Ke nānā aku nei paha ka ʻili a me nā lehelehe o kāu pēpē hānau hou i ke ʻano polū iki? Ke ʻano nei anei ʻo ia e pilikia iki ana i ka hanu ʻana? A i ʻole e hou nui ana ʻo ia i ka wā e inu ai ʻo ia i ka waiū? Mai hoʻokuke wale i kēia mau mea me he mea lā he mau hanana kūikawā. I kekahi manawa hiki i kēia ke lilo i mau hōʻailona o kahi maʻi puʻuwai i hānau ʻia ai kāu pēpē. I kēia lā, ke kamaʻilio nei mākou e pili ana i kahi maʻi puʻuwai kakaʻikahi āu, ma ke ʻano he makua, e makaʻala pono ai. ʻO ia ka Double Outlet Right Ventricle, a i ʻole DORV no ka pōkole.
I ka ʻōlelo maʻalahi, he aha ka Double Outlet Right Ventricle (DORV)?
I mea e hoʻomaopopo ai i kēia, e noʻonoʻo mua kākou pehea e hana ai ka puʻuwai olakino. Ua like ko kākou puʻuwai me kahi hale liʻiliʻi me ʻehā lumi. ʻElua lumi luna a ʻelua lumi haʻahaʻa. ʻO nā lumi nui ʻelua a ikaika ma lalo ua kapa ʻia ʻo ventricles. Aia kekahi ma ka ʻaoʻao ʻākau (Ventricle ʻĀkau), a ʻo kekahi ma ka ʻaoʻao hema (Ventricle Hema).
ʻO kēia ka mea maʻamau e hana ʻia:
- ʻO ke kīʻaha koko nui e lawe ana i ke koko "lepo" nele i ka oxygen i nā māmā, ʻo ia hoʻi ke aʻa pulmonary, e hoʻomaka ana ma ka ventricle ʻākau .
- ʻO ke kīʻaha koko nui loa, ʻo ka aorta, e hoʻomaka ana mai ka ventricle hema, e lawe ana i ke koko "maʻemaʻe" i hoʻopiha ʻia me ka oxygen mai nā māmā ma ke kino holoʻokoʻa.
He maikaʻi loa ia, ʻeā? Hoʻokahi kaʻa nui i loko o kēlā me kēia lumi.
I kēia manawa, ma ke ʻano o DORV, ua hoʻopilikia ʻia kēia kauoha. I loko o kahi pēpē me DORV, ʻo nā kīʻaha koko nui ʻelua a mākou i ʻōlelo ai, ʻo ka Pulmonary Artery a me ka Aorta, ua pili piha a hapa paha i ka Ventricle ʻĀkau. A laila, ʻaʻole i pili ka Ventricle Hema i kekahi kīʻaha koko nui, a i ʻole he ʻāpana liʻiliʻi loa.
I ka ʻōlelo maʻalahi, ʻo ka aorta, ka mea e lawe ana i ke koko oxygenated ma ke kino holoʻokoʻa, a me ke aʻa pulmonary, ka mea e lawe ana i ke koko deoxygenated i nā māmā, ua puka mai lāua ʻelua mai kahi hoʻokahi, ʻo ia hoʻi ka ventricle ʻākau. He maʻi puʻuwai hānau kēia.
Ke hāpai nei kēia i ʻelua mau nīnau:
1. Ua hui pū ʻia ke koko maʻemaʻe a waiwai i ka oxygen e pono ai ke hele i nā ʻāpana ʻē aʻe o ke kino me ke koko haumia a nele i ka oxygen.
2. Ke hoʻopuka ʻia nei ke koko ma o nā māmā ma mua o ka mea e pono ai. Hoʻokau kēia i ka nui o ke kaumaha ma ka puʻuwai a me nā māmā. I ka hala ʻana o ka manawa, hiki i kēia mau ʻōkana ke hōʻino ʻia.
He aha nā pilikia ʻē aʻe e ʻike ʻia me DORV?
I kekahi manawa, loaʻa i nā pēpē me DORV nā pilikia hānau ʻē aʻe. ʻO kekahi o kēia mau mea:
- Ka hana pono ʻole o ke kino o ka pēpē ma ke ʻano ciliary: Nā pilikia me ka hana ʻana o nā ʻāpana palupalu loa e like me ka lauoho o ke ala hanu o ka pēpē.
- Heterotaxy:Aia nā ʻōpū o ke pēpē ma ka umauma a me ka ʻōpū ma nā wahi ʻē aʻe ma mua o ko lākou wahi maʻamau.
- ʻŌpū malrotation: He wili ʻano ʻē o nā ʻōpū o ke pēpē.
- Stenosis pulmonary: Ka hoʻohaiki ʻana a i ʻole ka hoʻohaiki ʻana o ka valve ma waena o ka ventricle ʻākau a me ke aʻa pulmonary.
- Hypoplasia Ventricular: ʻOi aku ka liʻiliʻi o hoʻokahi a ʻelua paha o nā keʻena o ka puʻuwai ma mua o ka maʻamau.
Aia kekahi mau ʻano DORV like ʻole? Pehea e hiki ai ke hoʻokaʻawale i kēia mau mea?
ʻAe, aia kekahi mau ʻano o DORV. Hoʻoholo ʻia kēia hoʻokaʻawale ʻana e hoʻokahi mea nui loa. ʻO ia hoʻi, kokoke i kēlā me kēia pēpē me DORV he lua ma ka paia ma waena o nā keʻena ʻelua o ka puʻuwai (Ventricular Septal Defect - VSD). Pale kēia lua i ke koko mai ke kahe ʻana i mua a i hope ma waena o nā keʻena ʻelua.
Hoʻokaʻawale nā kauka i ka DORV ma muli o kahi o ka VSD, a i ʻole ka lua.
| ʻAno DORV | He wehewehe maʻalahi |
|---|---|
| ʻO DORV me ka VSD subaortic | Aia ka lua i loko o ka puʻuwai (VSD) ma lalo o ka aorta. |
| ʻO DORV me ka VSD subpulmonary (Taussig-Bing) | Aia ka lua i loko o ka puʻuwai ma lalo o ke aʻa pulmonary. Ua kapa ʻia hoʻi kēia ʻo ke kūlana Taussig-Bing. |
| ʻO DORV me ka VSD i hoʻopaʻa pālua ʻia | Aia ka lua ma lalo o nā kīʻaha koko nui ʻelua (ʻo Aorta a me Pulmonary Artery). |
| ʻO DORV me ka VSD i hoʻopaʻa ʻole ʻia (mamao) | Aia ka lua ma kahi mamao loa mai kēia mau kīʻaha koko nui ʻelua, ma kahi i pili ʻole ai. |
ʻO ka ʻike pono i ke ʻano o kēia he mea nui loa ia no ka hoʻolālā ʻana i ka mālama ʻana.
E Māmā a me Pāpā, e makaʻala i kēia mau hōʻailona!
ʻIke pinepine ʻia nā hōʻailona o ka DORV i loko o nā lā mua a i ʻole nā pule ma hope o ka hānau ʻana. Pono ʻoe e makaʻala loa i kēia mau mea.
| Hōʻailona | He aha ke ʻano o kēia? |
|---|---|
| Polū (Cyanosis) | Hiki i ka ʻili, nā lehelehe, a i ʻole nā kui lima o ke pēpē ke lilo i polū a poni paha. ʻO kēia ma muli o ka nele o ka oxygen i loko o ke koko. |
| Paʻakikī ka hanu ʻana | Ka hanu ʻana me ka leo nui, me ka wikiwiki (tachypnea) a i ʻole ka manaʻo pōkole o ka hanu. |
| Paʻakikī i ka ʻai ʻana/inu ʻana | ʻAʻole makemake e inu i ka waiū, a inā paha e inu iki ʻoe, e oki ʻoe ma hope o kekahi manawa. Hiki i kēia ke alakaʻi i ka emi ʻana o ke kaumaha. |
| ʻŌhumu Puʻuwai | Ke nānā ke kauka i ka puʻuwai me ka stethoscope, lohe ʻo ia i kahi leo ʻokoʻa ma waho aʻe o ka puʻuwai. |
| Ka wikiwiki o ka puʻuwai (Tachycardia) | ʻOi aku ka wikiwiki o ka puʻuwai ma mua o 100 mau kuʻi i kēlā me kēia minuke. |
| Ka hou nui ʻana | Nui ka hou o ke pēpē, ʻoiai i ka wā e hānai waiū ai. |
| Ka hiamoe ʻē | Ua hiamoe mau ka pēpē a make ke ola. |
He aha ke kumu o kēia maʻi? He aha nā pilikia e hiki mai ana?
ʻO kaʻoiaʻiʻo, ʻaʻole i loaʻa i nā kānaka noiʻi kahi kumu paʻa no ka DORV. Eia nō naʻe, ua ʻike ʻia aia ma kahi o 50% o nā pēpē me ke ʻano he ʻano loli i kā lākou mau chromosomes, nā ʻāpana o kā mākou ʻike genetic (DNA).
Akā, e hoʻomanaʻo i kēia. ʻAʻole kēia kou hewa, ʻaʻole kou hewa. No laila, mai noʻonoʻo e pili ana i ia mea a hoʻāhewa iā ʻoe iho.
Inā ʻaʻole mālama pono ʻia ka DORV, hiki ke ulu aʻe nā pilikia i ka hala ʻana o ka manawa.
- Nā ʻano ʻino o ka puʻuwai (Arrhythmias)
- Nā pilikia valve puʻuwai
- Ka hāʻule ʻana o ka puʻuwai
- Ke koko kiʻekiʻe o ka puʻuwai
- Ka maʻi o ka ʻili o loko o ka puʻuwai (Endocarditis)
Pehea e ʻike ai nā kauka i ke kūlana DORV?
I kekahi manawa, hiki ke ʻike ʻia kēia ʻano maʻi puʻuwai ma mua o ka hānau ʻana o ka pēpē, i ka wā o nā scan i kou wā hāpai. ʻO ke scan kūikawā i hoʻohana ʻia no kēia ua kapa ʻia ʻo "Fetal Echocardiogram".
Inā ʻaʻole hiki kēlā, e kānalua wale nā kauka i kēia ke ʻike ʻia nā hōʻailona a mākou i kamaʻilio ai ma mua ma hope o ka hānau ʻana o ka pēpē. A laila e nānā lākou i ka pēpē.
- E nānā e ʻike inā e polū ana ka ʻili o ke pēpē.
- Hoʻolohe ʻia ka puʻuwai me kahi stethoscope e nānā i nā leo ʻē aʻe (murmurs).
- ʻO ka Pulse Oximetry kahi hoʻāʻo e ana i ka nui o ka oxygen i loko o ke koko o ka pēpē ma ke kau ʻana i kahi mea liʻiliʻi e like me ke clip ma ka manamana lima a i ʻole ka pepeiao o ka pēpē. He hoʻāʻo maʻalahi kēia ʻaʻole e hōʻeha i ka pēpē.
Ma hope o kēia mau hoʻokolohua mua, hiki ke hana ʻia kekahi mau hoʻokolohua hou aʻe e hōʻoia i ka hōʻailona.
- Nā hoʻokolohua kiʻi ʻaʻole invasive: Nā scan CT, nā scan MRI, a me nā kukuna X i hana ʻia ma waho o ke kino o ke pēpē.
- Electrocardiogram (ECG a i ʻole EKG): He hoʻāʻo ʻeha ʻole e ana i ka hana uila o ka puʻuwai.
- Echocardiogram (echo): Ua like kēia me ke scan o ka puʻuwai. Hoʻohana ia i nā nalu kani e ʻike maopopo i nā mea āpau e pili ana i ka puʻuwai, me kona ʻano, ka hana, a pehea e pili ai kona mau kīʻaha koko.
- Catheterization Cardiac: Ma kēia, ua hoʻokomo ʻia kahi paipu lahilahi loa (catheter) i loko o ka puʻuwai ma o kahi kīʻaha koko ma ka ʻūhā a i ʻole ka lima o ke pēpē e loaʻa ai ka ʻike pololei loa e pili ana i ke kaomi o ka puʻuwai, nā pae oxygen, a me ke ʻano.
Mai hopohopo, aia kahi lāʻau lapaʻau no kēia! - E aʻo kākou e pili ana i ke ʻoki kino
Aneane kēlā me kēia pēpē me DORV e pono ai ke ʻoki puʻuwai hāmama i loko o ka makahiki mua o ke ola. He mea hopohopo paha kēia iā ʻoe. He mea maʻamau kēlā. Akā e hoʻomanaʻo, me ka ʻenehana lapaʻau o kēia wā, hiki ke hana ʻia kēia mau ʻoki me ka holomua loa.
E noʻonoʻo kāu kauka a me ke kauka lapaʻau puʻuwai i kekahi mau kumu i ka wā e hoʻoholo ai e pili ana i ke ʻoki ʻana.
- He aha ke ʻano DORV o ke pēpē?
- Inā paha he mau pilikia ʻē aʻe me ka puʻuwai o ka pēpē a i ʻole nā kikowaena ʻē aʻe.
- Ke olakino holoʻokoʻa o ka pēpē.
Aia kekahi mau ʻano hana ʻoki kino i hana pinepine ʻia.
- Hoʻoponopono intraventricular: Ma kēia kaʻina hana, hana ke kauka lapaʻau i kahi tunnel ma o ka VSD, a i ʻole ka lua i loko o ka puʻuwai, me ka hoʻohana ʻana i kahi ʻāpana kūikawā. Hana kēia tunnel i kahi ala no ke koko e kahe mai ka ventricle hema a i ka aorta.
- Hoʻoponopono biventricular a i ʻole ke kuapo arterial: Inā maikaʻi ke kūlana o nā ventricles ʻelua o ka pēpē a he ʻano subpulmonary VSD (Taussig-Bing) ka DORV, hiki i ke kauka lapaʻau ke hana i kahi ʻoki e wehe i ka aorta a hoʻohui iā ia i ka ventricle hema.
- Hoʻoponopono Univentricular (hana Fontan): Inā paʻakikī loa ka DORV, hiki i ke kauka lapaʻau ke ʻōlelo aku i kēia hana ʻoki kino. Pili kēia i ke kuhikuhi ʻana i ke koko nele i ka oxygen mai ke kino haʻahaʻa a i nā māmā. He hana ʻoki kino paʻakikī kēia e pili ana i kekahi mau pae.
Aia nā pilikia me kekahi ʻoki puʻuwai. ʻO kēia mau mea ke kahe koko, nā ʻūpā koko, ka maʻi, a me nā pilikia me ka anesthesia. Akā hana nā kauka i kā lākou mea maikaʻi loa e hōʻemi i kēia mau pilikia.
Pehea lā ke ʻano o ke ola ma hope o ke ʻoki kino?
Ma hope o ke ʻoki ʻana, pono ka pēpē e noho ma ka haukapila no hoʻokahi pule a ʻoi aku paha. E loli ka lōʻihi o ka manawa ma muli o ke ʻano o ke ʻoki ʻana i hana ʻia a me ke kūlana o ka pēpē.
ʻO ka mea nui, ma hope o ke ʻoki ʻana, hiki i ka hapa nui o nā pēpē me DORV ke ola olakino a maʻamau i ke ola makua.
Akā, hoʻokahi mea e hoʻomanaʻo ai, ʻo ke kanaka i hana ʻia i ke ʻoki DORV e pono e noho ma lalo o ka nānā ʻana o kahi kauka puʻuwai no ke koena o ko lākou ola. He mea nui e hele i nā nānā ʻana i ka manawa i hoʻonohonoho ʻia a hana i nā hoʻokolohua e pono ai.
Hiki i kekahi poʻe ke loaʻa i nā puʻuwai puʻuwai kūpono ʻole a i ʻole nā pilikia o ka puʻuwai i nā makahiki he nui ma hope o ke ʻoki ʻana, no laila he mea nui e mālama mau i kou olakino.
I ka manawa hea e ʻike ai i ke kauka a i ka manawa hea e hele ai i ka ETU
ʻOiai ma hope o ke ʻoki ʻana, e makaʻala pono i kāu keiki. Inā ʻike ʻia nā hōʻailona ma lalo nei, e hoʻomaopopo koke i kāu kauka.
- Inā ʻoe e manaʻo i kahi puʻuwai puʻuwai kūpono ʻole, wikiwiki, a i ʻole ke kuʻi ʻana.
- Inā ʻeha kou umauma a paʻakikī paha ka hanu ʻana (he mau hōʻailona paha kēia o ka hōʻeha puʻuwai).
I nā hihia aʻe, e lawe koke i ke keiki i ka Emergency Treatment Unit (ETU) o ka haukapila kokoke loa.
Inā pilikia nui kāu keiki i ka hanu ʻana a/a i ʻike ʻia ka uliuli o ko lākou mau kui lima, lehelehe, a i ʻole ka ʻili, e hele koke i ka ETU!
Nā nīnau koʻikoʻi e nīnau aku i kāu kauka
He mea maʻamau ka loaʻa ʻana o nā nīnau he nui ke ʻike ʻoe ua loaʻa i kāu pēpē ka DORV. Mai hūnā i kekahi mea iā ʻoe iho. E nīnau i kāu kauka i nā mea āpau a e wehewehe ʻia. Eia kekahi mau nīnau hiki iā ʻoe ke nīnau.
1. He aha ke ʻano o ka DORV i loaʻa i kaʻu keiki?
2. He aha ke ʻano o ke ʻoki kino āu e makemake ai e hana?
3. Āhea e hana ʻia ai ke ʻoki kino?
4. He aha nā pilikia o ke ʻoki kino?
5. He aha nā pilikia o ka hana ʻole ʻana i ke ʻoki kino?
6. Pono anei kaʻu keiki i nā ʻoki kino hou aʻe ma hope o kēia ʻoki kino?
7. Pono anei kaʻu keiki e lawe i ka lāʻau lapaʻau no ke koena o kona ola?
Ke ʻike pono ʻoe i nā mea āpau ma ka nīnau ʻana i nā nīnau e like me kēia, e hiki iā ʻoe ke hana i nā hoʻoholo naʻauao me ka makaʻu ʻole.
Leka Lawe-Home
- He maʻi puʻuwai hānau koʻikoʻi ka Double Outlet Right Ventricle (DORV), akā hiki ke mālama ʻia.
- ʻOiai ʻaʻole ʻike ʻia ke kumu maoli o kēia ʻano, mai manaʻo ʻoe he hewa kēia nou ma ke ʻano he makua.
- ʻO ka ʻike mua ʻana a me ke ʻoki ʻana i ka manawa kūpono e hāʻawi i nā keiki i ka manawa kūpono e ola olakino a lōʻihi.
- Ma hope o ke ʻoki ʻana, pono ʻoe e noho ma lalo o ka nānā ʻana o kahi cardiologist no ke koena o kou ola.
- E makaʻala mau no nā hōʻailona pilikia e like me ka paʻakikī nui o ka hanu ʻana a me ka ʻili polū. I kēlā hihia, e lawe koke i ke keiki i ka Emergency Treatment Unit (ETU).
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