Ua ʻike anei ʻoe i kahi maʻi laha ʻole i loko o kou puʻuwai: ʻo Ebstein's Anomaly?

He ʻōkana kupaianaha maoli ko kākou puʻuwai, ʻeā? Akā i kekahi manawa, ma muli o kekahi mau loli e kū mai ana i ka wā hānau, hiki ke loaʻa nā pilikia liʻiliʻi me ka hana o ka puʻuwai. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi puʻuwai kakaʻikahi i loaʻa ʻole i ka poʻe he nui. Ua kapa ʻia kēia ʻo Ebstein's Anomaly. ʻOiai paha he mea weliweli iki ka inoa, e hoʻomaopopo maʻalahi kākou.

No laila he aha ka Anomaly ʻo Ebstein?

I ka ʻōlelo maʻalahi, ʻo ka Anomaly ʻo Ebstein kahi maʻi puʻuwai i loaʻa i ka wā hānau . Ua kapa ʻia hoʻi ʻo "congenital," ʻo ia hoʻi ke loaʻa nei i ka wā hānau. ʻO ka "anomaly" ʻo ia hoʻi kahi mea ʻē a i ʻole i manaʻo ʻole ʻia. Ma kēia kūlana , ʻo ka valve tricuspid, kekahi o nā valve ʻehā i loko o ko kākou puʻuwai, ka mea i hoʻopilikia nui ʻia.

E noʻonoʻo e pili ana i ia mea, aia nā mea e like me nā puka ma waena o nā keʻena o ko kākou puʻuwai e kōkua ana i ke kahe ʻana o ke koko i hoʻokahi ala wale nō. ʻO ia ka mea a kākou e kapa nei he mau valves. ʻO ka valve tricuspid kekahi o lākou. Ua wehewehe mua ʻia kēia kūlana i ka makahiki 1866 e kekahi kauka Kelemania i kapa ʻia ʻo Wilhelm Ebstein. ʻO ia ke kumu i kapa ʻia ai ma hope ona.

He aha ka mea e hana ʻia ai ka valve tricuspid ma ke ʻano maʻi ʻo Ebstein?

ʻElua mau loli nui e ʻike ʻia ma ka valve tricuspid o kekahi me kēia maʻi:

1. ʻAʻole i hoʻokumu pono ʻia ka valve (deformed): ʻAʻole i hoʻohālikelike ʻia nā lau, nā ʻaoʻao e wehe a pani ana me kēlā me kēia kuʻi ʻana o ka puʻuwai, ma ke ʻano maʻamau. I kekahi manawa e pili paha lākou i ka paia o ka puʻuwai.

2. ʻOkoʻa ke kūlana o ka valve: Aia kēia valve ma kahi haʻahaʻa ma mua o ka mea maʻamau.

ʻO kēia mau pilikia ke kumu o ka pani pono ʻole ʻana o ka valve, e hoʻihoʻi ana i ke koko i loko o ka atrium ʻākau o luna. Ua kapa ʻia kēia e nā kauka he regurgitation. Ke hoʻomau nei ke kahe ʻana o ke koko i hope, e hoʻonui mālie ka atrium ʻākau. I ka hala ʻana o ka manawa, hiki i ka ʻaoʻao ʻākau o ka puʻuwai ke nāwaliwali a alakaʻi i ka hāʻule ʻana o ka puʻuwai . ʻAʻole kēia he hōʻeha puʻuwai, akā, ʻo ka hiki ʻole o ka puʻuwai ke pamu pono i ke koko.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia kūlana?

He nui ka poʻe me ka Ebstein's Anomaly i loaʻa i nā maʻi puʻuwai ʻē aʻe. Ma kahi o 94 pakeneka o ka poʻe me ka Ebstein's Anomaly he lua i loko o ka paia ma waena o nā keʻena ʻelua o luna o ka puʻuwai (atria). Ua kapa ʻia kēia he Atrial Septal Defect (ASD) a i ʻole he Patent Foramen Ovale (PFO).

Eia kekahi, hiki ke ʻike ʻia kekahi mau kīnā ma nā kiwikā ʻē aʻe o ka puʻuwai a me nā keʻena ʻaoʻao hema.

Pehea ka pinepine o ka Anomaly ʻo Ebstein?

He maʻi laha ʻole kēia. Hoʻopilikia ia i hoʻokahi i loko o kēlā me kēia ʻelua haneli tausani pēpē hānau hou. Ma waena o nā maʻi puʻuwai hānau, ʻike ʻia ma lalo o 1%.

He aha nā hōʻailona o ka anomaly ʻo Ebstein?

Hiki ke ʻokoʻa nā hōʻailona o kēia ʻano mai kēlā kanaka a kēia kanaka, mai ka ʻoluʻolu a i ka koʻikoʻi ma muli o ke kekelē o ka valve deformity.

Inā koʻikoʻi ke kūlana, ʻike pinepine ʻia nā hōʻailona i ka wā hānau a i ʻole i nā mahina mua o ke ola . I nā pēpē ʻōpio, i kekahi manawa:

• Hiki ke polū ka ʻili ma muli o ka nele o ka oxygen i loko o ke koko (cyanosis) .
• Hiki i nā hōʻailona mua o ka hāʻule ʻana o ka puʻuwai ke komo pū me:
• Ka hanu wikiwiki ʻana.
• Paʻakikī ka hanu ʻana.
• He mea maʻamau loa ka piʻi ʻana o ke kaumaha.
• Ka pehu ʻana (edema) ma nā wāwae, ka ʻōpū, a i ʻole a puni nā maka.

E noʻonoʻo, inā hoʻomau ka uliuli o kahi pēpē hānau hou a paʻakikī i ka hanu ʻana, e kānalua nā kauka i ia ʻano maʻi.

Inā he ʻoluʻolu ke ʻano, ʻaʻole paha ʻoe e loaʻa i kekahi mau hōʻailona , ​​a i ʻole e ʻike ʻia nā hōʻailona i ka wā makua. Ke ʻike ʻia nā hōʻailona i nā mākua, e ʻike paha ʻoe i nā mea e like me:

• Ka ʻili polū ʻana (ʻoi aku hoʻi nā lehelehe a me nā kui).
• Puʻuwai kūpono ʻole (arrhythmia).
• Inā loaʻa ka hāʻule ʻana o ka puʻuwai:
• ʻEha ka umauma.
• Ka poniuniu a i ʻole ka māʻule ʻana.
• Luhi mau ka manaʻo.
• Ka paʻakikī o ka hanu ʻana, ʻoiai i ka wā e hoʻoikaika kino ai.
• Ka pehu ʻana o nā wāwae a i ʻole ka ʻōpū.

He aha ke kumu o ka Anomaly ʻo Ebstein?

ʻO kaʻoiaʻiʻo, ʻaʻole hiki i nā kauka ke ʻōlelo pololei i ke kumu o kēia ʻano . Ua manaʻo ʻia ua hana ʻia e ka hui pū ʻana o nā kumu genetic (ʻo ia hoʻi, hoʻoilina) a me ke kaiapuni. Akā, ʻaʻole i hoʻoholo pono ʻia kēlā mau kumu.

Pehea e ʻike ʻia ai kēia maʻi? (Diagnosis)

Ke ʻike ʻoe i ke kauka, e nīnau lākou e pili ana i kāu mau hōʻailona a nānā i kou helu pulse a me ke koko. Hiki iā lākou ke hana i kekahi mau hoʻokolohua e ʻike ai i ka maikaʻi o ka hana ʻana o kou puʻuwai, e like me:

• Kiʻi o ka puʻuwai:
• X-ray o ka umauma: Hiki i kēia ke nānā inā ua hoʻonui ʻia ka puʻuwai.
• MRI Cardiac: Hiki i kēia ke hana i nā kiʻi maopopo a ʻoi aku ka kikoʻī o nā valves a me nā keʻena o ka puʻuwai.
• Echocardiogram: Ua like kēia me ka ultrasound o ka puʻuwai. Hiki i ke kauka ke ʻike i ke ʻano o nā valves a me nā keʻena o ka puʻuwai, a pehea lākou e hana ai. Hiki iā ia ke hōʻike maopopo i nā ʻano valve ma ka anomaly Ebstein.
• Electrocardiogram (ECG): Ke ana kēia i ka hana uila o ka puʻuwai. Hiki iā ia ke ʻike i kekahi mau puʻuwai puʻuwai kūpono ʻole (arrhythmias).
• Hoʻāʻo koʻikoʻi hoʻoikaika kino:Ma kēia, e kau ʻia ʻoe ma luna o kahi treadmill a i ʻole kahi paikikala a e lawe ʻia kahi ECG. Hana ʻia kēia e ʻike i ke ʻano o ka hana ʻana o kou puʻuwai ke kaumaha ʻoe.
• Nānā Holter: He mea liʻiliʻi kēia āu e ʻaʻahu ai no 24 a 48 mau hola. I loko o kēia manawa, hoʻopaʻa ia i kēlā me kēia puʻuwai. Hiki i kēia ke hāʻawi i kāu kauka i kahi manaʻo maikaʻi no ke ʻano o ka loli ʻana o kou puʻuwai a me kou mele i ka lā a pau.

Pehea e mālama ai i ka Anomaly ʻo Ebstein?

Ma muli o ke koʻikoʻi o kou mau hōʻailona ka lāʻau lapaʻau.

• Inā ʻaʻohe a ʻoluʻolu paha nā hōʻailona: E nānā mau kāu kauka i kou puʻuwai e ʻike inā he mau loli. ʻAʻole paha ʻoe e pono i kekahi lāʻau lapaʻau.
• Inā loaʻa iā ʻoe nā hōʻailona o ka puʻuwai puʻuwai a i ʻole ka puʻuwai puʻuwai kūpono ʻole (arrhythmia):
• Hiki ke hāʻawi ʻia nā lāʻau lapaʻau e kāohi i kēlā mau kūlana.
• Inā he puʻuwai nui kou a i ʻole he mōʻaukala o ka puʻuwai kūpono ʻole, e noi ʻia paha ʻoe e kaupalena i ka hana kino . Pono ʻoe e hahai pololei i nā ʻōlelo a kāu kauka.

• Inā koʻikoʻi nā hōʻailona: ʻO ke ʻoki ʻana i ka valve Tricuspid ke koho maikaʻi loa. Hiki i kēia ke hoʻomaikaʻi i ka hana o ka valve i ka wā lōʻihi.
• ʻO ka hoʻāʻo ʻana e hoʻoponopono iā ia me ka hoʻohana ʻana i kāu ʻiʻo valve ponoʻī ka lāʻau kūpono loa.
• Inā ʻoi aku ka nui o ka pōʻino e hiki ʻole ke hoʻoponopono, pono paha e hoʻololi ʻia ka valve . Hiki ke hana ʻia kēia me kahi valve mechanical a i ʻole kahi valve biological tissue. Eia nō naʻe, inā hoʻokomo ʻia kahi valve mechanical, pono ʻoe e lawe i ka lāʻau hoʻokahe koko no ke koena o kou ola.

I kekahi manawa, hiki ke hana ʻia nā ʻoki puʻuwai ʻē aʻe i ka manawa like me ke ʻoki ʻana o ka valve tricuspid. No ka laʻana, ke pani ʻana i ka lua ma waena o nā keʻena o luna o ka puʻuwai (hoʻoponopono ASD) a i ʻole ka mālama ʻana i ka puʻuwai puʻuwai kūpono ʻole (lāʻau arrhythmia). ʻO nā lāʻau lapaʻau no nā puʻuwai puʻuwai kūpono ʻole e komo pū me ka catheter ablation a i ʻole ke kau ʻana i kahi pacemaker.

He mea kākaʻikahi loa, pono paha kahi hoʻoili puʻuwai inā ʻaʻole holo pono nā lāʻau lapaʻau ʻē aʻe a pau.

Hiki ke pale ʻia ka Anomaly ʻo Ebstein?

No ka mea, ʻaʻole maopopo i nā kauka pehea e kū ai kēia ʻano, ʻaʻohe ala e pale aku ai i kēia manawa .

Pehea lā e holomua ai ke ola me kēia kūlana? (Outlook)

ʻOkoʻa loa ka nānā ʻana no ka poʻe me ka Anomaly ʻo Ebstein mai kēlā me kēia kanaka.ʻAʻole ola kekahi mau pēpē i kēia maʻi. Eia nō naʻe, ola kekahi i nā ola maʻamau a lōʻihi me ka ʻole o ka lāʻau lapaʻau. Ma keʻano laulā, ʻoi aku ka maikaʻi o ka nānā ʻana no ka poʻe me nā ʻano ʻoluʻolu o ke ʻano.

ʻOiai ma hope o ke ʻoki ʻana, hiki ke hoʻomau ʻia kekahi mau pilikia puʻuwai. Ua hōʻike ʻia nā haʻawina e hiki ke hoʻemi iki ʻia ka manaʻolana o ke ola ma hope o ke ʻoki ʻana. Ua hōʻike ʻia kekahi haʻawina (nāna i nānā i ka poʻe i hana ʻia ke ʻoki ma waena o 1972 a me 2006) he 20 mau makahiki ma hope o ke ʻoki ʻana, ua hoʻokomo ʻia he 65% o ka poʻe i ka haukapila no ka maʻi puʻuwai ma ka liʻiliʻi hoʻokahi, a ua ola nō 76%.

Akā he mea nui kēia: Me nā ʻano hana ʻokiʻoki holomua a me ka mālama ʻana i loaʻa i kēia lā, ʻoi aku ka maikaʻi o nā hopena no ka poʻe e hana ʻia ana i ke ʻoki ʻana i kēia lā.

Āhea wau e ʻike ai i ke kauka?

Inā loaʻa iā ʻoe ka Anomaly ʻo Ebstein, pono ʻoe e haʻi koke i kāu kauka inā e ʻoi aku ka ʻino o kāu mau hōʻailona a i ʻole inā loaʻa iā ʻoe nā hōʻailona hou . E makaʻala i kēia mau mea:

• Inā lilo ka ʻili i mea polū iki.
• Inā manaʻo ʻoe e nalowale ana ka ikaika o kou kino.
• Inā manaʻo ʻoe ua piha kou kino i ka wai (pehu).
• Inā wikiwiki ka puʻuwai.

ʻO ka leka koʻikoʻi loa e lawe i ka home

He maʻi puʻuwai hānau kakaʻikahi ʻo Ebstein's Anomaly. ʻOkoʻa loa nā hopena o kēia maʻi mai kēlā kanaka a kēia kanaka. Inā ʻike ʻia i ka wā kamaliʻi, he maʻalahi ka maʻi. ʻO ka poʻe e ola a hiki i ka wā makua, he ʻano maʻalahi ko lākou o ke ʻano. Eia nō naʻe, hiki iā lākou ke hoʻomohala i nā pilikia e like me ka puʻuwai puʻuwai kūpono ʻole (arrhythmia) a me ka hāʻule ʻana o ka puʻuwai i ka hala ʻana o ka manawa. Inā hana ʻia kēia, pono paha ke ʻoki ʻana e hoʻoponopono a hoʻololi paha i ka valve tricuspid, a me ka mālama ʻana i nā pilikia puʻuwai ʻē aʻe e pili ana.

ʻO ka mea nui loa, ʻo ia ka nānā mau ʻana i nā wahi e ʻike ai i nā loli i ka hana o kou puʻuwai a loaʻa koke ka lāʻau lapaʻau kūpono inā pono. Mai hopohopo, aia nā kauka e kōkua iā ʻoe.

ʻO ka Anomaly ʻo Ebstein, Maʻi Puʻuwai, Tricuspid Valve, Nā Kīnā Puʻuwai Hānau, Ka Holoʻole o ka Puʻuwai, ʻOki Puʻuwai, Nā Hōʻailona