He pilikia puʻuwai ko kāu keiki liʻiliʻi? E kamaʻilio kākou e pili ana i ka Hypoplastic Aortic Arch!

Ke pilikia nei kāu keiki liʻiliʻi i ka hānai waiū i kekahi manawa? Manaʻo anei ʻo ia ua ʻoi aku kona luhi ma mua o ka maʻamau i ka hanu ʻana? A i ʻole ke ʻano keʻokeʻo a me ka hou? He mea maʻamau iā ʻoe ma ke ʻano he makua ke hopohopo nui i ka wā e hiki mai ai kēia mau mea i kāu keiki liʻiliʻi. I kekahi manawa ʻo ke kumu o kēia he maʻi puʻuwai hānau. I kēia lā, e kamaʻilio mākou e pili ana i kahi maʻi e kū nei i loko o ka puʻuwai o nā pēpē, akā hiki ke ho'ōla ʻia inā ʻike mua ʻia a mālama pono ʻia.

He aha ke ʻano o ka Hypoplastic Aortic Arch?

I ka ʻōlelo maʻalahi, ʻo Hypoplastic Aortic Arch , i kapa ʻia ʻo Aortic Arch Hypoplasia (AAH) , he kīnā hānau ia e kū mai ana ma kahi ʻāpana o ka puʻuwai o kāu pēpē , ʻo ia hoʻi ka aorta . E noʻonoʻo i kēia ʻano, ua like ka aorta me kahi aʻa nui e lawe ana i ke koko ma o ko mākou kino. Hoʻomaka ia ma ka puʻuwai a lawe i ke koko oxygenated ma ke kino. Ua like kona ʻano me ke koʻokoʻo, kūlou iki. ʻO ke kūlou ma ka piko o ke koʻokoʻo ua kapa ʻia ʻo ke aortic arch , ʻo ia hoʻi 'ke kiko o ka aorta.'

ʻO ke ʻano o ka huaʻōlelo 'hypoplastic' ʻo ia hoʻi 'ʻaʻole i ulu pono', 'liʻiliʻi'. No laila, ʻo kahi pēpē me ke aortic arch hypoplastic he aortic arch i ulu ʻole. ʻO ia hoʻi, ʻoi aku ka haiki o ka puka, a i ʻole ke anawaena, i loko o kēlā ʻāpana ma mua o ka mea e pono ai. E like me ka paʻa ʻana o ka paipu wai, i ka wā e haiki ai ke aortic arch, lilo ia i mea paʻakikī no ke kahe ʻana o ke koko mai ka puʻuwai a i nā ʻāpana haʻahaʻa o ke kino, ma o ka descending aorta . Hiki i kēia ke hōʻemi i ka nui o ke koko e hiki aku i ke kino holoʻokoʻa.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia maʻi? Aia kekahi pilina me nā maʻi puʻuwai ʻē aʻe?

Hiki ke loaʻa ka Aortic arch hypoplasia (AAH) ma kāna iho. Eia nō naʻe, ʻike pinepine ʻia me kahi kīnā puʻuwai hānau ʻē aʻe , i kapa ʻia ʻo coarctation o ka aorta . He kūlana kēia kahi e haiki ai kekahi ʻāpana o ka aorta, ma kahi kokoke i ka ductus arteriosus, kahi kīʻaha koko kūikawā i loaʻa mau i loko o ka ʻōpū.

No laila e ʻōlelo paha ke kauka o kāu pēpē, "Loaʻa i kāu pēpē ka coagulopathy o ka aorta me ka aortic arch hypoplasia." ʻO ia hoʻi, aia kahi haiki o ka aorta, ʻaʻole wale ma kahi hoʻokahi, akā ma kahi ʻāpana lōʻihi.

Eia kekahi, hiki i nā pēpē me AAH ke loaʻa i nā maʻi puʻuwai ʻē aʻe. Eia kekahi laʻana:

• ʻO ke kīnā septal atrial (ASD) : He lua ma ka paia ma waena o nā keʻena ʻelua o luna o ka puʻuwai.
• ʻO ke kīnā Septal Atrioventricular : He kīnā paʻakikī i nā paia ma waena o nā keʻena o ka puʻuwai.
• ʻO ka Bicuspid Aortic Valve a me ka Aortic Valve Stenosis : Nā hemahema a me ka hāiki ʻana o ka aortic valve.
• ʻO ka Hypoplastic Left Heart Syndrome (HLHS) : ʻAʻole ulu pono ka ʻaoʻao hema o ka puʻuwai.
• ʻO ka Patent Ductus Arteriosus (PDA) : ʻO kahi kīʻaha koko i kapa ʻia ʻo ductus arteriosus e pani ʻia ma hope o ka hānau ʻana e waiho hāmama ana.
• ʻO ka Shone's Syndrome : He hui pū ʻana o kekahi mau kīnā e hoʻopilikia ai i ke kahe koko i ka ʻaoʻao hema o ka puʻuwai.
• ʻO ke kīnā septal ventricular (VSD) : He lua ma ka paia ma waena o nā keʻena haʻahaʻa ʻelua o ka puʻuwai.

He mea nui loa ka loaʻa ʻana o nā maʻi puʻuwai he nui e like me kēia i ka wā e hoʻolālā ai i ka mālama ʻana. No ka mea, pono kahi ʻoki kino a i ʻole kahi hoʻolālā lapaʻau i hoʻopilikino ʻia i nā kūlana paʻakikī a kikoʻī i loko o ka puʻuwai o ka pēpē.

He aha nā hōʻailona inā loaʻa i ke keiki kēia maʻi?

ʻO ka maʻamau, hoʻomaka nā pēpē me ka coarctation o ka aorta a me ka aortic arch hypoplasia e hōʻike i nā hōʻailona ma hope koke iho o ka hānau ʻana . E noʻonoʻo i kāu pēpē hānau hou, ka mea luhi ma hope o kekahi mau inu, me he mea lā e paʻakikī ana ka hanu ʻana. I kekahi manawa e hou lākou a lilo i kala uliuli-keʻokeʻo. He mea weliweli loa i kahi makuahine ke ʻike i kahi mea e like me kēia, ʻeā?

ʻO nā hōʻailona nui e ʻike ʻia:

• Ka pulse wikiwiki : Ua wikiwiki loa ka wikiwiki o ka pulse.
• Luhi : Manaʻo koke ka pēpē i ka luhi, ʻoiai i ka wā e hānai waiū ai.
• ʻIli hina a keʻokeʻo paha : I kekahi manawa hiki ke ʻike ʻia ka ʻili he uliuli.
• Hou nui .
• Uē pinepine, huhū .
• Ka hanu wikiwiki a hana nui paha .
• Pilikia me ka hānai ʻana: Hōʻole e inu i ka waiū, a hoʻōki paha ma hope o ka inu ʻana i kahi liʻiliʻi.
• Ka naʻau palupalu ma nā ʻāpana haʻahaʻa o ke kino : ʻOi loa ma nā wāwae, he nāwaliwali loa ka naʻau ma ke aʻa femoral i ka wā e pā ai.

Inā loaʻa i kāu pēpē kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​​​he mea nui loa e ʻimi koke i ke kōkua lapaʻau .

No ke aha lā kēia e hana nei? He aha nā kumu?

He kīnā puʻuwai hānau ʻo AAH. He mea ia e hana ʻia ana i ka wā e noho ana ke pēpē i loko o ka ʻōpū. Ke hoʻāʻo nei nā kānaka noiʻi e ʻike i ke kumu pololei o kēia ʻano kīnā puʻuwai hānau.

Akā ʻo ka mea i ʻike ʻia, hiki ke hana ʻia kēia ʻano inā ʻaʻole ulu pono ka ʻāʻī aortic o ka pēpē i nā pule mua o ka hāpai ʻana .

Pehea ʻoe e ʻike ai i kēia? He aha nā hoʻokolohua?

Hoʻohana nā kauka i kēia e ʻike ai i ke kūlana.Nā hoʻāʻo kiʻi kūikawā. Hana pinepine ʻia kēia mau hoʻāʻo ma hope koke o ka hānau ʻana o ka pēpē.

ʻO nā hoʻokolohua nui i hoʻohana ʻia no ka ʻike ʻana iā AAH:

• ʻO ka Tomography Computed Cardiac (CT scan)
• ʻO ka Angiogram Resonance Magnetic (MRA)
• ʻO ka Transthoracic Echocardiogram (TTE) : Ua like kēia me ke kiʻi ultrasound o ka puʻuwai.
• Ka Catheterization o ka Puʻuwai : I kekahi manawa pono e hana ʻia kēia hoʻāʻo.

Hiki i kēia mau hoʻokolohua ke nānā i ke ʻano o ka puʻuwai o ka pēpē, ʻo ia hoʻi, pehea e hoʻonohonoho ʻia ai nā ʻāpana o loko, me nā kikoʻī loa. Hiki iā lākou ke ʻike inā he mau kīnā puʻuwai ʻē aʻe ko ka pēpē. Ma ka hōʻiliʻili ʻana i kēia ʻike āpau e hoʻolālā ai nā kauka i ka lāʻau kūpono loa no ka pēpē.

No ka hoʻoholo ʻana inā loaʻa kahi AAH, nānā nā kauka i ka nui o ke kīkoʻo aortic o ka pēpē. ʻO ke kikoʻī, ana lākou i kona anawaena , ʻo ia ka laulā o ka puka i loko. Hoʻohālikelike ʻia kēia ana me ke anawaena o nā ʻāpana ʻē aʻe o ka aorta o ka pēpē (no ka laʻana, ka aorta ascending a i ʻole ka aorta descending). Hoʻohana ʻia kēia mau waiwai e hoʻoholo ai inā he haiki loa ke anawaena o ke kīkoʻo aortic.

E aʻo iki kākou e pili ana i nā ʻāpana like ʻole o ke ā aotic.

Ke kamaʻilio ke kauka o kāu pēpē e pili ana i kēia kūlana, e kamaʻilio paha ʻo ia e pili ana i nā ʻāpana like ʻole o ke aortic arch. Ke noʻonoʻo nei paha ʻoe, "Ua māhele ʻia ke aortic arch i nā ʻāpana ʻē aʻe? ʻAʻole anei ia he ʻāpana o ka aorta nui?" ʻAe, he ʻoiaʻiʻo kēlā. He ʻāpana liʻiliʻi ke aortic arch, "e like me ke koʻokoʻo" o ka aorta. Akā, e like me nā ʻāpana āpau o ka aorta, loaʻa i ke aortic arch nā kīʻaha koko (nā aʻa) e lālā ana i nā ʻaoʻao like ʻole. E like me ke alanui nui he mau alanui ʻaoʻao. Hoʻohana nā kauka i kēia mau kīʻaha koko like ʻole ma ke ʻano he palena, e hōʻailona ana i kahi e hoʻomaka ai a e pau ai kahi ʻāpana kikoʻī o ke aortic arch. He mea kōkua nui kēia "palapala ʻāina" i ka ʻike ʻana i ka AAH a me ka hoʻolālā ʻana i ka mālama ʻana.

Eia kekahi o nā ʻāpana o ke ānuenue aural a ke kauka o kāu pēpē e kamaʻilio ai paha:

• ʻO ke Kūlou Transverse Proximal : Aia kēia ma waena o ke Artery Brachiocephalic o ke pēpē (i kekahi manawa i kapa ʻia ʻo Innominate Artery) a me ka Left Common Carotid Artery . ʻO kēia ka ʻāpana kokoke loa i ka puʻuwai.
• ʻO ke apo transverse distal : Aia kēia ma waena o ke aʻa carotid maʻamau hema a me ke aʻa subclavian hema .
• ʻO ka Isthmus Aortic : Aia kēia ma waena o ke aʻa subclavian hema a me ke kīʻaha koko i ʻōlelo ʻia ma mua i kapa ʻia ʻo Ductus Arteriosus .

Na ke kauka o kāu pēpē e hoʻoholo pololei i kahi o ka hāiki ʻana ma ke apo pepeiao o kāu pēpē, a me ka nui o ka hāiki ʻana. Na kēia ʻike e alakaʻi i ka hoʻolālā lapaʻau.

He aha nā lāʻau lapaʻau no kēia?

Pono nā pēpē me ke kīkoʻo aortic hypoplastic i ke ʻoki puʻuwai e hoʻoponopono i ke kūlana. E wehewehe ke kauka puʻuwai o kāu pēpē iā ʻoe i ke ʻano o ke ʻoki e pono ai kāu pēpē. Hiki ke ʻokoʻa ke kaʻina hana ʻoki ma muli o nā kīnā ʻē aʻe a me ke ʻano o ka puʻuwai o kāu pēpē. Pono paha ʻoe e hana i kahi thoracotomy , ʻo ia hoʻi kahi ʻoki liʻiliʻi i hana ʻia ma ka ʻaoʻao hema o ka umauma o kāu pēpē, a i ʻole sternotomy, ʻo ia hoʻi kahi ʻoki i hana ʻia ma waenakonu o ka umauma.

ʻO ke kaʻina hana maʻamau i kapa ʻia ʻo resection me ka hoʻolōʻihi ʻia ʻana o ka end-to-end anastomosis . He mea kōkua nui kēia ʻoki no nā pēpē me ka coarctation o ka aorta a me ka aortic arch hypoplasia. Ma kēia kaʻina hana, wehe ke kauka ʻoki i ka ʻāpana haiki o ka aorta, lawe i ka ʻāpana haʻahaʻa o ka aorta (ka descending aorta), a hoʻopili iā ia i kahi ʻoki lōʻihi iki ma ke aortic arch.

ʻO ka manaʻolana o kēia ʻoki kino, ʻo ia ka hoʻomaikaʻi ʻana i ke kahe koko ma o ka aorta o ka pēpē, e hōʻoiaʻiʻo ana e loaʻa ana i nā ʻōkana a me nā ʻiʻo o ka pēpē ke koko momona i ka oxygen.

He aha ka manaʻolana no ka ho'ōla ʻana o kaʻu pēpē?

ʻO ke kauka o kāu pēpē ka pane maikaʻi loa. No ka mea, hilinaʻi ke olakino o ka pēpē i nā kumu he nui. ʻOi aku ka hopena inā he mau kīnā puʻuwai hānau ʻē aʻe.

Ma keʻano laulā, ʻoi aku ka nui o ka manawa o nā pēpē i mālama ʻia no kahi maʻi i kapa ʻia ʻo aotic arch hypoplasia e hoʻomohala i nā pilikia ma hope aku o ke ola:

• Ka hoʻohaiki pinepine ʻana o ko lākou aorta .
• Ke koko kiʻekiʻe (hypertension) .
• Paʻakikī i ka ʻai ʻana a i ʻole ke ale ʻana : Hiki ke hana kēia inā ua hōʻino ʻia kahi aʻalolo i ka wā o ke ʻoki ʻana (he mea kākaʻikahi loa kēia).

E kamaʻilio me ke kauka o kāu pēpē e pili ana i kēia mau pilikia a pehea e hoʻokele ai iā lākou.

Hiki ke pale ʻia kēia kūlana?

ʻAʻole hiki ke pale loa ʻia nā kīnā puʻuwai hānau e like me ke kīkoʻo aortic hypoplastic. Eia nō naʻe, ʻo ka hahai ʻana i nā ʻōlelo aʻo a kāu kauka e loaʻa kahi hāpai olakino hiki ke kōkua i ka hōʻemi ʻana i kou pilikia o ka hoʻomohala ʻana i kēia ʻano a i ʻole nā ​​​​hemahema ʻē aʻe.

Ma keʻano laulā, pono e pale ʻia kēia mau mea i ka wā hāpai:

• ʻAila ʻona .
• E pale i ka puhipaka, ka vaping a me nā huahana paka āpau . Eia kekahi, e pale i ka uahi lua .
• Nā lāʻau lapaʻau kū ʻole i ke kānāwai, ʻoi aku hoʻi ka cocaine .

A ʻo kāu hoʻi.He mea nui loa hoʻi e hele pinepine i nā hoʻokolohua prenatal. Inā loaʻa iā ʻoe kekahi mau maʻi olakino ʻē aʻe, e ʻimi i ka ʻōlelo aʻoaʻo lapaʻau e mālama i kēlā mau kūlana ma mua a i ka wā hāpai.

Pehea e mālama ai i ke keiki ma hope o ke ʻoki kino?

ʻOkoʻa paha nā pono o kēlā me kēia pēpē ma hope o ke ʻoki ʻana, no laila ʻoi aku ka maikaʻi o ka nīnau ʻana i ka hui lapaʻau o kāu keiki pehea e mālama ai i kāu pēpē i ka wā pōkole a me ka wā lōʻihi.

Eia kekahi mau nīnau hiki iā ʻoe ke nīnau i kāu kauka:

• Pehea lā wau e mālama ai i kaʻu keiki ke hoʻi au i ka home?
• He aha ka lāʻau lapaʻau e hāʻawi aku ai i kaʻu keiki? Pehea ka lōʻihi e hāʻawi aku ai?
• Pono kekahi mau lāʻau lapaʻau e kōkua i ka hana kino a i ʻole ka hana neurological o ke keiki?
• Aia kekahi mau kapu i nā hana a ke keiki?
• Āhea ka manawa palekana e hoʻouna aku ai i kaʻu keiki i kahi mālama keiki? He aha nā kuhikuhi kūikawā e pono iaʻu e hāʻawi aku iā lākou?
• E pono anei i kaʻu keiki ke mālama hou ʻia a i ʻole ke ʻoki kino?

He mea paʻakikī mau ka noho ʻana he makua. Akā, ʻo ka noho ʻana me kahi keiki me kahi maʻi puʻuwai e like me ka hypoplastic aortic arch (AAH) hiki ke lawe mai i nā pilikia hou i kēlā me kēia lā. ʻO kekahi o nā ʻōlelo a nā kauka e ʻōlelo ai a me nā hoʻolālā lapaʻau e like paha me he mea paʻakikī ke hoʻomaopopo. He mea maʻamau kēlā, a ʻaʻole ʻoe hoʻokahi. E nīnau i ke kauka o kāu keiki e pili ana i nā kumuwaiwai a me nā hui kākoʻo e hiki ke kōkua iā ʻoe. ʻO ke kūkulu ʻana i kahi kaiāulu kākoʻo no ʻoe a me kou ʻohana hiki ke kōkua i ka hōʻemi ʻana i ke kaumaha a hāʻawi iā ʻoe i ka ikaika e neʻe i mua me nā pono o kāu keiki.

E hoʻomanaʻo wale kākou i kā kākou i kamaʻilio ai (Leka Lawe-Home)

• ʻO ka Hypoplastic Aortic Arch (AAH) kahi kūlana kahi e ulu pono ʻole ai ke kakaka o ka aorta, kahi kīʻaha koko nui i loko o ka puʻuwai o ke pēpē, e hopena ana i ka haiki ʻana o ke kakaka.
• ʻO kēia ka mea e paʻakikī ai ke kahe ʻana o ke koko mai ka puʻuwai i nā ʻāpana ʻē aʻe o ke kino.
• ʻIke pinepine ʻia kēia ʻano me kahi maʻi puʻuwai ʻē aʻe i kapa ʻia ʻo Coarctation of the Aorta .
• ʻO nā hōʻailona e like me ka luhi, ka paʻakikī o ka hanu ʻana, a me ka ʻili keʻokeʻo e ʻike ʻia paha i nā wā mua o ke ola o ka pēpē.
• Hiki ke ʻike ʻia kēia kūlana me nā scan kūikawā.
• ʻO ke ʻoki puʻuwai ka lāʻau lapaʻau.
• He mea nui loa ka hahai pono ʻana i nā ʻōlelo lapaʻau a me ka hahai ʻana i ka nānā lōʻihi ma hope o ke ʻoki ʻana.
• ʻO ka hahai ʻana i nā hana olakino i ka wā hāpai hiki ke hōʻemi i ka pilikia o kekahi mau kīnā hānau.
• ʻAʻole ʻoe hoʻokahi. E kiʻi i ke kōkua mai nā kauka a me nā hui kākoʻo.

Lana ko mākou manaʻo he kōkua kēia ʻike iā ʻoe. Makemake mākou i kāu keiki liʻiliʻi e ho'ōla koke!

ʻO ke apo aortic hypoplastic, Aortic Arch Hypoplasia, kīnā puʻuwai hānau, ʻoki puʻuwai pēpē, coarctation o aorta, nā maʻi puʻuwai pēpē, ʻoki puʻuwai