

Health Library
October 10, 2025
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ʻO ka double outlet right ventricle (DORV) kahi hemahema puʻuwai hānau maʻamau ʻole kahi e pili ana nā kīʻaha koko nui ʻelua e haʻalele ana i ka puʻuwai i ka ventricle ʻākau ma mua o ka pili ʻana o kekahi i kēlā me kēia ventricle. ʻO ia hoʻi, ke pāpū nei ka puʻuwai o kāu pēpē i ke koko ma ke ʻano ʻokoʻa ma mua o ka mea e pono ai, hiki ke hoʻopilikia i ke ʻano o ka holo ʻana o ke koko momona i ka oxygen ma o ko lākou kino.
Hana ʻia kēia kūlana i ka wā hāpai mua ke hoʻokumu ʻia nei ka puʻuwai o kāu pēpē. ʻOiai ke kani nui nei, hiki i nā keiki he nui me DORV ke ola i ke ola olakino, ikaika me ka mālama lapaʻau kūpono a me ka mālama.
I loko o ka puʻuwai olakino, pili ka aorta i ka ventricle hema a pili ka pulmonary artery i ka ventricle ʻākau. Me DORV, puka mai nā kīʻaha koko nui ʻelua mai ka ventricle ʻākau.
E noʻonoʻo i kou puʻuwai e like me ka loaʻa ʻana o ʻelua keʻena pāpū nui. Hoʻouna maʻamau ka ʻaoʻao ʻākau i ke koko i nā māmā e kiʻi i ka oxygen, ʻoiai ke hoʻouna nei ka ʻaoʻao hema i ke koko momona i ka oxygen i ke koena o kou kino. Ke pili nā kīʻaha ʻelua i ka ventricle ʻākau, ua haki ʻia kēia ʻano kahe maʻamau.
Hoʻopilikia ka kūlana i ka maikaʻi o ke koko oxygenated i ka kino a me ka lolo o kāu pēpē. Aia ka nui o ka pilikia ma nā hemahema puʻuwai ʻē aʻe e loaʻa pinepine pū me DORV, e like me nā puka ma waena o nā keʻena puʻuwai.
Loaʻa iā DORV kekahi mau ʻano like ʻole, a hoʻokau nā kauka iā lākou ma muli o kahi o kahi puka i kapa ʻia ʻo ventricular septal defect (VSD). Kūleʻa kēia puka i ke kahe koko i nā pēpē me DORV.
ʻO nā ʻano nui e komo pū ana ʻo subaortic DORV, kahi e noho ai ka puka ma lalo o ka aorta, a me subpulmonary DORV, kahi e noho ai ma lalo o ka pulmonary artery. Aia nō hoʻi ka doubly committed DORV, kahi e pili ai ka puka i nā kīʻaha ʻelua, a me ka non-committed DORV, kahi ʻaʻole kūpono ka puka me kekahi kīʻaha.
Hoʻokoʻokoʻa ka hopena o kēlā me kēia ʻano ma ke kahe koko a pono nā ʻano mālama like ʻole. E hoʻoholo kāu kauka naʻau kamaliʻi i ke ʻano o kāu pēpē ma o nā kiʻi kikoʻī a me nā hoʻokolohua.
ʻIke pinepine ʻia nā hōʻailona o ka DORV i loko o nā pule mua a i ʻole nā mahina o ke ola, ʻoiai hiki ke ʻokoʻa nui ma muli o ka nui o ka hopena o ke kūlana ma ke kahe koko. Hōʻike kekahi mau pēpē i nā hōʻailona koke, ʻoiai ʻaʻole paha e loaʻa i nā mea ʻē aʻe nā hōʻailona maopopo a hiki i ka hope.
ʻO nā hōʻailona maʻamau āu e ʻike ai:
Hiki i kekahi mau pēpē ke loaʻa nā hōʻailona koʻikoʻi e like me nā maʻi hanu pinepine a i ʻole nā hanana kahi e lilo koke ai lākou i uliuli a pilikia. Pono kēia mau hanana, i kapa ʻia ʻo nā hypercyanotic spells, i ka mālama ʻana i ka lāʻau lapaʻau koke.
I nā hihia kakaikahi, hiki i nā pēpē me kekahi mau ʻano o ka DORV ke ʻike mua ʻia he ʻulaʻula a olakino, akā e hoʻomohala hou ana i nā hōʻailona o ka hāʻule o ka naʻau i ko lākou ulu ʻana. Hiki ke komo pū me ka pehu ʻana o ko lākou mau wāwae, ʻōpū, a i ʻole a puni ko lākou mau maka.
Hoʻomohala ʻia ka DORV i loko o nā hebedoma ʻewalu mua o ka hāpai ʻana i ka wā e hoʻokumu ai i ka naʻau o kāu pēpē. ʻAʻole maopopo loa ke kumu, akā ʻike ʻia ia e hana inā ʻaʻole huli pono nā arteries nui i ka wā e ulu ai ka naʻau.
Hana pinepine ʻia ka hapa nui o nā hihia me ka ʻole o kekahi mea hoʻonāukiuki kikoʻī a i ʻole ka hana o nā mākua i hoʻokumu ai. ʻO ia hoʻi, ʻaʻohe mea āu i hana a i ʻole ʻaʻole i hana i ka wā hāpai e alakaʻi ai i kēia kūlana.
Eia naʻe, aia kekahi mau kumu e hiki ai ke hoʻonui iki i ka pilikia. ʻO kēia mau mea, ʻo ia ka loaʻa ʻana o ka maʻi suka i ka wā hāpai, ka lawe ʻana i kekahi mau lāʻau lapaʻau, a i ʻole ka loaʻa ʻana o ka mōʻaukala ʻohana o nā hemahema naʻau i hānau ʻia. Hoʻopili pū ʻia kekahi mau kūlana genetic e like me ka DiGeorge syndrome a i ʻole ka Down syndrome me nā kiʻekiʻe kiʻekiʻe o DORV.
I nā hihia kakaikahi, ʻo ka hōʻike ʻia ʻana i kekahi mau maʻi i ka wā hāpai mua a i ʻole nā kumu kūlohelohe paha e pāʻani i kahi kuleana. Akā, hou, ʻo ka hapa nui o nā hihia e hana me ka ʻole o ke kumu i ʻike ʻia, kahi e hiki ai ke manaʻo huhū akā he mea maʻamau loa.
ʻOiai ʻo ka hapa nui o nā hihia o DORV e hana kūpono, hiki i kekahi mau kumu ke hoʻolilo iā ia i mea maʻalahi e hoʻomohala. Hiki ke kōkua ka hoʻomaopopo ʻana i kēia mau kumu pilikia, ʻoiai he mea nui e hoʻomanaʻo ʻaʻole ʻo ka loaʻa ʻana o nā kumu pilikia e hōʻoia ʻia ai ka DORV.
ʻO nā kumu makuahine e hiki ke hoʻonui i ka pilikia:
Hiki i nā kumu genetic ke pāʻani i kahi kuleana i kekahi mau hihia. Loaʻa i nā pēpē me kekahi mau kūlana chromosomal e like me ka Down syndrome, DiGeorge syndrome, a i ʻole 22q11.2 deletion syndrome nā kiʻekiʻe kiʻekiʻe o DORV.
ʻO nā hōʻike kūlohelohe i ka wā hāpai mua, e like me kekahi mau maʻi a i ʻole nā kemika, hiki ke hoʻopili i nā hihia kakaikahi. Eia naʻe, ʻo ka hapa nui o nā pēpē me DORV i hānau ʻia i nā mākua me ka ʻole o nā kumu pilikia i ʻike ʻia.
Pono ʻoe e hoʻokaʻaʻike koke i kāu pediatrician inā hōʻike kāu pēpē i nā hōʻailona o nā pilikia naʻau, ʻoi aku ka uliuli o ka ʻili, nā lehelehe, a i ʻole nā manikū. ʻO kēia ʻano uliuli, i kapa ʻia ʻo cyanosis, e hōʻike ana ʻaʻole lawa ka oxygen o kāu pēpē.
ʻO nā hōʻailona wikiwiki ʻē aʻe e komo pū ana ka pilikia nui o ka hanu, ka hiki ʻole ke hānai pono, a i ʻole nā manawa e lilo koke ai kāu pēpē i uliuli a pilikia. Pono kēia mau kūlana i ka mālama pilikia koke.
Pono nō hoʻi ʻoe e hele i kāu kauka inā manaʻo ʻia kāu pēpē i luhi loa i ka wā e hānai ana, ʻaʻole e loaʻa ka kaupaona kūpono, a i ʻole he mau maʻi hanu pinepine. ʻOiai hiki i kēia mau hōʻailona ke loaʻa nā kumu ʻē aʻe, pono lākou i ka loiloi ke loaʻa pū lākou.
Inā ua ʻike mua ʻia kāu pēpē me DORV, he mea nui nā hālāwai kūkākūkā maʻamau me kahi cardiologist kamaliʻi. E leka uila iā lākou ma waena o nā kipa inā ʻike ʻoe i kekahi loli i nā hōʻailona o kāu pēpē a i ʻole nā hōʻailona hou e hopohopo ai.
Me ka ʻole o ka mālama ʻana, hiki i ka DORV ke alakaʻi i nā pilikia koʻikoʻi he nui e hoʻopilikia i ke olakino a me ka ulu ʻana o kāu keiki. ʻO ka ʻoiaʻiʻo maikaʻi, me ka mālama lapaʻau kūpono, hiki ke pale ʻia a mālama ʻia paha nā pilikia he nui.
ʻO nā pilikia maʻamau e komo pū ana:
Hiki i kekahi mau keiki ke hoʻomohala i ka mea a nā kauka i kapa ai ʻo ka syndrome Eisenmenger, kahi kūlana maʻamau ʻole akā koʻikoʻi kahi e hoʻololi ai nā kaomi kiʻekiʻe o nā māmā i nā loli mau. Hana pinepine ʻia kēia i nā hihia i mālama ʻole ʻia a i ʻole nā mea i ʻike ʻia ma hope loa.
ʻO nā pilikia kikoʻī e hiki ai i kāu keiki ke kū pono i ke ʻano o ka DORV a lākou i loaʻa ai a me nā hemahema naʻau pili. E nānā kāu cardiologist no kēia mau pilikia a e hana e pale aku iā lākou ma o ka manawa mālama kūpono.
Hoʻomaka pinepine ka kānalua ʻana i ka DORV i ka lohe ʻana o ke kauka i kahi murmur o ka naʻau i ka wā o kahi hōʻoia maʻamau a i ʻole ka ʻike ʻana i nā hōʻailona e like me ka uliuli ʻana o ka ʻili. A laila, hōʻoia ʻia ka hōʻoia ma o kekahi mau hoʻokolohua naʻau kūikawā.
ʻO ka echocardiogram ka hoʻokolohua kikoʻī mua loa e hana ʻia. Hōʻike kēia ultrasound o ka naʻau i ke ʻano a me ka hana ma ka manawa maoli, e ʻae ana i nā kauka e ʻike pehea ka holo ʻana o ke koko ma o nā keʻena naʻau a me nā kīʻaha o kāu pēpē.
I kekahi manawa, pono nā hoʻokolohua hou aʻe no kahi kiʻi piha. Hiki paha kēia ke komo i kahi catheterization naʻau, kahi e hoʻokomo ʻia ai kahi tube lahilahi i nā kīʻaha koko e ana i nā kaomi a me nā pae oxygen pololei, a i ʻole nā kiʻi kiʻi holomua e like me ka MRI naʻau.
I kekahi mau hihia, hiki ke ʻike ʻia ka DORV ma mua o ka hānau ʻana i ka wā o nā ultrasounds hāpai maʻamau. Inā kānalua ʻia ma mua o ka hānau ʻana, e kuhikuhi ʻia paha ʻoe i kahi loea lapaʻau makuahine-pēpē a me ka cardiologist pediatric no ka loiloi kikoʻī a me ka hoʻolālā hānau.
ʻO ka lapaʻau no ka DORV ʻaneʻane mau loa e pili ana i ka ʻoki kino e hoʻohuli i ke kahe koko a kōkua i ka naʻau e hana me ka maikaʻi. ʻO ke ala ʻoki kino kūikawā e hilinaʻi ana i ke ʻano kino o kāu keiki a me nā hemahema naʻau e pili ana.
Pono nā keiki he nui i kahi moʻo o nā ʻoki kino ma mua o hoʻokahi hana wale nō. Hoʻomaka pinepine ka ʻoki kino mua i ka wā kamaliʻi e hoʻomaikaʻi i nā hōʻailona koke a me ke kahe koko. Hiki paha kēia ke komo i ka hana ʻana i nā pilina ma waena o nā kīʻaha koko a i ʻole ka waiho ʻana i nā shunts manawa.
ʻO ka pahuhopu hope loa ʻo ka hoʻokō ʻana i ka mea i kapa ʻia e nā kauka he
I nā hihia kahi ʻaʻole hiki ke hana hou i ka lua ventricle, hiki i nā kauka ʻoki ke ʻōlelo aku i kahi ala hoʻokahi ventricle. Hoʻopili kēia i kahi papa o ʻekolu mau hana ʻoki e ʻae ai i ka hopena i hoʻokahi ventricle e lawelawe i nā hana pau ʻana o ka naʻau.
Ma waena o nā hana ʻoki, pono paha kāu keiki i nā lāʻau lapaʻau e kōkua i ka hana maikaʻi ʻana o ko lākou naʻau a i ʻole e pale aku i nā pilikia. Hiki paha kēia ke komo pū me nā diuretics e hōʻemi i ka waihona wai a i ʻole nā lāʻau lapaʻau e kākoʻo i ka hana o ka naʻau.
ʻO ka mālama ʻana i kahi keiki me DORV ma ka home e komo pū me kekahi mau manaʻo nui e mālama ai i ko lākou olakino a me ka ʻoluʻolu ma waena o nā hālāwai lapaʻau. E hāʻawi kāu hui olakino i nā kuhikuhi kūikawā i kūpono i nā pono o kāu keiki.
ʻO ka hānai pinepine e pono ai ka nānā kūikawā ʻana, ʻoiai hiki i nā pēpē me DORV ke luhi maʻalahi. Pono paha ʻoe e hāʻawi i nā meaʻai liʻiliʻi a pinepine a i ʻole e hoʻohana i nā ʻōmole kūikawā e maʻalahi ai ka hānai. Loaʻa i kekahi mau pēpē ka pōmaikaʻi mai nā pūhui kiʻekiʻe-calorie e kākoʻo i ka ulu kūpono.
E nānā i nā hōʻailona e pono ai ka nānā lapaʻau koke, e like me ka hoʻonui ʻana i ka kala uliuli, ka pilikia nui o ka hanu, a i ʻole nā hoʻololi nui i nā ʻano ʻai a i ʻole hana. E mālama i nā helu kelepona pilikia ma kahi maʻalahi.
Pono paha kāu keiki i ka palekana mai kekahi mau maʻi, ʻoi aku hoʻi nā maʻi hanu. Hiki paha kēia ke manaʻo i ka palena ʻana i ka hōʻike ʻana i nā lehulehu i ka wā o ke kau anu a me ka maʻi a i ʻole e hōʻoia i ka mālama ʻana o nā lālā ʻohana a pau i nā lāʻau lapaʻau.
He mea nui nā papa manawa lāʻau lapaʻau maʻamau inā lawe kāu keiki i nā lāʻau naʻau. Mai haʻalele i nā mahele me ka ʻole o ka nīnau ʻana i kāu cardiologist, a mālama i nā lako kākoʻo i loaʻa inā loaʻa nā pilikia a i ʻole ka huakaʻi.
Hiki i ka hoʻomākaukau ʻana no nā hālāwai cardiology ke kōkua i ka hōʻoia ʻana e loaʻa iā ʻoe ka mea nui loa mai kēlā me kēia kipa a ʻaʻole e poina i nā nīnau a i ʻole nā manaʻo nui. E mālama i kahi papa inoa holo o nā hōʻailona a i ʻole nā hoʻololi āu i ʻike ai ma waena o nā hālāwai.
E lawe mai i ka papa inoa piha o nā lāʻau āpau a kāu keiki e lawe ai, me nā nui kūpono a me ka manawa. E lawe mai pū i nā moʻolelo o ka hoʻonui ʻana o ke kaumaha, nā ʻano hānai, a i ʻole nā paepae hana inā ua mālama ʻoe i kēia mau mea ma ka home.
E kākau i nā nīnau ma mua no ka mea hiki ke manaʻo nui nā hālāwai lapaʻau. ʻO nā nīnau maʻamau paha e pili ana i ka nīnau ʻana e pili ana i nā palena hana, nā hōʻailona e nānā ai, a i ʻole ka wā e pono ai ka ʻoki kino hou.
Inā hiki, e lawe mai i kahi pākeke ʻē aʻe me ʻoe i nā hālāwai. Hiki i ka loaʻa ʻana o kahi kanaka ʻē aʻe ke kōkua iā ʻoe e hoʻomanaʻo i ka ʻike i kūkākūkā ʻia a hāʻawi i ke kākoʻo manaʻo i nā kamaʻilio paʻakikī.
Mai kānalua e noi i ka wehewehe inā paʻakikī nā huaʻōlelo lapaʻau a i ʻole nā hoʻolālā mālama. Makemake kāu hui olakino e hoʻomaopopo ʻoe i ke kūlana o kāu keiki a manaʻo hilinaʻi i kā lākou mālama.
ʻAʻole maikaʻi, ʻaʻohe ala i ʻike ʻia e pale aku ai i ka DORV no ka mea e ulu kūlohelohe ia i ka wā hāpai mua i ka hapa nui o nā hihia. Hiki ke manaʻo kaumaha kēia, akā he mea nui ia e hoʻomaopopo ʻaʻohe mea āu i hana ai i kēia kūlana.
Eia naʻe, hiki i kekahi mau hana maʻamau i ka wā hāpai ke kākoʻo i ka hoʻomohala ʻana o ka naʻau o ka pēpē. ʻO kēia mau mea e komo pū ana i ka lawe ʻana i nā huaora hāpai me ka folic acid, ka mālama pono ʻana i ka maʻi diabetes inā loaʻa iā ʻoe, a me ka pale ʻana i ka waiʻona a me ka paka.
Inā ʻoe e hoʻolālā nei i nā hāpai ʻana i ka wā e hiki mai ana a loaʻa iā ʻoe ka mōʻaukala ʻohana o nā hemahema naʻau i hānau ʻia, hiki ke kōkua ka ʻōlelo aʻoaʻo genetics. Hiki i kahi kākāʻōlelo ke kūkākūkā i kāu mau pilikia kūikawā a me nā koho hoʻokolohua i loaʻa.
ʻAe ka mālama hāpai maʻamau i ka ʻike mua inā e ulu nā hemahema naʻau. ʻOiai ʻaʻole kēia e pale aku i ka DORV, ʻae ia i ka hoʻomākaukau maikaʻi a me ka hoʻolālā ʻana no ka mālama ʻana i kāu pēpē ma hope o ka hānau ʻana.
ʻO ka DORV he hemahema puʻuwai hānau nui akā hiki ke mālama ʻia, e hoʻopilikia ana i ke kahe ʻana o ke koko ma o ka puʻuwai o kāu keiki. ʻOiai hiki i ka ʻike ʻana ke manaʻo nui, nui nā keiki me ka DORV e ola ana i nā ola piha a hana nui me ka mālama lapaʻau kūpono.
ʻO ke kī i nā hopena maikaʻi loa, ʻo ia ka hana pili kokoke me kahi hui kālepa puʻuwai pediatric akamai e hiki ke alakaʻi iā ʻoe ma o nā hoʻoholo mālama a me ka manawa. He kū hoʻokahi ke kūlana o kēlā me kēia keiki, a ua hoʻonohonoho pono ʻia nā papahana mālama i kā lākou anatomy a me nā pono.
E hoʻomanaʻo he mea maʻamau loa ka manaʻo makaʻu, huikau, a i ʻole kaumaha i ka wā e kūʻē ana i kahi ʻike e like me ka DORV. Mai kānalua e nīnau i nā nīnau, e ʻimi i nā manaʻo ʻē aʻe inā pono, a e hoʻopili me nā hui kākoʻo a i ʻole nā ʻohana ʻē aʻe i hele ma kēia ala.
Me ka holomua o ka ʻoki kino puʻuwai pediatric a me ka mālama mau, ke hoʻomaikaʻi mau nei ka manaʻolana no nā keiki me ka DORV. E kau i ka manaʻo i ka lawe ʻana i nā mea i hoʻokahi kapuaʻi i ka manawa a e hoʻolauleʻa i nā lanakila liʻiliʻi ma ke ala.
Hiki i nā keiki he nui me ka DORV i mālama ʻia me ka holomua ke komo i nā hana kino, ʻoiai ke hilinaʻi nei nā palena kūikawā i kā lākou hana puʻuwai pilikino a me ka mōʻaukala ʻoki kino. E loiloi kāu cardiologist pediatric i ka hana puʻuwai o kāu keiki a e hāʻawi i nā kuhikuhi hana pilikino.
ʻAʻohe palena paha i kekahi mau keiki, ʻoiai e pono paha i nā mea ʻē aʻe ke pale aku i nā haʻuki hoʻokūkū ikaika loa. ʻO ka pahuhopu, ʻo ia ka loaʻa ʻana o ke kaulike kūpono e hiki ai i kāu keiki ke noho ikaika a olakino me ka pale ʻana i ko lākou puʻuwai.
He ʻokoʻa loa ka nui o nā ʻoki kino ma muli o ke ʻano kūikawā o kāu keiki o ka DORV a me nā hemahema puʻuwai e pili ana. Pono kekahi mau keiki i hoʻokahi a ʻelua paha mau kaʻina hana, ʻoiai e pono paha i nā mea ʻē aʻe ʻekolu a ʻoi aku paha nā ʻoki kino ma luna o kekahi mau makahiki.
E hoʻomohala kāu hui ʻoki kino i kahi hoʻolālā piha i ka wā mua, ʻoiai paha e pono ai nā hoʻoponopono i ka ulu ʻana o kāu keiki. E hoʻāʻo mau lākou e hoʻemi i ka nui o nā kaʻina hana āpau ʻoiai e hoʻokō ana i ka hana naʻau maikaʻi loa.
Ua holomua nui ka nānā lōʻihi i nā makahiki i hala iho nei me nā holomua i ka ʻoki kino naʻau kamaliʻi. He nui nā keiki me DORV e ulu a alakaʻi i nā ola maʻamau, hele i ke kula, alualu i nā ʻoihana, a loaʻa pū kekahi i nā ʻohana ponoʻī.
Eia naʻe, pono ka hapa nui i ka nānā cardiologist ola lōʻihi e nānā i kā lākou hana naʻau a nānā i nā pilikia ma hope. ʻO ka prognosis kūikawā e hilinaʻi ana i nā mea e like me ke ʻano o DORV, nā hemahema i pili, a me ka hana maikaʻi ʻana o nā hoʻoponopono ʻoki kino ma mua o ka manawa.
Hiki i nā wāhine he nui me ka DORV i hoʻoponopono ʻia me ka holomua ke loaʻa nā hapai palekana, ʻoiai e pono ai ka hoʻolālā akahele a me ka mālama kūikawā. ʻO ka hoʻoholo e hilinaʻi ana i nā mea e like me ka hana naʻau o kēia manawa, nā hemahema i koe, a me ke kūlana olakino holoʻokoʻa.
Pono nā wāhine e noʻonoʻo ana i ka hapai e hana pū me kā lākou cardiologist a me kahi loea lapaʻau makuahine-pēpē e loiloi i nā pilikia a hoʻomohala i kahi hoʻolālā mālama piha. Me ka nānā pono, he nui nā wāhine me DORV i loaʻa nā hapai holomua a me nā pēpē olakino.
ʻAʻole pono nā keiki a pau me DORV i nā lāʻau ola lōʻihi, akā pono paha kekahi i ka mālama mau e pili ana i kā lākou hana naʻau ma hope o ka ʻoki kino. ʻO nā lāʻau maʻamau paha e komo pū ana i nā mea hoʻonāwaliwali koko, nā lāʻau naʻau, a i ʻole nā lāʻau e kākoʻo i ka hana naʻau.
E loiloi hou ʻia ka pono o kāu keiki no nā lāʻau i ka wā e ulu ai lākou a nānā ʻia kā lākou hana naʻau. Hiki i kekahi mau keiki ke hoʻōki i kekahi mau lāʻau, ʻoiai pono paha kekahi e hoʻomau iā lākou no ka lōʻihi no ke olakino naʻau maikaʻi loa.
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