Loaʻa i kāu keiki liʻiliʻi kēia pilikia puʻuwai? E aʻo kākou e pili ana i ka Pulmonary Artery Stenosis!

Ke manaʻo nei kāu keiki i ka hanu pōkole i kekahi manawa? A i ʻole luhi koke ʻo ia ma hope o ka pāʻani ʻana no kekahi manawa? Hiki i kekahi mau keiki liʻiliʻi ke loaʻa i nā maʻi puʻuwai hānau. I kēia lā e kamaʻilio mākou e pili ana i kekahi maʻi puʻuwai, he mea kakaikahi iki, akā he mea nui loa e ʻike ai. Kāhea mākou i kēia '(Pulmonary Artery Stenosis)'. I ka ʻōlelo maʻalahi, he pani ʻia ia o ke aʻa nui e lawe ana i ke koko i nā māmā.

He aha ka Pulmonary Artery Stenosis?

I ka ʻōlelo maʻalahi, aia kekahi kīʻaha koko nui e lawe ana i ke koko mai ka ʻaoʻao ʻākau o ko kākou puʻuwai (a mākou e kapa nei ʻo "ventricle ʻākau") i nā māmā. Ua kapa ʻia kēia ʻo "pulmonary artery". Ke hiki i nā māmā, ua hoʻopiha ʻia kēia koko me ka oxygen, a laila ua lawe ʻia kēlā koko i hoʻopiha ʻia me ka oxygen ma ke kino holoʻokoʻa.

I kēia manawa, ma kēia kūlana i kapa ʻia ʻo `(Pulmonary Artery Stenosis)`, ua ālai ʻia a ua ʻūmiʻi paha ke kīʻaha koko e lawe ana i ke koko i nā māmā ma kekahi wahi a i ʻole ma kekahi o kona mau lālā. Ua like ia me kahi paipu wai e ālai ʻia ana ma waenakonu. He aha ka mea e hana ʻia a laila? Lilo ia i mea paʻakikī no ke koko ke kahe i nā māmā. Inā ʻaʻole e kahe pono ke koko, ʻaʻole e loaʻa i ke kino ka nui o ka oxygen e pono ai. Ma muli o kēia, ʻaʻole hiki i ka puʻuwai o ke keiki a me ke kino holoʻokoʻa ke hana pono.

Hiki ke hana ʻia kēia ālai ʻana ma ke aʻa pulmonary nui (`(main pulmonary artery)`), a i ʻole ma kona mau lālā hema a ʻākau paha (`(lālā hema a ʻākau paha)`). Ke hana ʻia kēia ālai ʻana, pono ka ʻaoʻao ʻākau o ka puʻuwai (`(ventricle ʻākau)`) e hana ikaika loa e pamu i ke koko ma mua o ka ālai ʻana. Ua like ia me ka hoʻohana ʻana i ka ikaika nui e pahu i ka wai ma o kahi paipu ālai ʻia. I ka hala ʻana o ka manawa, hiki i kēia hoʻoikaika mau ke hōʻino i ka ʻiʻo o ka puʻuwai.

He aha nā pilikia e hiki ke kū mai mai kēia maʻi?

Inā ʻaʻole mālama pono ʻia kēia, hiki i ka ʻaoʻao ʻākau o ka puʻuwai ke lilo i mea hana ʻole i ka hala ʻana o ka manawa. ʻO ia hoʻi, aia kahi hiki ke loaʻa i kahi maʻi i kapa ʻia ʻo "Right-sided heart failure". No laila, he mea nui loa ka mālama ʻana i kēia.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia kūlana?

ʻO kēia kūlana i kapa ʻia ʻo `(Pulmonary Artery Stenosis)` he mea kākaʻikahi loa ia. ʻO ia hoʻi, ʻaʻole ia e hiki i nā kānaka a pau. Hiki i kekahi mau keiki ke loaʻa iā ia me nā maʻi puʻuwai ʻē aʻe (`(congenital heart disease)` - ʻo ia hoʻi, nā maʻi puʻuwai i loaʻa i ka wā hānau). I kekahi manawa hiki iā ia ke lilo i hoʻokahi wale nō me ka ʻole o nā pilikia puʻuwai ʻē aʻe. Eia kekahi, hiki ke hana ʻia kēia kūlana ma hope o kekahi mau ʻoki puʻuwai.

He aha nā hōʻailona o kēia? E nānā inā loaʻa i kāu keiki kēia mau mea pū kekahi.

Pili kēia mau hōʻailona i ke ʻano o ka haiki o ka ʻōmole. I kekahi manawa, inā he ʻoluʻolu loa ka stenosis (`mild stenosis`), ʻaʻohe paha he mau hōʻailona . Akā i ka wā e ʻoi aku ka ʻino o ka stenosis, hiki i kāu keiki ke hōʻike i nā mea e like me:

• Manaʻo e like me ka paʻakikī o ka hanu ʻana (`(Pōkole o ka hanu)`).
• Luhi mau ka manaʻo (`(Fatigue)`).
• Me he mea lā e hanu wikiwiki ana ʻoe , a malia paha e hanu nui ana ʻoe.Penei ke ʻano? (`(Hanu kaumaha a wikiwiki paha)`).
• Ke manaʻo nei anei kou puʻuwai e like me ka wikiwiki loa o ke kuʻi ʻana ? (Rapid heart rate).
• Aia kekahi pehu ma kou mau wāwae, kuʻekuʻe wāwae, maka, lihilihi maka, a i ʻole ka ʻōpū? (`(Pehu)`).
• Ke manaʻo nei ʻoe i kekahi manawa ua poniuniu a nalowale paha kou manaʻo ? (`(Dizziness or passing out)`).
• Polū anei kou mau lehelehe, kou mau manamana lima, a me kou mau manamana wāwae ? ʻO kēia ka mea a mākou e kapa nei i ka lāʻau lapaʻau he "Cyanosis".
• Ke luhi koke nei ʻoe i ka wā e pāʻani ana me nā keiki ʻē aʻe? ʻAʻole hiki iā ʻoe ke holo a lele e like me nā keiki ʻē aʻe? (`(Hoʻemi ʻia ka hoʻomanawanui hoʻoikaika kino)`).

E hoʻomanaʻo, ʻo ka loaʻa ʻana o hoʻokahi a ʻelua paha o kēia mau hōʻailona ʻaʻole ia he manaʻo ua loaʻa iā ʻoe ka maʻi. Akā inā loaʻa i kāu keiki kekahi o kēia mau mea, ʻoi aku ka maikaʻi o ka ʻimi ʻana i ke kōkua lapaʻau.

No ke aha i ālai ʻia ai kēia aʻa pulmonary? He aha nā kumu?

Hānau ʻia kekahi mau keiki me kēia maʻi (Pulmonary Artery Stenosis), a loaʻa paha nā pilikia me nā valves, nā paia, a i ʻole nā ​​​​​​ʻāpana ʻē aʻe o ka puʻuwai. Hiki i kekahi ke hānau ʻia me kēia maʻi akā ʻaʻohe pilikia puʻuwai ʻē aʻe. Eia kekahi, hiki ke hana ʻia kēia maʻi ma hope o ke ʻoki puʻuwai a i ʻole ma muli o nā maʻi ʻē aʻe, kakaikahi loa.

Nā kumu hānau

Ma keʻano holoʻokoʻa, ma kahi o 40% o nā kānaka i hānau ʻia me kēia maʻi (Pulmonary Artery Stenosis), akā ʻaʻohe o lākou pilikia puʻuwai ʻē aʻe.

Eia nō naʻe, ma waena o 2% a me 3% o ka poʻe me nā kīnā puʻuwai hānau ʻē aʻe e loaʻa paha kēia maʻi (Pulmonary Artery Stenosis). Eia kekahi o nā kīnā puʻuwai maʻamau:

• (Tetralogy o Fallot): Ma kēia, ʻehā mau pilikia o ke keiki me ka puʻuwai, kahi e hoʻopilikia ai i ke kahe o ke koko.
• (Pulmonary atresia): Ma kēia, ʻaʻole i hana ʻia ka valve (pulmonary valve) ma waena o ka ventricle ʻākau o ka puʻuwai a me ke aʻa pulmonary (pulmonary artery). No laila, ʻaʻole kahe ke koko i nā māmā.
• (Truncus arteriosus): He kīʻaha koko nui hoʻokahi ma kahi o nā ʻelua maʻamau. Hoʻohui kēia i ke koko i loaʻa ka oxygen me ke koko i nele i ka oxygen.
• ʻO ka stenosis valve aortic: ʻO kahi pilikia me ka valve ma ke aʻa nui e lawe i ke koko mai ka puʻuwai a i ke kino, kahi e hoʻemi ai i ka nui o ke koko e kahe ana i ke kino.
• ʻO ke kīnā septal atrial: He lua ma ka paia ma waena o nā keʻena ʻelua o luna o ka puʻuwai (ka atria). ʻAe kēia i ke koko i loaʻa ka oxygen a me ke koko i nele i ka oxygen e hui pū.
• ʻO ke kīnā septal ventricular: He lua ma ka paia e hoʻokaʻawale ana i nā keʻena haʻahaʻa ʻelua (ventricles) o ka puʻuwai. Hiki i kēia ke hoʻoulu i ka nui o ke koko e kahe i loko o nā māmā.
• (Ka hoʻololi ʻana o nā kīʻaha koko nui): Ua hoʻohuli ʻia nā kīʻaha koko nui ʻelua e lawe ana i ke koko mai ka puʻuwai mai. Hoʻopilikia kēia i ke kahe koko maʻamau a kaupalena i ka nui o ka oxygen e hiki aku ana i nā ʻāpana o ke kino.
• (Patent ductus arteriosus):He pilina kēia ma waena o ke aʻa pulmonary o ke pēpē a me ke aʻa nui e lawe ana i ke koko i ke kino (ka aorta). Pono kēia e pani ma hope o ka hānau ʻana, akā inā ʻaʻole, e kahe nui ke koko i nā māmā.

Nā kumu ʻē aʻe

Ma waho aʻe o kēia, aia paha kekahi mau kumu ʻē aʻe:

• ʻO ka maʻi Rubella: Inā loaʻa iā ʻoe ka rubella i ka wā hāpai, loaʻa paha i kāu pēpē nā pilikia puʻuwai a me nā pilikia olakino ʻē aʻe.
• (Williams syndrome): Hoʻoulu kēia i nā ʻano ʻē aʻe i loko o ka puʻuwai o ke keiki a me nā ʻōpū ʻē aʻe.
• (Alagille syndrome): Hoʻopilikia kēia i nā pilikia o ke akepaʻa a me nā maʻi puʻuwai.
• ʻO ka arteritis o Takayasu: He mumū kēia e hōʻino ai i nā kīʻaha koko nui.
• ʻO kekahi mau pilikia me ke ʻano o ka hoʻokuke ʻia ʻana o ke aʻa pulmonary o ke keiki i waho.

Nā kumu hana ʻoki kino

Hiki i kekahi poʻe ke loaʻa i kēia maʻi ma hope o ke ʻoki ʻana. Eia kekahi laʻana:

• He hoʻoili ʻana o ka māmā.
• ʻO ke ʻoki kino e hoʻoponopono ai i kahi kīnā puʻuwai hānau i loko o kahi keiki, a i ʻole e hoʻomaikaʻi i ke kahe o ke koko ma o ka puʻuwai.
• He ʻoki kino i kapa ʻia ʻo "Pulmonary artery banding". Ma kēia, ua hoʻohaiki ʻia ke aʻa e hōʻemi i ka nui o ke koko e hele ana i nā māmā.

Pehea e ʻike ai i kēia maʻi?

Ke nānā ʻia kāu keiki e ke kauka, lohe paha lākou i kahi leo puʻuwai ʻē (he "murmur"). Inā pēlā, e kauoha lākou i kekahi mau hoʻokolohua hou aʻe. ʻO kekahi o kēia mau mea:

• Electrocardiogram - ECG a i ʻole EKG: Hoʻopaʻa kēia i nā loli uila e hana ʻia ana i ka wā e paʻi ai ka puʻuwai. Hiki iā ia ke ʻike i nā puʻuwai puʻuwai kūpono ʻole (arrhythmias) a me ke kaumaha ma ka ʻiʻo puʻuwai.
• ʻO kahi X-ray umauma: Hiki i kēia ke hōʻike i ka nui a me ke ʻano o ka puʻuwai, nā māmā, a me nā aʻa pulmonary.
• Echocardiogram: Ua like kēia me ke kiʻi neʻe o ka puʻuwai. Hoʻohana ia i nā nalu kani e nānā i nā ʻiʻo puʻuwai a me nā valves.
• ʻO ke kiʻi hoʻohālikelike magnetic cardiac (MRI): He ʻano hana kiʻi ʻekolu-dimensional (3D) kēia e hiki ai ke ʻike i ke kahe ʻana o ke koko ma o ka puʻuwai a me nā kīʻaha koko o kāu keiki.
• (CT scan): He hoʻāʻo `(X-ray)` nō hoʻi kēia. Hoʻohana kahi kamepiula i kahi moʻo o nā kiʻi `(X-ray)` o ka puʻuwai o ke keiki e hana i nā hiʻohiʻona ʻokiʻoki. Hoʻokomo ʻia kahi kala kūikawā (`(IV contrast)`) i loko o ka puʻuwai e nānā i ke ʻano a me ke kahe o ke koko o ka puʻuwai.
• Catheterization cardiac: Ma kēia kaʻina hana, hoʻokomo ʻia kahi ʻōmole lahilahi (catheter) i loko o kahi aʻa a i ʻole ke aʻa a alakaʻi ʻia i loko o ka puʻuwai. ʻAe kēia i ke kauka e nānā i nā pae oxygen, nā loli kaomi, a lawe i nā kukuna X o ka puʻuwai.
• ʻO ka angiography pulmonary: Lawe ʻia kahi X-ray o nā aʻa pulmonary a me nā veins o ka puʻuwai me ka hoʻohana ʻana i kahi dye.
• (Perfusion scan): Ma kēia, hoʻokomo ʻia kahi liʻiliʻi o ka mea radioactive. A laila, hoʻohana ʻia kahi mīkini kūikawā e nānā i ke kahe maikaʻi o ke koko i kēlā me kēia māmā.

Inā hoʻoholo kāu kauka he Pulmonary Artery Stenosis kāu keiki, e kuhikuhi lākou iā ʻoe i kahi loea puʻuwai hānau. Loaʻa i kēia ʻano kauka nā mākau a me ke aʻo ʻana e ʻike pololei i ka pilikia puʻuwai o kāu keiki, hana i nā hoʻokolohua e pono ai, hāʻawi i ka lāʻau lapaʻau, hana i ke ʻoki puʻuwai, a hāʻawi i nā nānā hou aku . Hiki iā lākou ke hana i nā hoʻokolohua hou aku inā pono.

I ke kamaʻilio ʻana e pili ana i kēia kūlana, hiki i kāu kauka ke kuhikuhi iā ia ma ke ʻano he Type I, II, III, a i ʻole IV. He hoʻokaʻawale kēia e pili ana i nā ʻāpana o ke aʻa i ālai ʻia a me ka nui.

He aha nā lāʻau lapaʻau no kēia? (Lāʻau lapaʻau)

ʻO ka lāʻau lapaʻau maikaʻi loa e pili ana i nā hōʻailona o kāu keiki a me nā kumu ʻē aʻe. Inā he ʻūlū iki a ʻoi aku paha o kekahi mau lālā o ke aʻa pulmonary, ʻaʻole pono paha ka lāʻau lapaʻau . Eia nō naʻe, inā koʻikoʻi ka ʻūlū ʻana, pono ka lāʻau lapaʻau .

ʻO nā lāʻau lapaʻau no ka `(Pulmonary Artery Stenosis)` penei:

Hoʻonui ʻana i ka baluna / Angioplasty

Ma kēia, hana ke kauka:

• Hoʻokomo ʻia kahi catheter e like me ka balloon (balloon dilation catheter) i loko o ke kahawai i paʻa.
• A laila, me ke akahele loa, ua hoʻopiha ʻia ka pōpō mai ke kaomi haʻahaʻa a i ke kaomi kiʻekiʻe.
• A laila hoʻonui ʻia kēlā ʻauwai i paʻa.
• A laila hoʻohemo ʻia ka ea mai loko mai o ka pōpō a lawe ʻia i waho.

ʻO ka hoʻolaha ʻana o ka balloon a me ke kau ʻana o ka stent (hana pinepine ʻia kēia)

Ma kēia, hana ke kauka:

• Hoʻohana ʻia kahi stent hiki ke hoʻonui ʻia i loko o ka baluna (he ʻōmole mesh liʻiliʻi ka stent) e wehe i ke aʻa i paʻa.
• Hoʻokomo ʻia kēia stent ma luna o kahi catheter angioplasty balloon a uhi ʻia me kahi ʻūpā.
• Ke hoʻoneʻe ʻia nei ka stent i kahi kūpono.
• Ua wehe ʻia ka uhi ma luna o ka stent-balloon.
• A laila ua hoʻopiha ʻia ka pōpō i ke kaomi kūpono, e hoʻonui ana i ka stent a hoʻopaʻa ʻia ma laila.

He baluna kūikawā i kapa ʻia ʻo ``The Cutting Balloon™``

Ua like kēia pōpō me ka pōpō maʻamau. Eia nō naʻe, he nui nā lau liʻiliʻi ma kona lōʻihi. Ke hoʻopiha ke kauka i ka pōpō, hoʻāla kēia mau lau a ʻoki i ka wahi i ālai ʻia. ʻOi aku ka maʻalahi o ka hoʻonui ʻana i ka ʻōmole, e hana ana i kahi puka nui aʻe.

Loaʻa i ka hapa nui o ka poʻe me nā maʻi puʻuwai hānau ʻē aʻe nā hopena maikaʻi me kēia kaʻina hana. Eia nō naʻe, ma kahi o 21% o ka poʻe e pani hou ʻia paha ko lākou aʻa ma hope o kekahi mau mahina.

ʻoki kino

Hoʻohana nā kauka lapaʻau i nā ʻano hana like ʻole e mālama ai i ka stenosis o ke aʻa pulmonary. Aia ke koho i ke ʻano o ka paʻa ʻana, nā kīʻaha koko e hoʻopuni ana, a me nā ʻano hana ʻē aʻe.

Aia kekahi mau pilikia i ka mālama ʻana?

Hoʻemi ka hoʻonui ʻana o ka baluna i ke ālai ʻana i ka hapa nui o nā kānaka. Eia nō naʻe, i ka hala ʻana o ka manawa, hiki ke ālai hou ʻia ke aʻa ma 15% a 20%, a pono e hana hou ʻia ke kaʻina hana. Ke noiʻi nei nā kānaka noiʻi i nā ʻano baluna like ʻole e hāʻawi i nā hopena maikaʻi a lōʻihi.

Nā pilikia e hiki mai ana i ka wā o ka hoʻolaha ʻana o ka balloon:

• ʻO ka aneurysm o ke aʻa puʻuwai.
• ʻOki ʻana i ke aʻa puʻuwai (ʻoki ʻana i ke aʻa puʻuwai).
• Ka haki ʻana o ke aʻa puʻuwai (`(Ka haki ʻana o ke aʻa puʻuwai)`).
• ʻEdema puʻuwai (`(ʻEdema puʻuwai)`).
• He mea kākaʻikahi loa, hiki ke loaʻa nā make.

Nā pilikia e hiki mai ana mai ka hoʻohana ʻana i ka stent:

• Nā ʻūpā koko.
• Nā ʻano ʻē o ka puʻuwai (`(Ventricular arrhythmias)`).
• Hiki ke nalowale a hemo paha ka stent.
• Pono e hoʻonui hou i ke aʻa (he mea kākaʻikahi loa kēia).

He aha nā pono o ka lāʻau lapaʻau?

Makemake nā kauka e kau i nā stents no ka Pulmonary Artery Stenosis no kēia mau kumu:

• He 96% ka hopena koke o lākou.
• He lōʻihi ka manawa e wehe ai ke aʻa.
• Hiki ke hoʻonui ʻia ka ʻāpana i hāiki ʻia ma kahi o ʻelua ka nui .
• He emi ke kumukūʻai a ʻoi aku ka maikaʻi ma mua o ke hoʻokomo wale ʻana i nā pōpō pehu a i ʻole ka hana ʻana i ke ʻoki kino.
• ʻOi aku ka holomua ma mua o ka angioplasty balloon.

Hiki i nā kauka ke koho i ka angioplasty balloon ma kahi o kahi stent i nā hihia aʻe:

• Inā pilikia loa ke kūlana o ke keiki .
• Inā paʻakikī ke ʻano o loko o ke kino o ke keiki (`(complex anatomy)`).
• Inā liʻiliʻi loa ke keiki .

He aha ka mea e hana ʻia ma hope o ke ʻoki kino?

Inā he stent kāu keiki, pono lākou e lawe i nā antibiotics a me nā lāʻau hoʻokahe koko paha no kekahi manawa. Hāʻawi ʻia kēia mau lāʻau lapaʻau e pale aku i ka maʻi a hoʻōki i ke kāpili ʻana o ke koko.

ʻEhia ka manawa e ʻoi aku ai ka maikaʻi o ke keiki ma hope o ka lāʻau lapaʻau?

Hana koke ke kaʻina hana o ka hoʻonui ʻana i ke aʻa pulmonary o kāu pēpē. ʻO ke ʻano kēia, ua maʻalahi loa i kāu pēpē ke komo i ke koko i loko o ko lākou mau māmā a loaʻa ka oxygen.

Inā loaʻa kēia maʻi i kaʻu keiki, pehea lā ka wā e hiki mai ana? (Wānana)

Me nā lāʻau lapaʻau holomua o kēia wā, nui ka poʻe me kēia maʻi e ola a hiki i ka wā makua. Eia nō naʻe, ʻaʻole paha e pane maikaʻi ka poʻe me nā maʻi e like me ka "Williams syndrome" a me ka "Alagille syndrome" i ka lāʻau.

Āhea ʻoe e lawe ai i kāu keiki i ke kauka?

Pono ʻoe e lawe i kāu keiki no nā nānā maʻamau e ʻike ai pehea lākou e hana nei. Pono nō hoʻi ʻoe e loaʻa i nā echocardiograms maʻamau e ʻike ai inā pono e hoʻonui hou ʻia ke aʻa i ka wā e hiki mai ana.

He aha kaʻu e nīnau aku ai i ke kauka?

Hiki iā ʻoe ke nīnau i ke kauka i nā nīnau penei:

• No ke aha i loaʻa ai i kaʻu keiki ka `(Pulmonary Artery Stenosis)`?
• He aha ka lāʻau lapaʻau maikaʻi loa no kaʻu keiki?
• He aha nā manawa kūpono e pono ai kaʻu keiki e mālama hou ʻia no kēia?

Inā pono kāu keiki i ka lāʻau no ka Pulmonary Artery Stenosis, e noʻonoʻo ke kauka i ka lāʻau maikaʻi loa no ke kūlana o kāu keiki. Mai kānalua e nīnau i nā nīnau e pili ana i kekahi mea āu i maopopo ʻole ai e pili ana i ke kaʻina hana o kāu keiki.

Leka Lawe-Home

I ka wā e pili ana i kēia kūlana i kapa ʻia ʻo `(Pulmonary Artery Stenosis)`, ʻo ka mea nui loa, ʻo ia ka ʻike mua ʻana i nā hōʻailona a me ka ʻimi ʻana i ke kōkua lapaʻau . He mea nui e ʻimi i ke kōkua a kahi `(congenital heart professional)`, ʻoiai inā loaʻa i nā keiki kēia maʻi.

Mai hopohopo, aia nā lāʻau lapaʻau no kēia . Hiki ke hoʻomalu ʻia kēia kūlana me nā mea e like me ka hoʻonui ʻana o ka balloon, ke kau ʻana o ka stent, a, inā pono, ke ʻoki kino. ʻOiai ma hope o ka lāʻau lapaʻau, he mea nui loa ia no ke olakino o ke keiki e hele no nā nānā lapaʻau maʻamau a hahai i nā ʻōlelo a ke kauka . E kamaʻilio me ke kauka e pili ana i nā nīnau a hopohopo paha āu e loaʻa ai.

ʻO ka Stenosis o ke Ala Puʻuwai, ka maʻi puʻuwai o nā keiki, nā kīnā o ka puʻuwai hānau, ka paʻakikī o ka hanu ʻana, ke ʻoki puʻuwai, ka Stent