ʻAukā ʻaukā pāpālua

ʻO ke austic arch ʻelua kahi hana maʻamau o ke aorta, ke aʻa nui e hāpai i ke koko mai ka puʻuwai a i ke koena o ke kino. He pilikia kūpuna ia, ʻo ia hoʻi aia ia ma ka wā hānau.

ʻO ke austic arch ʻelua kahi ʻano maʻamau o kahi hui o nā kīnā e hoʻopili i ka ulu ʻana o ka aorta i loko o ka ʻōpū. Hoʻokumu kēia mau hemahema i kahi hoʻokumu maʻamau i kapa ʻia he apo vascular (kahi pōʻai o nā kīʻaha koko).

ʻO ka maʻamau, hoʻomohala ka aorta mai kekahi o nā ʻāpana o ke kiko (arches). Ke ulu nei nā pēpē i loko o ka ʻōpū, ua māhele ʻia nā pio i nā ʻāpana he nui. Haki ke kino i kekahi o nā pio, a ʻo nā mea ʻē aʻe e hana i nā aʻa. ʻO kahi aorta e hoʻomohala mau ʻia kahi pana hoʻokahi e haʻalele i ka puʻuwai a neʻe i ka hema.

I ke kia ʻaukā pāpālua, aia kekahi o nā pio e nalo e noho mau nei i ka wā hānau ma waho o ka piʻo maʻamau. ʻO nā pēpē me ka ʻaukā aorta ʻelua he aorta i hana ʻia i ʻelua moku ma kahi o hoʻokahi.Loaʻa nā ʻāpana ʻelua i ke aorta i nā lālā ʻoi aku o ka liʻiliʻi e pili ana i nā lālā o lākou. ʻO ka hopena, hele a puni nā lālā ʻelua a kaomi i lalo i ka puka makani a me ka paipu (esophagus) e halihali ana i ka meaʻai mai ka waha a hiki i ka ʻōpū.

Hiki ke hana ʻia kahi pio aortic papalua i nā kīnā puʻuwai hānau ʻē aʻe, e like me:

• ʻO Tetralogy o Fallot
• Truncus arteriosus
• Transposition o nā aʻa nui
• Hewa septal ʻo Ventricular

Kākaʻikahi loa ka pio aorta ʻelua. Hana nā apo vascular i kahi pākēneka liʻiliʻi o nā pilikia puʻuwai congenital āpau. ʻO kēia mau mea, ʻoi aku ka liʻiliʻi ma mua o ka hapalua e kumu ʻia e ka aortic arch. Kū like ke ʻano i nā kāne a me nā wahine. Aia pinepine ia i ka poʻe me kekahi ʻano chromosome abnormalities.

Ma muli o ka maʻalahi o nā ʻōuli o ka austic arch ʻelua, ʻaʻole ʻike ʻia ka pilikia a hiki i ke ʻano o ke keiki i kekahi mau makahiki.

E kaomi paha ka piʻo aortic pāpālua ma ka trachea a me ka esophagus, e alakaʻi ana i ka pilikia e hanu a me ka ale ʻana. ʻO ke koʻikoʻi o nā ʻōuli ke pili i ka nui o ka ʻaukā aortic e kaomi ana i kēia mau hale.

Nā hōʻailona hanu me:

• Kani kani kiʻekiʻe i ka hanu ʻana (stridor)
• Hanu walaʻau
• Pākuʻi hou i hana hou ʻia
• ʻUhihi

Hiki ke hoʻopili ʻia nā hōʻailona Digestive:

• Kāpena
• Paʻa i ka ʻai ʻana a me ka ale ʻana
• ʻO ka luaʻi

Hiki i nā ʻōuli ke alakaʻi i kahi mea mālama ola e kānalua i ka austic arch. E makemake ʻia nā hoʻokolohua ʻē aʻe e hōʻoia i ka ʻike.

Hiki i nā hoʻāʻo aʻe ke kōkua i ke kuhi ʻana i nā ʻanuʻu aorta ʻelua:

• Pākuʻi x-ray
• Nā scans e hana i nā kiʻi ʻāpana o ke kino (CT a i ʻole MRI scan)
• ʻIniu ultrasound o ka puʻuwai (echocardiography)
• X-ray e hoʻohana nei i kahi mea e hōʻike i ka esophagus (barium ale)

Hiki ke hana i ke kahaʻoki e hoʻoponopono i nā pio aorta ʻelua. Hoʻopili ka mea hana lāʻau i ka lālā liʻiliʻi a hoʻokaʻawale iā ia mai ka lālā nui. A laila pani ka lāʻau lapaʻau i nā wēlau o ke aorta me nā humuhumu. Hoʻomaha kēia i ke kaomi ma ka esophagus a me ka windpipe.

ʻOi aku ka maikaʻi o ka hapanui o nā keiki ma hope o ke kahakaha ʻana, ʻoiai ke hoʻomau mau nei kekahi i nā hōʻailona hanu no kekahi manawa ma hope o ka hoʻoponopono ʻana. ʻO kēia ka mea pinepine ma muli o ka nāwaliwali o ka trachea ma muli o ke kaomi ma luna o ia ma mua o ka hoʻoponopono ʻana.

I nā hihia maʻamau, inā kaomi nui ka piʻo ma ke ala ākea, hiki i ke keiki ke loaʻa i ka paʻakikī hanu paʻakikī e hiki ai i ka make.

Komo pū nā pilikia:

• Ka holomua ʻole ʻana
• Nā maʻi hanu
• Ke kāpae nei i ka uhi o ka esophagus (esophageal erosion) a me ka puka makani
• Kākaʻikahi loa, kahi pili pono ʻole ma waena o ka esophagus a me ke aorta (aortoesophageal fistula)

Kāhea i kāu mea hāʻawi inā loaʻa kāu pēpē i nā ʻōuli o ka austic arch ʻelua.

ʻAʻohe ala i ʻike ʻia e pale ai i kēia ʻano.

ʻAnopika arch anomaly; Puku ʻelua; Hōʻino puʻuwai Congenital - arch aortic pāpālua; Hema kīnā hānau - arch aortic ʻelua

• Kaha vaskular
• ʻAukā ʻaukā pāpālua

ʻO Bryant R, Yoo S-J. ʻO nā apo vascular, ka maʻa o ka pulmonary, a me nā kūlana e pili ana. I: Wernovsky G, Anderson RH, Kumar K, Mussatto K, et al, eds. Ka Pediatric Cardiology o Anderson. ʻEhā ed. Piladelapia, PA: Elsevier; 2020: chap 47.

Fraser CD, Kane LC. Maʻi maʻi ʻōpū. I: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. ʻO Sabiston Textbook of Surgery. 20 ouk. Piladelapia, PA: Elsevier; 2017: chap 58.

ʻO Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. ʻO nā puʻuwai congenital a me nā malformations vascular. I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 459.

Webb GD, Smallhorn JF, Therrien J, Redington AN. ʻO ka maʻi puʻuwai Congenital i ke kanaka makua a me ka mea maʻi pediatric. I: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. ʻO ka maʻi puʻuwai ʻo Braunwald: He puke aʻo no ka lāʻau Cardiovascular. 11th hua. Piladelapia, PA: Elsevier; 2019: chap 75.