Transposition o nā aʻa nui

ʻO Transposition o nā aʻa nui (TGA) kahi kīnā o ka puʻuwai i kū mai ka hānau ʻana (congenital). ʻO nā aʻa nui ʻelua e lawe ana i ke koko mai ka puʻuwai - ke aorta a me nā aʻa pulmonary - ua hoʻololi ʻia (transosed).

ʻAʻole ʻike ʻia ke kumu o TGA. ʻAʻole pili ia me kekahi o nā kūlohelohe maʻamau. ʻAʻohe loaʻa iki i nā lālā ʻohana ʻē aʻe.

ʻO TGA kahi kīnā naʻau cyanotic. ʻO kēia ke kumu o ka hōʻemi ʻana o ka oxygen i loko o ke koko e hoʻokahe ʻia mai ka puʻuwai a i ke koena o ke kino.

I nā puʻuwai maʻamau, hele ke koko e hoʻi mai ke kino ma ka ʻaoʻao ʻākau o ka puʻuwai a me ke aʻa o ka pulmonary i nā akemāmā e loaʻa ai ka oxygen. Hele hou ke koko i ka ʻaoʻao hema o ka puʻuwai a hele i waho aorta i ke kino.

I ka TGA, hoʻi hou ke koko venous i ka puʻuwai ma o ka atrium ʻākau. Akā, ma kahi o ka hele ʻana i nā ake māmā e omo i ka oxygen, hoʻokahe ʻia kēia koko ma o ka aorta a hoʻi i ke kino. ʻAʻole i hoʻonui hou ʻia kēia koko me ka oxygen a alakaʻi i ka cyanosis.

Hōʻike nā ʻōuli i ka wā hānau a i ʻole ma hope koke iho. Aia ka maikaʻi ʻole o nā ʻōuli i ke ʻano a me ka nui o nā kīnā o ka puʻuwai (e like me ka hemahema atrial septal, ventricular septal defect, a i ʻole patent ductus arteriosus) a me ka nui o ke kōkō e hiki ai ke kāwili ʻia ma waena o nā kahe ʻelua.

Pākuʻi ʻia paha nā ʻōuli:

• ʻEnessuli o ka ʻili
• ʻO ka clubbing o nā manamana lima a me nā manamana wāwae
• ʻAi hānai maikaʻi ʻole
• Pokole o ka hanu

Hoʻomaopopo paha ka mea mālama ola i kahi ʻōhumu o ka puʻuwai ke hoʻolohe nei i ka umauma me kahi stethoscope. ʻO ka waha a me ka ʻili o ka pēpē e lilo i kala polū.

Hoʻokomo pinepine nā hoʻokolohua i kēia mau mea:

• ʻO ka catheterization Cardiac
• Pākuʻi x-ray
• ECG
• Echocardiogram (inā hana ma mua o ka hānau ʻana, ua kapa ʻia he echocardiogram fetal)
• Pulse oximetry (e nānā i ka pae oxygen o ke koko)

ʻO ka hana mua i ka mālama ʻana e ʻae i ke koko momona o oxygen e hui pū me ke koko maikaʻi ʻole o oxygen. E loaʻa koke i ka pēpē kahi lāʻau i kapa ʻia ʻo prostaglandin ma o ka IV (laina intravenous).Kōkua kēia lāʻau i ka mālama ʻana i kahi kīʻaha koko i kapa ʻia ʻo ductus arteriosus hāmama, e ʻae ana i kekahi hui ʻana o nā kahe koko ʻelua. I kekahi mau hihia, hiki ke hana i kahi wehe ma waena o ka atrium ʻākau a me ka hema me ka hana e hoʻohana ana i ka catheter balloon. ʻAe kēia e huikau ke koko. ʻIke ʻia kēia hana ma ke ʻano he septostomy balloon atrial.

Hoʻopili ka mālama paʻa ʻana i ka ʻoki puʻuwai kahi e ʻoki ʻia ai nā aʻa nui a humuhumu ʻia i ko lākou kūlana kūpono. Kāhea ʻia kēia he hana kuapo arterial (ASO). Ma mua o ka hoʻomohala ʻana o kēia ʻoki, ua hoʻohana ʻia kahi ʻoki i kapa ʻia he atrial switch (a i ʻole ke kaʻina ʻo Mustard a i ʻole ke ʻano ʻo Senning).

E hoʻomaikaʻi hou nā ʻōuli o ke keiki ma hope o ke kaha ʻana e hoʻoponopono i ka hemahema. ʻO ka hapa nui o nā pēpē i loaʻa i nā kuapo arterial ʻaʻole i loaʻa nā ʻōuli ma hope o ke kaha ʻana a ola i nā ola maʻamau. Inā ʻaʻole e hoʻokō ʻia ke kaha pololei, he mau mahina wale nō ke ola.

Komo pū nā pilikia:

• Nā pilikia o ke aʻa coronary
• Nā pilikia o ka puʻuwai puʻuwai
• Nā ritme puʻuwai ʻole kaulike (arrhythmia)

Hiki ke kuhi ʻia kēia ʻano ma mua o ka hānau ʻana me ka hoʻohana ʻana i ka echocardiogram fetal. Inā ʻaʻole, ʻike pinepine ʻia ia ma hope koke o ka hānau ʻia ʻana o kahi pēpē.

E hele i ka lumi pilikia a kāhea paha i ka helu kūloko kūloko (e like me 911) inā ulu ka ʻili o kāu pēpē i kahi kala bluish, keu hoʻi i ka maka a i ʻole kumu.

Kāhea i kāu mea hāʻawi inā loaʻa kāu pēpē i kēia ʻano a hoʻomohala i nā ʻōuli hou, lilo i ʻino, a i ʻole hoʻomau ma hope o ka lāʻau.

ʻO nā wahine e hoʻolālā e hāpai e pono e pale ʻia i ka rubella inā ʻaʻole lākou e pale aku. Maikaʻi ka ʻai ʻana, ka hōʻalo ʻana i ka wai ʻona, a me ka kaohi ʻana i ka maʻi kō ma mua a ma ka wā hāpai.

d-TGA; Hapa puʻuwai Congenital - transposition; Maʻi naʻau cyanotic - transposition; Hewa hānau - transposition; Hoʻololi o nā moku nui; TGV

• ʻOki puʻuwai Pediatric - hoʻokuʻu

• Puʻuwai - ʻāpana ma waena o ka waena
• Puʻuwai - ʻike mua
• ʻO ka Transposition o nā moku nui

ʻO Bernstein D. Cyanotic maʻi puʻuwai congenital: loiloi i ka neonate maʻi nui me ka cyanosis a me ka hanu hanu. I loko o: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21th hua. Piladelapia, PA: Elsevier; 2019: chap 456.

Fraser CD, Kane LC. Maʻi maʻi ʻōpū. I: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. ʻO Sabiston Textbook of Surgery: Ke kumu kumu olaola o ka hana ʻoki ʻana o kēia ao. 20 ouk. Piladelapia, PA: Elsevier; 2017: chap 58.

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.