ʻO ke kiwikā ākea Bicuspid

ʻO kahi kiwikā aicic bicuspid (BAV) kahi kiwikā aortic i loaʻa i ʻelua mau leaflets wale nō, ma kahi o ʻekolu.

Hoʻonohonoho ka valve aortic i ke kahe o ke koko mai ka puʻuwai i ke aorta. ʻO ke aorta ke kīʻaha koko nui e lawe mai i ke koko momona i ke kino.

Hāʻawi ka valve aortic i ke koko waiwai o oxygen e kahe mai ka puʻuwai a ke aorta. Pale ia i ke kahe o ke koko mai ke aorta a i ka puʻuwai ke hoʻomaha ka lumi pauma.

Aia ʻo BAV i ka wā hānau (congenital). Hoʻomohala ʻia kahi kiwikā aortic maʻamau i nā pule mua o ka hāpai ʻana, ke ulu nei ka puʻuwai o ka pēpē. ʻAʻole maopopo ke kumu o kēia pilikia, akā ʻo ia ka maʻi puʻuwai congenital ʻoi loa. Holo pinepine ʻo BAV i loko o nā ʻohana.

ʻAʻole maikaʻi paha kahi BAV i ka hoʻōki ʻana i ke koko mai ka hoʻi hou ʻana i ka puʻuwai. Kapa ʻia kēia leakage regurgitation aortic. E hele a paʻa hoʻi ka aorta aʻole e wehe. Kapa ʻia kēia aortic stenosis, ka mea e kumu i ka puʻuwai e pauma ʻoi aku ka paʻakikī ma mua o ka maʻamau e loaʻa i ke koko ma o ka va. Hiki ke hoʻonui ʻia ka aorta me kēia ʻano.

ʻOi aku ka maʻamau o ka BAV ma waena o nā kāne ma mua o nā wahine.

Noho mau kahi BAV i loko o nā pēpē me ka coarctation o ka aorta (ʻākiki o ka aorta). ʻIke ʻia ka BAV i nā maʻi i loaʻa kahi ālai i ke kahe o ke koko ma ka ʻaoʻao hema o ka puʻuwai.

ʻO ka hapa nui o ka manawa, ʻaʻole ʻike ʻia ka BAV i loko o nā pēpē a i ʻole nā ​​keiki no ka mea ʻaʻohe kumu. Eia nō naʻe, hiki i ke kiwikā maʻamau ke kulu a hele a hāiki paha i ka manawa.

ʻO nā ʻōuli o ia ʻano pilikia e hoʻopili pū paha:

• Pōpē wale ka pēpē a keiki paha
• ʻEha ʻeha
• Hanu paʻakikī
• ʻO ke kuʻi puʻuwai wikiwiki a kaulike ʻole (palpitations)
• Ka nalowale o ka ʻike (maʻule)
• ʻIli pale

Inā loaʻa i nā pēpē kekahi mau pilikia puʻuwai congenital, hana paha lākou i mau hōʻailona e alakaʻi i ka loaʻa ʻana o kahi BAV.

I ka wā o kahi hoʻokolohua, hiki i ka mea mālama ola ke loaʻa nā hōʻailona o kahi BAV me:

• Puʻuwai hoʻonui ʻia
• Ohumu puuwai
• Pulse nāwaliwali i nā pulima a me nā kuʻekuʻe wāwae

ʻO nā hoʻokolohua i kauoha ʻia e komo pū:

• MRI, kahi e hāʻawi ai i kahi kiʻi kikoʻī o ka puʻuwai
• ʻO Echocardiogram, kahi ultrasound e ʻike i nā ʻano puʻuwai a me ke kahe o ke koko i loko o ka puʻuwai

Inā manaʻo ka mea hāʻawi i nā pilikia a i ʻole nā ​​kīnā o ka puʻuwai hou, e hoʻopili ʻia nā hoʻokolohua ʻē aʻe:

• Pākuʻi x-ray
• Electrocardiogram (ECG), kahi e ana ai i ka hana uila 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.
• MRA, kahi MRI e hoʻohana i kahi kala e ʻike ai i nā kīʻaha koko o ka puʻuwai

Pono paha ke pēpē a keiki paha i kahi ʻoki e hoʻoponopono a pani ai i kahi leaky a i ʻole pāʻī ʻia, inā he koʻikoʻi nā pilikia.

Hiki ke wehe ʻia kahi kiwikā liʻiliʻi ma o ka catheterization puʻuwai. Kuhi ʻia kahi ʻōmole maikaʻi (catheter) i ka puʻuwai a i loko o ka hāiki hāmama o ka nohona aorta. Hoʻopili ʻia kahi pāluna i ka hopena o ka Tube e hoʻonui i ka wehe o ka puka.

I ka poʻe mākua, ke lilo ka paipu bicuspid i mea leaky a iiki loa paha, pono paha e hoʻololi.

I kekahi manawa pono e hoʻoponopono hou ʻia ke aorta inā ua ākea a ʻākiki loa paha.

Pono ʻia ka lāʻau e hōʻoluʻolu i nā ʻōuli a pale i nā pilikia. Pākuʻi ʻia paha nā lāʻau:

• Nā lāʻau i hoʻohaʻahaʻa i ka ukana hana ma ka puʻuwai (beta-blockers, ACE inhibitors)
• Nā lāʻau e hoʻonā i ka puʻuwai puʻuwai paʻakikī (inotropic agents)
• Pila wai (diuretics)

Aia ka maikaʻi o ka pēpē i ka hiki a me ke koʻikoʻi o nā pilikia o BAV.

ʻO ke kū ʻana o nā pilikia kino ʻē aʻe i ka wā hānau hiki ke hoʻopili i ka maikaʻi o ka pēpē.

ʻO ka hapa nui o nā pēpē me kēia ʻano ʻaʻohe o lākou hōʻailona, ​​a ʻaʻole ʻike ʻia ka pilikia a hiki i ko lākou wā makua. ʻAʻole ʻike kekahi poʻe ua loaʻa iā lākou kēia pilikia.

Nā pilikia o BAV me:

• Hewa puʻuwai
• Kulu o ke koko ma o ka pahu i hoʻi i loko o ka puʻuwai
• Pāliʻi ʻia o ka wehe o ka puka
• ʻO ka maʻi o ka puʻuwai puʻuwai a i ʻole ke kiwikā aorta

Kāhea i ka hāʻawi o kāu keiki inā kāu pēpē:

• ʻAʻohe ona makemake
• ʻIli ʻulaʻula a ʻulaʻula paha
• Me ka maʻalahi o ka luhi

Holo ʻo BAV i loko o nā ʻohana. Inā ʻike ʻoe i kēia ʻano i kou ʻohana, e ʻōlelo i kāu mea hoʻolako ma mua o ka hāpai ʻana. ʻAʻohe ala i ʻike ʻia e pale ai i ke ʻano.

Kiwikā ʻaʻano Bicommissural; Maʻi valvular - bicuspid aortic valve; ʻO BAV

• ʻO ke kīpoki puʻuwai puʻuwai - hoʻokuʻu

• ʻO ke kiwikā ākea Bicuspid

Borger MA, Fedak PWM, Stephens EH, et al. Nā kuhikuhi kuʻikahi AATS e pili ana i ka aortopathy e pili ana i ka aorta bicuspid: piha piha ma ka pūnaewele wale nō. ʻO J Thorac Cardiovasc Surg. 2018; 156 (2): e41-74. hana: 10.1016 / j.jtcvs.2018.02.115. PMID: 30011777 pubmed.ncbi.nlm.nih.gov/30011777/.

ʻO Braverman AC, ʻo Cheng A. ʻO ke kiʻiona aorta bicuspid a me ka maʻi ʻaʻa e pili ana. I: Otto CM, Bonow RO, hoʻoponopono. ʻO ka maʻi puʻuwai valvular: kahi hoa hele i ka maʻi puʻuwai o Braunwald. 5th lā. Piladelapia, PA: Elsevier; 2021: chap 11.

Fraser CD, Cameron DE, McMillan KN, Vricella LA. Nā maʻi puʻuwai a me nā maʻi maʻi hoʻopili. 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 53.

ʻO Lindman BR, Bonow RO, Otto CM. Maʻi maʻi aorta. 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 68.