nā hopena lōʻihi maikaʻi loa.
Me nā ʻenehana ʻoki kino hou, ulu ka hapa nui o nā keiki e loaʻa ana i ka lapaʻau kūpono e ola i nā ola piha a hana nui. Hiki iā lākou ke komo i nā haʻuki, hele i ke kula maʻamau, a hahai i kā lākou mau moe e like me nā keiki ʻē aʻe.
ʻO ke kī i ka holomua, ʻo ia ka ʻike mua a me ka lapaʻau e nā loea naʻau pediatric akamai. Inā hopohopo ʻoe e pili ana i nā hōʻailona o kāu keiki, e hilinaʻi i kāu mau manaʻo a e ʻimi koke i ka mālama lapaʻau.
E hoʻomanaʻo ʻaʻole ʻoe wale nō i kēia huakaʻi. Loaʻa i nā hui naʻau pediatric nā kauka, nā nēnē, nā limahana hanauna, a me nā loea ʻē aʻe e hoʻomaopopo ana i nā ʻaoʻao lapaʻau a me ka manaʻo o ka mālama ʻana i nā keiki me nā kūlana naʻau.
ʻAe, ola ka hapa nui o nā keiki e loaʻa ana i ka ʻoki kino holomua no ka transposition i nā ola maʻamau loa. Hiki iā lākou ke hele i ke kula, pāʻani haʻuki, a komo i nā hana kamaliʻi maʻamau. Pono paha kekahi i ka nānā mau ʻana e kahi cardiologist, akā ʻaʻole pinepine kēia e kaupalena i kā lākou mau hana i kēlā me kēia lā. ulu ka nui o nā mākua i loaʻa kēia ʻoki kino i nā pēpē e loaʻa i kā lākou mau ʻohana ponoʻī a hahai i kekahi ʻoihana ā lākou e koho ai.
ʻAʻole pono ka hapa nui o nā keiki e loaʻa ana i ka arterial switch operation i nā ʻoki kino naʻau hou aku. Eia naʻe, pono paha kekahi i nā kaʻina hana liʻiliʻi inā hoʻomohala nā pilikia me nā arteries coronary a i ʻole nā valves naʻau ma hope o kekahi manawa. E nānā mau kāu cardiologist i ka naʻau o kāu keiki a e haʻi aku iā ʻoe inā pono kekahi mau lapaʻau hou aku. ʻO ka pono no ka ʻoki kino hou e ʻokoʻa loa mai ke keiki a ke keiki.
ʻAʻole, ʻaʻohe mea āu e hiki ke hana ʻokoʻa e pale aku ai i kēia kūlana. Hana ʻia ka Transposition o nā arteries nui i ka wā hoʻomohala naʻau mua a ʻaʻole i hoʻokumu ʻia e kekahi mea a nā mākua e hana ai a i ʻole ʻaʻole e hana. Hiki i nā makuahine e hahai ana i nā kuhikuhi hāpai āpau ke loaʻa nā pēpē me kēia kūlana. He hoʻololi hoʻomohala kūpono ʻole ia e hana ʻia ma kahi o 1 i 4,000 mau hānau.
Ke hilinaʻi nei ka manawa i ke kūlana kūikawā o kāu pēpē a me nā hōʻailona. Pono kekahi mau pēpē i ka ʻoki kino i loko o nā lā mua a i ʻole nā pule o ke ola, ʻoiai hiki i kekahi ke kūpaʻa lawa e kali iki. E nānā pono kāu hui naʻau pediatric i kāu pēpē a e ʻōlelo aku i ka manawa kūpono loa no ka ʻoki kino ma muli o kā lākou mau pono pilikino a me ko lākou olakino holoʻokoʻa.
Inā loaʻa iā ʻoe hoʻokahi keiki me ka transposition o nā arteries nui, ʻoi aku ka nui o ka manawa o ka loaʻa ʻana o ke keiki ʻē aʻe me kekahi ʻano o ka hemahema naʻau ma mua o ka awelika, akā emi loa ia ma kahi o 2-3%. ulu ka nui o nā ʻohana e loaʻa nā keiki hou aku me nā naʻau maʻamau loa. Hiki i kāu kauka ke kūkākūkā i ka ʻōlelo aʻo genetic inā makemake ʻoe i ka ʻike kikoʻī hou aku e pili ana i nā pilikia no nā hāpai e hiki mai ana.
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