Loaʻa i kāu keiki liʻiliʻi kēia ʻano pilikia puʻuwai? E aʻo kākou e pili ana i ka Endocardial Cushion Defect!

Ke hānau ʻia kāu pēpē, hopohopo nui ʻoe e pili ana i nā mea āpau e pili ana iā ia, ʻeā? I kekahi manawa hiki i nā keiki liʻiliʻi ke loaʻa nā kīnā puʻuwai hānau. ʻO kekahi o ia ʻano maʻi puʻuwai, ʻoiai he mea paʻakikī iki, he mea nui loa ia e makaʻala ʻia, ʻo ia ka Endocardial Cushion Defect (ECD) . He mea weliweli iki paha ka inoa, akā mai hopohopo. E kamaʻilio kākou e pili ana i kēia me ka maʻalahi, ma ke ʻano e hiki ai iā ʻoe ke hoʻomaopopo, ʻae?

He aha kēia kīnā o ka cushion endocardial?

I ka ʻōlelo maʻalahi, he kīnā kēia i loko o ka puʻuwai o kā kākou poʻe liʻiliʻi, he mea hānau mai , ʻo ia hoʻi, he kīnā e kū mai ana i ko lākou wā i loko o ka ʻōpū o ka makuahine. E noʻonoʻo i ko kākou puʻuwai me he hale lā me ʻehā lumi. Aia ʻelua lumi ma ka papahele kiʻekiʻe (a mākou e kapa nei he atria), a ʻelua lumi ma ka papahele lalo (ʻo ia hoʻi nā ventricles). Ua hoʻokaʻawale ʻia kēia mau lumi kekahi i kekahi e nā paia (septums). Ma muli o kēia mau paia, ke kahe nei ke koko ma ke ʻano maʻamau, ma hoʻokahi wale nō ʻaoʻao.

Eia kekahi, aia nā kiwikā e like me ka puka ma waena o kēia mau keʻena e kōkua ana i ke kahe ʻana o ke koko i hoʻokahi ala wale nō. ʻO ka mea nui, ʻo ka kiwikā tricuspid a me ka kiwikā mitral, aia ma waena o nā keʻena o luna (atria) a me nā keʻena o lalo (nā keʻena mitral ), pono e hana pono ʻia.

Eia nō naʻe, i loko o kahi pēpē me kēia kīnā endocardial cushion (ECD), ʻaʻole e ulu pono nā ʻāpana o ka puʻuwai i kapa ʻia ʻo endocardial cushion - ʻo ia hoʻi nā ʻiʻo kumu e hana ai i kēia mau paia a me nā valves. ʻO ka hopena, ʻaʻole paha e ulu pono nā paia e hoʻokaʻawale ana i nā keʻena o ka puʻuwai, a i ʻole e nalowale loa lākou. ʻAʻole paha e ulu pono nā valves koʻikoʻi ʻelua a mākou i ʻōlelo ai (tricuspid a me mitral).

E noʻonoʻo, he aha ka mea e hana ʻia ke hana pono ʻole kēia mau māhele i loko o ka puʻuwai, kēia mau paia, a ʻaʻole hana pono nā valves, e like me nā ʻīpuka? Ua hoʻopilikia ʻia ke kaʻina o ke kahe ʻana o ke koko i loko o ka puʻuwai.

• ʻOi aku ka nui o ke koko ma mua o ka maʻamau e kahe ana i loko o nā māmā o ka pēpē , e hoʻoulu ai i ke koko kiʻekiʻe i loko o nā kīʻaha koko i loko o nā māmā (pulmonary high blood pressure).
• Ma muli o kēia kaomi kiʻekiʻe i loko o nā māmā, hoʻomaka ke koko e kahe i ke ala kūpono ʻole. A laila , hoʻomaka ke koko maʻemaʻe a waiwai i ka oxygen a me ke koko lepo a nele i ka oxygen e hui pū .
• Pono ka puʻuwai e hana ikaika e pamu i ke koko me kēia kahe koko i hoʻopilikia ʻia. Ua like ia me ka piʻi ʻana i kahi mauna. I ka hala ʻana o ka manawa, hiki i kēia ke hoʻonāwaliwali i ka ʻiʻo puʻuwai o ka pēpē.

Aia kekahi mau ʻano kīnā endocardial cushion (ECD) like ʻole?

ʻAe, aia kekahi mau ʻano o kēia ʻano, ma muli o ka nui o ka pōʻino. Loaʻa paha i kekahi mau pēpē nā pilikia me ka paia ma waena o nā keʻena o luna o ka puʻuwai (atria). Loaʻa paha i kekahi poʻe nā pilikia me ka paia ma waena o nā keʻena haʻahaʻa (ventricles). I kekahi manawa hiki ke hui pū ʻia nā pilikia ʻelua. Eia kekahi, hiki ke ʻokoʻa nā hemahema i loko o nā valves mitral a me tricuspid.

ʻO kēia kūlana, i kapa ʻia ʻo endocardial cushion defect (ECD), ua ʻike ʻia hoʻi ʻo atrioventricular septal defect (AVSD) a i ʻole atrioventricular canal defect .

Hiki ke pili kēia kūlana i kekahi manawa me nā kīnā puʻuwai hānau ʻē aʻe. Eia kekahi laʻana:

• ʻO ke Tetralogy o Fallot
• ʻO ka ventricle ʻākau puka pālua
• Ke hoʻololi ʻana o nā aʻa koko nui (ke hoʻololi ʻana o nā kīʻaha koko nui ʻelua i nā wahi kūpono ʻole)
• ʻO ka ductus arteriosus patent
• ʻAʻa hoʻokahi (ʻaʻa hoʻokahi)

Pehea ka maʻamau o kēia ʻano?

Inā ʻoe e nānā i nā helu helu ma nā ʻāina e like me ʻAmelika Hui Pū ʻIa, ma kahi o hoʻokahi i loko o 1,800 mau pēpē i hānau ʻia i kēlā me kēia makahiki e loaʻa paha kēia kīnā endocardial cushion (ECD). ʻO kekahi mea nui ʻē aʻe, ʻo ia ma kahi o 20% o ka poʻe me Down syndrome e loaʻa ana kēia maʻi ECD.

Pehea ka hopena o ka ECD i ke kino o kaʻu pēpē?

Inā ʻaʻole mālama pono ʻia kēia kūlana ECD, hiki iā ia ke lilo i mea koʻikoʻi a hoʻoulu i nā pilikia koʻikoʻi.

• Ka hōʻino ʻana o ka māmā: E like me kā mākou i kūkākūkā ai ma mua, ʻo ke kahe nui o ke koko i nā māmā, ka hoʻonui ʻia o ke kaomi, a me ka hala ʻana o ka manawa, hiki ke hōʻeha mau loa i nā māmā.
• ʻAno ʻē ka ʻōlinolino o ka puʻuwai: Ua hoʻopilikia ʻia ka hana uila o ka puʻuwai, a hiki ke lilo ka puʻuwai i mea ʻē.
• ʻO ka hāʻule ʻana o ka puʻuwai paʻa: ʻO ke ʻano e nāwaliwali ai ka puʻuwai a hiki ʻole ke pamu i ke koko kūpono i ke kino.
• ʻO ka maʻi Eisenmenger: He kūlana koʻikoʻi loa kēia. Piʻi nui ke kaomi i loko o nā māmā, e hoʻohuli loa ana i ke kahe o ke koko, e hoʻopilikia ana i ke koko nele i ka oxygen e kaʻapuni i loko o ke kino.

I kekahi mau hihia koʻikoʻi, hiki i kēia kūlana ke hoʻoweliweli i ke ola .

Eia kekahi, ʻoiai ma hope o ka hoʻoponopono ʻia ʻana o ke kīnā me ke ʻoki ʻana, hiki i kāu keiki ke pilikia i ka loaʻa ʻana o kahi maʻi puʻuwai i kapa ʻia ʻo endocarditis . No laila, hiki i nā kauka ke ʻōlelo aku i ka lawe ʻana i nā antibiotics ma mua o kekahi mau kaʻina hana niho.

He aha nā hōʻailona o kēia maʻi?

I kekahi manawa, ʻaʻole paha e hōʻike nā pēpē me kahi kīnā endocardial hapa i kekahi mau hōʻailona a hiki i ko lākou wā ʻōpiopio loa. Eia nō naʻe, hiki i nā pēpē me kahi kīnā endocardial piha (ECD piha) ke loaʻa nā hōʻailona i loko o kekahi mau pule o ka hānau ʻana. He mea nui e makaʻala i kēia mau hōʻailona:

• Ka wikiwiki o ka puʻuwai
• ʻIli keʻokeʻo a polū paha:E nānā i ka polū ʻole o ke kala, ʻoiai hoʻi ma nā lehelehe, ke alelo, a me nā manamana lima. Ua kapa ʻia kēia he cyanosis.
• Pilikia i ka ʻai ʻana a me ka piʻi ʻana o ke kaumaha: E nānā inā he palupalu kāu pēpē, hou ka hou ma hope o ka inu ʻana i ka waiū, a ʻaʻole paha e piʻi pono ana ke kaumaha.
• ʻAʻohe ikaika: Ua hiamoe mau anei kāu pēpē a ʻaʻole makemake e pāʻani?
• Ka hanu wikiwiki a paʻakikī paha: E nānā e ʻike inā paʻa ka umauma o ke pēpē, ke hanu wikiwiki nei, a inā paha e komo ana ka umauma i loko i ka wā e hanu ana.
• Nā maʻi pinepine: E makaʻala i nā maʻi pinepine, ʻoiai nā maʻi hanu.

He aha ke kumu o kēia kīnā endocardial cushion?

ʻAʻole ʻike ʻia ke kumu kikoʻī o ka hemahema cushion endocardial (ECD). Eia nō naʻe, ua ʻike ʻia he pili loa ia me Down syndrome . Hiki mai kēia hemahema i ka wā mua o ka ulu ʻana o ka pēpē i loko o ka ʻōpū. ʻO ka pilikia nui, ʻo ka ʻiʻo i kapa ʻia ʻo endocardial cushion a mākou i kamaʻilio ai ma mua ʻaʻole ia e ulu pono. ʻO kēia nā paia a me nā valves e hoʻokaʻawale hope i nā keʻena o ka puʻuwai.

Pehea e ʻike ai nā kauka i kēia maʻi?

E hāʻawi mua ke kauka o kāu pēpē i kāu pēpē i kahi hoʻokolokolo kino piha . A laila, inā lohe ʻia kahi leo ʻē (heart murmur) i ka wā e hoʻolohe ai i ka puʻuwai me ka stethoscope, he hōʻailona paha ia no kēia maʻi.

Eia kekahi, hiki ke hana ʻia kekahi mau hoʻokolohua ʻē aʻe:

• Electrocardiogram (EKG/ECG): Hoʻāʻo kēia i ka hana uila o ka puʻuwai.
• Echocardiogram: ʻO kēia ka hoʻāʻo koʻikoʻi loa. Hoʻohana ia i nā nalu kani e kiʻi i ke kiʻi o ka puʻuwai. Hiki iā ia ke ʻike maopopo i nā mea āpau mai ke ʻano o ka puʻuwai, a i nā lua i loko o kona mau paia a pehea e hana ai nā valves.
• X-ray o ka umauma: Hiki i kēia ke nānā i ka nui o ka puʻuwai a me ke kūlana o nā māmā.
• MRI o ka puʻuwai: I kekahi manawa hana ʻia kēia hoʻāʻo inā pono nā kikoʻī hou aku.
• Catheterization cardiac: Ma kēia kaʻina hana, hoʻokomo ʻia kahi paipu liʻiliʻi (catheter) ma o kahi kīʻaha koko i loko o ka puʻuwai e ana i ke kaomi i loko o ka puʻuwai a lawe i nā hāpana koko. Pono paha kēia ma mua o ke ʻoki ʻana.

ʻO ka mea nui loa, ʻo ia ke hoʻomau i ka ʻike ʻana i kāu kauka i kou wā hāpai a hana i nā scan ultrasound.Ke hana pololei nei. I kekahi manawa, hiki ke ʻike ʻia ka ECD ma mua o ka hānau ʻana o ka pēpē, ma o kahi scan i hana ʻia ʻoiai ke pēpē i loko o ka ʻōpū (fetal echocardiogram). Ke ʻike mua ʻia kēia, hiki ke hoʻolālā no ka lāʻau e pono ai e hoʻomaka i ka wā e hānau ai ka pēpē.

Pehea e mālama ʻia ai kēia?

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

I ka wā o ke ʻoki ʻana, pani nā kauka lapaʻau puʻuwai i nā lua e hoʻokahe ai ke koko ma waena o nā keʻena o ka puʻuwai. Hoʻohana lākou i kahi ʻāpana kūikawā e hana ai i kēia. Eia kekahi, inā hoʻokahi wale nō valve o ka pēpē, e hana lākou i ʻelua mau valve mitral a me tricuspid kaʻawale mai ia mea. I kekahi manawa, inā e liki ka valve mitral o ka pēpē, hiki ke hoʻoponopono ʻia a hoʻololi ʻia paha inā pono.

Hiki ke hana ʻia kēia ʻoki kino ma kahi pēpē ma waena o 3 a me 6 mahina . Eia nō naʻe, inā he kīnā piha ko kāu pēpē i ka endocardial cushion, he mea nui e hana ʻia ke ʻoki kino ma mua o ka piha ʻana o ka pēpē i hoʻokahi makahiki . ʻO kēia wale nō ke ala e pale aku ai i ka hōʻino mau ʻana o ka māmā.

I kekahi manawa, maʻi loa paha ka pēpē a kaumaha ʻole paha e hana i kahi ʻoki piha. I kēlā hihia, e hana mua nā kauka i kahi ʻoki maʻalahi ʻē aʻe e kaupalena i ka nui o ke koko e hele ana i nā māmā o ka pēpē. Ua kapa ʻia kēia he pulmonary artery banding . Hiki i kēia ke kōkua i ka kaohi ʻana i nā hōʻailona a hiki i ka wā e ikaika ai ka pēpē a piʻi ke kaumaha. A laila, i ka manawa kūpono, hiki ke hana ʻia ka hoʻoponopono piha.

Aia kekahi mau pilikia i ka mālama ʻana?

E like me nā ʻoki kino āpau, aia kekahi mau pilikia e hiki ke kū mai ma hope o ke ʻoki kino ECD. Eia nō naʻe, i ka hapa nui o ka manawa, hiki ke mālama ʻia kēia mau mea.

• ʻO kahi valve mitral e liki ana: I kekahi manawa ma hope o ke ʻoki ʻana, ʻaʻole paha e pani pono ka valve mitral a hiki i kekahi koko ke liki i hope.
• ʻAno ʻē ka puʻuwai: Hiki ke loaʻa kekahi mau hewa i loko o ka puʻuwai.

Hiki ke kū mai kēia mau pilikia i kekahi manawa ke ʻelemakule iki ke keiki, ʻoiai i ko lākou wā ʻōpiopio. No laila he mea nui nā nānā lapaʻau maʻamau.

Hiki ke pale ʻia kēia kūlana?

ʻOiai ʻaʻole ʻike ʻia ke kumu kikoʻī o ka ECD, he paʻakikī ke ʻōlelo pehea e pale loa ai. Eia nō naʻe, inā ua loaʻa i kekahi o kou ʻohana kēia ʻano kīnā puʻuwai (ECD) a i ʻole nā ​​​​pilikia genetic ʻē aʻe, he manaʻo maikaʻi paha e loaʻa ka ʻōlelo aʻoaʻo genetic ma mua o kou hāpai ʻana.

Inā loaʻa ka ECD i kaʻu pēpē, he aha kaʻu e manaʻo ai?

ʻO kēia ka nīnau nui loa a nā mākua he nui. ʻO ka nūhou maikaʻi, ma hope o ke ʻoki ʻana e hoʻoponopono i ka ECD, hoʻomau ka hapa nui o nā pēpē i ke ola maʻamau a olakino. Eia nō naʻe, pili ke olakino o ka pēpē i ka wā e hiki mai ana i nā mea e like me ke koʻikoʻi o kā lākou ECD, inā ua hōʻino ʻia ko lākou mau māmā, a me ko lākou olakino holoʻokoʻa.

Wahi a nā helu helu, ʻo ka manawa kūpono e ola ai i ka makahiki mua ma hope o ke ʻoki ʻana ma kahi o 80%. ʻO ka manawa kūpono e ola ai no kekahi 10 mau makahiki ma kahi o 75%, a ʻo ka manawa kūpono e ola ai no 20 mau makahiki ma kahi o 65%.

ʻOiai ma hope o ke ʻoki ʻana, hiki i kekahi mau keiki ke loaʻa nā pilikia ma hope. ʻO kahi pilikia maʻamau, ʻaʻole pani pono ka valve mitral a kahe ke koko. Inā hana ʻia kēia, hiki ke hana ʻia kekahi ʻoki ʻē aʻe e hoʻoponopono ai.

Pehea ka lōʻihi o ke kūlana ECD?

He mea nui loa kēia. ʻAʻole ka hemahema o ka cushion endocardial (ECD) he maʻi e ho'ōla pono'ī. E mau ana ka pilikia a hiki i ka wā e hana ai ke kauka puʻuwai i ke ʻoki ʻana e hoʻoponopono i ke kīnā. Me ka ʻole o ke ʻoki ʻana, ʻaʻole e ola nā pēpē he nui ma mua o 2 a 3 paha mau makahiki. ʻO ia ke kumu he mea nui ka ʻoki ʻana i ka manawa kūpono.

Pehea wau e mālama ai i kaʻu pēpē?

Ma hope o ka ʻike ʻia ʻana o kāu pēpē me ka ECD, a ma hope o ke ʻoki ʻana, pono kāu pēpē e hana i nā nānā maʻamau me kahi kauka puʻuwai . Hiki ke hana ʻia kēia mau manawa hoʻokolokolo hoʻokahi a ʻelua paha i ka makahiki. E hoʻomau ʻia kēia mau nānā ʻana i ka wā kamaliʻi o kāu keiki a hiki i ka wā makua. Hiki i ke kauka ke nānā i ke kūlana o kāu pēpē, e nānā i nā pilikia me ka ʻāpana a i ʻole ka valve, e like me nā leaka, a hana koke inā pono.

Āhea wau e lawe ai i kaʻu pēpē i ke kauka?

Inā hōʻike kāu pēpē i kekahi o nā hōʻailona ma lalo nei, e ʻike koke i ke kauka:

• Ke paʻakikī nei ka hanu ʻana
• Luhi me ka ʻole o ka hoʻoikaika nui ʻana ma hope o ka holo ʻana a me ka pāʻani ʻana no kekahi manawa
• ʻAʻole e ulu a loaʻa paha ke kaumaha e like me ka mea i manaʻo ʻia
• Loaʻa kahi ʻili polū

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

He mea nui loa ia e hoʻomaopopo i nā kānalua āu e loaʻa ai. Mai makaʻu e nīnau i kāu kauka i nā nīnau e like me:

• "E ke kauka, he kīnā piha a hapa paha ko kaʻu pēpē i ka cushion endocardial?"
• "He aha ka hoʻolālā lapaʻau a ke kauka e ʻōlelo ai no ke kūlana o kaʻu pēpē?"
• "E ke kauka, ehia mau ʻoki kino ECD āu i hana ai e like me kēia?"
• "He aha nā mea kūikawā e pono ai iaʻu e mālama ma hope o ke ʻoki kino?"

Nā mea nui e hoʻomanaʻo (Leka Lawe-Home)

ʻAʻole paha ʻoe i moeʻuhane e hana ʻoki no kāu keiki liʻiliʻi. Eia nō naʻe, ʻo ke ʻoki ʻana ke ala maikaʻi loa a maikaʻi loa e hoʻoponopono ai i kahi kīnā endocardial cushion (ECD). ʻAʻole e hoʻomaikaʻi kēia pilikia iā ia iho. No laila, inā he mea āu e maopopo ʻole ai, e noi i kāu kauka e wehewehe iā ʻoe.

ʻO ka mea nui loa, ʻaʻole ʻoe hoʻokahi. Aia nā kauka, nā kahu maʻi, a me ke kime mālama ola holoʻokoʻa e kōkua iā ʻoe a hoʻoikaika iā ʻoe ma kēia huakaʻi. ʻOiai ma hope o ke ʻoki ʻana, mai poina i nā keʻena lapaʻau o kāu pēpē a e hahai i nā ʻōlelo a ke kauka e like me ke kauoha. Ma kēlā ala, hiki iā ʻoe a me kāu keiki liʻiliʻi ke ʻike i nā pilikia e kū mai ana i ka wā e hiki mai ana a hoʻoponopono pono iā lākou. E noho ikaika!

ʻO ke kīnā o ka Endocardial Cushion, AVSD, maʻi puʻuwai, nā kīnā hānau, maʻi puʻuwai pediatric, ʻoki puʻuwai, Down syndrome, maʻi puʻuwai hānau, cardiology pediatric