Wahi pulmonary nele

ʻO ke kīʻaha pulmonary absent kahi hemahema kīnā kahi o ka pulmonary valve e nalowale ana a i hana maikaʻi ʻole ʻia paha. Kahe ka oxygen-wai maikaʻi ʻole ma waena o kēia kiwikā mai ka puʻuwai a i nā akemāmā, kahi e ʻohi ai i ka oxygen hou. Aia kēia ʻano i ka wā hānau (congenital).

Kūpono ke kīpē pulmonary absent ke hana ʻole ka valve pulmonary a hoʻomohala pono paha i ka wā o ka pēpē i loko o ka ʻōpū o ka makuahine. Ke loaʻa, hana pinepine ia ma ke ʻano he ʻano puʻuwai i kapa ʻia ʻo tetralogy o Fallot. Loaʻa ia ma kahi o 3% a i 6% o ka poʻe i loaʻa ka tetralogy o Fallot.

Ke nalowale nei a ʻole holo pono ka pahū pulmonary, ʻaʻole kahe pono ke kahe i ke akemāmā e loaʻa ai ka oxygen.

I ka hapanui o nā hihia, aia kekahi puka ma waena o ka ventricle hema a me ka ʻākau o ka puʻuwai (ventricular septal defect). ʻO kēia kīnā e alakaʻi pū ʻia hoʻi i ke kahe o ka oxygen haʻahaʻa e kaʻi ʻia i waho i ke kino.

E loaʻa i ka ʻili kahi hiʻohiʻona polū (cyanosis), no ka mea, he haʻahaʻa ka oxygen o ke kino o ke kino.

ʻO ka hopena o ka pulmonary absent ka hopena i hoʻonui ʻia (dilated) nā aʻa pulmonary lālā (nā aʻa e hāpai i ke koko i nā akemāmā e kiʻi i ka oxygen). Hiki iā lākou ke hoʻonui aʻe a kaomi lākou i nā paipu e lawe i ka oxygen i loko o nā akemāmā (bronchi). ʻO kēia ke kumu o nā pilikia hanu.

ʻO nā kīnā ʻole o ka puʻuwai e hiki ke hana me ka valve pulmonary absent e komo pū me:

• ʻO ka paepae tricuspid maʻamau
• Hewa septal Atrial
• Pālua ventricle ʻaoʻao ʻākau
• ʻO Ductus arteriosis
• Hewa cushion Endocardial
• Maʻi ʻo Marfan
• Tricuspid atresia
• ʻO ke aʻa aʻa hema hema

ʻO nā pilikia puʻuwai e kū nei me ka pulmonary valve absent ma muli o nā hemahema i kekahi mau ʻāpana.

Hiki ke loli nā ʻōuli ma muli o nā kīnā ʻē aʻe o ke pēpē, akā hiki ke hoʻopili ʻia:

• ʻO ka pena ʻulaʻula i ka ʻili (cyanosis)
• ʻ Clelo huna
• Ka holomua ʻole ʻana
• Makemake maikaʻi ʻole
• Hanu wikiwiki
• Hoʻoikaika hanu ʻole
• ʻUhihi

Hiki ke ʻike ʻia ka valve pulmonary absent ma mua o ka hānau ʻia ʻana o ka pēpē me kahi hōʻike e hoʻohana ai i nā nalu kani e hana i kiʻi o ka puʻuwai (echocardiogram).

I ka wā o kahi hoʻokolohua, hiki i ka mea mālama ola ke lohe i kahi ʻōhumu i ka umauma o ka pēpē.

ʻO nā hoʻāʻo no ka pulmonary valve absent e komo pū:

• ʻO kahi hōʻoia e ana i ka hana uila o ka puʻuwai (electrocardiogram)
• Scanner CT Heart
• Pākuʻi x-ray
• ʻEchocardiogram
• ʻImehana resonance kiʻi (MRI) o ka puʻuwai

ʻO nā pēpē i loaʻa i nā ʻōuli hanu pono pono ke ʻoki koke. ʻO nā pēpē me ka ʻole o nā ʻōuli ʻino e hana pinepine ʻia i loko o ka 3 a 6 mau mahina o ke ola.

Kaukaʻi ʻia i ke ʻano o nā kīnā o nā puʻuwai i loaʻa i ke pēpē, pili paha ke ʻoki ʻana:

• Ke pani nei i ka lua i ka paia ma waena o ka ventricle hema a me ka akau o ka puʻuwai (ventricular septal defect)
• ʻO ka pani ʻana i kahi kīʻaha koko e hoʻopili i ka aorta i ke aʻa o ka pulmonary (ductus arteriosis)
• Ke hoʻonui nei i ke kahe mai ka ventricle ʻākau i nā akemāmā

Nā ʻano o ke ʻoki ʻana no ka valve pulmonary absent e komo pū:

• Ke hoʻoneʻe nei i ke aʻa o ka pulmonary i mua o ke aorta a mamao aku mai nā alaloa
• ʻO ke kūkulu hou ʻana i ka paia artery i nā akemāmā e hoʻēmi i ke kaomi ma nā alaloa (pulmonary plication a me ka hoʻēmi arterioplasty)
• Ke kūkulu hou ʻana i ka ʻōpala a me nā paipu hanu i nā ake māmā
• Ke pani nei i ka nohona pulmonary abnormal me kekahi i lawe ʻia mai ke kino kanaka a holoholona paha

Pono paha nā kamaliʻi me nā ʻōuli hanu nui e loaʻa ka oxygen a kau ʻia ma luna o kahi mīkini hanu (ventilator) ma mua a ma hope o ke kaha ʻana.

Me ka ʻole o ke kaha ʻana, e make ka hapa nui o nā pēpē i loaʻa i nā kūpikipiki maʻi māmā

I nā manawa he nui, hiki i ke kīpoki ke mālama i ke ʻano a hōʻoluʻolu i nā ʻōuli. ʻO nā hopena pinepine maikaʻi loa.

Komo pū nā pilikia:

• Maʻi maʻiʻaʻai (abscess)
• Hū ka maʻi (atelectasis)
• Pulumonia
• Hoka puʻuwai ʻaoʻao ʻākau
• Hahau

Kāhea i kāu mea hāʻawi inā loaʻa kāu pēpē i nā ʻōuli o ka abs pulmonary valve. Inā loaʻa iā ʻoe kahi moʻolelo ʻohana o nā kīnā o ka puʻuwai, e kamaʻilio me kāu mea hoʻolako i mua a i ʻole i ka wā hāpai.

ʻOiai ʻaʻohe ala e pale ai i kēia ʻano, e loiloi ʻia paha nā ʻohana e hoʻoholo ai i ko lākou pilikia no nā kīnā hānau.

Loaʻa ka maʻi o ka pulmonary valve; Ka nele o ka nohona kiwikā; Pulseary valve agenesis; Maʻi maʻi puʻuwai Cyanotic - pulmonary valve; Maʻi maʻi Congenital - pulmonary valve; Hanau kīnā ʻole o ka hānau

• Wahi pulmonary nele
• ʻO Cyanotic 'Spell spell'
• ʻO Tetralogy o Fallot

ʻO Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. ʻO ka maʻi puʻuwai acyanotic: nā liona regurgitant. 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 455.

ʻO Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Nā maʻi puʻuwai cyanotic congenital: nā leona e pili ana me ka hoʻemi 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 457.

ʻO Scholz T, Reinking BE. Maʻi maʻi ʻōpū. I: Gleason CA, Juul SE, eds. Nā maʻi o Avery o ke keiki hānau. 10 o ka lā. Piladelapia, PA: Elsevier; 2018: chap 55.

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.