He tino okana whakamiharo tō tātou ngākau, kāore? Engari i ētahi wā, nā ētahi huringa e puta mai ana i te whānautanga, ka puta pea he raruraru iti ki te mahi a te ngākau. I tēnei rā ka kōrero tātou mō tētahi mate ngākau onge kāore i te maha ngā tāngata e pāngia ana. Ko te ingoa o tēnei ko te Ebstein's Anomaly. Ahakoa he ahua whakamataku te ingoa, me mārama noa iho.
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Nā, he aha te Anomaly a Ebstein?
He whakarāpopototanga noa iho, ko te Ebstein's Anomaly he mate ngākau e kitea ana i te whānautanga . E kiia ana hoki ko te "congenital," ko tōna tikanga kei te whānautanga. Ko te tikanga o te "anomaly" he mea rerekē, he mea ohorere rānei. I roto i tēnei āhuatanga , ko te takirere tricuspid, tētahi o ngā takirere e whā o te ngākau, te mea nui ka pāngia.
Whakaarohia, he mea pērā i ngā tatau i waenganui i ngā rūma o tō tātou ngākau e āwhina ana i te rere o te toto ki te taha kotahi anake. Koinei te mea e kīia nei e tātou he takirere. Ko te takirere tricuspid tētahi o aua mea. I whakaahuahia tuatahitia tēnei āhuatanga i te tau 1866 e te tākuta Tiamana ko Wilhelm Ebstein tōna ingoa. Koia te take i tapaina ai ki tōna ingoa.
He aha te mea ka tupu ki te takirere tricuspid i roto i te mate Ebstein anomaly?
E rua ngā huringa matua ka kitea i roto i te takirere tricuspid o te tangata whai mate pēnei:
1. Kāore te takirere i te hanga tika (kua rerekē te āhua): Kāore ngā rau, arā, ngā papa e tuwhera ana, e kati ana i ia pao o te ngākau, i te hanga ōrite. I ētahi wā ka piri pea ki te pakitara o te ngākau.
2. He rerekē te tūnga o te takirere: Kei raro iho tēnei takirere i tōna tūnga tūturu.
Nā ēnei raruraru ka kore e kati tika te takirere, ka turuturu te toto ki te atrium matau o runga. Ka kiia tēnei e ngā tākuta he regurgitation. I te rere tonu o te toto ki muri, ka nui haere te atrium matau. I roto i te wā, ka ngoikore te taha matau o te ngākau, ā, ka arahi ki te ngoikore o te ngākau . Ehara tēnei i te mate ngākau, engari ko te korenga o te ngākau ki te pupuhi tika i te toto.
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He maha ngā tāngata whai Ebstein's Anomaly he mate manawa anō ō rātou. Tata ki te 94 ōrau o ngā tāngata whai Ebstein's Anomaly he kōhao kei te pakitara i waenganui i ngā rūma e rua o runga o te manawa (atria). Ka kiia tēnei he Atrial Septal Defect (ASD) he Patent Foramen Ovale rānei (PFO).
Ka kitea anō hoki ētahi hapa i roto i ngā takirere kē atu o te ngākau me ngā rūma o te taha maui.
He pēhea te noa o te mate urutā a Ebstein?
He tino onge tēnei mate . Tata ki te kotahi i roto i te rua rau mano ngā pēpi hou ka pāngia. I roto i ngā mate manawa whānau, ka kitea i raro i te 1%.
He aha ngā tohu o te Anomaly a Ebstein?
Ka rerekē ngā tohu o tēnei āhua i ia tangata, mai i te ngawari ki te kino, i runga i te nui o te hē o te takirere.
Mena he kino te mate, ka puta ngā tohu i te whānautanga, i ngā marama tuatahi rānei o te oranga . I ētahi wā, i roto i ngā pēpi nohinohi:
- Tērā pea ka kikorangi te kiri nā te kore o te hāora i roto i te toto (cyanosis) .
- Ko ngā tohu wawe o te ngoikore o te ngakau ka uru atu ki:
- Te manawa tere.
- Te uaua ki te manawa.
- He mea tino noa te pikinga o te taumaha.
- Te pupuhi (edema) i ngā waewae, i te puku, i te taha rānei o ngā kanohi.
Whakaarohia, ki te mea ka kikorangi tonu te tae o tētahi pēpi whānau hou, ā, ka uaua ki te manawa, ka whakapae ngā tākuta mō tēnei āhua.
Mena he ngawari te āhua, kāore pea he tohu e pā mai ana ki a koe, ka puta rānei ngā tohu i te pakeketanga. Ina puta ngā tohu i roto i ngā pakeke, ka kite pea koe i ngā mea pēnei i:
- Te tae kikorangi o te kiri (ina koa ko ngā ngutu me ngā maikuku).
- Te pāorooro o te ngakau (arrhythmia).
- Mena ka puta te ngoikore o te ngakau:
- Mamae o te uma.
- Pōuriuri, ngoikore rānei.
- Te ngenge tonu.
- Te uaua ki te manawa, ina koa i te wā e whakakorikori ana.
- Te pupuhi o ngā waewae, o te puku rānei.
He aha te take o te Anomaly a Ebstein?
Ki te pono, kāore anō kia taea e ngā tākuta te kī tika he aha i puta ai tēnei mate . E whakaponohia ana nā te huinga o ngā āhuatanga ira (arā, te tuku iho) me te taiao i puta ai. Engari kāore anō kia tino whakatauhia he aha aua āhuatanga.
Me pēhea te tautuhi i tēnei mate? (Tautuhinga)
Ina kite koe i tētahi tākuta, ka pātai atu rātou mō ō tohumate, ka tirohia hoki tō tere o te manawa me tō toto toto. Tērā pea ka whakahaerehia ētahi whakamātautau hei tirotiro i te pai o te mahi a tō ngākau, pēnei i:
- Whakaahua o te ngākau:
- X-ray o te uma: Mā tēnei ka taea te tirotiro mēnā kua rahi te ngākau.
- MRI o te ngākau: Ka taea e tēnei te whakaputa i ngā whakaahua mārama ake, taipitopito ake hoki o ngā takirere me ngā rūma o te ngākau.
- Echocardiogram: He rite tēnei ki te ultrasound o te ngākau. Mā tēnei ka taea e te tākuta te kite i te āhua o ngā takirere me ngā rūma o te ngākau, me te āhua o tā rātou mahi. Ka taea e ia te whakaatu mārama i ngā hapa takirere i roto i te Ebstein anomaly.
- He whakamātautau hiko-karu (ECG): Mā tēnei ka ine i te ngohe hiko o te ngākau. Ka taea te kite i ngā pāorooro o te ngakau (arrhythmias).
- Whakamātautau ahotea whakakori tinana:I tēnei, ka whakanohoia koe ki runga i te mīhini oma, ki runga rānei i te pahikara, ā, ka tangohia he ECG. Ka mahia tēnei hei tirotiro i te mahi a tō ngākau ina ahotea koe.
- Aroturuki Holter: He taputapu iti tēnei ka mau koe mō ngā haora 24 ki te 48. I tēnei wā, ka hopukina e ia ngā pao o te ngakau. Mā tēnei ka taea e tō tākuta te mōhio ki te panoni o te tere o tō ngakau me te auau o te manawa puta noa i te rā.
Me pēhea te rongoā i te Anomaly a Ebstein?
Ko te maimoatanga e whakawhirinaki ana ki te kaha o ō tohumate.
- Mena he ngawari, he ngaro rānei ngā tohu: Ka tirotirohia tō ngākau e tō tākuta i ia wā, i ia wā, kia kite ai mēnā he huringa kei te puta. Kāore pea koe e hiahia ki tētahi maimoatanga.
- Mena kei a koe ngā tohu o te ngoikore o te ngākau, te koretake rānei o te ngakau (arrhythmia):
- Ka taea te hoatu rongoā hei whakahaere i aua āhuatanga.
- Mena he nui tō ngākau, he hītori rānei o te pā o te ngakau kāore i te tika, ka tonoa pea koe kia whakawhāiti i te korikori tinana . Me whai tonu koe i ngā tohutohu a tō tākuta.
- Mena he kino rawa ngā tohu: Ko te pokanga mō te takirere tricuspid te kōwhiringa pai rawa atu. Ka taea e tēnei te whakapai ake i te mahi a te takirere mō te wā roa.
- Ko te ngana ki te whakatika mā te whakamahi i tō ake kiko takirere te maimoatanga tika rawa atu.
- Mena he kino rawa te pakaru, me whakakapi pea te takirere . Ka taea tēnei mā te takirere ā-ringa, mā te takirere kiko koiora rānei. Heoi, ki te whakauruhia he takirere ā-ringa, me tango e koe he rongoā whakangawari toto mō te toenga o tō oranga.
I ētahi wā, ka taea te mahi i ētahi atu pokanga ngākau i te wā kotahi me te pokanga takirere tricuspid. Hei tauira, te kati i te kōhao i waenganui i ngā rūma o runga o te ngākau (te whakatikatika ASD) te rongoā rānei i te ngakau pāhekeheke (te rongoā arrhythmia). Ko ngā maimoatanga mō te ngakau pāhekeheke ko te tango i te catheter, te whakauru rānei i te pacemaker.
He tino onge pea te hiahia ki te whakawhitinga ngākau mēnā kāore e angitu ngā maimoatanga katoa.
Ka taea te ārai i te Anomaly a Ebstein?
Nā te mea kāore ngā tākuta i te tino mōhio me pēhea te puta mai o tēnei mate, kāore he huarahi hei ārai i tēnei wā .
Me pēhea te haere whakamua o te ao me tēnei āhuatanga? (Tirohanga)
He rerekē rawa te āhua o te tangata whai mate urutā a Ebstein i ia tangata, i ia tangata.Kāore ētahi pēpi e ora i tēnei mate. Heoi, ko ētahi ka roa te ora, ka ora noa, kāore he maimoatanga. I te nuinga o te wā, he pai ake te āhua mō te hunga he ngawari ake te āhua o te mate.
Ahakoa i muri i te pokanga, tera pea ka mau tonu ētahi raruraru o te ngākau. Kua whakaatuhia e ngā rangahau ka paku whakaitihia te roa o te oranga i muri i te pokanga. I whakaatuhia e tētahi rangahau (i tirohia ai te hunga i pokangahia i waenga i te tau 1972 me te 2006) i roto i te 20 tau i muri i te pokanga, 65% o te hunga i whakaurua ki te hōhipera mō te mate ngākau i te iti rawa kotahi, ā, 76% kei te ora tonu.
Engari he mea nui tēnei: Nā ngā tikanga pokanga matatau me te tiaki whai muri i ēnei rā, tera pea ka pai ake ngā hua mō te hunga e pokangahia ana i ēnei rā.
Āhea ahau me toro atu ki te tākuta?
Mena kei a koe te Anomaly a Ebstein, me kōrero tonu atu koe ki tō tākuta mena ka kino haere ō tohu, ka puta rānei he tohu hou . Kia tūpato ki ēnei mea:
- Mena ka piki haere te kikorangi o te kiri.
- Ki te mea kei te ngoikore haere tō tinana.
- Ki te mea kei te pupuhi tō tinana (kei te kī tonu i te wai).
- Mena he tere te pao o te ngakau.
Ko te karere tino nui hei kawe ki te kāinga
He mate ngakau onge, whānau mai te Ebstein's Anomaly. He rerekē ngā pānga o tēnei mate i ia tangata, i ia tangata. Ki te kitea i te wā pēpi, he ngawari noa iho te mate. Ko te hunga e ora ana ki te pakeketanga he maha ngā wā ka ngawari ake te ahua o te mate. Heoi, ka taea hoki e rātou te whakawhanake i ngā raruraru pēnei i te ngakau pāhekeheke (arrhythmia) me te ngoikore o te ngakau i roto i te wā. Ki te tupu tēnei, tera pea me pokanga hei whakatika, hei whakakapi rānei i te takirere tricuspid, me te rongoā i ētahi atu raruraru ngakau e pā ana.
Ko te mea nui ko te tirotiro i tō ngākau i ia wā, i ia wā, kia kitea ai ngā huringa o te mahi a tō ngākau, ā, kia whiwhi wawe tonu i te maimoatanga whai hua mēnā e tika ana. Kaua e manukanuka, kei reira ngā tākuta hei āwhina i a koe.
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