Tricuspid atresia

ʻO Tricuspid atresia kahi ʻano maʻi maʻi puʻuwai e kū nei i ka wā hānau (maʻi puʻuwai congenital), kahi e nalowale nei a hoʻomohala pono ʻole ʻia paha ka puʻuwai tricuspid. Hoʻopili ka hemahema i ke kahe o ke koko mai ka atrium ʻākau a i ka ventricle ʻākau. Aia nā hemahema o ka puʻuwai a i ʻole ka moku i ka manawa like.

ʻO Tricuspid atresia kahi ʻano maʻamau o nā maʻi puʻuwai hānau. Hoʻopili ia ma kahi o 5 i kēlā me kēia 100,000 hānau hānau. Hoʻokahi o ka poʻe ʻelima me kēia ʻano e loaʻa pū kekahi i nā pilikia puʻuwai.

ʻO ka maʻamau, kahe ke koko mai ke kino i ke atrium ʻākau, a laila ma o ka pulupulu tricuspid i ka ventricle ʻākau a ma i nā akemāmā. Inā ʻaʻole wehe ka puka tricuspid, ʻaʻole hiki i ke koko ke kahe mai ke atrium akau a i ka ventricle ʻākau. Ma muli o ka pilikia me ka pahū tricuspid, ʻaʻole hiki i ke koko ke komo i loko o nā akemāmā. ʻO kēia kahi e pono ai e hele e ʻohi i ka oxygen (lilo i oxygenated).

Akā, hele ke koko i loko o kahi lua ma waena o ka atrium ʻākau a me ka hema. I ka atrium hema, hui pū ia me ke koko momona o ka oxygen e hoʻi mai ana mai nā akemāmā. Hoʻohui ʻia kēia hui ʻana o ka oxygen-waiwai a me ke koko-ʻilihune i loko o ke kino mai ka ventricle hema. ʻO kēia ke kumu o ka pae o ka oxygen ma ke koko i lalo o ka maʻamau.

I ka poʻe me atresia tricuspid, loaʻa ke akemāmā i ke koko ma o ka puka ma waena o nā ventricle ʻākau a me ka hema (i wehewehe ʻia ma luna), a i ʻole ma o ka mālama ʻana i kahi moku fetal i kapa ʻia ʻo ductus arteriosus. Hoʻopili ka ductus arteriosus i ke aʻa puʻupuʻu (artery to the lungs) i ke aorta (artery main to the body). Aia ia ke hānau ʻia kahi pēpē, akā pani pinepine ʻia e ia ma hope koke iho o ka hānau ʻana.

ʻO nā hōʻailona me:

• ʻO kaʻula Bluish i kaʻili (cyanosis) ma muli o ka haʻahaʻa o ka oxygen i loko o ke koko
• Hanu wikiwiki
• Ka luhi
• Piʻi maikaʻi ʻole
• Pokole o ka hanu

ʻIke ʻia kēia ʻano i ke kiʻi maʻamau o prenatal ultrasound a i ʻole ke nānā ʻia ka pēpē ma hope o ka hānau ʻana. Aia ka ʻili polū i ka wā hānau. Aia pinepine kahi ʻōhumu o ka puʻuwai i ka wā hānau a hoʻonui paha i ka leo nui i loko o kekahi mau mahina.

Hiki i nā hoʻokolohua ke hoʻopili i kēia mau mea:

• ECG
• ʻEchocardiogram
• Pākuʻi x-ray
• ʻO ka catheterization Cardiac
• MRI o ka puʻuwai
• CT scan o ka puʻuwai

Ke hōʻike ʻia ke kuhi, e ʻae pinepine ka pēpē i ka neonatal intensive care unit (NICU). Hiki ke hoʻohana ʻia kahi lāʻau i kapa ʻia ʻo prostaglandin E1 e mālama i ka ductus arteriosis i hiki i ke koko ke holo i nā akemāmā.

ʻO ka maʻamau, ʻo nā mea maʻi me kēia ʻano e koi i ke ʻoki. Inā hiki ʻole i ka puʻuwai ke hoʻoheheʻe i ke koko i waho i nā akemāmā a me ke koena o ke kino, hana pinepine ka ʻoki mua i loko o nā lā mua o ke ola. I kēia kaʻina hana, hoʻokomo ʻia kahi shunt ʻimi e hoʻomau i ke kahe o ke koko i nā akemāmā. I kekahi mau hihia, ʻaʻole pono kēia ʻoki mua.

Ma hope, hoʻi ka pēpē i ka home i ka nui o nā hihia. Pono ke keiki e lawe i hoʻokahi a ʻoi paha o nā lā i kēlā me kēia lā lāʻau lapaʻau a ukali pono ʻia e ka psyatric cardiologist. E hoʻoholo kēia kauka i ka manawa e hana ai ka pae ʻelua.

Kāhea ʻia ka pae hou o ke ʻoki i ke kaʻina hana ʻo Glenn shunt a i ʻole hemi-Fontan. Hoʻohui kēia kaʻina hana i ka hapalua o nā veins e lawe ana i ka oxygen-ke koko ʻilihune mai ka hapalua o luna o ke kino i pololei i ke aʻa o ka pulmonary. Hana pinepine ʻia ke ʻokiʻoki ke ke keiki ma waena o 4 a 6 mau makahiki paha.

Ma ke kahua I a me II, e nānā polū pea ke keiki (cyanotic).

ʻO ke kahua III, ka anuu hope loa, ua kapa ʻia ka hana Fontan. ʻO ke koena o nā uaua e hāpai ana i ka oxygen-ke koko ʻino mai ke kino e hoʻopili pololei ʻia i ke aʻa puʻupuʻu e alakaʻi i nā māmā. ʻO ka ventricle hema i kēia manawa wale nō e pīpī i ke kino, ʻaʻole i nā akemāmā. Hana pinepine ʻia kēia ʻokiʻoki ke keu keiki he 18 mau mahina a i ka 3 mau makahiki. Ma hope o kēia kaʻina hope loa, ʻaʻole uliuli ka ʻili o ka pēpē.

I ka hapanui o nā hihia, hoʻomaikaʻi ke ʻōpala i ke ʻano.

Komo pū nā pilikia:

• Hewa, wikiwiki nā puʻuwai puʻuwai (arrhythmia)
• ʻO ka diarrhea maʻi (mai kahi maʻi i kapa ʻia ʻo enteropathy-protein keu)
• Hewa puʻuwai
• Fluid i loko o ka ʻōpū (ascites) a i nā akemāmā (pleural effusion)
• Ka poloka o ka shunt ʻimi hoʻopunipuni
• Nā hahau a me nā pilikia ʻōnaehana ʻōnaehana ʻē aʻe
• Make make koke

Kāhea koke aku i kāu kahu mālama ola inā loaʻa i kāu pēpē:

• Hoʻololi hou i nā mana hanu
• Pilikia i ka ʻai ʻana
• ʻO ka ʻili e uliuli nei

ʻAʻohe ala i ʻike ʻia e pale aku ai i ka atresia tricuspid.

Tri atresia; Maʻi Valve - tricuspid atresia; Puʻuwai hānau - tricuspid atresia; Maʻi maʻi Cyanotic - tricuspid atresia

• Puʻuwai - ʻāpana ma waena o ka waena
• Tricuspid atresia

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.

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.