Loaʻa i kāu keiki liʻiliʻi kēia ʻano pilikia puʻuwai? (Total Anomalous Pulmonary Venous Return - TAPVR) E kamaʻilio kākou e pili ana i kēia!

Ke hānau ʻia kāu pēpē, manaʻo paha ʻoe i kekahi manawa he paʻakikī ke hanu? Ke nānā aku nei paha ʻoe i kekahi manawa me he mea lā e uliuli ana ka ʻili? He mea maʻamau ke makaʻu nui ke ʻike ʻoe i kahi mea e like me ia. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi puʻuwai hānau e hōʻike ana i kēia mau hōʻailona. Ua kapa ʻia kēia ʻo TAPVR (Total Anomalous Pulmonary Venous Return) . ʻOiai paha he mea paʻakikī iki ka inoa, e hoʻomaopopo maʻalahi kākou.

He aha ka TAPVR? I ka ʻōlelo maʻalahi...

E noʻonoʻo e pili ana i ia mea, loaʻa i ko kākou kino ka oxygen mai nā māmā, ʻeā? No laila, mai kēia mau māmā, lawe ʻia ke koko i piha i ka oxygen (e kapa kākou i kēia he "koko maikaʻi") i ka puʻuwai e nā aa koko , kahi ʻano kīʻaha koko. ʻO ka maʻamau, pono e hele mai kēia "koko maikaʻi" i ka atrium hema ma ka ʻaoʻao hema o ka puʻuwai. Mai laila mai, hoʻopuka ʻia ke koko e hāʻawi i ka oxygen i ke kino holoʻokoʻa.

Eia nō naʻe, i loko o kahi pēpē me TAPVR, ma kahi o ka hoʻopili ʻana i ka atrium hema o ka puʻuwai, hoʻopili kēia aa pulmonary i kahi kūpono ʻole, ʻo ia ka atrium ʻākau . Loaʻa i ka atrium ʻākau ke "koko maikaʻi ʻole" i hoʻohana ʻia e ke kino, he haʻahaʻa i ka oxygen. I kēia manawa, hui pū ke "koko maikaʻi" i piha i ka oxygen me kēia "koko maikaʻi ʻole." He aha ka mea e hana ai? ʻO ka nui o ka oxygen i loko o ke koko i manaʻo ʻia e hele i ke kino holoʻokoʻa e emi ana. He kūlana weliweli loa kēia a he mea hoʻoweliweli i ke ola no kahi pēpē. He maʻi puʻuwai hānau kēia.

ʻAneʻane nā pēpē a pau me TAPVR he lua liʻiliʻi (Atrial Septal Defect - ASD) ma waena o nā keʻena ʻelua o ko lākou puʻuwai, ʻo ia ka atrium ʻākau a me ka atrium hema. ʻAe kēia lua i kekahi koko oxygenated e kahe i ka ʻaoʻao hema o ka puʻuwai a laila i ke koena o ke kino. Inā ʻaʻole, ua ʻoi aku ka ʻino o ke kūlana.

ʻO ka mea nui loa, ʻo ia ka pono o ke ʻoki ʻana i nā pēpē me TAPVR. Inā hana mua ʻia ke ʻoki ʻana, hiki i ka hapa nui o nā keiki ke ola olakino a hiki i ka wā makua. Eia nō naʻe, i kekahi manawa, ʻo nā mea e like me ka hāiki ʻana o nā aʻa koko ma hope o ke ola e pono ai ke ʻoki hou aʻe a i ʻole nā ​​​​lāʻau lapaʻau ʻē aʻe. No laila, ʻoiai ma hope o ke ʻoki ʻana o TAPVR, pono ʻoe e nānā ʻia e kahi kauka cardiologist no ke koena o kou ola.

I kekahi manawa kapa pū nā kauka i kēia kūlana ʻo TAPVC (Total Anomalous Pulmonary Venous Connection). He ʻano like nā mea ʻelua.

Aia kekahi mau ʻano TAPVR like ʻole?

ʻAe, aia kekahi mau ʻano nui o TAPVR. Ua hoʻokaʻawale ʻia kēia mau mea e like me ke ʻano o ka hoʻopili pono ʻole ʻia ʻana o ke "koko maikaʻi" mai nā māmā i ka atrium ʻākau.

• ʻO Supracardiac TAPVR: Ma kēia, hui pū nā aa pulmonary a pili i kahi aa nui i loaʻa ma luna o ka puʻuwai (i kapa ʻia ʻo superior vena cava , he aa nui ia e lawe i ke koko mai nā ʻaoʻao o luna o ke kino a i ka ʻaoʻao ʻākau o ka puʻuwai).
• Cardiac TAPVR: Maanei, ke kahe pololei nei nā aa pulmonary i loko o ka sinus coronary ma ka ʻaoʻao ʻākau o ka puʻuwai.Hoʻohui. ʻO kēia aʻa coronary kahi e hōʻiliʻili pinepine ai ke koko nele i ka oxygen mai ka ʻiʻo puʻuwai.
• ʻO ka TAPVR Infracardiac: Ma kēia ʻano, hele ka aʻa pulmonary ma lalo o ka puʻuwai, ma o ke akepaʻa, a pili i kahi aʻa nui ( inferior vena cava ) e lawe ana i ke koko mai nā ʻaoʻao haʻahaʻa o ke kino a i ka ʻaoʻao ʻākau o ka puʻuwai.
• TAPVR Hui Pū ʻia: ʻO kēia ke ʻano kakaikahi loa a paʻakikī loa e hoʻoponopono. Maanei, hiki ke hoʻopili ʻia ma mua o hoʻokahi aa koko mai nā māmā, ma ka hui pū ʻana o hoʻokahi a ʻoi aku paha o nā ʻano i ʻōlelo ʻia ma luna.

He mea nui kēia hoʻokaʻawale ʻana no nā kauka e hoʻolālā i ke ʻoki kino a hoʻoholo i nā ʻano lapaʻau.

Pehea ka maʻamau o ka TAPVR?

He maʻi puʻuwai laha ʻole ʻo TAPVR. ʻOiai ma kahi ʻāina e like me ʻAmelika, hoʻokahi wale nō o nā pēpē hānau hou e pili ana i kēia maʻi. He mea laha ʻole nō hoʻi ia ma Sri Lanka.

He aha nā hōʻailona o ka TAPVR?

ʻIke pinepine ʻia nā hōʻailona o TAPVR i ka wā hānau , ʻoiai inā he ʻūlū o nā aʻa pulmonary kahi e pili ai lākou i ka puʻuwai. Eia nō naʻe, ʻaʻole paha e hōʻike kekahi mau pēpē i nā hōʻailona no kekahi mau pule.

ʻO nā hōʻailona nui kēia:

• Cyanosis: He ʻano polū a hina paha ke kala o ka ʻili, nā kui, a me nā lehelehe (i nā pēpē me ka ʻili ʻeleʻele). Hoʻokumu ʻia kēia e ka nele o ka oxygen i loko o ke koko.
• Paʻakikī omo: He paʻakikī i ka pēpē ke omo a luhi ma hope o ka inu ʻana i kahi mea liʻiliʻi.
• Luhi a me ka palaualelo: Luhi mau ka pēpē a me he mea lā palaualelo.
• ʻŌhumu puʻuwai: Ke hoʻolohe ke kauka i ka puʻuwai o ke pēpē (me ka stethoscope), lohe ʻia kahi leo ʻē.
• Pōkole o ka hanu (dyspnea): Ke hanu wikiwiki nei ka pēpē a me he mea lā ke paʻakikī nei ka hanu ʻana.
• ʻO ka ulu maikaʻi ʻole a me ka piʻi ʻana o ke kaumaha: ʻAʻole ke piʻi pono nei ke kaumaha o ka pēpē a ua palupalu.

Inā loaʻa i kāu pēpē kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​​​pono ʻoe e ʻimi koke i ke kōkua lapaʻau .

He aha ke kumu o ka TAPVR?

ʻO kaʻoiaʻiʻo, ʻaʻole maopopo i nā kauka ke kumu pololei o TAPVR. Hoʻokumu ʻia ia e kahi kīnā i ka puʻuwai a me nā kīʻaha koko i ka wā o ka ulu ʻana o ka fetal .

ʻAʻole ʻike pinepine ʻia he maʻi hoʻoilina. Eia nō naʻe, ua loaʻa nā hōʻike o nā hihia i loaʻa i nā hoahānau i loko o ka ʻohana hoʻokahi. Ua hōʻike kekahi mau noiʻi aia paha kahi pilina ma waena o nā keiki me kekahi mau maʻi a me ka ʻike ʻana i nā mea wehe pena, nā pesticides, a me ke kēpau i ka wā hāpai. Eia naʻe, ʻaʻole i hōʻoia ʻia kēia.

He aha nā pilikia e hiki mai ana o TAPVR?

Ua loaʻa i kahi pēpē me TAPVR ka hāʻule ʻana o ka puʻuwai a me ke koko kiʻekiʻe o ka pulmonary.ʻOiai ma hope o ke ʻoki ʻana, i kekahi manawa hiki ke loaʻa nā ʻano puʻuwai ʻē aʻe a i ʻole nā ​​​​​​pale ʻana i nā aʻa pulmonary. No laila he mea nui ka hoʻomau ʻana i nā nānā lapaʻau.

Pehea e ʻike ʻia ai ka TAPVR?

I kekahi manawa, hiki ke ʻike ʻia ke kūlana ma mua o ka hānau ʻana o ka pēpē, me kahi echocardiogram (kahi scan o ka puʻuwai) i hana ʻia i ka wā hāpai. Eia nō naʻe, ʻo ka hapa nui o ka manawa i hana ʻia ai ka ʻike ma hope o kekahi mau pule a i ʻole mau mahina ma hope o ka hānau ʻana o ka pēpē.

Ke hānau ʻia ke pēpē, e hana ke kauka i kahi hoʻokolokolo kino a hoʻolohe i ka puʻuwai o ke pēpē. Hiki ke hoʻohana ʻia kahi mea liʻiliʻi i kapa ʻia he pulse oximeter (he clip e pili ana a puni ka manamana wāwae nui o ke pēpē) e ana i ka pae oxygen i loko o ke koko.

Eia kekahi, hiki ke hana ʻia nā hoʻokolohua e like me kēia e nānā i ke kūlana o ka puʻuwai a me nā kīʻaha koko:

• X-ray umauma
• ʻImi CT (ʻimi CT - tomography i helu ʻia)
• MRI puʻuwai (kiʻi resonance magnetic)
• ʻEchocardiogram
• EKG (EKG a i ʻole ECG - Electrocardiogram)

Pono paha kekahi o kēia mau hoʻokolohua (e like me ka MRI) e hoʻomoe māmā ʻia ka pēpē. Akā, ʻaʻole komo kēia mau hoʻokolohua a pau. Hiki i kēia mau hoʻokolohua i nā kauka ke kiʻi i nā kiʻi maopopo o ka puʻuwai a me nā māmā o ka pēpē, e ʻike i ke kahe ʻana o ke koko, a e nānā i nā mea ʻē.

I kekahi manawa hoʻohana ʻia kahi hoʻāʻo i kapa ʻia ʻo cardiac catheterization e ʻike ai i ka maʻi. Eia nō naʻe, ʻoiai hiki ke loaʻa ka ʻike e pono ai mai nā scans i ʻōlelo ʻia ma luna, ʻaʻole i hoʻokau pinepine ʻia ka pēpē i ka hoʻāʻo invasive.

Pehea e mālama ʻia ai ʻo TAPVR?

ʻAneʻane pono nā pēpē a pau me TAPVR i ke ʻoki kino e ola ai. Hoʻōla nā kauka iā ia me ke ʻoki puʻuwai hāmama . I ka manawa pinepine, hana ʻia kēia ʻoki kino i ka hikiwawe loa ma hope o ka ʻike ʻia ʻana o ke kūlana.

ʻOiai he mea kakaikahi loa, ua ʻike ʻia kekahi poʻe me TAPVR i ko lākou wā makua me ka ʻole o ke ʻoki ʻana i ko lākou wā kamaliʻi. Eia nō naʻe, i kēlā manawa, loaʻa paha iā lākou nā kūlana e like me ke koko kiʻekiʻe pulmonary e hoʻolilo ai i ke ʻoki ʻana i mea paʻakikī loa.

Lapaʻau ma mua o ke ʻoki ʻana

ʻOiai e kali ana no ke ʻoki kino, pono paha e hāʻawi ʻia ka oxygen hou i ka pēpē a i ʻole e hoʻopili ʻia i kahi ventilator e kōkua iā ia e hanu. Hiki ke hāʻawi ʻia iā ʻoe kahi ʻano lāʻau i kapa ʻia he inotrope , kahi e kōkua ai i ka puʻuwai e kuʻi ikaika.

Pehea e hana ʻia ai ke ʻoki kino

Ma hope o ka hiamoe loa ʻana o ke pēpē ma lalo o ka anesthesia laulā , e hana ke kauka lapaʻau i kēia mau mea:

1. Hana ʻia nā ʻoki ma ka umauma a me ka puʻuwai o ka pēpē.

2. Hoʻopili hou ʻia nā aʻa pulmonary i kahi kūpono, ʻo ia hoʻi, ka atrium hema o ka puʻuwai.

3. Ua pani ʻia nā aa koko i pili pono ʻole ʻia.

4. Inā he mea e ālai ana i nā aa koko pulmonary, e wehe ʻia.

5. I ka manawa pinepine, ua pani ʻia ka lua ma waena o ka atria hema a me ka ʻākau ( Atrial Septal Defect - ASD ).

ʻOiai he hana ʻoki kino paʻakikī loa kēia, hana pono nā hui lapaʻau akamai.

Nā pilikia o ka mālama ʻana

Eia kekahi o nā pilikia e hiki mai ana ma hope o ke ʻoki kino:

• Nā ʻano ʻino o ka puʻuwai (Arrhythmias)
• Ke koko kiʻekiʻe o ka puʻuwai
• Ka pale ʻana o nā aʻa koko pulmonary
• I kekahi manawa, ma hope o ka hoʻohana ʻana i ka mīkini puʻuwai-māmā (mīkini bypass) i ka wā o ke ʻoki kino, paʻakikī i ka puʻuwai ke kuʻi iā ia iho.

Ke makaʻala mau nei nā kauka i nā mea e like me kēia.

Hiki ke pale ʻia ka TAPVR?

ʻO ka mea pōʻino, ʻaʻohe ala e pale aku ai i kēia ʻano maʻi puʻuwai hānau. Inā loaʻa i kekahi o kou ʻohana ka maʻi puʻuwai hānau, a inā he TAPVR kou, he manaʻo maikaʻi e kamaʻilio me kāu kauka ma mua o ka hoʻomaka ʻana i kahi ʻohana.

Inā loaʻa i kaʻu keiki ʻo TAPVR, he aha kaʻu e manaʻo ai?

Me ka ʻole o ke ʻoki ʻana, hiki i kekahi mau ʻano TAPVR ke make i loko o nā pule o ka hānau ʻana. Eia nō naʻe, inā ʻike mua ʻia ka maʻi a mālama ʻia ma ke ʻoki ʻana, maikaʻi loa ka nānā ʻana no nā pēpē me TAPVR. ʻO ka nui o ke ola ma hope o ke ʻoki ʻana ma kahi o 97%. He kiʻekiʻe loa kēlā o ka holomua.

I ka ulu ʻana o kāu keiki a hiki i ka wā makua, pono ʻo ia e ʻike pinepine i kahi kauka puʻuwai . Hiki i kēia mau nānā maʻamau i ke ola holoʻokoʻa ke kōkua i ka ʻike ʻana a me ka mālama ʻana i nā pilikia e like me nā ʻano ʻē o ka puʻuwai a me nā poloka o nā kīʻaha koko. I kekahi manawa, inā ulu kahi poloka, pono paha ke ʻoki hou a paʻakikī paha ke mālama. ʻO ia ke kumu he mea nui e hoʻomau i ka hahai ʻana i nā ʻōlelo a kāu kauka.

Pono paha kāu keiki e hoʻomau i ka lawe ʻana i ka lāʻau lapaʻau a i ʻole e hana i nā lāʻau lapaʻau e like me ka catheterization cardiac .

Pehea lā wau e mālama ai i kaʻu keiki?

Hiki i nā keiki me TAPVR ke loaʻa i kekahi manawa ka pilikia me nā pae hoʻomohala maʻamau, e like me ka hana ʻana o ka neʻe maikaʻi. Hiki iā ʻoe ke nānā i nā pae hoʻomohala o kāu keiki a kuhikuhi iā lākou i kahi mea hiki ke kōkua iā lākou e hoʻomohala i kēlā mau mākau inā pono.

E kamaʻilio me ke kauka o kāu keiki e pili ana i ka hiki i kāu keiki ke komo i nā haʻuki a me nā hana kino. Pono paha ʻoe e kaupalena i ka hoʻoikaika kino ikaika .

Āhea e pono ai i kaʻu keiki ke ʻike i ke kauka?

Ma hope o ke ʻoki ʻana, e ʻike ʻia kāu pēpē i kēlā me kēia 6 a 12 mau mahina.Nā nānā lapaʻau. Pono ʻoe e hoʻomau i ka ʻike mau ʻana i kāu kauka i kou ola makua. Makemake paha ke kauka o kāu keiki e hana i nā hoʻokolohua e like me ka EKG, ka hoʻāʻo koʻikoʻi hoʻoikaika kino, a i ʻole ka echocardiogram .

Āhea ʻoe e pono ai e hele i ka lumi pilikia (ETU) ?

E ʻimi koke i ke kōkua lapaʻau (e hele i ka ER) inā loaʻa i kāu pēpē kekahi o kēia mau hōʻailona:

• Inā polū/hina ka ʻili, nā kui, a i ʻole nā ​​lehelehe.
• Inā pilikia ʻoe i ka hanu ʻana a i ʻole ka ʻai ʻana/inu ʻana i ka waiū.
• Inā ʻike ʻia ʻoe he hiamoe loa a make paha.

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

Eia kekahi mau nīnau koʻikoʻi e nīnau aku ai i ke kauka o kāu keiki:

• He aha ke ʻano o ka TAPVR a kaʻu keiki?
• Manaʻo ʻoe e pono ana kaʻu keiki i kahi ʻoki lua?
• ʻEhia mau ʻoki kino āu i hana ai e hoʻoponopono iā TAPVR?

He leka koʻikoʻi e lawe ai i ka home

Ua hahai paha ʻoe i ka ʻōlelo aʻo a kāu kauka e noho olakino i ka wā hāpai. Akā i kekahi manawa, hiki i kahi pēpē hānau hou ke loaʻa i kahi maʻi puʻuwai. ʻAʻohe hewa o kekahi. Me ka ʻepekema lapaʻau holomua o kēia wā, hiki i kāu pēpē ke manaʻo i kahi hopena ʻoi aku ka maikaʻi ma mua o nā keiki o nā hanauna i hala. He kūpono loa ka lāʻau lapaʻau.

E hoʻomanaʻo, ʻoiai he maʻi koʻikoʻi ʻo TAPVR, me ka ʻike mua ʻana a me ka mālama ʻoki kūpono, loaʻa i nā keiki kahi manawa kūpono e ola ai i kahi ola maikaʻi. Ma hope o ke ʻoki ʻana, he mea nui e lawe i kāu keiki no nā nānā lapaʻau maʻamau. ʻO kēia i hiki ke nānā ʻia ke olakino o kāu keiki a hiki ke mālama koke ʻia nā pilikia e kū mai ana. ʻAʻole ʻoe hoʻokahi, aia nā kauka a me nā limahana mālama ola e kōkua iā ʻoe a me kāu pēpē.

ʻO TAPVR, maʻi puʻuwai, pēpē, ʻoki kino, nā aʻa pulmonary, cyanosis, nā maʻi hānau