Pulseary atresia

ʻO ka atresia pulmonary kahi ʻano o ka maʻi puʻuwai kahi e kū pono ʻole ai ka pahu pulmonary. Aia ia mai ka wā hānau (maʻi puʻuwai congenital). ʻO ka pahū pulmonary kahi hāmama ma ka ʻaoʻao ʻākau o ka puʻuwai e hoʻoponopono ai i ke kahe o ke koko mai ka ventricle ʻākau (ʻaoʻao ʻūhā ʻaoʻao ʻākau) i nā ake māmā

I ka atresia pulmonary, hoʻohui ʻia nā leaflets ākea. ʻO kēia ke kumu o kahi ʻāpana paʻa o nā kikoʻī e hana ma kahi e wehe ai ka pahu. ʻO ka hopena o ke kahe o ke kahe kūlohelohe i ka hopena. Ma muli o kēia hemahema, kaohi ʻia ke koko mai ka ʻaoʻao ʻākau o ka puʻuwai mai ka hiki ʻana i nā māmā e ʻohi i ka oxygen.

E like me ka nui o nā maʻi puʻuwai congenital, ʻaʻohe kumu i ʻike ʻia o atresia pulmonary. Pili kēia ʻano me kekahi ʻano kīnā o ka naʻau hānau i kapa ʻia he patent ductus arteriosus (PDA).

Hiki i nā atresia pulmonary me ka ʻole o ka hemahema septal ventricular (VSD).

• Inā ʻaʻohe kahi VSD o ke kanaka, kapa ʻia ke ʻano he atresia pulmonary me ka septum ventricular paʻa (PA / IVS).
• Inā loaʻa nā pilikia ʻelua i ke kanaka, kapa ʻia ke ʻano he atresia pulmonary me VSD. ʻO kēia kahi ʻano tetralogy o Fallot.

ʻOiai ʻo nā kūlana ʻelua i kapa ʻia he atresia pulmonary, ʻokoʻa nā kīnā ʻokoʻa. Kūkākūkā kēia ʻatikala i ka atresia pulmonary me ka ʻole o VSD.

Loaʻa paha i ka poʻe me PA / IVS kahi pahū tricuspid maikaʻi ʻole i hoʻomohala ʻia. Loaʻa paha iā lākou kahi ventricle ʻākau ma lalo o ka hoʻomohala a mānoanoa loa, a me nā kīʻaha koko ʻole e hānai ana i ka puʻuwai. ʻO ka mea liʻiliʻi, pili nā kūkulu i ka ventricle hema, ka aorta aorta, a me ka atrium ʻākau.

Kū pinepine nā ʻōuli i nā hola mua o ke ola, ʻoiai e lawe ʻia i kekahi mau lā.

Pākuʻi ʻia paha nā ʻōuli:

• ʻO kaʻili kala polū (cyanosis)
• Hanu wikiwiki
• Ka luhi
• Nā ʻano ʻai maikaʻi ʻole (luhi paha nā pēpē i ka hānai ʻana a i ʻole ka hou i ka wā o ka hānai ʻana)
• Pokole o ka hanu

E hoʻohana ka mea mālama ola i kahi stethoscope e hoʻolohe ai i ka puʻuwai a me nā māmā. ʻO ka poʻe me kahi PDA kahi ʻōhumu o ka naʻau e hiki ke lohe ʻia me kahi stethoscope.

E kauoha ʻia nā hōʻike aʻe:

• Pākuʻi x-ray
• ʻEchocardiogram
• Electrocardiogram (ECG)
• Hoʻohui puʻuwai puʻuwai
• Pulse oximetry - hōʻike i ka nui o ka oxygen i loko o ke koko

Hoʻohana ʻia kahi lāʻau i kapa ʻia ʻo prostaglandin E1 e kōkua i ke neʻe ʻana o ke koko (circulate) i loko o nā māmā. Mālama kēia lāʻau i kahi kīʻaha koko e hāmama ma waena o ke aʻa aʻa a me ka aorta. Kapa ʻia ka moku he PDA.

Hiki i nā ʻano lāʻau he nui, akā kaukaʻi ʻia i ka nui o nā kīnā ʻole o ka puʻuwai e pili pū me ka hemahema o ka pulmonary valve. Loaʻa i nā ʻano hana kūpono invasive:

• Hoʻoponopono Biventricular - Hoʻokaʻawale kēia ʻoki i ke kahe o ke koko i nā akemāmā mai ke kaapuni ʻana i ke koena o ke kino e ka hana ʻana i ʻelua ventricle pumping.
• Hoʻololi ʻo Univentricular - Hoʻokaʻawale kēia ʻoki i ke kahe o ke koko i nā akemāmā mai ke kaapuni ʻana i ke koena o ke kino e ke kūkulu ʻana i hoʻokahi ventricle pumping.
• Ke kuʻi puʻuwai.

Hiki ke kōkua i ka hapa nui o nā hihia me ke ʻoki. Maikaʻi ka hilinaʻi ʻana o kahi pēpē i:

• Ka nui a me nā pilina o ke aʻa pulmonary (ke aʻa e lawe i ke koko i nā akemāmā)
• Pehea ke kuʻi ʻana o ka puʻuwai
• Pehea ka maikaʻi o ka hoʻokumu ʻia ʻana o nā valves naʻau a i ʻole ka nui o ka liʻu ʻana

Hoʻololi nā hopena ma muli o nā ʻano like ʻole o kēia kīnā. Pono i kahi pēpē wale nō i kahi kaʻina hana a i ʻole hiki ke pono ʻekolu a ʻoi paha o nā ʻoki ʻana a hoʻokahi wale nō ventricle hana.

Komo pū nā pilikia:

• Hoʻoneʻe ka ulu a me ka hoʻomohala
• Ka hopu ʻana i nā maʻi
• Hahau
• Endocarditis maʻi maʻi
• Hewa puʻuwai
• Make

Kāhea i kāu mea hāʻawi inā loaʻa ka pēpē i:

• Pilikia i ka hanu ʻana
• ʻIli, nā kui a i ʻole nā ​​lehelehe e ʻike polū (cyanosis)

ʻAʻohe ala i ʻike ʻia e pale ai i kēia ʻano.

Pono nā wahine hāpai a pau e mālama i ka mālama prenatal maʻamau. Hiki ke ʻike ʻia nā hemahema he nui ma nā hōʻike ultrasound maʻamau.

Inā loaʻa ka hemahema ma mua o ka hānau ʻana, hiki i nā loea lapaʻau (e like me ka cardiologist pediatric, surgeon cardiothoracic, a me neonatologist) i ka wā hānau, a mākaukau e kōkua inā pono. Hiki i kēia hoʻomākaukau ke ʻokoʻa ma waena o ke ola a me ka make no kekahi mau pēpē.

Pulmonary atresia - kōpaʻa ventricular paʻa; PA / IVS; Maʻi maʻi ʻōpū - atresia pulmonary; Maʻi naʻau cyanotic - atresia pulmonary; Valve - maʻi pulmonary atresia

• Puʻuwai - ʻāpana ma waena o ka waena
• Puʻuwai - ʻike mua

Fraser CD, Kane LC. Maʻi maʻi ʻōpū. I: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. ʻO Sabiston Textbook of Surgery. 20 ouk. Piladelapia, PA: Elsevier; 2017: chap 58.

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.