He aha ka Pulmonary Atresia me ka Ventricular Septal Defect? Nā Hōʻailona, Nā Kumu, a me ka Lapaʻau

ʻO ka pulmonary atresia me ka ventricular septal defect he pilikia nui o ka naʻau i hānau ʻia nā pēpē me ia, kahi i hana ʻole pono ʻia ai ka puka pulmonary a aia kekahi puka ma waena o nā keʻena ʻoihana nui o ka naʻau. ʻO ia hoʻi, ʻaʻole hiki i ke koko ʻole o ka oxygen ke kahe maʻamau mai ka naʻau i nā māmā e kiʻi i ka oxygen.

E noʻonoʻo i kou naʻau e like me ka loaʻa ʻana o ʻehā lumi me nā puka ma waena o lākou. Ma kēia kūlana, ua pani loa kekahi o kēlā mau puka (ka puka pulmonary), a aia kekahi wehe ʻana ma ka paia ma waena o ʻelua lumi ʻaʻole pono ke loaʻa. Hana kēia i kahi kūlana paʻakikī kahi e pono ai ka naʻau o kāu pēpē e hana nui aku e kiʻi i ka oxygen i ke kino.

He aha ka Pulmonary Atresia me ka Ventricular Septal Defect?

Hana ʻia ka pulmonary atresia me ka ventricular septal defect (PA-VSD) i ka wā e loaʻa pū ai ʻelua pilikia naʻau mai ka hānau ʻana. ʻO ka puka pulmonary, kahi e wehe ai e ʻae i ke koko e kahe mai ka naʻau i nā māmā, ua pani loa ʻia a nalowale paha.

I ka manawa like, aia kekahi puka ma ka paia (septum) e hoʻokaʻawale ai i nā keʻena haʻahaʻa ʻelua o ka naʻau (ventricles). Hana kēia hui ʻana i ka paʻakikī loa no ke koko e hiki aku i nā māmā e kiʻi i ka oxygen.

E ho'āʻo ke kino o kāu pēpē e uku ma ka hoʻomohala ʻana i nā kīʻaha koko ʻē aʻe i kapa ʻia ʻo ka collateral circulation. Kūkulu kēia mau ala hou i kekahi koko e hiki i nā māmā, akā ʻaʻole lākou e like me ka maikaʻi o ke ala maʻamau.

Hoʻopili kēia kūlana i kahi 1 i kēlā me kēia 10,000 pēpē i hānau ʻia. ʻOiai he mea hopohopo ia, ua hoʻomaikaʻi nui nā holomua ma ka ʻoki naʻau pediatric i ka lapaʻau nui aku ma mua o ka wā ma mua.

He aha nā Hōʻailona o ka Pulmonary Atresia me ka Ventricular Septal Defect?

Hōʻike pinepine nā hōʻailona o PA-VSD i loko o nā lā mua a i ʻole nā pule o ke ola o kāu pēpē. Malia paha e ʻike ʻoe i ka ʻili, nā lehelehe, a i ʻole nā kui o kāu pēpē e lilo i polū, ʻoi aku hoʻi i ka wā e uē a hānai paha.

Eia nā hōʻailona nui e nānā ai:

• Ke kala polū o ka ʻili, nā lehelehe, a i ʻole nā kui (cyanosis)
• ʻO ka pilikia o ka hanu a i ʻole ka hanu wikiwiki
• Ka hānai maikaʻi ʻole a i ʻole ka luhi koke i ka wā e hānai ana
• Ka hāʻule ʻole o ke kaupaona maʻamau
• Hoʻonui ʻia ka huhū a i ʻole ka hoʻonāukiuki
• Clubbing o nā manamana lima a me nā manamana wāwae (lilo nā manamana lima i puni a hoʻonui ʻia)
• Nā manawa fainting a i ʻole nā hanana kahi e lilo ai kāu pēpē i palupalu

Malia paha e hoʻomohala kekahi mau pēpē i nā pilikia koʻikoʻi e like me ka naʻau naʻau, kahi e hakakā ai ka naʻau e hoʻopuka i ke koko me ka maikaʻi. I nā hihia kakaikahi, hiki i nā keiki ke ʻike i nā abscesses lolo a i ʻole nā hahau ma muli o nā clots koko.

Hiki i ka nui o nā hōʻailona ke ʻokoʻa nui ma muli o ka nui o ke koko e hiki ke hiki i nā māmā ma o kēlā mau ala ʻē aʻe a mākou i ʻōlelo ai ma mua.

He aha nā ʻAno o ka Pulmonary Atresia me ka Ventricular Septal Defect?

Hoʻokaʻawale pinepine nā kauka i ka PA-VSD ma muli o ke ʻano o ke koko e hiki ai i nā māmā a me ke ʻano kūikawā o ka naʻau o kēlā me kēia keiki. ʻO ka ʻokoʻa nui, ʻo ia inā loaʻa i kāu keiki ka kahe koko lawa i nā māmā a ʻaʻole paha.

Hoʻopili ka ʻano 1 i nā keiki i loaʻa iā lākou nā kīʻaha koko collateral maikaʻi i hoʻomohala kūlohelohe e hoʻolako i nā māmā. Hiki i kēia mau ala ʻē aʻe ke hoʻolako i ka kahe koko maikaʻi, ʻoiai ʻaʻole nui e like me ka naʻau maʻamau e hāʻawi ai.

Hoʻopili ka ʻano 2 i nā keiki me ka collateral circulation maikaʻi ʻole a i ʻole lawa ʻole. Pono pinepine kēia mau pēpē i ka hoʻoponopono wikiwiki no ka mea ʻaʻole lawa ka kahe koko o kā lākou māmā.

Aia nō hoʻi kahi hoʻokaʻawale ʻana ma muli o ka hoʻomohala maikaʻi ʻia a liʻiliʻi paha nā arteries pulmonary nui (nā kīʻaha nui e lawe i ke koko i kēlā me kēia māmā). Loaʻa pinepine i nā keiki me nā arteries pulmonary nui, hoʻomohala maikaʻi ʻia nā hopena maikaʻi.

E hoʻoholo ka cardiologist o kāu keiki i ke ʻano o kāu pēpē ma o nā haʻawina kiʻi kikoʻī, kahi e kōkua ai i ka hoʻolālā lapaʻau.

He aha ka mea e kumu ai i ka Pulmonary Atresia me ka Ventricular Septal Defect?

Hoʻomohala ʻia ka PA-VSD i loko o nā hebedoma 8 mua o ka hāpai ʻana i ka wā e hoʻomohala ana ka naʻau o kāu pēpē. ʻAʻole maopopo loa ke kumu pololei, akā ʻike ʻia he hui pū ʻana o nā kumu genetic a me nā kumu kūlohelohe.

Hana pinepine ʻia ka hapa nui o nā hihia me ka ʻole o kekahi kumu maopopo. ʻAʻole hoʻomohala maʻamau nā ʻano o ka naʻau o kāu pēpē i kēlā mau hebedoma mua koʻikoʻi i ka wā e hoʻomohala ana nā kino.

Eia kekahi mau mea e hoʻonui paha i ka pilikia:

• Nā syndrome genetic e like me ka DiGeorge syndrome a i ʻole ka CHARGE syndrome
• Mōʻaukala ʻohana o nā hemahema naʻau congenital
• Ka maʻi diabetes makuahine i ka wā hāpai
• Nā lāʻau lapaʻau i lawe ʻia i ka wā hāpai mua
• Nā maʻi makuahine e like me ka rubella i ka wā hāpai
• Ka hoʻohana ʻana o ka waiʻona makuahine i ka wā hāpai
• Ka makahiki makuahine holomua (ma mua o 35)

I nā hihia kakaikahi, hiki i ka PA-VSD ke lilo i ʻāpana o kahi syndrome genetic nui e hoʻopilikia i nā ʻōnaehana kino he nui. Eia naʻe, ʻo ka hapa nui o nā keiki me kēia kūlana he hemahema naʻau kaʻawale ia.

He mea nui e ʻike ʻaʻole ʻoe i hana a i ʻole i hana ʻole i kekahi mea i ka wā hāpai i kumu ai i kēia kūlana. Hana ʻia nā hemahema naʻau e like me PA-VSD i ka wā hāpai mua loa, pinepine ma mua o kou ʻike ʻana e manaʻo ana ʻoe.

I ka wā e ʻike ai i ke Kauka no ka Pulmonary Atresia me ka Ventricular Septal Defect?

Hoʻomaopopo pinepine ʻia ka PA-VSD i ka wā hāpai ma o nā hoʻokolohua ultrasound maʻamau, ma kahi o 18-22 mau wiki. Inā kānalua ʻia, e kauoha aku kāu kauka i kahi ultrasound kūikawā i kapa ʻia ʻo ka fetal echocardiogram e kiʻi i nā kiʻi kikoʻī o ka naʻau o kāu pēpē.

Ma hope o ka hānau ʻana, hoʻopili ka hoʻomaopopo ʻana i nā hoʻokolohua he nui e hoʻomaopopo i ke ʻano kūikawā o ka naʻau o kāu pēpē. Hāʻawi pinepine ka kala ʻili o kāu pēpē, nā ʻano hanu, a me nā pilikia hānai i nā ʻōlelo mua e pono ai ka nānā.

Hoʻopili pinepine ke kaʻina hana hoʻomaopopo ʻana i:

1. Nānā kino a me ka hoʻolohe ʻana i ka naʻau
2. X-ray umauma e ʻike i ka nui o ka naʻau a me ka kahe koko māmā
3. Echocardiogram (ultrasound naʻau) no ke ʻano kikoʻī o ka naʻau
4. Electrocardiogram (EKG) e nānā i ke ʻano o ka naʻau
5. Pulse oximetry e ana i nā pae oxygen
6. Cardiac catheterization no nā ana pololei
7. MRI a i ʻole CT scan no nā kiʻi kikoʻī

He mea nui loa ka cardiac catheterization no ka mea ʻae ia i nā kauka e ʻike pono i ke ʻano o ke koko e kahe ana ma o ka naʻau o kāu pēpē a ana i nā kaomi ma nā keʻena like ʻole. He mea nui kēia ʻike no ka hoʻolālā ʻana i ka lapaʻau.

E nānā pū ka cardiologist o kāu pēpē i nā kūlana genetic a i ʻole nā hemahema naʻau ʻē aʻe e loaʻa paha. Kūkulu kēia loiloi piha i ka hoʻolālā lapaʻau maikaʻi loa no ke kūlana kūikawā o kāu keiki.

He aha ka Lapaʻau no ka Pulmonary Atresia me ka Ventricular Septal Defect?

ʻO ka lapaʻau no ka PA-VSD kokoke mau e hoʻopili i ka ʻoki, akā ʻo ka manawa a me ke ala e hilinaʻi ana i ke kūlana kūikawā o kāu keiki a me nā hōʻailona. ʻO ka pahuhopu, ʻo ia ke kōkua i ka nui o ke koko e hiki aku i nā māmā a hoʻemi i ka hana o ka naʻau o kāu pēpē.

Pono ka hapa nui o nā pēpē i kā lākou ʻoki mua i loko o nā mahina mua o ke ola. Kēia kaʻina hana mua, i kapa pinepine ʻia ʻo ka Blalock-Taussig shunt, hana i kahi ala hou no ke koko e hiki aku i nā māmā me ka hoʻohana ʻana i kahi tube liʻiliʻi.

Hoʻopili pinepine ka hoʻolālā lapaʻau piha i nā ʻoki he nui ma nā makahiki he nui:

1. ʻOki shunt mua (maʻamau i nā mahina mua o ke ola)
2. ʻOki waena e hoʻomaikaʻi i ka kahe koko māmā (ma kahi o 4-6 mau mahina)
3. ʻOki hoʻoponopono piha (maʻamau ma waena o 6 mau mahina a 2 mau makahiki)
4. Nā ʻoki hou e like me ka ulu ʻana o kāu keiki

Ma waena o nā ʻoki, pono kāu keiki i nā lāʻau lapaʻau e kōkua i ka naʻau e hana me ka maikaʻi a pale aku i nā pilikia. Hiki i kēia ke hoʻopili i nā diuretics e hoʻemi i ka waihona wai a me nā lāʻau lapaʻau e hoʻoikaika i nā ʻoihana naʻau.

Malia paha e pono kekahi mau keiki i nā kaʻina hana hou e like me ka balloon angioplasty e wehe i nā kīʻaha koko haiki a i ʻole ka hoʻonoho ʻana i nā mea hana e pani i nā pilina makemake ʻole ma waena o nā kīʻaha koko.

E hana pū ka hui ʻoki me ʻoe e hoʻonohonoho i kēlā me kēia kaʻina hana me ka maikaʻi, e noʻonoʻo ana i ka ulu ʻana o kāu keiki, nā hōʻailona, a me ke olakino holoʻokoʻa. Loaʻa i ka ʻoki naʻau pediatric hou nā helu holomua maikaʻi no ka hoʻoponopono ʻana i ka PA-VSD.

Pehea e Hāʻawi ai i ka Mālama Home i ka wā o ka Pulmonary Atresia me ka Ventricular Septal Defect?

ʻO ka mālama ʻana i kahi keiki me PA-VSD ma ka home e pono ai kekahi nānā kūikawā, akā hoʻololi maikaʻi ka hapa nui o nā ʻohana me ka alakaʻi kūpono. ʻO kāu mea nui e nānā i nā hōʻailona o kāu keiki a hōʻoia i ka loaʻa ʻana o ka hānai a me ka hoʻomaha lawa.

Hiki ke paʻakikī ka hānai no ka mea luhi koke nā pēpē me PA-VSD. E hāʻawi i nā hānai liʻiliʻi, pinepine a noʻonoʻo i nā fomula kiʻekiʻe-calorie inā ʻōlelo kāu kauka iā lākou e kōkua i ke kaupaona.

Eia nā ʻāpana nui o ka mālama home:

• E nānā i ke kala ʻili, ʻoi aku hoʻi a puni nā lehelehe a me nā kui
• E nānā i nā loli i nā ʻano hanu
• E hahai i ke kaupaona a me nā ʻano hānai
• E hāʻawi i nā lāʻau lapaʻau e like me ka mea i kuhikuhi ʻia
• E pale i nā maʻi me ka hoʻomaʻemaʻe maikaʻi
• E kaupalena i ka hōʻike ʻana i nā poʻe maʻi i ka hiki
• E mālama i nā papa hoʻomaha maʻamau
• E hahai i nā palena hana e like me ka ʻōlelo aʻo

E mālama i kahi moʻolelo o kēlā me kēia lā o nā hōʻailona o kāu keiki, nā nui hānai, a me nā loli hopohopo. Kūkulu kēia ʻike i kāu hui lapaʻau e hana i nā hoʻoholo ʻike e pili ana i ka manawa lapaʻau a me nā hoʻoponopono.

E hana i kahi wahi mālie, kākoʻo ma ka home. Hana maikaʻi pinepine nā keiki me nā kūlana naʻau me nā hana maʻamau a me ka haʻahaʻa o ke kaumaha. Mai kānalua e noi i nā ʻohana a me nā hoaaloha no ke kōkua me nā hana o kēlā me kēia lā i hiki iā ʻoe ke kālele i ka mālama ʻana o kāu keiki.

Pehea ʻoe e hoʻomākaukau ai no kāu Hoʻokolokolo Kauka?

ʻO ka hoʻomākaukau ʻana no nā hoʻokolokolo cardiology e kōkua i ka hōʻoia ʻana e loaʻa iā ʻoe ka mea maikaʻi loa mai kēlā me kēia kipa a ʻaʻole poina i nā nīnau a i ʻole nā manaʻo nui. E mālama i kahi papa inoa o nā nānā e pili ana i ke kūlana o kāu keiki ma waena o nā kipa.

E lawe mai i ka moʻolelo lapaʻau piha o kāu keiki, me nā hoʻokolohua a i ʻole nā kaʻina hana hou mai nā kauka ʻē aʻe. Inā ua mālama ʻoe i kahi moʻolelo hōʻailona, e lawe mai i kēlā no ka mea hāʻawi ia i ka ʻike waiwai e pili ana i nā ʻano a me nā loli.

Eia ka mea e hoʻomākaukau ai ma mua o kēlā me kēia hoʻokolokolo:

• Papa inoa o nā lāʻau lapaʻau a me nā dosages
• Nā nīnau e pili ana i ke kūlana o kāu keiki a me ka lapaʻau
• Nā manaʻo e pili ana i nā hōʻailona a i ʻole nā loli ʻano
• ʻIke e pili ana i ka hānai, ka ulu, a me ka hoʻomohala
• Nā kāleka ʻinikua a me nā pepa hana pono
• ʻIke pilikia pilikia
• Nā mea hōʻoluʻolu no kāu keiki

E kākau i nā nīnau nui ma mua, e like me ka nīnau ʻana e pili ana i nā palena hana, i ka wā e pono ai ka ʻoki hou, a i ʻole nā hōʻailona e pono ai ka nānā koke. Mai hopohopo e nīnau i nā nīnau he nui - makemake kāu hui lapaʻau iā ʻoe e ʻike maikaʻi.

E noʻonoʻo e lawe mai i kahi lālā ʻohana a hoaaloha paha no ke kākoʻo, ʻoi aku hoʻi i nā hoʻokolokolo kahi e kūkākūkā ʻia ai nā hoʻoholo nui. Hiki i ka loaʻa ʻana o kekahi kanaka ʻē aʻe ke kōkua iā ʻoe e hoʻomanaʻo i ka ʻike nui a hāʻawi i ke kākoʻo manaʻo.

He aha ka lawe nui e pili ana i ka Pulmonary Atresia me ka Ventricular Septal Defect?

ʻO ka PA-VSD he kūlana naʻau koʻikoʻi akā hiki ke mālama ʻia e pono ai ka mālama lapaʻau kūikawā a me nā ʻoki he nui i ka manawa. ʻOiai hiki i ka hoʻomaopopo ʻana ke manaʻo ʻia he mea hopohopo, ua hoʻomaikaʻi nui nā holomua ma ka ʻoki naʻau pediatric i nā hopena no nā keiki me kēia kūlana.

Hiki i ka hapa nui o nā keiki me PA-VSD ke ola i nā ola piha, hana ma hope o ka lapaʻau kūleʻa. ʻOiai e pono lākou i ka hahai ʻana i ka naʻau ola lōʻihi a loaʻa paha iā lākou kekahi mau palena hana, hiki i ka hapa nui ke komo i ke kula, nā haʻuki, a me nā hana ʻē aʻe o ke kamaliʻi. ʻO ke kī, ʻo ia ka hana pū ʻana me kāu hui lapaʻau e hoʻokele pono i ke kūlana a nānā i nā loli ma mua o ka manawa.

E hoʻomanaʻo ʻaʻole ʻoe wale nō ma kēia huakaʻi. Hiki i kāu hui lapaʻau, ʻohana, a me nā hui kākoʻo ke hāʻawi i ke kōkua a me ka hoʻoikaika waiwai. Loaʻa i nā ʻohana he nui ka mea kōkua e hoʻopili me nā poʻe ʻē aʻe i hele ma kēia ala ma mua.

Nā Nīnau Pinepine e pili ana i ka Pulmonary Atresia me ka Ventricular Septal Defect

Hiki i kaʻu keiki me PA-VSD ke ola i kahi ola maʻamau?

Hiki i nā keiki he nui me PA-VSD ke ola i nā ola piha, hana ma hope o ka lapaʻau kūleʻa. ʻOiai e pono lākou i ka hahai ʻana i ka naʻau ola lōʻihi a loaʻa paha iā lākou kekahi mau palena hana, hiki i ka hapa nui ke komo i ke kula, nā haʻuki, a me nā hana ʻē aʻe o ke kamaliʻi. ʻO ke kī, ʻo ia ka hana pū ʻana me kāu hui lapaʻau e hoʻokele pono i ke kūlana a nānā i nā loli ma mua o ka manawa.

Ehia mau ʻoki e pono ai kaʻu keiki?

Pono ka hapa nui o nā keiki me PA-VSD i 2-3 mau ʻoki nui i loko o kā lākou mau makahiki mua o ke ola, me ka hiki ke loaʻa nā kaʻina hana hou i ka wā e ulu ai lākou. ʻO ka helu pololei e hilinaʻi ana i ke ʻano kūikawā o kāu keiki a me ke ʻano o kā lākou pane ʻana i ka lapaʻau. E kūkākūkā kāu kauka i ke ala ʻoki i hoʻolālā ʻia a kōkua iā ʻoe e hoʻomaopopo i ka mea e manaʻo ai i kēlā me kēia kahua.

He aha nā hopena lōʻihi o PA-VSD?

Me ka lapaʻau kūleʻa, hiki i nā keiki he nui me PA-VSD ke manaʻo i nā hopena maikaʻi lōʻihi. Eia naʻe, e pono lākou i ka hahai ʻana i ka cardiology ola lōʻihi e nānā i ka hana naʻau a nānā i nā pilikia hiki ke loaʻa. Loaʻa paha i kekahi ka palena hana a pono paha i nā kaʻina hana hou ma hope i ke ola, akā nui ka poʻe e alakaʻi i nā ola maʻamau me ka mālama kūpono.

Hiki ke pale ʻia ka PA-VSD?

I kēia manawa, ʻaʻohe ala e pale aku ai i ka PA-VSD no ka mea e hoʻomohala ana ia i ka wā hāpai mua ma muli o nā mea ma mua o ka mana o kekahi. Eia naʻe, ʻo ka mālama ʻana i ka mālama hāpai maikaʻi, ka hoʻokele ʻana i ka maʻi diabetes inā loaʻa, ka pale ʻana i ka waiʻona a me ka puhipaka i ka wā hāpai, a me ka lawe ʻana i nā mea hoʻohui folic acid i kuhikuhi ʻia hiki ke kākoʻo i ka hoʻomohala ʻana o ka pēpē olakino holoʻokoʻa.

Pehea ka hopena o PA-VSD i ka ulu a me ka hoʻomohala ʻana o kaʻu keiki?

Ma mua o ka ʻoki, ʻike pinepine nā keiki me PA-VSD i ka loaʻa ʻana o ke kaupaona lohi a hiki ke hiki i nā hōʻailona hoʻomohala ma hope o ka maʻamau. Ma hope o ka lapaʻau kūleʻa, loaʻa i ka hapa nui o nā keiki ka hopena nui i ka ulu a me ka hoʻomohala. E nānā pono kāu hui lapaʻau i kēia mau wahi a hāʻawi i nā lawelawe kākoʻo inā pono e kōkua i kāu keiki e hiki i kā lākou hiki piha.