He uaua te manawa o tāu tamaiti pēnei? Me kōrero tātou mō te "Double Aortic Arch"!

Kei te ahua uaua te manawa o tāu tamaiti i ētahi wā? Kei te kōkō ia i a ia e whāngai ana, e whāngai ana rānei? Kei te puta rānei he haruru rerekē i a ia e manawa ana? Ahakoa he mate noa ēnei o te tamarikitanga, tera pea he mate kino ake, engari ka taea te rongoā, kei muri i tēnei. Ko tētahi mate onge engari he mea nui kia mōhiotia ko te "Double Aortic Arch." Me kōrero taipitopito tātou mō tēnei i tēnei rā.

Ki te kī ngāwari, ko te aorta te oko toto matua e kawe ana i te toto mai i te ngākau o tō pēpi ki te toenga o tōna tinana. He oko toto kotahi, nui te nuinga. Ko te wāhanga piko o tēnei oko toto i runga ake i te ngākau e kiia ana ko te aortic arch .

Heoi, i roto i te pēpi he "Double Aortic Arch", ka hangaia tēnei "aortic arch" kia rua. Arā, ka honoa tētahi atu oko toto ki tēnei "aortic arch" matua, ka hanga he āwhata tuarua. Ka honoa ēnei āwhata e rua, ka hanga he mowhiti huri noa i te pūhā o te pēpi (trachea) me te paipa kai (esophagus) . Whakaarohia, he aha ka tupu mēnā ka āraia tēnei mowhiti? Ko te wā tēnā ka tīmata te pēpi ki te pāngia e ngā tohu kino pēnei i te uaua ki te manawa, te tangi i te wā e manawa ana, me te uaua ki te horomi kai.

Ka hangaia tēnei "āwhata aorta" i ngā wāhanga tino tīmatanga o te hapūtanga, i waenganui i ngā wiki e rua me te whitu. I tēnei wā, ka whanake te ngākau me ngā uaua toto i roto i te huarahi tino uaua. Ka hangaia ētahi wāhanga, ā, i muri i tētahi wā, kāore e hiahiatia ana, ka ngaro. Ka hangaia te "āwhata aorta takirua" nā tētahi mea pēnei. I te wiki tuarima o te hapūtanga, kāore e ngaro tētahi uaua toto i tika kia ngaro, mō tētahi take kāore i te mōhiotia, ā, ka hangaia tēnei "āwhata aorta takirua". He mate ngākau whānau tēnei, ko te tikanga ka whānau mai te pēpi me taua mate.

Ko tēnei āhuatanga, e kiia nei ko te "double aortic arch," nō roto i tētahi rōpū o ngā mate ngākau whānau e kiia nei ko ngā "vascular rings." Ka pēhia te puku o te pēpi me te esophagus e ēnei.

He aha ēnei "mowhiti toto"?

Āe, ko ngā "Mowhiti Toto", e ai ki taku kōrero i mua, he āhuatanga e whanake rerekē ai te "āwhata o te aorta", ka pēhia ai te pūhā o te pēpi (trachea), te pūhā kai (esophagus), ngā mea e rua rānei. He tino onge ēnei mate ngākau whānau . Whakaarohia, tata ki te kotahi i roto i te 100 pēpi ka whānau mai me tētahi momo mate ngākau whānau. Engari ko te "mowhiti toto" ka pā ki te kotahi i roto i te 10,000 pēpi. He tino onge.

He aha ngā momo matua o ngā "Mōwhiti Toto"?

He maha ngā momo matua o ēnei "mowhiti toto". Kia tirohia tātou he aha ēnei:

• Āwhata Aorta Takirua: I roto i tēnei, e rua ngā "āwhata aorta" (maui me matau) e karapoti ana i te trachea me te esophagus. Koinei te kaupapa matua e kōrerohia ana e tātou.
• Te āwhata aorta matau me te uaua subclavian maui rerekē me te ligamentum maui: Kotahi te āwhata aorta i konei, engari kei te taha matau o te trachea (kei te taha maui tonu). I tēnei wā, ko te uaua subclavian maui, e tuku toto ana ki ngā ringa o te pēpi, ka tīmata i te aorta ka haere i muri i te trachea me te esophagus. Ka oti tēnei mowhiti vascular mā te hono atu ki te ligamentum arteriosus maui, he toenga o te uaua toto i noho i te wā o te tamaiti, kātahi ka ngaro.
• Te āwhata aorta maui me te uaua subclavian matau rerekē: I konei, ka haere te uaua subclavian matau ki muri i te trachea me te esophagus, ka pēhi i a rāua.
• Te pēhanga o te uaua innominate: Ko te uaua innominate (brachiocephalic) he peka o te aorta. I tēnei wā, ka tīmata i te taha maui o te trachea, kātahi ka haere i mua o te trachea ki te taha matau. Ka pēhia te trachea e tēnei.
• Te whitiki uaua pūkahukahu: Ka tīmata te uaua pūkahukahu maui i te uaua pūkahukahu matau ka haere i waenganui i te trachea me te esophagus.

Ko te mea ka tupu i roto i ēnei mea katoa, ka pāngia te ara manawa, te korokoro rānei i tētahi ara.

He momo "Double Aortic Arch" kei reira?

Ae, e toru ngā momo matua o te "āwhata aorta takirua." Ka wehewehea ēnei e te āwhata nui ake, me te mea kei te taha matau, kei te taha maui rānei o te trachea taua āwhata nui ake.

• Te kōpiko matau matua me te kōpiko maui iti: Koinei te momo tino noa, e puta ana i te 80 ōrau o ngā take.
• Te kōpuku maui matua me te kōpuku matau iti: Ka kitea tēnei i roto i te 10 ōrau o ngā take.
• Ngā āwhata taurite: Ka kitea anō hoki tēnei i roto i te 10 ōrau o ngā take.

He aha te momo "mowhiti toto" e kitea nuitia ana?

I whakaaro ngā tākuta i mua ko te "rua o te aorta" te momo "mowhiti toto" tino noa. Engari kua kitea e ngā rangahau hou ko te "matau o te aorta" te mea tino noa.

Engari he mea anō tēnei. Ko ngā pēpi he "āwhata aorta matau" he nui ake te tūponotanga kia pāngia e ētahi atu mate manawa whānau i ngā pēpi he "āwhata aorta takirua". Heoi, he iti ake te tūponotanga kia kitea i te whānautanga. Nā te mea kāore pea he tohu o te "āwhata aorta matau" i ngā wāhanga tōmua. Nō reira, me pokanga pea tēnei raruraru i waenga i te 6 me te 18 marama te pakeke.

Ko te āhua e kiia nei ko te "Double Aortic Arch" te momo mowhiti toto tino noa . Nō reira, ka taea e te tākuta o tō pēpi te tātaritanga wawe i te raruraru. Ki te whakatauritea ki ngā pēpi he momo mowhiti toto kē atu, me pokanga pea ngā pēpi he "Double Aortic Arch" i muri tata mai i te whānautanga.

He aha ngā tohu o te "Double Aortic Arch"?

Ko ngā tohu o te "āwhati aorta takirua" ka puta i te nuinga o te wā ina pēhia te puku o te pēpi, te korokoro, ngā mea e rua rānei. Ka pā pea tēnei pēhitanga ki te manawa, te whāngai, te mahi rānei o te ngākau o te pēpi.

I te nuinga o te wā, ka puta mai ēnei tohu i te wā kōhungahunga (ina koa mēnā he kino rawa te mate), engari i ētahi wā ka puta mai i te toru tau te pakeke, i te taiohitanga rānei.

Tērā pea ka hē te tautuhi i ētahi tamariki he mate huango rātou nā te mea he rite ngā tohu. Ka tautuhia te mate ina kore e angitu te maimoatanga huango, ā, ka mahia he whakamātautau anō.

Ngā tohu manawa

Tata ki te 91 ōrau o ngā pēpi me ngā tamariki he "double aortic arch" ka pāngia e ēnei tohu e pā ana ki te manawa. Ko ēnei:

• He tangi rerekē i te manawa (he tangi teitei e kiia nei ko te 'stridor', he tangi wheezing rānei e kiia nei ko te 'wheezing') .
• He maremare e tangi ana me te mea he "au o te kekeno." He rite tēnei ki te maremare e puta ana i te wā e kiia nei ko te "croup".
• Ngā mate urutā auau o te ara manawa o raro .

Ngā tohu o te puku

Tata ki te 40 ōrau o ngā pēpi me ngā tamariki he "rua te aortic arch" ka pāngia e ēnei tohumate nakunaku. Kei roto i ēnei:

• Te kōroke i te wā e whāngai ana, e whāngai ana rānei.
• Te uaua ki te horomi (dysphagia) . Ka kitea pinepine tēnei i muri i te tīmatanga o te pēpi ki te kai i ngā kai totoka.
• Te uaua ki te whakanui i te taumaha .
• Te pikinga ake o te kai ki roto i te korokoro ('Regurgitation/reflux') .

Ngā tohu o te ngākau

Tata ki te 30 ōrau o ngā pēpi me ngā tamariki he "rua te aortic arch" ka pāngia e ngā tohu e pā ana ki te ngākau. Kei roto i ēnei:

• Te mamae o te uma .
• He tangi o te ngākau rerekē ('Hear murmur') .
• Kiri kikorangi (cyanosis) .

He aha te take o te "Aortic Arch Takirua"?

Kāore anō kia kitea te tino take o te "rua o te āwhata o te puku" . Ahakoa e kitea whānuitia ana tēnei āhuatanga i roto i ngā pēpi i whānau mai i te "whakatōnga in-vitro" (IVF) , kāore anō kia mōhiotia te tino take.

Ka taea hoki te hono atu ki te āwhata aorta takirua me ētahi atu mate hauora. Tata ki te 12 ōrau o ngā pēpi he āwhata aorta takirua he mate ngākau whānau anō tō rātou, pērā i te mate ventricular septal defect (VSD) me te tetralogy of Fallot . Hei tāpiri, tata ki te 20 ōrau o ngā pēpi he āwhata aorta takirua he mate ira pērā i te mukunga o te chromosome 22q11 me te trisomy 21 (Down syndrome) .

Me pēhea te mōhio ki te "Double Aortic Arch"?

Ka kitea te āwhata rua o te aorta mā te tūponotanga, mā te tīmatanga rānei o ngā tohu. I ētahi wā, ka kitea tūpono i te wā e hapu ana, i muri i te whānautanga, i ngā whakamātautau whakaahua rānei mō tētahi atu take. Mai i te tau 2004, kua piki ake te tokomaha o te hunga kua kitea tēnei mate mā roto i ngā matawai i te wā e hapu ana. He tino whai hua tēnei, nā te mea ka taea te whakarite maimoatanga i te wā tonu ka whānau te pēpi.

Ka taea rānei te tautuhi i te mate ina puta mai ngā tohu i muri i te whānautanga o te pēpi. Ka kōrero te tākuta o tō pēpi ki a koe mō ngā tohu, kātahi ka whakahaere i ētahi whakamātautau.

Ngā whakamātautau hei tautuhi i te "Double Aortic Arch"

Ko ngā whakamātautau matua e whakamahia ana hei tautuhi i tēnei mate ko:

• X-ray o te uma: Koinei te whakamātautau tuatahi e mahia ana i te nuinga o te wā .
• Matawai CT.
• Whakamātautau Echocardiogram (Echo): He mea tino āwhina tēnei hei kimi mēnā he hapa anō kei te ngākau.
• Whakamātautau MRI (MRI).

I ētahi wā, ka taea te whakamahi i te bronchoscopy hei tirotiro i te puku o te pēpi. Tērā pea ka mahia tēnei hei whakautu i ngā uauatanga manawa i mua i te tautuhi i te āwhata aorta takirua. Tērā pea ka mahia i mua i te pokanga hei āwhina i te taote kia mārama ake ki te āhua o te mea e tupu ana.

Mā te whakamātautau ira ka āwhina i te tautuhi i ngā āhuatanga ira.

Me pēhea te rongoā i te "Double Aortic Arch"?

Me mahi pokanga hei whakatika i te āhua o te "āwhata aorta takirua." Ahakoa mō ngā pēpi kāore he tohu, he pai ake te mahi pokanga i waenga i te 6 me te 9 marama te pakeke. Ko ngā pēpi e whakaatu ana i ngā tohu, inā koa he kino ngā tohu, me pokanga wawe tonu.

Whakaarohia, i kitea tuatahitia te mate "double aortic arch" i te tau 1737. I mahia te pokanga tuatahi mōna i te tau 1947. Āe, kua tae mai tātou mai i taua wā! Nā te pokanga, kua roa te ora o ngā pēpi me tēnei koha whānau.

Pokanga "Aortic Arch Takirua"

He tino haumaru te pokanga "Double Aortic Arch", ā, he tino angitu ngā hua . Kāore e hiahiatia kia honoa tō pēpi ki te "cardiopulmonary bypass" (mīhini ngākau-pūkahukahu) .

Ka mahia te pokanga mā te whakamahi i tētahi tikanga e kiia nei ko te "lateral thoracotomy." Ko te tikanga tēnei ka tapahia e te taote pokanga tētahi taha o te uma o te pēpi, i waenganui i ngā rara e rua. He maha ngā wā ka mahia tēnei ki te taha maui, nā te mea koinei te wāhi e "tāpiri" ai te nuinga o ngā pēpi i te rara iti. Mā ngā whakamātautau whakaahua i mua i te pokanga ka taea e te taote pokanga te tirotiro pai i te roto o te tinana o te pēpi, me te whakamahere i te pokanga kia rite.

Ka whakamahia e te taote pokanga te tikanga "here me te wehewehe" . Ko te tikanga o tēnei ka herea te āwhata iti hei aukati i te toto i roto. Kātahi ka tapahia, ka tuia. Ka whakahokia tēnei mahi kia wehea rawatia te āwhata, kia kore rawa hoki te pēhanga i runga i te puku me te korokoro.

Ka noho tō pēpi ki te hōhipera mō te wiki pea, kātahi ka ora ake ka hoki ki te kāinga.

Te maimoatanga o "Kommerell diverticulum" (`Kommerell diverticulum - KD`)

He onge tonu, ko ētahi pēpi he "rua te āwhata o te aorta" ka pāngia pea e tētahi mea e kiia nei ko te "Kommerell diverticulum (KD)." He mea noa ake tēnei i roto i ngā pēpi he "tika te āwhata o te aorta," engari he tino onge anō hoki me te "rua te āwhata o te aorta."

He 'aneurysm' tēnei 'KD', he oko toto e puta ana i roto i te 'aorta' heke e tata ana ki te 'aortic arch'. Ka taea e tēnei pupuhi te noho wāhi, ka pēhi i te ngongo manawa me te paipa kai o te pēpi. Kāore pea e puta he raruraru tonu. Heoi, e pai ana ki ngā taote pokanga te tango i tēnei i te wā e hangaia ana te 'double aortic arch' hei ārai i ngā raruraru ā muri ake nei.

Te maimoatanga o ētahi atu hapa o te ngākau

Mena he mate ngākau anō tō pēpi e hiahia ana kia pokaina, ka taea te rongoā i te wā kotahi me te rua o te āwhata aorta. Tērā pea ka hiahiatia he tikanga e kiia nei ko te "median sternotomy" , arā, ko te tapahi i waenganui o te uma. Ka matapakihia e te taote pokanga o tō pēpi te kōwhiringa pai rawa atu māu, i runga i ngā take e tika ana kia rongoātia.

He aha ngā raruraru ka pā mai i te pokanga?

He tino iti te mōrearea o ngā raruraru mai i te pokanga āwhata aorta takirua . He onge te wā ka uaua te pēpi ki te whāngai, ka raru pea rānei te manawa tonu. Mena he raru manawa tonu, ka ngaro i roto i te tau kotahi i muri i te pokanga.

Ka rerekē pea ngā uauatanga o ngā pokanga hei whakatika i te āwhata aorta takirua me ētahi atu hapa o te ngākau. Kōrerohia ēnei mōrearea me ngā uauatanga katoa ki te taote pokanga o tō pēpi.

Ka taea te ārai i te "Double Aortic Arch"?

Kāo, kāore e taea te ārai i tētahi āhuatanga e kīia nei ko te "double aortic arch". He mea tēnei e puta wawe ana i te wā e hapu ana, ā, kei te whānau mai anō.

He aha te heke mai mō te hunga whai "Double Aortic Arch"?

He tino kanapa te heke mai o ngā pēpi me ngā tamariki kua oti te pokanga "Double Aortic Arch". Ka taea e rātou te noho me te whai wāhi ki ngā mahi noa, me te kore he here.

I muri i te pokanga, tera pea ka mau tonu te haruru o te manawa o tō pēpi mō te tau. Nā te mea kāore anō kia tino whanake te pūaha o te pēpi nā te "rua o te aorta". Nō reira, ka roa pea te wā ka kaha ake te pūaha.

He tino onge pea te pānga o ngā tohu manawa ki ngā pēpi me ngā tamariki neke atu i te tau i muri i te pokanga.

Ko te mea nui ko te kawe i tāu pēpi ki ngā tirotiro hauora i ia wā , me te kōrero ki tō tākuta mēnā kei te pāngia koe e ētahi tohumate. He mea tino nui te tiaki ā-muri mō te wā roa mō ia tamaiti kua pokangahia te ngākau.

Āhea ahau me waea atu ki te tākuta o taku pēpi?

Ki te kite koe i ētahi tohu hou, ki te āhua kino haere rānei ngā tohu o nāianei, kōrero tonu atu ki te tākuta o tō pēpi. He nui rawa ngā panonitanga o ngā pēpi me ngā tamariki nohinohi, ā, he uaua ki te mōhio he aha te mea noa, he aha te mea rerekē. Engari ki te mea he maremare "rite ki te hiri" tō pēpi , he tangi wheezing rānei, kōrero tonu atu ki tō tākuta. He mate noa pea tēnei nō te tamarikitanga. Engari he onge, he tohu pea tēnei nō te raruraru ngākau e pā ana ki te kaha o tō pēpi ki te manawa.

Āhea koe me waea atu ki te 911 (nama āwhina)?

Ka taea e te "rua o te aorta" te pēhi i te puku o tō pēpi kia mate ai . Mēnā he kikorangi te kiri o tō pēpi (cyanosis) , mēnā rānei he uaua rawa te manawa o tō pēpi, waea atu ki te 911 tonu.

He aha ngā pātai me pātai atu e au ki te tākuta o taku pēpi?

Mena kua tāpaetia tō pēpi he "āwhata aorta takirua," he mea noa kia maha āu pātai. He mea nui kia mōhio koe ki te āhua o tō pēpi, tae atu ki te wā e hiahiatia ana te pokanga, me te mea he raruraru hauora anō tō tō pēpi. Akene he pai ki a koe te pātai i ēnei pātai:

• Āhea te wā me pokanga ai taku pēpi?
• Kei hea te pokanga e mahia ana? Ko wai kei te mahi?
• He aha ngā tohu me tūpato ahau ki te kāinga?
• He aha taku e taea ai kia māmā ake ai te noho o taku pēpi ki te kāinga?
• He mate manawa anō kei taku pēpi?
• Me whakamātauria he whakamātautau ira mō taku pēpi, mō te toenga rānei o tōku whānau?
• He aha te āhua o taku pēpi i te heke mai?

He aha te "Aortic Arch Syndrome"? He rerekē tēnei i te "Double Aortic Arch"?

Āe, kaua e whakapoauau i ngā mea e rua. Ko te "Aortic Arch Syndrome" he momo "Vasculitis" tino onge. E mōhiotia ana hoki ko te "Takayasu's arteritis" .

Ko te "Vasculitis" he mumura o ngā pakitara o ō uaua toto. Ka taea te puta i roto i ngā uaua toto katoa, tae atu ki ngā uaua toto iti rawa, arā, ngā capillaries. Ko te "Takayasu's arteritis" he mumura o ngā uaua toto rahi-waenga me ngā uaua toto nui. Ko te nuinga o te wā ka puta i roto i te "aortic arch" me ōna peka nui. Koia te take i huaina ai ko te "aortic arch syndrome".

Ka pā te mate pūkahukahu o Takayasu (e mōhiotia ana ko te "aortic arch syndrome") ki ngā taiohi me ngā taiohi . He rerekē rawa tēnei raruraru i te "double aortic arch" whānau i kōrerotia i mua ake nei, ā, he rerekē te maimoatanga e hiahiatia ana.

He wā harikoa, he wā whakanui hoki te whānautanga o tētahi pēpi. Heoi, ina kitea e koe he mate manawa tō pēpi, i te wā e hapu ana, i muri rānei i te whānautanga, ka whakakapia taua harikoa e te manukanuka me te koretake. Mena kua kitea e koe he "āwhata aorta takirua" tō pēpi, tera pea kei te whakaaro koe he aha te mahi e whai ake nei.

Kaua e manukanuka. Mā tō tīma hauora koe e āwhina i roto i ēnei mahi katoa, taahiraa i ia taahiraa. Kua oti kē i a koe te taahiraa tuatahi, arā, ko te ako atu mō te āhua o tō pēpi. Ko te rongo pai, he tino haumaru, he angitu hoki te pokanga āwhata rua o te aorta. Mena he mate ngākau kē atu tō pēpi, he mate ira rānei, ka uaua ake te maimoatanga. Heoi, me ngā whanaketanga o te tiaki pēpi hou, ka taea e koe te āwhina i tō pēpi kia whiwhi i te tiaki tika e hiahiatia ana e rātou, kia ora ai, kia harikoa ai hoki tōna tamarikitanga.

Nā, he aha ngā mea tino nui hei maumahara mā tātou i roto i ngā kōrero kua kōrerotia nei? (Karere hei kawe ki te kāinga)

Āe, me whakarāpopoto e tātou ētahi o ngā mea hei maumahara māu mai i ngā mea kua kōrerotia e tātou mō tēnei "Double Aortic Arch":

• He hapa whānau te "rua o te aorta". E rua ngā ara o te aorta o te pēpi, ā, ka pēhia te trachea me te esophagus.
• Ko ngā tohu matua ko te uaua ki te manawa, te wheezing, te uaua ki te horomi, me ngā mate manawa auau .
• Ka tātaritatia tēnei mate mā roto i ngā whakamātautau pēnei i te `CT scan`, `MRI`, `Echocardiogram` . I ētahi wā ka taea te tātaritatia i te wā e hapu ana.
• He pokanga te maimoatanga. He tino haumaru, he angitu hoki tēnei pokanga.
• Mō te pēpi i muri i te pokangaKa taea e koe te noho hauora, noa iho.
• Mena ka pā mai ngā tohu ki tō pēpi pēnei i te uaua ki te manawa, te kōkō rānei, rapua tonutia he tohutohu hauora .
• Kaua e manukanuka . Mā te whanaketanga o te rongoā hou, ka taea te rongoā angitu i tēnei mate. Mā tō rōpū hauora koe e tautoko.

` Ara Puku Takirua, Mate Manawa Whānau, Mowhiti Toto, Asphyxia Pēpi, Hauora Pēpi, Pokanga Manawa