Hewa cushion Endocardial

He kīnā ʻole naʻau endocardial cushion (ECD) ʻO nā paia e hoʻokaʻawale ana i nā keʻena ʻehā o ka puʻuwai e hana maikaʻi ʻole a hele ʻole paha. Eia kekahi, ʻo nā kiwikā e hoʻokaʻawale ana i nā keʻena luna a me lalo o ka puʻuwai he mau kīnā ʻole i ka wā o ka hoʻokumu ʻana. He maʻi puʻuwai hānau ʻo ECD, ʻo ia hoʻi aia ia mai ka wā hānau.

Kū ʻo ECD ʻoiai e ulu ana kahi pēpē i loko o ka ʻōpū. ʻO nā cushions endocardial ʻelua mau mānoanoa e hoʻomohala i nā paia (septum) e hoʻokaʻawale i nā keʻena ʻehā o ka puʻuwai. Hoʻokumu pū lākou i nā kiwikā mitral a me tricuspid. ʻO kēia nā kiwikā e hoʻokaʻawale i ka atria (luna e hōʻiliʻili ana i nā keʻena) mai nā ventricle (nā keʻena pumping o lalo).

ʻO ka nele o ka hoʻokaʻawale ma waena o nā ʻaoʻao ʻelua o ka puʻuwai e hana i kekahi mau pilikia:

• Hoʻonui i ke kahe o ke koko i nā akemāmā. ʻO kēia ka hopena i ka hoʻonui ʻia o ke kaomi i nā māmā. I ka ECD, kahe ke koko ma o ka puka wehe ʻole mai ka hema a i ka ʻaoʻao ʻākau o ka puʻuwai, a laila i nā akemāmā. ʻO ke kahe hou ʻana o ke koko i loko o nā akemāmā e piʻi ai ke kiʻekiʻe o ke koko i nā māmā.
• Hewa puʻuwai. ʻO ka hana hou e pono ai ka pauma e hana ka naʻau i ka ʻoi aku ka paʻakikī ma mua o ka maʻamau. Hiki ke hoʻonui ka nāwaliwali o ka puʻuwai a hoʻonāwaliwali hoʻi. Hiki i kēia ke hoʻoulu i ka pēpē, nā pilikia i ka hanu ʻana, a me ka paʻakikī i ka hānai ʻana a me ka ulu ʻana.
• ʻO Cyanosis. Ke piʻi nei ke kiʻekiʻe o ke koko i nā akemāmā, hoʻomaka ke kahe o ke koko mai ka ʻaoʻao ʻākau o ka puʻuwai i ka hema. Hoʻohui ke koko ʻilihune oxygen me ke koko momona o ka oxygen. A ʻo kahi hopena, hoʻokahe ʻia ke koko me ka oxygen liʻiliʻi ma mua o ka maʻamau. ʻO kēia ke kumu o ka cyanosis, a i ʻole ka ʻili bluish.

ʻElua ʻano o ECD:

• Piha ECD. Hoʻopili kēia ʻano i ka hemahema septial atrial (ASD) a me kahi kīnā septal ventricular (VSD). Loaʻa i ka poʻe me ka ECD piha hoʻokahi hoʻokahi puʻuwai puʻuwai nui (ka pauma AV maʻamau) ma kahi o ʻelua mau kiwikā ʻokoʻa (mitral a me tricuspid).
• Māhele (a i ʻole piha) ECD. I kēia kūlana, he ASD wale nō, a i ʻole ASD a me VSD e noho nei. Aia he ʻelua mau kiʻiona ʻokoʻa, akā ʻo kekahi o lākou (ka mitral valve) he mea maʻamau ʻole me ka wehe ("cleft") i loko. Hiki i kēia kīnā ke hoʻokahe i ke koko ma o ka paula.

Ua hoʻopili ikaika ʻia ʻo ECD i Down Syndrome. Pili pū kekahi mau hoʻololi kūʻē i ka ECD. Eia nō naʻe, ʻaʻole ʻike ʻia ke kumu kūpono o ka ECD.

E pili pū paha ʻo ECD me nā kīnā o ka naʻau congenital, e like me:

• Pālua ventricle ʻaoʻao ʻākau
• Ventricle hoʻokahi
• ʻO ka Transposition o nā moku nui
• ʻO Tetralogy o Fallot

Pākuʻi ʻia paha nā ʻōuli o ECD:

• Pōhaku na pēpē
• ʻO kaʻili o kaʻili polū, ʻike ʻia ʻo cyanosis (polū paha nā lehelehe)
• Pilikia hānai
• ʻAʻole e loaʻa ke kaupaona a ulu
• ʻO ka maʻi pneumonia pinepine a maʻi paha
• ʻO kaʻili pale (pallor)
• Hanu wikiwiki
• Kuʻi puʻuwai wikiwiki
• Hoʻoulu
• Nā wāwae pehu a ʻōpū paha (ʻike pinepine ʻia i nā keiki)
• Hoʻopilikia i ka hanu ʻana, ʻoiai hoʻi i ka wā e hānai ana

I ka wā o kahi hoʻokolohua, ʻike paha ka mea mālama ola i nā hōʻailona o ECD, me:

• ʻO kahi electrocardiogram abnormal (ECG)
• Hoʻonui ʻia ka puʻuwai
• Ohumu puuwai

ʻAʻole hiki i nā keiki me ka hapa ECD i nā hōʻailona a i ʻole nā ​​ʻōuli o ka maʻi i ka wā kamaliʻi.

ʻO nā hoʻokolohua e kuhikuhi iā ECD me:

• ʻO Echocardiogram, kahi ultrasound e ʻike i nā ʻano puʻuwai a me ke kahe o ke koko i loko o ka puʻuwai
• ECG, kahi e ana ai i ka hana uila o ka puʻuwai
• Pākuʻi x-ray
• MRI, kahi e hāʻawi ai i kahi kiʻi kikoʻī o ka puʻuwai
• Cardiac catheterization, kahi hana e hoʻokomo ʻia ai kahi paipu lahilahi (catheter) i loko o ka puʻuwai e ʻike i ke kahe o ke koko a lawe i nā ana pono o ke kaomi koko a me nā pae oxygen.

Pono ka ʻoki kino e pani i nā lua ma waena o nā keʻena puʻuwai, a hana i nā valves tricuspid a me mitral ʻokoʻa. Aia ka manawa o ke ʻoki ʻana i ke kūlana o ke keiki a me ke koʻikoʻi o ka ECD. Hiki ke hana pinepine ʻia ke 3 a 6 mau makahiki paha o ka pēpē. ʻO ka hoʻoponopono ʻana i kahi ECD koi paha ma mua o hoʻokahi ʻoki.

Kauoha ke kauka o kāu keiki i ka lāʻau:

• E mālama i nā hōʻailona o ka puʻuwai puʻuwai
• Ma mua o ke kaha ʻana inā ua maʻi ʻo ECD i kāu pēpē

E kōkua nā lāʻau i kāu keiki e loaʻa ka paona a me ka ikaika ma mua o ke kaha ʻana. Hoʻohana pinepine ʻia nā lāʻau i:

• Diuretics (wai huaale)
• ʻO nā lāʻau i hana i ka puʻuwai e ʻaelike me ka ikaika, e like me digoxin

Pono e hana i ke kaha no kahi ECD piha i ka makahiki mua o ke pēpē o ke ola. Inā ʻole, hiki i ka hōʻino o ka māmā i hiki ʻole ke hoʻohuli ʻia. ʻO nā pēpē me Down Syndrome e hoʻomohala i nā maʻi māmā ma mua. No laila, he mea nui ka ʻoki mua no kēia mau pēpē.

Maikaʻi ka hilinaʻi o kāu pēpē:

• ʻO ke koʻikoʻi o ka ECD
• ʻO ke olakino olakino o ke keiki
• Inā ua ulu mua ka maʻi maʻi

Noho nā keiki he nui i nā ola maʻamau, nā ola hana ma hope o ka hoʻoponopono ʻia ʻana o ka ECD.

Komo pū nā pilikia mai ECD:

• Hoka puʻuwai Congestive
• Make
• Eisenmenger maʻi
• ʻO ke kiʻekiʻe o ke koko i nā akemāmā
• Hoʻopilikia kūpono ʻole i nā māmā

ʻAʻole hiki ke ʻike ʻia kekahi mau pilikia o ke kahakaha ECD a hiki i ke keiki makua. Hoʻopili kēia i nā pilikia o ka puʻuwai puʻuwai a me ka valve mitral leaky.

Pilikia paha nā keiki me ka ECD no ka maʻi o ka puʻuwai (endocarditis) ma mua a ma hope o ke kaha ʻana. E noi i ke kauka o kāu keiki inā pono kāu keiki e lawe i nā lāʻau ʻaloʻalo ma mua o kekahi mau kaʻina niho.

Kāhea i ka hāʻawi o kāu keiki inā kāu keiki:

• Pākuʻi maʻalahi
• He pilikia i ka hanu ʻana
• He ʻili a he lehelehe paha kona

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

Hoʻopili ʻia ʻo ECD me kekahi mau ʻano ʻē aʻe. Makemake paha nā kāne me ka mōʻaukala ʻohana o ECD e ʻimi i nā ʻōlelo aʻoaʻo kūlohelohe ma mua o ka hāpai ʻana.

Hewa kīnā ʻo Atrioventricular (AV); Hewa ʻino Atrioventricular; AVSD; Orifice AV maʻamau; Nā hemahema o ka ʻostium primum atrial septal; Hapa puʻuwai Congenital - ECD; Hewa hānau - ECD; Maʻi Cyanotic - ECD

• Hewa septal ʻo Ventricular
• Hewa septal Atrial
• Kanal Atrioventricular (kīnā ʻole cushion endocardial)

ʻO Basu SK, Dobrolet NC. Nā kīnā hānau o ka ʻōnaehana cardiovascular. I: Martin RJ, Fanaroff AA, Walsh MC, hua. ʻO Fanaroff lāua ʻo Martin's Neonatal-Perinatal Medicine. 11th hua. Piladelapia, PA: Elsevier; 2020: chap 75.

ʻO Ebels T, Tretter JT, Spicer DE, Anderson RH. Nā hemahema septtro Antroventricular. I: Wernovsky G, Anderson RH, Kumar K, et al. Ka Pediatric Cardiology o Anderson. ʻEhā ed. Piladelapia, PA: Elsevier; 2020: chap 31.

ʻO Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. ʻO ka maʻi puʻuwai acyanotic: nā liona shunt hema-ʻākau. 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 453.