Ua ālai ʻia ke kīʻaha koko nui o ka puʻuwai o kāu keiki liʻiliʻi? E aʻo kākou e pili ana i kēia (Coarctation of the Aorta)!

Ua noʻonoʻo paha ʻoe i ke ʻano kupaianaha o ka hana ʻana o ko kākou mau puʻuwai liʻiliʻi? Akā i kekahi manawa, ma muli o kekahi mau pilikia hānau, hiki ke hoʻopilikia iki ʻia ka hana o kēia puʻuwai. ʻO kekahi ʻano maʻi puʻuwai e hiki ke loaʻa i kekahi mau pēpē ua kapa ʻia ʻo `(Coarctation of the Aorta)`. ʻOiai paha he paʻakikī iki ka inoa, mai hopohopo. E kamaʻilio maʻalahi kākou e pili ana i kēia.

He aha ka `(Coarctation of the Aorta)`? I ka ʻōlelo maʻalahi loa...

I ka ʻōlelo maʻalahi, ʻo ka Coarctation o ka Aorta kahi ʻoki a i ʻole ka ʻoki ʻana o ka aorta, ke kīʻaha koko nui e lawe ana i ke koko momona oxygen mai ka puʻuwai o kāu pēpē i ke koena o ke kino. He kīnā puʻuwai hānau kēia.

E noʻonoʻo, he nui nā kaʻa e neʻe wikiwiki ana ma ke alanui nui. Akā he aha ka hopena inā ʻoe e hoʻohaiki i ke alanui ma kekahi wahi a ʻae wale i hoʻokahi alahele o ke kaʻa? Hiki mai nā pilikia kaʻa, a hoʻomaka nā mea hoʻokele e neʻe, ʻeā? Pēlā nō hoʻi ka hopena ma kēia kūlana. Lilo ia i mea paʻakikī no ke koko ke kahe pono ma o ka mea i paʻa i loko o ka aorta.

ʻO ka hapa pinepine, hana ʻia kēia ʻano haiki (coarctation) ma mua o ka māhele ʻana o nā lālā e lawe ana i ke koko mai ka aorta a i ke kino haʻahaʻa, e like me nā wāwae. He aha ke kumu o kēia? Piʻi ke koko ma ka ʻaoʻao o luna o ke kino o ka pēpē, e like me nā lima, akā emi iho ma ka ʻaoʻao o lalo o ke kino, e like me nā wāwae. ʻO kēia ʻokoʻa nui o ke koko ma waena o nā ʻaoʻao o luna a me lalo o ke kino he hiʻohiʻona koʻikoʻi ia o kēia maʻi. ʻOiai inā ʻaʻohe hōʻailona ʻē aʻe, kānalua paha nā kauka ke ʻike lākou i kēia ʻokoʻa koko.

Wahi a nā helu helu ma ʻAmelika Hui Pū ʻIa, ma kahi o hoʻokahi i loko o 1,700 mau pēpē i hānau ʻia i kēlā me kēia makahiki e loaʻa paha kēia maʻi (Aortic Coarctation). Hiki i kekahi mau pēpē ke loaʻa pū me kēia mau maʻi puʻuwai hānau ʻē aʻe.

He aha nā hōʻailona e hōʻike ai nā keiki i ka wā e loaʻa ai kēia maʻi iā lākou?

Pili kēia mau hōʻailona i ka nui o ka haiki ʻana o ka aorta. ʻO ka maʻamau, inā he haiki iki, e hoʻomaka nā hōʻailona e ʻike ʻia i loko o hoʻokahi a ʻelua paha pule ma hope o ka hānau ʻana. Eia nō naʻe, inā ʻaʻole ʻoi aku ka koʻikoʻi o ka haiki ʻana, ʻaʻole paha e ʻike ʻia nā hōʻailona a hiki i ka wā e ʻelemakule ai ke pēpē, a i ʻole ʻaʻole loa lākou e ʻike ʻia.

Nā hōʻailona o nā pēpē hānau hou

Inā loaʻa i kahi pēpē hānau hou kēia ʻano maʻi, hiki iā ia ke ʻike i nā hōʻailona e like me:

• Ka hiamoe mau a me ka makemake ʻole e inu i ka waiū
• Ka puʻuwai wikiwiki (pulse wikiwiki)
• Ka hou nui ʻana
• Hoʻonāukiuki mau, uē
• ʻIli keʻokeʻo a hina paha
• Ka hanu wikiwiki a luhi paha
• Ka paʻakikī o ka inu ʻana i ka waiū

Inā loaʻa i kahi pēpē ka coarctation koʻikoʻi o ka aorta , hiki iā ia ke lilo i ka haʻalulu a hoʻoweliweli i ke ola inā ʻaʻole ʻike ʻia a mālama koke ʻia. Eia nō naʻe, ʻaʻole paha e hōʻike kekahi mau pēpē i kekahi mau hōʻailona no ka mea he liʻiliʻi loa ka paʻa ʻana.

Nā hōʻailona o ka maʻi i nā keiki ʻōpio a me nā ʻōpio

Hiki i nā ʻōpio a me nā ʻōpio me ka Aortic Coarctation ke ʻike i nā hōʻailona e like me:

• ʻEha poʻo pinepine
• Ke koko kiʻekiʻe
• Ka kahe pinepine ʻana o ka ihu
• ʻEha ma nā wāwae i ka wā e hoʻoikaika kino ai, holo ai, a lele paha

ʻAʻole paha e ʻike kekahi mau keiki i kekahi mau hōʻailona. Akā, ʻike mua ʻia ka pilikia ma ke koko kiʻekiʻe i ka wā o ka nānā pono ʻana.

Ke ʻike ʻia kēia mau hōʻailona e kahi pākeke, pinepine ia ma muli o ka hoʻi hou ʻana o ka aorta e hāiki ana ma hope o ka mālama ʻana ma mua. He mea kākaʻikahi loa ka ʻike ʻole ʻia ʻana o kēia maʻi a hiki i ka wā makua.

No ke aha i kū ai kēia kūlana? He aha nā kumu?

ʻAʻole ʻike maopopo nā loea i ke kumu kikoʻī o CoA. Eia nō naʻe, ua manaʻoʻiʻo ʻia aia kahi ʻāpana genetic . ʻO ia hoʻi, ʻo kekahi mau loli gene e hana ʻia ana i ka wā o ka pēpē i loko o ka ʻōpū hiki ke kumu i ka ulu pono ʻole ʻana o ka aorta o ka pēpē.

I kekahi manawa hiki ke hoʻoili ʻia kēia mau loli genetic ma o nā ʻohana. Ua hōʻike ʻia e ka noiʻi inā loaʻa iā ʻoe ʻo CoA, ʻoi aku ka nui o ka loaʻa ʻana o kāu pēpē i ka CoA a i ʻole nā ​​maʻi puʻuwai ʻē aʻe ma mua o ka mea i loaʻa ʻole iā CoA.

Eia kekahi, ʻoi aku ke kiʻekiʻe o ka pilikia o ka hoʻomohala ʻana i ka CoA me kekahi mau kūlana genetic. No ka laʻana, ʻoi aku ka nui o nā pēpē i hānau ʻia me ke kūlana genetic ʻo Turner syndrome ʻaʻole wale ka Coarctation of the Aorta, akā ʻo nā kīnā hānau ʻē aʻe e like me ka bicuspid aortic valve, kahi kīnā i nā valves o ka puʻuwai.

Ke kumu o ka `(CoA)` wale nō me ka ʻole o nā kīnā puʻuwai ʻē aʻe

ʻO ke kumu nui o ka CoA i hoʻokaʻawale ʻia ʻo ia ka pani ʻana o kahi kīʻaha koko liʻiliʻi i kapa ʻia ʻo ductus arteriosus i ka wā o ka ulu ʻana o ka fetal. ʻO ka ductus arteriosus kahi kīʻaha koko liʻiliʻi e hoʻopili ana i ka aorta fetal i ke aʻa pulmonary. ʻAʻole hana nā māmā i ka wā o ka pēpē i loko o ka ʻōpū, no laila kōkua kēia paipu i ka pēpē e loaʻa ke koko momona oxygen e pono ai.

Ma hope o ka hānau ʻana o ke pēpē, hoʻomaka ka hana ʻana o nā māmā. A laila, ʻoiai ʻaʻole pono hou kēia `(ductus arteriosus)`, hoʻomaka ia e pani iā ​​ia iho i loko o kekahi mau lā. Eia naʻe, i ka wā e pani ʻia ai, hiki i kekahi o nā ʻiʻo o kēlā duct ke hui pū me nā ʻiʻo o `(Aorta)`. I kēlā hihia, i ka wā e koi ʻia ai ka `(ductus arteriosus)` e pani, hiki nō hoʻi i ka aorta ke lilo i mea haiki (coarctation)`.

He aha nā pilikia e hiki ke hana ma muli o kēia ʻano?

Inā ʻaʻole mālama ʻia kēia ʻano (Coarctation of the Aorta) no ka manawa lōʻihi, hiki mai nā pilikia e like me:

• Ke koko kiʻekiʻe (hypertension) ma ke kino holoʻokoʻa.
• Lilo ka mānoanoa o ka ʻiʻo o ka puʻuwai ma mua o ka maʻamau (hypertrophy ventricular hema).
• ʻO ka hōʻiliʻili ʻana o ka plaque i loko o nā aʻa coronary, ka mea e hoʻolako koko i ka puʻuwai, e hana ʻia ma mua o ka maʻamau - ua kapa ʻia kēia he maʻi coronary artery.
• He puʻupuʻu kūpono ʻole (aneurysm) a i ʻole ka haehae ʻana (dissection) i loko o ka aorta.
• ʻO kahi aneurysm o ka lolo (pehu ʻana o kahi kīʻaha koko i loko o ka lolo).
• ʻO ka hemahema o ka puʻuwai kahi kūlana i hiki ʻole ai i ka puʻuwai ke pamu i ke koko me ka pono.

ʻOi aku ka weliweli o kēia kūlana `(CoA)` inā ʻaʻole i ʻike ʻia a mālama ʻia. No laila, he mea nui loa ka ʻike ʻana iā ia i ka wā hikiwawe loa, i ka wā ʻōpiopio, a e hana i ka lāʻau lapaʻau e pono ai a me nā nānā mau ʻana (nānā ʻana).

Pehea e ʻike ai nā kauka i kēia? (Diagnosis)

ʻO ka maʻamau, ʻike nā kauka puʻuwai keiki i kēia maʻi (Coarctation of the Aorta) i ka wā kamaliʻi a i ʻole ka wā kamaliʻi. ʻO ka wikiwiki o ka ʻike ʻia ʻana ma muli o ke koʻikoʻi o nā hōʻailona.

ʻIke ʻia nā pēpē me nā hōʻailona ʻoi aku ka liʻiliʻi i loko o kekahi mau lā o ka hānau ʻana. Eia nō naʻe, ʻaʻole paha e ʻike ʻia nā pēpē me nā hōʻailona ʻoi aku ka liʻiliʻi, a ʻaʻohe loa, a hiki i ko lākou wā ʻelemakule a loaʻa ke koko kiʻekiʻe. He mea kākaʻikahi loa kēia e ʻike ʻia i ka wā makua.

Hoʻomaopopo pinepine ʻia kēia ʻano maʻi i nā pēpē a me nā keiki liʻiliʻi ma muli o kekahi mau hiʻohiʻona kānalua (nā hae ʻulaʻula) i ʻike ʻia i ka wā o ka hoʻokolokolo kino. ʻO ia mau hiʻohiʻona:

• He kiʻekiʻe ke koko o ka pēpē ma nā lima a me ke kino o luna, akā he haʻahaʻa ke koko ma nā wāwae a me ke kino o lalo.
• Aia kahi ʻokoʻa ma waena o ka helu pulse ma ka ʻāʻī a me ka helu pulse ma ka ʻūhā.
• Ke kau ke kauka i ka stethoscope ma ke kua o ke pēpē a hoʻolohe, lohe ʻia kahi leo ʻokoʻa a ʻoʻoleʻa e puka mai ana mai ka puʻuwai mai (he 'harsh heart murmur') .

Ua ʻike ʻia kekahi mau pēpē hānau hou me ka maʻi ma mua o ko lākou hōʻike ʻana i nā hōʻailona. Pehea ʻoe e ʻike ai? ʻO kahi hoʻāʻo i kapa ʻia ʻo "pulse oximetry" e ana ana i nā pae oxygen i loko o ko lākou koko e hōʻike ana he haʻahaʻa nā pae oxygen. Hiki i nā pae oxygen haʻahaʻa ke lilo i hōʻailona o kahi maʻi puʻuwai hānau koʻikoʻi, no laila ua hoʻāʻo ʻia kēia mau pēpē e ʻike pono i ka mea i hewa.

No ka hōʻoia ʻana i kēia kūlana `(CoA)`, hoʻohana pinepine nā kauka i kahi `(Echocardiogram)`, kahi scan puʻuwai, a i ʻole `(echo)`. Hiki iā lākou ke hana i nā hoʻokolohua e like me `(CT scan)` a i ʻole `(MRI scan)` e aʻo hou e pili ana i ke ʻano a me ka hoʻonohonoho ʻana o ka aorta o ke pēpē.

Aia kekahi mau maʻi puʻuwai ʻē aʻe e pili ana me ka "Coarctation of the Aorta"?

ʻAe, hiki ke ʻike ʻia nā pēpē me CoA me nā maʻi puʻuwai ʻē aʻe. No ka laʻana, ma waena o 45% a me 75% o ka poʻe me CoA he maʻi i kapa ʻia ʻo bicuspid aortic valve. ʻO ke ʻano kēia, ʻelua wale nō leaflet o ka valve e lawe ana i ke koko mai ka puʻuwai i ka aorta ma kahi o nā ʻekolu e pono ai ke loaʻa.

ʻAʻole wale kēlā, akā hiki ke hele pū ʻia ka `(CoA)` me kēia mau kūlana:

• ʻO ka loaʻa ʻana o kahi lua ma ka puʻuwai - ua kapa ʻia kēlā he `(atrial septal defect)` a i ʻole `(ventricular septal defect)`.
• ʻO kahi kīʻaha koko i loaʻa i ka wā o ke ola fetal (`ductus arteriosus`) e noho hāmama ana no ka lōʻihi ma mua o ka mea i manaʻo ʻia - ua kapa ʻia kēia ʻo "patent ductus arteriosus".
• ʻO ka hypoplasia aortic arch hypoplastic left heart syndrome a i ʻole ka maʻi puʻuwai hema hypoplastic.
• ʻOʻoleʻa o ka valve puʻuwai - ʻo ia hoʻi, ka stenosis valve aortic a i ʻole ka stenosis valve mitral.

Nānā nā kauka lapaʻau puʻuwai i kēia mau mea āpau i loko o ka puʻuwai o kāu pēpē a hana i kahi hoʻolālā lapaʻau e pili pono ana iā lākou. ʻO ke ʻano kēia, ʻoi aku ka paʻakikī o ka mālama ʻana i kahi pēpē me nā kīnā puʻuwai he nui ma mua o ka mālama ʻana i kahi pēpē me hoʻokahi CoA wale nō.

He aha nā lāʻau lapaʻau no kēia?

Aia nā koho lapaʻau i ka makahiki o kāu keiki, ka nui o ka haiki ʻana o ka aorta, a inā he mau kīnā puʻuwai ʻē aʻe kona. ʻO ke ʻoki kino ke ala maikaʻi loa e mālama ai i ka coagulopathy i nā pēpē a me nā keiki liʻiliʻi. No nā keiki makua, inā ʻaʻole ʻoi aku ka koʻikoʻi o ka haiki ʻana, kūpono paha ka catheterization cardiac, kahi hana i emi iki ka invasive ma mua o ke ʻoki kino. Hoʻohana ʻia nō hoʻi ia i nā keiki a me nā pākeke i loaʻa hou ka haiki ʻana ma hope o ka mālama ʻana ma mua.

ʻoki kino

Aia kekahi mau ʻano hana nui i hana ʻia e hoʻoponopono i kēia kūlana `(CoA)`:

• ʻO ka ʻoki ʻana me ka anastomosis mai ka hopena a i ka hopena: Inā liʻiliʻi ka coarctation, hiki i ke kauka lapaʻau ke ʻoki i ka ʻāpana i paʻa ʻia o ka aorta o ke pēpē a hoʻohui hou i nā wēlau i koe.
• ʻO ka ʻoki ʻana me ka anastomosis hoʻonui ʻia mai ka ʻaoʻao a i ka ʻaoʻao:Inā ua ālai ʻia ke kīkoʻo aortic o ka pēpē, hiki i ke kauka lapaʻau ke ʻoki i ka ʻāpana i ālai ʻia o ka aorta a hoʻohui i ka ʻaoʻao haʻahaʻa o ka aorta descending i kahi ʻoki lōʻihi ma ke kīkoʻo aortic. ʻO kēia ke koho maikaʻi loa i ka wā e loaʻa ai i ka pēpē ka transverse aortic arch hypoplasia a me ka aortic coarctation.

Pono paha e hāʻawi ʻia nā lāʻau lapaʻau i nā pēpē e hōʻike ana i nā hōʻailona koʻikoʻi ma hope o ka hānau ʻana ma mua o ke ʻoki ʻana. Eia kekahi laʻana:

• Hiki ke hāʻawi ʻia kahi lāʻau lapaʻau i kapa ʻia ʻo `(Prostaglandin (PGE-1))` e wehe i ka `(ductus arteriosus)` o ka pēpē. E ʻae kēia i ka pēpē e loaʻa ka oxygen e pono ai a mālama iā lākou i ka paʻa a hiki i ka mākaukau no ke ʻoki ʻana.
• Hiki nō hoʻi iā ʻoe ke hāʻawi i ka pēpē i ka lāʻau lapaʻau e kōkua i kona puʻuwai e pamu i ke koko.

Ka hoʻopili ʻana o ka puʻuwai

Inā ua hōʻemi kāu keiki i ka hoʻohaiki aortic, a inā paha ua hoʻi hou ka ʻoki ʻana ma hope o ke ʻoki ʻana, hiki i ke kauka ke kuhikuhi i nā lāʻau lapaʻau e like me:

• ʻO ka angioplasty balloon: Hoʻohana ke kauka lapaʻau i kahi balloon liʻiliʻi e hoʻonui i ka ʻāpana haiki o ka aorta o ke keiki.
• ʻO ka angioplasty balloon me ke kau ʻana o ka stent: ʻOiai ke hoʻonui nei ke kauka lapaʻau i ka aorta o ke keiki, hoʻokomo pū ʻo ia i kahi ʻōmole liʻiliʻi, i kapa ʻia he stent, i ʻole e ālai hou ʻia.

Āhea wau e lawe ai i kaʻu keiki i ke kauka? (Nā hoʻokolokolo hou ʻana)

E haʻi aku ke kime lapaʻau o kāu keiki iā ʻoe i ka manawa e lawe mai ai i kāu keiki. Ma ke ola holoʻokoʻa o kāu keiki, pono lākou e loaʻa i nā hoʻokolohua hahai me kahi loea maʻi puʻuwai hānau. E hana kēia loea:

• E nānā mau i ke koko o ke keiki.
• Ke hana nei i nā scan (nā hoʻāʻo kiʻi) i nā manawa maʻamau e nānā i ka hana o ka puʻuwai o ke keiki a no nā hōʻailona o nā pilikia.
• Ke hāʻawi nei i nā ʻōlelo aʻoaʻo no nā ʻano ʻai olakino no ka puʻuwai a me ka hoʻoikaika kino.
• Ke kuhikuhi ʻana i nā lāʻau lapaʻau a me ka hoʻololi ʻana i kā lākou dosage inā pono.

ʻEhia ka lōʻihi e hiki ai i kekahi me kēia maʻi ke ola?

Me nā ʻano hana loiloi a me ka mālama ʻana i ka holomua, hiki i ka poʻe me Coarctation of the Aorta ke ola a hiki i ka liʻiliʻi he 60 mau makahiki. I ka wā ma mua, ʻo ka awelika o ke ola no ka poʻe me kēia maʻi ma kahi o 35 mau makahiki. No laila he nūhou maikaʻi maoli kēia.

Inā loaʻa i kāu pēpē ka Coarctation of the Aorta, e noʻonoʻo paha ʻoe i ke kumu i hana ʻia ai. ʻAʻole hiki iā mākou ke kuhikuhi mau i ke kumu pololei o ka maʻi puʻuwai hānau. Akā ua ʻike mākou hiki i ka ʻike mua ʻana a me ka mālama ʻana ke hōʻemi nui i ka hopena o kēia ʻano ma ke ola o kahi keiki.

Aia mau ke kime lapaʻau ma laila e kōkua i kāu keiki. Ua mākaukau lākou e pane i nā nīnau āu e loaʻa ai a e kōkua iā ʻoe e hoʻomaopopo pehea e hoʻopilikia ai kēia hōʻailona `(CoA)` i kāu keiki. Hiki paha iā ʻoe ke ʻike he mea kōkua ke kamaʻilio me nā mākua ʻē aʻe o nā keiki me kēia ʻano maʻi puʻuwai.

Nā mea nui e hoʻomanaʻo (Leka Lawe-Home)

Ke lana nei koʻu manaʻo ua maopopo iā ʻoe ke ʻano o ke kūlana a mākou i kūkākūkā ai i kapa ʻia ʻo "Coarctation of the Aorta". Eia kekahi o nā mea nui e hoʻomanaʻo ai:

• He kīnā puʻuwai hānau kēia; kahi paʻa ʻana i loko o ke aʻa nui e lawe ana i ke koko mai ka puʻuwai a i ke kino.
• Hiki ke loaʻa nā hōʻailona i ka wā kamaliʻi a i ʻole e ulu aʻe ma hope. Inā ʻike ʻoe i ka hiamoe nui, ka paʻakikī o ka hanu ʻana, a i ʻole ka makemake ʻole e hānai i kāu pēpē i ka waiū, e ʻimi koke i ke kōkua lapaʻau.
• ʻO ka ʻike mua ʻana a me ka mālama ʻana i ka maʻi e hāʻawi i ke keiki i kahi manawa kūpono nui aʻe e ola i kahi ola maʻamau a olakino hoʻi.
• Hiki ke mālama pono ʻia kēia kūlana me ke ʻoki ʻana a me nā lāʻau ʻē aʻe.
• He mea nui ka nānā lapaʻau kūikawā no ke ola holoʻokoʻa ma hope o ka mālama ʻia ʻana.

Mai makaʻu. Ua holomua loa ka ʻepekema lapaʻau i kēia lā. Aia nā kauka akamai e hiki ke hāʻawi i ka lāʻau lapaʻau maikaʻi loa no kāu keiki. ʻO ka mea nui loa, ʻo ia kou ʻike i kēia, e hoʻolohe i nā loli o kāu keiki, a e ʻimi i ke aʻo lapaʻau i ka manawa kūpono.

ʻO ke Coarctation o ka Aorta, Maʻi Puʻuwai, Pediatrics, Maʻi Puʻuwai Hānau, Aortic Stenosis, Ola Kino o ke Keiki, Kaomi Koko