Pālua ventricle ʻaoʻao ʻākau

ʻO ka ventricle ʻaoʻao ʻākau puka pāpālua (DORV) kahi maʻi puʻuwai e kū mai ka hānau (congenital). Hoʻohui ka aorta i ka ventricle ʻākau (RV, ke keena o ka puʻuwai e pīpī i ke koko o ka oxygen-maikaʻi ʻole i nā akemāmā), ma kahi o ka ventricle hema (LV, ke keʻena e hoʻomaʻamaʻa maʻamau i ke koko waiwai oxygen i ke kino).

ʻO ke aʻa o ka pulmonary (ka mea lawe i ke koko nawaliwali o ka oxygen i nā akemāmā) a me ke aorta (e lawe ana i ke koko momona o ka oxygen mai ka puʻuwai a i ke kino) mai ka lumi pumping hoʻokahi ʻAʻole pili nā aʻa i ka ventricle hema (ke keena e ninini maʻamau i ke koko i ke kino).

I loko o kahi naʻau maʻamau, hoʻopili ka aorta i ka LV. Hoʻopili maʻamau ke aʻa pulmonary i ka RV. I DORV, kahe nā aʻa ʻelua i waho o ka RV. He pilikia kēia ma muli o ka lawe ʻana o ka RV i ke koko ʻilihune o ka oxygen. Hoʻomāhele ʻia kēia koko i loko o ke kino.

ʻO kekahi kīnā i kapa ʻia he kīnā septal ventricular (VSD) i nā manawa āpau me DORV.

ʻO ke koko momona o ka oxygen mai nā akemāmā e kahe ana mai ka ʻaoʻao hema o ka puʻuwai, ma o ka wehe ʻana o VSD a i ka RV. Kōkua kēia i ke pēpē me DORV ma ka ʻae ʻana i ke koko momona o oxygen e hoʻohuihui me ke koko maikaʻi ʻole o oxygen. ʻOiai me kēia hui ʻana, ʻaʻole lawa paha ka oxygen i ke kino. Hana nui kēia i ka puʻuwai e hoʻokō i nā pono o ke kino. Nui a hewahewa nā ʻano like o DORV.

ʻO ka ʻokoʻa ma waena o kēia mau ʻano ka wahi o ka VSD e pili ana i ka wahi o ke aʻa aʻa a me ka aorta. E pili nā ʻōuli a me ke koʻikoʻi o ka pilikia i ke ʻano o DORV. ʻO ke kū ʻana o ka stenosis pulmonary valve e hoʻopili pū i ke ʻano.

Loaʻa pinepine ka poʻe me DORV i nā kīnā o ka naʻau, e like me:

• Endocardial cushion defect (ʻo nā paia e hoʻokaʻawale ana i nā keʻena ʻehā o ka puʻuwai e hana maikaʻi ʻole ʻia a i ʻole ʻole)
• Coarctation o ka aorta (hoʻoliʻiliʻi o ka aorta)
• Nā pilikia mitral valve
• Pulmonary atresia (ʻaʻohe kūpono kūpono o ka pulmonary valve)
• Pulenary kiwikā stenosis (narrowing o ka pulmonary kiwikā)
• Austic ʻaoʻao ʻaoʻao ʻākau (aia ke ākea aortic ma ka ʻākau ma kahi o ka hema)
• Transposition o nā aʻa nui (ua hoʻololi ʻia ke aorta a me nā aʻa pulmonary)

Pākuʻi ʻia paha nā hōʻailona o DORV:

• Puʻuwai hoʻonui ʻia
• Ohumu puuwai
• Hanu wikiwiki
• Kuʻi puʻuwai wikiwiki

Pākuʻi ʻia paha nā ʻōuli o DORV:

• Hānai hānai maikaʻi ʻole i ka luhi ʻana
• ʻO kaʻula Bluish o kaʻili a me nā lehelehe
• ʻO ka clubbing (ka mānoanoa o nā moena kui) o nā manamana wāwae a me nā manamana lima (hōʻailona hope)
• ʻAʻole e loaʻa ke kaupaona a ulu
• Pale kala
• Hoʻoulu
• Nā wāwae pehu a ʻōpū paha
• Hī pilikia

ʻO nā hoʻokolohua e kuhikuhi iā DORV:

• Nā kukui uila
• Ke hōʻike ultrasound o ka puʻuwai (echocardiogram)
• Ke hele nei i kahi paipu lahilahi a maʻalahi i ka puʻuwai e ana i ke kahe o ke koko a hoʻokomo i ka pena no nā kiʻi kūikawā o ka puʻuwai a me nā aʻa (catheterization cardiac)
• Puʻuwai MRI

Pono ka lapaʻau i ka ʻoki ʻana e pani i ka lua o ka puʻuwai a kuhikuhi i ke koko mai ka ventricle hema i ka aorta. Pono paha ke ʻoki e neʻe i ke aʻa aʻa a i ʻole aorta.

Nā kumu e hoʻoholo ai i ke ʻano a me ka helu o nā hana a ka pēpē e pono ai:

• Ke ʻano o DORV
• ʻO ke koʻikoʻi o ka kīnā
• Ke kū nei o nā pilikia ʻē aʻe i ka puʻuwai
• ʻO ke kūlana holoʻokoʻa o ke keiki

Maikaʻi ka hilinaʻi ʻana o ka pēpē i:

• Ka nui a me kahi o ka VSD
• ʻO ka nui o nā keʻena pumping
• ʻO kahi o ke aorta a me ke aʻa aʻa
• Ke kū ʻana o nā pilikia ʻē aʻe (e like me ka coarctation o ka aorta a me nā pilikia mitral valve)
• ʻO ke olakino olakino o ke pēpē i ka manawa o ka diagnostics
• Inā paha ua hōʻino ka lolo mai ka nui o ke koko e kahe ana i nā akemāmā no kahi manawa lōʻihi

Komo paha nā pilikia mai DORV:

• Hewa puʻuwai
• ʻO ke koko kiʻekiʻe i loko o nā akemāmā, ka mea i mālama ʻole ʻia e hiki ai i ka hōʻino mau loa o ka māmā
• Make

Pono paha nā keiki me kēia ʻano puʻuwai e lawe i nā lāʻau antibiotic ma mua o ka mālama ʻana i nā niho. Pale kēia i nā maʻi a puni ka puʻuwai. Pono paha nā lāʻau antibiotic ma hope o ke kaha ʻana.

Kāhea i kāu mea mālama ola inā luhi maʻalahi kāu keiki, pilikia i ka hanu ʻana, a i ʻole ka ʻili a me nā lehelehe bluish. Pono ʻoe e nīnau aku i kāu mea hāʻawi inā ʻaʻole e ulu ana kāu pēpē a loaʻa ʻole paha ke kaumaha.

DORV; Taussig-Bing anomaly; DORV me VSD i hana pālua ʻia; DORV me VSD noncommitted; ʻO DORV me ka VSD subaortic; Hapa puʻuwai Congenital - DORV; Hewa naʻau cyanotic - DORV; Hānau hānau - DORV

• Pālua ventricle ʻaoʻao ʻākau

ʻO Bichell D. ventricle ʻaoʻao pālua. I: Ungerleider RM, Meliones JN, McMillian KN, Cooper DS, Jacobs JP, hua. ʻO ka maʻi puʻuwai koʻikoʻi i nā pēpē a me nā keiki. ʻEkolu huaʻōlelo. Piladelapia, PA: Elsevier; 2019: chap 58.

ʻO Haller C, Van Arsdell GS, Yoo S-J, George-Hyslop CST, Spicer DE, Anderson A. ʻO ventricle pālua. I: Wernovsky G, Anderson RH, Kumar K, et al. Ka Pediatric Cardiology o Anderson. ʻEhā ed. Piladelapia, PA: Elsevier; 2020: chap 39.

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.