Loaʻa i kāu pēpē kēia pilikia puʻuwai? E kamaʻilio kākou e pili ana i ka Coarctation o ka Aorta

He mea maʻamau ka makaʻu a me ka haʻalulu ke haʻi ʻia kāu keiki liʻiliʻi e ke kauka he kīnā puʻuwai hānau ko lākou. Akā, mai hopohopo. Inā ʻike piha ʻoe i ke kūlana a mākou e kamaʻilio ai i kēia lā, i kapa ʻia ʻo Coarctation of the Aorta, hiki iā ʻoe ke hoʻoponopono pono iā ia. E nānā pono kākou i ke ʻano o ia mea, ke kumu e hana ai, a me nā mea hiki ke hana ʻia.

He aha ka Coarctation o ka Aorta (CoA)?

I ka ʻōlelo maʻalahi, ʻo ka Coarctation of the Aorta (CoA) he maʻi puʻuwai hānau . Hana ia i ka wā e haiki ai kahi ʻāpana o ka aorta, ke kīʻaha koko nui e lawe ana i ke koko oxygenated mai ko mākou puʻuwai a i ke koena o ko mākou kino . ʻO ka huaʻōlelo "coarctation" ʻo ia hoʻi "ka haiki ʻana."

E noʻonoʻo i ka mea e hana ʻia inā pani koke ʻia kekahi ala o ke alanui i ka wā o ka paʻa nui o ke kaʻa ma ke Alanui ʻo Colombo-Galle? E hoʻomaka ana nā kaʻa a pau e neʻe, a e nui loa ke kaʻa. ʻO ia ka mea e hana ʻia ke ālai ʻia kekahi o nā kīʻaha koko nui loa o ko kākou kino. ʻAʻole hiki i ke koko ke kahe pono.

ʻO ka maʻamau, hana ʻia kēia paʻa ʻana ma mua o ka māhele ʻana o nā aʻa koko e lawe ana i ke koko i nā ʻāpana haʻahaʻa o ke kino. Hoʻonui kēia i ke kaomi koko mai ka puʻuwai a i nā ʻāpana o luna (nā lima, ke poʻo), a emi iho ke kaomi mai ke koko a i nā ʻāpana haʻahaʻa o ke kino (nā wāwae). ʻO ka ʻoiaʻiʻo, ʻo kēia ʻokoʻa nui a ke kauka e ʻike ai i ka wā e nānā ai i ke kaomi koko ma nā lima a me nā wāwae o kāu pēpē kekahi o nā hōʻailona nui e ʻike ai i kēia maʻi.

He aha nā hōʻailona? Aia kekahi ʻokoʻa ma waena o nā pēpē a me nā keiki makua?

ʻOkoʻa nā hōʻailona ma muli o ke koʻikoʻi o ka paʻa ʻana. Inā koʻikoʻi ka paʻa ʻana, hiki i ka pēpē ke hoʻomaka e hōʻike i nā hōʻailona i loko o hoʻokahi a ʻelua paha pule o ka hānau ʻana. Eia nō naʻe, inā liʻiliʻi loa ka paʻa ʻana, ʻaʻole paha e ʻike ʻia nā hōʻailona a hiki i ka wā kamaliʻi, a i ʻole ʻaʻole loa.

ʻAʻole paha e ʻike kekahi mau keiki i kekahi mau hōʻailona. I kekahi manawa, ʻo ka manawa mua e ʻike ai lākou i kēia pilikia ke nānā ke kauka i ko lākou koko ma kahi keʻena lapaʻau maʻamau a ʻike he kiʻekiʻe ia.

No ke aha lā kēia e hana nei? He aha nā kumu?

ʻOiai ʻaʻole ʻike ʻia ke kumu kikoʻī o kēia, manaʻoʻiʻo nā kauka he ʻāpana genetic kona. ʻO ke ʻano kēia, ʻo kekahi mau loli genetic e hana ʻia ana i ka wā e ulu ana ke pēpē i loko o ka ʻōpū e pale aku i ka aorta mai ka hoʻokumu pono ʻana.

ʻO kekahi kumu nui o kēia he loli i ke ʻano o ka pani ʻana o kahi kīʻaha koko liʻiliʻi i kapa ʻia ʻo ductus arteriosus .

Eia kekahi, ʻo nā pēpē me kekahi mau kūlana genetic, e like me ka Turner syndrome, ua nui ka pilikia o ka hoʻomohala ʻana i kēia kūlana CoA.

He aha nā pilikia e hiki ke kū mai inā ʻaʻole mālama ʻia?

Inā ʻaʻole i ʻike ʻia a mālama ʻia kēia maʻi i ka wā mua, hiki ke loaʻa nā pilikia like ʻole i ka hala ʻana o ka manawa.

• Ke koko kiʻekiʻe (hypertension) ma ke kino holoʻokoʻa.
• Ka mānoanoa nui o ka ʻiʻo o ka puʻuwai (hypertrophy ventricular hema) .
• Nā waihona momona i loko o nā aʻa e hoʻolako koko i ka puʻuwai (ka maʻi artery coronary) ma mua o ka manawa maʻamau.
• He puʻupuʻu e like me ka pōpō pehu (aneurysm) i loko o ka aorta a i ʻole kekahi kīʻaha koko i loko o ka lolo.
• ʻO ke kūlana kahi i hiki ʻole ai i ka puʻuwai ke pamu i ke koko lawahiki mai (ka hāʻule ʻana o ka puʻuwai).

Pehea e ʻike pololei ai nā kauka i kēia maʻi?

ʻO nā kauka puʻuwai keiki ka poʻe e ʻike i kēia maʻi. Hana nui ʻia kēia ma o ka nānā kino ʻana i ke pēpē.

• Ke nānā nei i ka ʻokoʻa o ke kaomi koko ma nā lima a me nā wāwae .
• Ke nānā nei i ka pulse ma ka ʻāʻī a me ka ʻūhā.
• ʻO ka murmur puʻuwai he leo ʻokoʻa ia e lohe ʻia ke kau ʻia ʻana o kahi stethoscope ma ka umauma a me ke kua.

Eia kekahi, hana ʻia kahi Echocardiogram (echo) , kahi scan o ka puʻuwai, e hōʻoia i ka hōʻailona a hoʻoholo i ka nui o ka paʻa ʻana. I kekahi manawa, pono paha nā scan CT a MRI paha.

Nā maʻi puʻuwai ʻē aʻe e hiki ke hana me kēia

Ma kahi o 45% a 75% o nā pēpē me CoA he maʻi i kapa ʻia ʻo bicuspid aortic valve . ʻO ke ʻano kēia, ʻelua wale nō cusps o ka valve e lawe ana i ke koko mai ka puʻuwai a i ka aorta ma kahi o ʻekolu. Eia kekahi, hiki ke ʻike ʻia nā kūlana e like me nā lua i loko o ka puʻuwai (atrial septal defect a i ʻole ventricular septal defect) me kēia maʻi.

He aha nā koho lapaʻau?

Hoʻoholo ʻia ka lāʻau lapaʻau ma muli o ka makahiki o ke keiki, ke ʻano o ka stenosis aortic, a inā he mau maʻi puʻuwai ʻē aʻe.

ʻoki kino

ʻO ke ʻoki ʻana ka lāʻau lapaʻau maikaʻi loa no nā pēpē a me nā kamaliʻi.

• ʻO ka hoʻihoʻi ʻana me ka anastomosis end-to-end: ʻO kēia ka ʻoki ʻana i ka ʻāpana liʻiliʻi o ka blockage a me ka hoʻopili hou ʻana i nā ʻāpana olakino ʻelua.
• ʻO ka hoʻihoʻi ʻana me ka anastomosis hoʻonui ʻia mai ka hopena a i ka hopena: Inā he poloka ma ke kūlou o ka aorta, hana ʻia kēia ʻoki ʻoi aku ka paʻakikī.

Pono paha e hāʻawi ʻia nā lāʻau lapaʻau e like me prostaglandin (PGE-1) i nā pēpē i loaʻa nā hōʻailona koʻikoʻi i ka wā hānau e like me ka prostaglandin (PGE-1) e hoʻopaʻa i ke kūlana o ka pēpē ma mua o ke ʻoki ʻana.

Ka hoʻokomo ʻana o ka catheter cardiac

He ʻano maʻalahi kēia ma mua o ke ʻoki ʻana. Hoʻohana ʻia kēia ʻano hana inā he liʻiliʻi ka paʻa ʻana, a inā paha e hoʻi hou ka paʻa ʻana ma hope o ke ʻoki ʻana.

• ʻO ka angioplasty balloon: Hoʻokomo ʻia kahi mea liʻiliʻi e like me ka balloon i loko o kahi kīʻaha koko a hoʻonui ʻia e hoʻonui i ka wahi i paʻa.
• ʻO ka angioplasty balloon me ke kau ʻana o ka stent: Hoʻonui ʻia ka wahi i paʻa ʻia me kahi balloon, a ua kau ʻia kahi ʻōmole liʻiliʻi e like me ka mesh (stent) i loko e pale ai i ka wahi mai ka paʻa hou ʻana.

Pehea lā ka wā e hiki mai ana o kaʻu keiki?

Me nā ʻano hana loiloi holomua a me nā lāʻau lapaʻau o kēia wā, hiki i kahi keiki me CoA ke ola i kahi ola maʻamau a olakino. ʻOiai i ka wā ma mua, ʻo ka awelika o ke ola no kēia mau maʻi ma kahi o 35 mau makahiki, hiki iā lākou ke ola olakino no 60 mau makahiki a ʻoi aku paha.

ʻO ka mea nui loa, ʻoiai ma hope o ka mālama ʻia ʻana, pono ʻoe e hele i ke keʻena o ke kauka cardiologist no ke koena o kou ola.

A laila, e Kauka,

• Hoʻopaʻa mau ʻia ke koko o ke keiki.
• Hana ʻia nā hoʻokolohua e like me nā scan e nānā i ka hana o ka puʻuwai.
• Hāʻawi i nā ʻōlelo aʻoaʻo no nā ʻano ʻai olakino no ka puʻuwai a me ka hoʻoikaika kino.
• Hoʻolako i ka lāʻau lapaʻau inā pono.

Ke ʻike ʻoe ua loaʻa i kāu keiki kēia maʻi, nui paha kāu mau nīnau. E noʻonoʻo paha ʻoe no ke aha i hana ʻia ai kēia. E hoʻomanaʻo mau, ʻaʻole kēia kou hewa. E hana kāu kauka a me ka hui lapaʻau i nā mea āpau e hiki ai iā lākou ke kōkua iā ʻoe a me kāu keiki. Mai kānalua e nīnau iā lākou a kamaʻilio e pili ana i kou mau makaʻu.

Coarctation o ka Aorta Sinhala, Coarctation o ka Aorta, Maʻi puʻuwai hānau, Maʻi puʻuwai o ka pēpē, Maʻi puʻuwai pediatric, Ke koko kiʻekiʻe, ʻAʻohe kīnā puʻuwai hānau Sinhala