ʻO ka maʻi puʻuwai hema hema

Hana ʻia ʻo ka hypoplastic left heart syndrome ke hana ʻole nā ​​ʻāpana o ka ʻaoʻao hema o ka puʻuwai (mitral valve, left ventricle, aortic valve, a aorta). Aia ke ʻano i ka wā hānau (congenital).

ʻO ka puʻuwai hema Hypoplastic kahi ʻano laha ʻole o ka maʻi puʻuwai hānau. ʻOi aku ka maʻamau i nā kāne ma mua o nā wahine.

E like me nā kīnā o ka naʻau congenital, ʻaʻohe kumu i ʻike ʻia. Ma kahi o 10% o nā pēpē me ka maʻi hypoplastic hema puʻuwai i loaʻa pū kekahi kīnā hānau. Pili pū ʻia ia me kekahi mau maʻi genetics e like me Turner Syndrome, Jacobsen Syndrome, trisomy 13 a me 18.

E ulu ka pilikia ma mua o ka hānau ʻana ke ulu ʻole ka ventricle hema a me nā hanana ʻē aʻe, e like me ka:

• Aorta (ke kīʻaha koko e lawe i ke koko waiwai-oxygen mai ka ventricle hema i ke kino holoʻokoʻa)
• Ke komo a me ka puka o ka ventricle
• ʻO nā valves Mitral a me nā aorta

ʻO kēia ke kumu e hoʻomohala maikaʻi ʻole ʻia ai ka ventricle hema a me ka aorta, a i ʻole hypoplastic. I ka hapanui o nā hihia, ʻoi aku ka liʻiliʻi o ka ventricle hema a me ka aorta ma mua o ka maʻamau.

I nā pēpē me kēia ʻano, ʻaʻole hiki i ka ʻaoʻao hema o ka puʻuwai ke hoʻouna i ke koko i ke kino. ʻO ka hopena, pono ka ʻaoʻao ʻākau o ka puʻuwai e mālama i ka holo o nā māmā a me ke kino. Hiki i ka ventricle ʻākau ke kākoʻo i ke kaapuni i nā māmā ʻelua a me ke kino no kekahi manawa, akā ʻo kēia kaumaha hana keu i kumu e hāʻule ka ʻaoʻao ʻākau o ka puʻuwai.

ʻO ka hiki ke ola wale nō kahi pilina ma waena o ka ʻākau a me ka ʻaoʻao hema o ka puʻuwai, a i ʻole ma waena o nā aʻa a me nā aʻa pulmonary (nā kīʻaha koko e lawe i ke koko i nā akemāmā). Hānau ʻia nā pēpē me ʻelua o kēia mau pilina:

• Foramen ovale (kahi puka ma waena o ka atrium ʻākau a hema)
• ʻO Ductus arteriosus (kahi kīʻaha koko liʻiliʻi e hoʻopili i ka aorta i ke aʻa o ka pulmonary)

Pili mau kēia mau pilina i kā lākou iho i kekahi mau lā ma hope o ka hānau ʻana.

I nā pēpē me ka maʻi puʻuwai hema hypoplastic, haʻalele ke koko i ka ʻaoʻao ʻākau o ka puʻuwai ma o ke aʻa pulmonary e hele ai ma o ka ductus arteriosus i ke aorta. ʻO kēia wale nō ke ala e hiki ai ke koko i ke kino. Inā ʻae ʻia ka ductus arteriosus e pani i loko o kahi pēpē me ka maʻi puʻuwai hema hypoplastic, make koke paha ka pēpē no ka mea ʻaʻole e hoʻokahe ʻia ke koko i ke kino. ʻO nā pēpē me ka maʻi hypoplastic hema i ʻike ʻia e hoʻomaka pinepine ʻia i ka lāʻau e wehe i ka ductus arteriosus hāmama.

Ma muli o ka liʻiliʻi a ʻaʻohe kahe o ka puʻuwai hema, pono ke koko e hoʻi i ka puʻuwai mai nā ake māmā e hele ma waena o ka foramen ovale a i ʻole ka hemahema atrial septal (kahi puka e hoʻopili ana i nā keʻena hōʻiliʻili ma ka ʻaoʻao hema a me ka ʻākau o ka puʻuwai) hoʻi i ka ʻaoʻao ʻākau o ka puʻuwai. Inā ʻaʻohe foramen ovale, a inā liʻiliʻi ia, hiki i ka pēpē ke make. Loaʻa i nā pēpē me kēia pilikia ka puka ma waena o kā lākou atria i wehe ʻia, a i ʻole me ke ʻoki ʻana a i ʻole ka hoʻohana ʻana i kahi paipu lahilahi a maʻalahi (catheterization puʻuwai).

I ka manawa mua, he mea maʻamau ke hānau hou me ka puʻuwai hema hypoplastic. Hiki ke loaʻa nā ʻōuli i nā hola mua o ke ola, ʻoiai e lawe ʻia i kekahi mau lā e hoʻomohala i nā ʻōuli. Pākuʻi ʻia paha kēia mau ʻōuli:

• Bluish (cyanosis) a i ʻole ʻili kala maikaʻi ʻole
• ʻO nā lima anuanu a me nā wāwae (ʻoi loa)
• Lethargy
• Pulse maikaʻi ʻole
• Hānai hānai maikaʻi ʻole
• Puʻuwai kuʻi
• Hanu wikiwiki
• Pokole o ka hanu

I nā keiki hānau olakino, ke kala ʻulaʻula i nā lima a me nā wāwae he pane i ke anuanu (kapa ʻia kēia hopena he cyanosis peripheral).

ʻO ke kala bluish i ka umauma a i ʻole ka ʻōpū, nā lehelehe, a me ke alelo he mea maʻamau (i kapa ʻia ʻo cyanosis waena). He hōʻailona ia ʻaʻole lawa ka oxygen ma ke koko. Hoʻonui pinepine ka cyanosis waena me ka uē.

Hōʻike paha kahi hoʻokolohua kino i nā hōʻailona o ka puʻuwai puʻuwai:

• ʻOi aku ka wikiwiki ma mua o ka helu maʻamau o ka puʻuwai
• Lethargy
• Hoʻonui ākea
• Hanu wikiwiki

Eia kekahi, nāwaliwali loa ka lāʻau ikiʻai ma nā wahi like ʻole (ka pulima, ke aʻa, a me nā mea ʻē aʻe). Aia pinepine (akā ʻaʻole mau) nā leo puʻuwai maʻamau ke hoʻolohe nei i ka umauma.

E komo pū paha nā hoʻāʻo:

• ʻO ka catheterization Cardiac
• ECG (electrocardiogram)
• ʻEchocardiogram
• X-ray o ka umauma

Ke hoʻoholo ʻia ka naʻau hypoplastic hema, e ʻae ʻia ka pēpē i ka neonatal intensive care unit. Pono paha kahi mīkini hanu (ventilator) e kōkua i ka pēpē hanu. Hoʻohana ʻia kahi lāʻau i kapa ʻia ʻo prostaglandin E1 e mālama i ke kahe o ke koko i ke kino e ka hāmama ʻana i ka ductus arteriosus.

ʻAʻole hoʻoholo kēia mau ana i ka pilikia. Pono ke ʻano i ke kaha ʻana.

ʻO ke ʻoki kino mua, i kapa ʻia ka hana Norwood, hiki i loko o nā lā mua o ke ola o ka pēpē. ʻO ke kaʻina hana Norwood ke kūkulu ʻana i aorta hou e:

• Ke hoʻohana nei i ka pahu pulmonary a me ke aʻa
• Hoʻohui i ka aorta kahiko hypoplastic a me nā aʻa coronary i ke aorta hou
• Ke wehe nei i ka paia ma waena o ka atria (atrial septum)
• Ke hana nei i kahi pilina pilikino mai ka ventricle ʻākau a i ʻole ke aʻa kino ma ke aʻa o ka pulmonary e mālama i ke kahe o ke koko i nā akemāmā (i kapa ʻia he shunt)

Hiki ke hoʻohana ʻia kekahi ʻano o ka hana Norwood, i kapa ʻia ʻo ke kaʻina hana Sano. Hoʻokumu kēia kaʻina hana i kahi ventricle ʻākau i ka pilina o ka pulmonary artery.

Ma hope, hoʻi ka pēpē i ka home i ka nui o nā hihia. Pono ke keiki e lawe i nā lāʻau i kēlā me kēia lā a ukali pono ʻia e ka psyatric cardiologist, nāna e hoʻoholo i ka manawa o ka ʻelua o ke kaha e hana ai.

Kapa ʻia ke kahua II o ka hana ʻo Glenn shunt a i ʻole hemi-Fontan. Kuhi ʻia ia ma ke ʻano he cavopulmonary shunt. Hoʻohui kēia kaʻina hana i ka aa nui e lawe ana i ke koko polū mai ka hapa kiʻekiʻe o ke kino (ʻo ka vena cava kiʻekiʻe) pololei i nā kīʻaha koko i nā akemāmā (nā aʻa o ka pulmonary) e loaʻa ai ka oxygen. Hana pinepine ʻia ke ʻokiʻoki ke hiki ke keiki i ka 4 a 6 mau mahina o kona makahiki.

Ma nā pae I a me II, ʻike mau ke keiki i kahi polū (cyanotic).

ʻO ke kahua III, ka anuu hope loa, ua kapa ʻia ka hana Fontan. ʻO ke koena o nā uaua e lawe i ke koko polū mai ke kino (ka vena cava inferior) e hoʻopili pololei ʻia i nā kīʻaha koko i nā akemāmā. ʻO ka ventricle ʻākau i kēia manawa ke lawelawe ʻia ma ke keʻena pumping no ke kino (ʻaʻole nā ​​māmā a me ke kino). Hana pinepine ʻia kēia ʻoki ke 18 mau makahiki a hiki i ka 4 mau makahiki o ka pēpē. Ma hope o kēia kaʻina hope loa, ʻaʻole ke keiki cyanotic hou a loaʻa kahi pae oxygen maʻamau i ke koko.

Pono paha kekahi poʻe i nā hana hou i ko lākou 20 a 30 paha inā ulu lākou paʻakikī e kaohi i nā arrhythmia a i ʻole nā ​​pilikia ʻē aʻe o ke kaʻina hana Fontan.

Manaʻo kekahi mau kauka i ke kanu ʻana o ka naʻau i kahi ʻokoʻa i ke ʻoki ʻekolu. Akā loaʻa he mau puʻuwai manawaleʻa i loaʻa no nā pēpē liʻiliʻi.

Inā haʻalele ʻole ʻia, make hewa ka maʻi maʻi hypoplastic hema. Ke piʻi mau nei nā helu ola no ka hana hou ʻana i ke ʻano me ka hoʻomaikaʻi ʻana i nā ʻoki a me ka mālama ʻana ma hope o ke kaha ʻana. ʻO ke ola ma hope o ka pae mua ma mua o 75%. ʻO nā keiki i ola i ko lākou makahiki mua he manawa kūpono loa lākou no ke ola lōʻihi.

ʻO ka hopena o ke keiki ma hope o ke kaha ʻana e pili ana i ka nui a me ka hana o ka ventricle ʻākau.

Komo pū nā pilikia:

• Ka poloka o ka shunt ʻimi hoʻopunipuni
• Hiki i ke kahe koko ke alakaʻi i ka hahau a i ʻole embolism pulmonary
• ʻO ka wā lōʻihi (mau) diarrhea (mai kahi maʻi i kapa ʻia ʻo enteropathy-protein i nalowale)
• Fluid i loko o ka ʻōpū (ascites) a i nā akemāmā (pleural effusion)
• Hewa puʻuwai
• Hewa, wikiwiki nā puʻuwai puʻuwai (arrhythmia)
• Nā hahau a me nā pilikia ʻōnaehana ʻōnaehana ʻē aʻe
• Ka hoʻohaunaele Neurological
• Make make koke

Kāhea koke aku i kāu kahu mālama ola inā kāu pēpē:

• ʻAi liʻiliʻi (hoʻemi i ka hānai ʻana)
• He ʻili polū (cyanotic)
• Loaʻa nā hoʻololi hou i nā mana hanu

ʻAʻohe pale e ʻike ʻia no ka maʻi hypoplastic hema puʻuwai. E like me nā maʻi kūmaka he nui, ʻaʻole maopopo nā kumu o ka hypoplastic left heart syndrome a ʻaʻole i hoʻopili ʻia i ka maʻi a me ka lawena a ka makuahine.

HLHS; Puʻuwai hānau - puʻuwai hema hypoplastic; Maʻi maʻi Cyanotic - puʻuwai hema hypoplastic

• Puʻuwai - ʻāpana ma waena o ka waena
• Puʻuwai - ʻike mua
• Hypoplastic hema puʻuwai maʻi

Fraser CD, Kane LC. Maʻi maʻi ʻōpū. I: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. ʻO Sabiston Textbook of Surgery: Ke kumu kumu olaola o ka hana ʻoki ʻana o kēia ao. 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.