Pākuʻi hema hema ventricle

Pālua puka hema hema (DILV) kahi kīnā o ka puʻuwai i kū mai ka hānau (congenital). Hoʻopili ia i nā kiwikā a me nā keʻena o ka puʻuwai. ʻO nā pēpē i hānau ʻia me kēia ʻano he hoʻokahi wale nō keena pumping lumi (ventricle) i loko o ko lākou puʻuwai.

ʻO DILV kekahi o nā kīnā o ka puʻuwai i ʻike ʻia ma ke ʻano he hoʻokahi (a i ʻole maʻamau) nā kīnā o ka ventricle. Loaʻa i ka poʻe me DILV kahi ventricle hema nui a me kahi ventricle ʻākau liʻiliʻi. ʻO ka ventricle hema ka lumi pumping o ka puʻuwai e hoʻouna i ke koko waiwai oxygen i ke kino. ʻO ka ventricle ʻākau ke keʻena pumping e hoʻouna i ke koko-maikaʻi ʻole i nā māmā.

I ka puʻuwai maʻamau, loaʻa nā ventricle ʻākau a hema i ke koko mai ka atria ʻākau a hema. ʻO nā atria nā keʻena kiʻekiʻe o ka puʻuwai.ʻO ke koko maikaʻi ʻole o oxygen e hoʻi mai ana mai ke kino e kahe ana i ka atrium ʻākau a me ka ventricle ʻākau. ʻO ka ventricle ʻākau a laila e pīpī i ke koko i ke aʻa puʻupuʻu. ʻO kēia ke kīʻaha koko e hāpai i ke koko i nā akemāmā e ʻohi i ka oxygen.

ʻO ke koko me ka oxygen hou e hoʻi i ka atrium hema a me ka ventricle hema. Lawe ka aorta i ke koko waiwai oxygen i ke koena o ke kino mai ka ventricle hema. ʻO ke aorta ka aʻa nui e alakaʻi ana i waho o ka puʻuwai.

I ka poʻe me DILV, hoʻomohala wale ka ventricle hema. ʻO ke koko hakahaka ʻelua o atria i loko o kēia ventricle. ʻO kēia ka hui ʻana o ke koko waiwai oxygen me ka oxygen-maikaʻi ʻole o ke koko. Hoʻopili ʻia ka hui ʻana i ke kino a me nā akemāmā.

Hiki i ka DILV ke kū i nā kīʻaha koko nui e kū aʻe mai ka puʻuwai i nā kūlana kūpono ʻole. Kū aʻe ka aorta mai ka ventricle ʻākau liʻiliʻi a kū i luna ke aʻa pulmonary mai ka ventricle hema. Hiki iā ia ke hana i ke ʻano o nā aʻa i nā kūlana maʻamau a ala aʻe mai nā ventricle maʻamau. I kēia hihia, kahe ke koko mai ka hema a i ka ʻākau ventricle ma o kahi puka ma waena o nā keʻena i kapa ʻia he kīnā septal ventricular (VSD).

Kākaʻikahi loa ʻo DILV. ʻAʻole ʻike ʻia ke kumu kūpono. Kūpono ka pilikia ma ka wanaʻao, ke ulu ka puʻuwai o ka pēpē. ʻO ka poʻe me DILV ka pilikia pinepine o ka naʻau, e like me:

• Coarctation o ka aorta (hoʻoliʻiliʻi o ka aorta)
• Pulmonary atresia (ʻaʻohe kūpono kūpono o ka pulmonary valve o ka puʻuwai)
• Pulenary kiwikā stenosis (narrowing o ka pulmonary kiwikā)

Pākuʻi ʻia paha nā ʻōuli o DILV:

• ʻO kaʻula Bluish i kaʻili a me nā lehelehe (cyanosis) ma muli o ka haʻahaʻa o ka oxygen i loko o ke koko
• ʻAʻole e loaʻa ke kaupaona a ulu
• ʻO kaʻili pale (pallor)
• Hānai hānai maikaʻi ʻole i ka luhi ʻana
• Hoʻoulu
• Nā wāwae pehu a ʻōpū paha
• Hī pilikia

Pākuʻi ʻia paha nā hōʻailona o DILV:

• Rhythm puʻuwai maʻamau, e like me ka mea i ʻike ʻia ma ka electrocardiogram
• ʻO ke kūkulu ʻana i ka wai a puni nā akemāmā
• Hewa puʻuwai
• Ohumu puuwai
• Kuʻi puʻuwai wikiwiki

Hoʻokomo ʻia paha nā hoʻāʻo e hōʻike iā DILV:

• Pākuʻi x-ray
• Ke ana o ka hana uila i ka puʻuwai (electrocardiogram, a i ʻole ECG)
• Ke hōʻike ultrasound o ka puʻuwai (echocardiogram)
• Ke hele nei i kahi paipu lahilahi a maʻalahi i ka puʻuwai e nānā i nā aʻa (catheterization cardiac)
• Puʻuwai MRI

Pono ka ʻoki kino e hoʻomaikaʻi i ke kahe o ke koko ma o ke kino a i loko o nā akemāmā. ʻO nā ʻoki maʻamau loa e mālama iā DILV kahi mahele o ʻelua a ʻekolu paha mau hana. Ua like kēia mau ʻoki ʻana me nā mea i hoʻohana ʻia e mālama i ka maʻi puʻuwai hema hypoplastic a me atresia tricuspid.

Pono paha ke ʻōpala mua i ka wā he mau lā wale nō o ka pēpē. I ka hapanui o nā hihia, hiki i ka pēpē ke hoʻi i ka home mai ka haukapila ma hope. Pono ke keiki e lawe pinepine i nā lāʻau i kēlā me kēia lā a ukali pono ʻia e ke kauka puʻuwai pediatric (cardiologist). E hoʻoholo ke kauka o ke keiki i ka manawa e hana ai i ka pae ʻelua.

ʻO ke kīʻaha hou (a i ʻole ke ʻoki mua, inā ʻaʻole pono ka pēpē i kahi kaʻina hana me he keiki hānau hou) ua kapa ʻia ʻo bidirectional Glenn shunt a i ʻole he hana Hemifontan. Hana pinepine ʻia kēia ʻoki ʻana inā 4 a 6 mau makahiki paha ke keiki.

ʻOiai ma hope o nā hana ma luna, e polohi mau paha ke keiki (cyanotic). Kapa ʻia ka anuu hope loa ʻo ke kaʻina hana Fontan. Hana pinepine ʻia kēia ʻokiʻoki ke piha ke keiki i 18 mau mahina a i ka 3 mau makahiki. Ma hope o kēia anuu hope loa, ʻaʻole polū ka pēpē hou.

ʻAʻole hana ka hana Fontan i ka holo maʻamau i ke kino. Akā, hoʻomaikaʻi ia i ke kahe o ke koko e lawa ai no ke keiki e ola a ulu ai.

Pono paha kahi keiki i kahi ʻoki hou aʻe no nā kīnā ʻē aʻe a i ʻole e hoʻolōʻihi i ke ola ke kali nei no ke kaʻina hana Fontan.

Pono paha kāu keiki e lawe i nā lāʻau ma mua a ma hope o ke kaha ʻana. Pākuʻi ʻia kēia mau mea:

• ʻO nā Anticoagulants e pale i ka hoʻopili ʻana o ke koko
• Nā mea pāpā ACE e hōʻemi i ke kahe o ke koko
• Digoxin e kōkua i ka ʻaelike puʻuwai
• Pila wai (diuretics) e hoʻemi i ka pehu ʻana i loko o ke kino

Kūkā ʻia kahi transplant puʻuwai, ke holo pono ʻole nā ​​hana i luna.

He maʻi puʻuwai paʻakikī ʻo DILV i maʻalahi ʻole e mālama. Maikaʻi ka hilinaʻi ʻana o ka pēpē i:

• ʻO ke kūlana holoʻokoʻa o ka pēpē i ka manawa o ke kuhi a me ka lāʻau.
• Inā loaʻa nā pilikia puʻuwai ʻē aʻe.
• Pehea ka ʻino o ka hemahema.

Ma hope o ka lapaʻau ʻana, nui nā pēpē me DILV e noho makua. Akā, e koi lākou i nā ukali o ke ola. Hiki paha iā lākou ke alo i nā pilikia a ua kaupalena paha i kā lākou hana kino.

Nā pilikia o DILV me:

• ʻO ka clubbing (ka mānoanoa o nā moena kui) ma nā manamana wāwae a me nā manamana lima (hōʻailona hopena)
• Hewa puʻuwai
• Pneumonia pinepine
• Nā pilikia puʻuwai puʻuwai
• Make

Kāhea i kāu kahu mālama ola inā kāu keiki:

• Me ka maʻalahi o ka luhi
• He pilikia i ka hanu ʻana
• He ʻili a he lehelehe paha kona

E kamaʻilio pū i kāu mea hāʻawi inā ʻaʻole e ulu ana kāu pēpē a loaʻa ʻole paha ke kaumaha.

ʻAʻohe pale e ʻike ʻia.

DILV; Single ventricle; ʻO ka ventricle maʻamau; Puʻuwai univentricular; Puʻuwai univentricular o ka ʻano ventricular hema; Hapa puʻuwai Congenital - DILV; Hewa naʻau cyanotic - DILV; Hewa hānau - DILV

• Pākuʻi hema hema ventricle

ʻO Kanter KR. ʻO ka mālama ʻana i ka ventricle hoʻokahi a me nā pilina cavopulmonary. I: Sellke FW, del Nido PJ, Swanson SJ, eds. ʻO Sabiston lāua ʻo Spencer Surgery o ka umauma. 9th ed. Piladelapia, PA: Elsevier; 2016: chap 129.

ʻO Kliegman RM, St. Geme JW, Blum NJ. Shah SS, Tasker RC, Wilson KM. ʻO Schor NF. ʻO ka maʻi puʻuwai Cyanotic congenital: nā leona e pili ana me ka hoʻonui ʻia o ke kahe o ka pulmonary. 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 458.

ʻO Wohlmuth C, Gardiner HM. Ka puʻuwai. I: Pandya PP, Oepkes D, Sebire NJ, Wapner RJ, eds. Ka lāʻau lapaʻau Fetal: ʻEpekema ʻepekema a me nā hana lapaʻau. ʻEkolu huaʻōlelo. Piladelapia, PA: Elsevier; 2020: chap 29.