Loaʻa i kāu pēpē kēia ʻano pilikia puʻuwai? E aʻo kākou e pili ana i (Atrioventricular Canal Defect)!

I kekahi manawa ua ʻike paha ʻoe ua paʻakikī kāu pēpē hānau hou i ka hanu ʻana, hakakā e hānai, a i ʻole e lilo i polū. I kekahi manawa hiki i kēia ke lilo i mau hōʻailona o kahi pilikia liʻiliʻi me ka puʻuwai o kāu pēpē. ʻAe, ʻo kahi maʻi puʻuwai i loaʻa i ka wā hānau ua kapa ʻia ʻo Atrioventricular Canal Defect , a mākou e kamaʻilio nei i kēia lā.

He aha kēia (Atrioventricular Canal Defect)?

I ka ʻōlelo maʻalahi, ʻo ka Atrioventricular Canal Defect kahi kīnā puʻuwai i loaʻa i ka wā hānau. I kekahi manawa kapa ʻia e nā kauka ʻo AVSD - Atrioventricular Septal Defect . I ka pololei, he hui pū ʻana ia o kekahi mau pilikia puʻuwai. ʻO ka mea nui, aia kahi lua a i ʻole nā ​​​​lua i loko o ka paia ma waenakonu o ka puʻuwai (kapa mākou i kēia he septum) . Pili pū ia i nā valves i loko o ka puʻuwai e hana pono ʻole ana.

E noʻonoʻo e like me ka lua i loko o ka paipu wai. I loko o ka puʻuwai olakino, ʻaʻole hui pū ke koko waiwai oxygen mai ka ʻaoʻao hema o ko kākou puʻuwai me ke koko nele oxygen mai ka ʻaoʻao ʻākau. Akā ma muli o kēia lua, hui pū kēlā koko waiwai oxygen me ke koko nele oxygen. I ka hapa nui o ka manawa, hana kēia lua i ka nui o ke koko e hele i nā māmā. A laila emi ka nui o ke koko e hele ana i ke koena o ke kino. Inā hoʻomau kēia no ka manawa lōʻihi, hiki iā ia ke hele i kekahi ʻaoʻao ʻē aʻe. ʻO ia hoʻi, ʻoi aku ka nui o ke koko nele oxygen e hele i ke kino, a emi iho ka hele ʻana i nā māmā. Ke hana ʻia kēlā, emi ka pae oxygen i loko o ke kino a hiki i ka ʻili ke lilo i polū. Kāhea mākou iā ia he cyanosis .

ʻO kēia ke kumu o ka hana ʻana o ka puʻuwai i mea ikaika aʻe, hiki ke alakaʻi i nā pilikia olakino ʻē aʻe e like me ka congestive heart failure .

Inā ʻaʻole mālama pono ʻia kēia (Atrioventricular Canal Defect) , hiki iā ia ke hoʻopilikia i ke ola o ke keiki. Me ka ʻole o ke ʻoki ʻana, hiki i nā keiki me kēia maʻi ke ola no ka manawa pōkole wale nō, ma kahi o ʻelua a ʻekolu mau makahiki. Akā, mai hopohopo! ʻO ka mea maikaʻi loa, ʻo ke ʻoki ʻana e hoʻoponopono ai i kēia he holomua loa. Hiki i nā kauka ke ʻike i kēia maʻi ma mua o ka lilo ʻana i mea koʻikoʻi, ʻoiai ma mua o ka hānau ʻana o ka pēpē.

Ma ka awelika, ma nā ʻāina e like me ʻAmelika Hui Pū ʻIa, ma kahi o hoʻokahi i loko o 1,700 mau pēpē i hānau ʻia me kēia kīnā i kēlā me kēia makahiki. ʻO kēia ka helu ma waena o 3% a me 5% o nā kīnā puʻuwai hānau āpau.

Ua kapa ʻia kēia kūlana e kekahi mau inoa ʻē aʻe. Hiki i kāu kauka ke hoʻohana i kekahi o kēia mau inoa:

• ʻO ke kīnā septal atrioventricular (AVSD)
• Ka hemahema o ka uluna endocardial

Aia kekahi mau ʻano like ʻole o ke kīnā ʻana o ke kahawai atrioventricular?

ʻAe, hiki i kēia kīnā puʻuwai ke loaʻa i nā ʻano like ʻole. ʻO ia hoʻi, pili ia i nā mea e like me ka nui o ka lua i loko o ka puʻuwai a me ka helu o nā valves.

Piha (Kīnā Canal Atrioventricular)

ʻO ka mea e hana ʻia ma kēia, ʻo ia nā keʻena ʻelua o luna o ka puʻuwai (kapa mākou iā lākou he atria) a me nā keʻena ʻelua o lalo (kapa mākou iā lākou he ventricles)(i kapa ʻia) He lua nui e hana ʻia ma waena o nā keʻena ʻelua o ka puʻuwai. ʻO ia hoʻi, ua pili pū nā keʻena ʻehā o ka puʻuwai. ʻO ka maʻamau, pono e loaʻa ʻelua mau kiwikā ma waena o nā keʻena o luna a me lalo. Akā i kēia hihia, hoʻokahi wale nō kiwikā nui. ʻAʻole paha e wehe a pani pono ʻia kona ʻaoʻao .

ʻĀpana (ʻĀpana ʻĀpana Atrioventricular Canal Defect)

I kēia hihia, aia ka lua ma waena o nā keʻena ʻelua o luna o ka puʻuwai (ʻo kēia ka mea maʻamau) a i ʻole ma waena o nā keʻena ʻelua o lalo. ʻAʻole pili nā keʻena ʻehā a pau. ʻElua mau kiwikā ma waena o nā keʻena o luna a me lalo. Akā, hoʻokahi kiwikā (ʻo ka pinepine ka kiwikā mitral, aia ma waena o ke keʻena o luna (atrium hema) a me ke keʻena o lalo (ventricle hema) ma ka ʻaoʻao hema) ʻaʻole hana pono.

Ka hemahema o ke kahawai Atrioventricular Transitional

Ma kēia, aia kahi lua ma waena o nā keʻena ʻelua o luna a me kahi lua liʻiliʻi ma waena o nā keʻena ʻelua o lalo. Ua kaʻawale nā ​​​​kiwikā ʻelua ma waena o nā keʻena o luna a me lalo (nā kiwikā AV) .

ʻO ke kīnā piha o ke kahawai Atrioventricular piha ʻole

E hoʻomanaʻo i ke ʻano o kahi kīnā puʻuwai piha aia kekahi kiwikā nui? Aia kēlā kiwikā kokoke i kekahi keʻena haʻahaʻa (ventricle) ma mua o kekahi. A laila lilo ke keʻena e loaʻa ana i ke koko hou aku i ʻoi aku ka nui ma mua o kekahi. Kāhea nā kauka i kēlā keʻena liʻiliʻi he 'hypoplastic' .

He aha nā hōʻailona o kēia?

Hiki ke ʻike ʻia nā hōʻailona o kēia kīnā puʻuwai ma mua o ka hānau ʻana o kāu pēpē. E nānā e ʻike inā loaʻa i kāu pēpē kekahi o kēia mau mea:

• ʻIli polū (Cyanosis): ʻOi loa aku ma nā wahi e like me nā lehelehe, ke alelo, a me nā manamana lima.
• Paʻakikī i ka inu waiū: Manaʻo luhi a palaualelo hoʻi ma hope o ka inu ʻana i kahi waiū liʻiliʻi.
• E hou ana ma hope o ka inu ʻana i ka waiū.
• Luhi koke: Luhi ma hope o ka hana ʻana i kahi mea liʻiliʻi.
• Nā palpitations o ka puʻuwai.
• Ka paʻakikī o ka hanu ʻana (Dyspnea) a i ʻole ka hanu wikiwiki.
• Lohi ka piʻi ʻana o ke kaumaha.
• Ka pehu ʻana (edema) ma ka ʻōpū a i ʻole nā ​​wāwae.
• Ka naʻau palupalu.

Eia nō naʻe, ʻaʻole paha e hōʻike mua nā pēpē me kahi ʻano pilikia hapa a i ʻole ke ʻano hoʻololi i kēia mau hōʻailona. Hiki ke ʻike ʻia kēia mau hōʻailona ma hope o ka wā kamaliʻi, a i ʻole i ka wā ʻōpio, a i ʻole i ka wā makua.

No ke aha e hana ʻia ai kēia (Atrioventricular Canal Defect)?

ʻAʻole maopopo loa i nā kānaka noiʻi ke kumu o ka Atrioventricular Septal Defect (AVSD) . Ua manaʻo ʻia he hui pū ʻana o nā kumu genetic a me ke kaiapuni . Akā hoʻokahi mea i maopopo: aia kahi pilina ikaika ma waena o kēia maʻi puʻuwai a me Down syndrome . Ma kahi o 40% o nā pēpē me Down syndrome he AVSD .

He aha nā kumu pilikia e hoʻopilikia ai i kēia?

ʻAʻole i ʻike ʻia e nā loea nā kumu pilikia kikoʻī e hoʻokumu ai i kēia. Aia paha he mana hoʻoilina. Malia paha he gene ʻano ʻē i hoʻoili ʻia mai ka makuahine a i ʻole ka makuakāne e hoʻonui i ka manawa kūpono o ka pēpē e loaʻa ai ka maʻi puʻuwai i ka wā e noho ana i loko o ka ʻōpū.

Ma waho aʻe o kēlā, hiki i nā mea aʻe i ka wā hāpai ke hoʻonui i ka pilikia o kāu pēpē e loaʻa i kahi maʻi puʻuwai hānau:

• Kekahi mau lāʻau lapaʻau.
• Nā lāʻau lapaʻau kū ʻole i ke kānāwai (Nā lāʻau lapaʻau leʻaleʻa).
• Nā mea inu me ka waiʻona.
• Ka ʻike ʻia ʻana i nā mea ʻawahia a i ʻole nā ​​kemika o ke kaiapuni.
• Ka nele i nā meaʻai a i ʻole nā ​​​​wikamina.
• Ka momona.
• Nā huahana paka.
• ʻAʻole kaohi ʻana i nā maʻi mau e like me ka Diabetes Mellitus a i ʻole ke Hypertension.
• Nā maʻi viral e like me ka influenza (flu) a i ʻole ka maʻi palū.

He aha nā pilikia e hiki mai ana o ka Atrioventricular Canal Defect?

Inā koʻikoʻi kēia ʻano, ʻo ia hoʻi, ʻaʻole i mālama pono ʻia, hiki ke loaʻa nā pilikia e like me kēia:

• Nā ʻano helu puʻuwai maʻamau ʻole (Arrhythmia).
• Ka hāʻule ʻana o ka puʻuwai paʻa.
• Nā maʻi puʻuwai (Endocarditis).
• Ke koko kiʻekiʻe i loko o nā kīʻaha koko e hiki aku ai i nā māmā (Pulmonary hypertension).

Pehea e ʻike ʻia ai kēia maʻi?

ʻO ka nūhou maikaʻi, ʻo ka hapa nui o ka manawa, hiki i nā kauka ke ʻike i kēia (Atrioventricular Septal Defect) ma mua o ka hānau ʻana o ka pēpē me kekahi mau hoʻokolohua:

• ʻO ka ultrasound prenatal: Hiki i kēia ke ʻike i nā kiʻi o nā neʻe ʻana o ka pēpē i loko o ka ʻōpū, me ka puʻuwai. Hiki iā ia ke ʻike i kahi kīnā septal nui.
• ʻO ka echocardiogram fetal: Hōʻike kēia i nā kiʻi o ka puʻuwai me ka maopopo ma mua o ka ultrasound. Hiki i ke kauka ke loaʻa kahi manaʻo maikaʻi aʻe i ke ʻano o ka hoʻonohonoho ʻana o ka puʻuwai a pehea e pamu ai i ke koko.

Ma hope o ka hānau ʻana o ke pēpē, hiki i ke kauka ke hoʻolohe i ka puʻuwai o ka pēpē me ka stethoscope . Inā lohe ʻia kahi leo "leo nui" ʻē (kapa mākou he murmur puʻuwai ), hiki paha ke kani o ke koko e kahe ana ma ka lua.

ʻO nā hoʻokolohua ʻē aʻe i hana ʻia ma hope o ka hānau ʻana:

• Nā kiʻi X-ray o ka umauma: E nānā i ka nui a me ke ʻano o ka puʻuwai o ke pēpē.
• Electrocardiogram (EKG/ECG): E nānā i ka hana uila o ka puʻuwai o ke pēpē.
• Echocardiogram: He nānā kikoʻī loa i ke ʻano o ka puʻuwai o ke pēpē.
• Ka catheterization cardiac: E aʻo hou e pili ana i ke koʻikoʻi o ke kūlana o ke keiki.
• Kiʻi Resonance Magnetic Cardiac (Heart MRI): E ʻike i nā ʻāpana o ka puʻuwai o ke pēpē a pehea lākou e hana ai.

I kekahi manawa, inā he kīnā septal liʻiliʻi ko kahi pēpē, ʻaʻole paha e hōʻike i kekahi mau hōʻailona i ka wā mua. I kēlā hihia, he mau makahiki paha ma mua o ka ʻike ʻana o ke kauka i ke kūlana.

He aha nā lāʻau lapaʻau no kēia?

ʻO ka lāʻau lapaʻau maʻamau no ka Atrioventricular Canal Defect ʻo ia ke ʻoki puʻuwai hāmama . Ma kēia ʻoki ʻana, hoʻopili ke kauka lapaʻau i ka lua ma ka puʻuwai o ke pēpē a pani iā ​​ia. Inā pau ka hemahema, e māhele ʻia ka valve puʻuwai hoʻokahi i ʻelua, e hana ana i ʻelua mau valve kaʻawale, hoʻokahi ma ka ʻaoʻao ʻākau a hoʻokahi ma ka hema.

Pono paha i kahi pēpē me kahi kīnā AV canal kaulike ʻole e hana i kekahi mau ʻoki kino, a hiki i kahi ʻoki kino i kapa ʻia ʻo ke kaʻina hana Fontan .

ʻOi aku ka maikaʻi o ka hana ʻana i kēia ʻoki kino i ka wā hikiwawe loa, ma mua o ka hōʻeha mau ʻana o ke kūlana i ka puʻuwai. Loaʻa i ka hapa nui o nā pēpē kēia ʻoki kino i loko o nā mahina ʻeono mua o ke ola. Hiki i kekahi mau pēpē me kahi kīnā hapa akā ʻaʻohe hōʻailona ke hana i kēia ʻoki kino i loko o nā makahiki ʻekolu mua o ke ola.

I kekahi manawa, inā ʻaʻole lawa ke olakino o ke pēpē no ke ʻoki ʻana, a i ʻole ua kaumaha iki, hiki ke hāʻawi ʻia ka lāʻau lapaʻau e kāohi i nā hōʻailona a hiki i kona loaʻa ʻana o ke kaumaha a me ka ikaika.

• ʻO nā diuretics nā lāʻau lapaʻau e wehe i ka wai keu mai ke kino.
• Kōkua ʻo Digoxin i ka puʻuwai o ke pēpē i ʻoi aku ka ikaika.
• Hoʻonui nā mea hoʻopaʻa ACE i nā kīʻaha koko a hoʻomaʻamaʻa i ke kahe koko.

ʻO kekahi hopena pōkole he kaʻina hana i kapa ʻia ʻo pulmonary artery banding . Hoʻemi kēia i ka nui o ke koko e kahe ana ma o ke aʻa pulmonary i nā māmā o ka pēpē. Hiki i nā pēpē i loaʻa ka pulmonary artery banding ke hana ʻia kahi hoʻoponopono mau loa ma hope.

Hiki ke loaʻa nā pilikia ma hope o ka mālama ʻana?

ʻAe, i kekahi manawa hiki ke hana ʻia nā mea e like me kēia ma hope o ke ʻoki ʻana:

• Pale puʻuwai: ʻO ke ʻano kēia, ʻaʻole hiki pono ka hōʻailona pulse o ka puʻuwai i ka ʻaoʻao haʻahaʻa o ka puʻuwai.
• ʻO kahi kiwikā AV hema a ʻākau paha e liki ana.
• He lua ma ka paia o ke keʻena haʻahaʻa (Ventricular septal defect).
• Ka hāiki ʻana (Stenosis) o ka valve AV hema a ʻākau paha.
• ʻO kahi ālai ʻana i ke kahe koko ma lalo o ka valve aortic (subaortic stenosis).

ʻAʻole hiki ke pale ʻia kēia (Atrioventricular Canal Defect)?

ʻAʻohe ala maoli e pale aku ai i kēia (Atrioventricular Septal Defect) mai ka hana ʻana, no ka mea, pili pinepine ia i nā kumu hoʻoilina.

Eia nō naʻe, inā ʻoe e hāpai ana, hiki iā ʻoe ke hana i kēia mau mea e hōʻemi i ka pilikia o kāu pēpē e loaʻa ana i ka maʻi puʻuwai hānau:

• E pale loa i nā lāʻau lapaʻau i kū ʻole i ke kānāwai, ka waiʻona, a me nā huahana paka.
• E kiʻi i nā lāʻau pale āpau e pono ai ʻoe e pale aku iā ʻoe iho mai nā maʻi.
• E mālama i ke kaumaha olakino.
• Inā loaʻa iā ʻoe nā maʻi olakino mau loa, e mālama pono iā lākou.
• E lawe i nā huaora prenatal, ʻoiai ka waikawa folic, e like me ka ʻōlelo a kāu kauka.

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

Me ke ʻole o ke ʻoki ʻana, hiki i nā keiki me ke kīnā o ke kahawai AV ke ola no ʻelua a ʻekolu mau makahiki wale nō. Ola kekahi a hiki i ka wā ʻōpio.

Akā, ʻo ka nūhou maikaʻi, ma kahi o 90% o nā keiki i loaʻa i kēlā ʻoki hoʻoponopono e ola ana he 10 mau makahiki ma hope o kēlā lāʻau lapaʻau. ʻO ia hoʻi, ola lākou ma ka liʻiliʻi he 10 mau makahiki ma hope o ke ʻoki ʻana. Ma kahi o 65% e ola ana he 20 mau makahiki ma hope o ke ʻoki ʻana.

Akā e hoʻomanaʻo i kēia: ʻOiai ma hope o ke ʻoki ʻana, ʻaʻole e loaʻa i kahi kanaka me kahi Atrioventricular Canal Defect (AVCD) kahi puʻuwai maʻamau. Pono lākou e hoʻomau i ka loaʻa ʻana o nā echocardiograms i nā manawa maʻamau e nānā i ka hana o ko lākou puʻuwai. E kōkua kēia i ka hopu ʻana i nā pilikia i ka wā mua.

ʻO ka ʻāpana i kau ʻia ma luna o ka lua e mau loa ia no ke ola holoʻokoʻa. Akā i ka hala ʻana o ka manawa, hiki i kekahi o nā kiwikā puʻuwai i hoʻoponopono ʻia ke hoʻomaka e liki. Inā hana ʻia kēlā, ma waena o 10% a me 20% o ka poʻe e pono ai ke ʻoki lua .

ʻAʻole pono ka hapa nui o ka poʻe i ka lāʻau lapaʻau a i ʻole ke ʻoki kino hou aʻe ma hope o ke ʻoki kino. Eia nō naʻe, hiki ke loaʻa nā arrhythmias cardiac ma hope. Inā hana ʻia kēia, hiki i ke kauka ke ʻōlelo i nā kaʻina hana minimally invasive e like me ka ablation .

Pehea lā wau e mālama ai i kaʻu keiki?

Pono paha ʻoe e kaupalena i ka hana kino o kāu keiki . E nīnau i ke kauka puʻuwai pediatric o kāu keiki - he kauka loea i nā maʻi puʻuwai i nā keiki - e pili ana i kēia.

Hiki ke aʻo ʻia nā keiki me kahi kīnā septal atrioventricular (AVSD) e lawe i nā antibiotics ma mua o ke kipa ʻana i ke kauka niho. Hiki i kēia ke kōkua i ka pale ʻana i kahi ʻano maʻi puʻuwai i kapa ʻia ʻo endocarditis . E kamaʻilio pū me ke kauka cardiologist o kāu keiki e pili ana i kēia.

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

Pono ʻoe e lawe i kāu keiki i kā lākou kauka puʻuwai pediatric i nā manawa maʻamau. Hiki iā lākou ke nānā i ka puʻuwai o kāu keiki no kekahi mau pilikia. Pono paha kekahi mau keiki me nā kīnā o ke kahawai AV i ka mālama hou ʻana ma hope o ke ʻoki ʻana. Hiki ke liki ka ʻāpana, a i ʻole e liki a haiki paha kahi valve i hoʻoponopono ʻia.

Hiki i ke keiki me kahi kīnā AV canal ke loaʻa i nā maʻi neurological a i ʻole nā ​​​​maʻi ulu . Pono paha lākou i ke kōkua me kēlā.

Ke nui aʻe kāu keiki, pono lākou e neʻe i kahi loea puʻuwai hānau makua a hele i nā nānā kino ma ka liʻiliʻi i hoʻokahi manawa i ka makahiki.

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

Hiki iā ʻoe ke nīnau i ke kauka o kāu keiki e like me kēia:

• He aha ke ʻano o ke kīnā o ke kahawai AV i loaʻa i kaʻu keiki?
• He aha nā hōʻailona e hōʻike ana e ʻoi aku ka ʻino o ke kūlana?
• Āhea ʻo ia e pono ai ke ʻoki kino?
• E pono anei kaʻu keiki i ka lāʻau lapaʻau ma hope o ke ʻoki ʻana?
• Pono anei au e kaupalena i nā hana a kaʻu keiki?

He mea maʻamau ka loaʻa ʻana o nā ʻano manaʻo like ʻole ke lohe ʻoe ua loaʻa i kāu pēpē hānau hou kahi maʻi puʻuwai e like me ka Atrioventricular Canal Defect . Akā, aia nā kauka o kāu pēpē ma laila e kōkua iā ʻoe, a makemake lākou e hana i ka mea maikaʻi loa no kāu pēpē. E ʻike pono i ka mea kikoʻī i ke kūlana o kāu pēpē. Inā he mea āu i maopopo ʻole ai, e nīnau i kāu kauka e pili ana. ʻO kēia ka manawa e kākoʻo ai i ke olakino o kāu pēpē.

No laila, he aha nā mea e pono ai kākou e hoʻomanaʻo mai kēia mau mea a pau?

• ʻO ka Atrioventricular Canal Defect kahi maʻi puʻuwai i loaʻa i ka wā hānau. ʻO ke kumu nui o ia mea he lua i loko o ka puʻuwai a me kahi pilikia me nā valves.
• He mea nui loa ka ʻike mua ʻana a me ke ʻoki ʻana i ka manawa kūpono . He holomua nui kēia mau ʻoki ʻana.
• ʻOi aku ka nui o ka loaʻa ʻana o kēia maʻi i nā pēpē me Down syndrome .
• Pono ka nānā ʻana o ke kauka no ka manawa lōʻihi ma hope o ke ʻoki ʻana. Pono ʻoe e launa pū me kahi kauka puʻuwai no ke koena o kou ola.
• Inā ʻoe e hāpai ana, hiki i ka hahai ʻana i kahi nohona olakino a me ka hahai ʻana i nā ʻōlelo aʻoaʻo lapaʻau ke kōkua i ka hōʻemi ʻana i ka pilikia o kāu pēpē i ka maʻi puʻuwai hānau.
• Mai makaʻu, aia nā kauka ma laila e kōkua iā ʻoe a me kāu pēpē. E nīnau i nā nīnau, e ʻike.

ʻO ka maʻi puʻuwai, nā kīnā puʻuwai hānau, ka puʻuwai o ka pēpē, ka lua i loko o ka puʻuwai, Atrioventricular Canal Defect, AVSD, maʻi puʻuwai pediatric