Ke hopohopo nei ʻoe e pili ana i ke kuʻina o kou puʻuwai? E aʻo kākou e pili ana i ka Short QT Syndrome (SQTS)!

Ke manaʻo nei ʻoe i kekahi manawa ua poniuniu a poʻo paha? A i ʻole ua loaʻa koke i kekahi āu i ʻike ai ka hāʻule puʻuwai? I kekahi manawa ʻo ke kumu o kēia mau mea he ʻano maʻi genetic laha ʻole a mākou i lohe nui ʻole ai. ʻO kekahi o ia ʻano ʻo ia ka Short QT Syndrome, a i ʻole e like me ka kapa ʻana o nā kauka, ʻo Short QT Syndrome (SQTS) . ʻOiai paha he paʻakikī iki ka inoa, e kamaʻilio maʻalahi kākou e pili ana.

He aha ka wā QT? ʻO nā hōʻailona uila o ka puʻuwai!

ʻAe, e nānā mua kākou i ke ʻano o kēia wā QT. Ua ʻike anei ʻoe e kuʻi ana ko kākou puʻuwai me ka hoʻohana ʻana i kahi ʻōnaehana o nā hōʻailona uila? Ua like ia me kahi ʻōnaehana uea i loko o kahi hale e hoʻā i nā kukui. Ma muli o kēia mau hōʻailona uila e ʻūlū ai nā keʻena o ka puʻuwai a hoʻonui i ke ʻano maʻamau, ʻo ia ka mea e pamu ai i ke koko.

Ua ʻike paha ʻoe i kekahi kauka e hana ana i kahi hoʻāʻo ECG (electrocardiogram) ke nānā lākou i kou puʻuwai. Hana lākou i kēia hoʻāʻo ma ka hoʻomoe ʻana iā ʻoe ma luna o kahi moe a hoʻopili i nā pepili liʻiliʻi ma kou umauma a me kou mau lima. ʻO ia ka mea e hoʻopaʻa ai i ka hana uila o ka puʻuwai. Ke nānā ke kauka i kēia hoʻopaʻa leo, ʻike ʻo ia i kahi ʻano nalu kikoʻī i kapa ʻia ʻo P, Q, R, S, T. Hōʻike kēlā me kēia o kēia mau leka iā ʻoe pehea e hana ai nā ʻāpana like ʻole o ka puʻuwai.

I ka ʻōlelo maʻalahi:

• Nalu P: ʻO ka hōʻailona uila e hoʻoulu ai i nā keʻena o luna o ka puʻuwai, ʻo ia hoʻi ka atria, e ʻuʻuku.
• Paʻakikī QRS: ʻO ka hōʻailona uila e hoʻoulu ai i nā keʻena haʻahaʻa o ka puʻuwai, i kapa ʻia ʻo ventricles, e ʻūlū. ʻO kēia ka mea nui e pamu i ke koko i ke kino.
• Nalu T: Ua pau ka ʻaelike ʻana o nā ventricles, hoʻomaha hou, a ke hoʻomākaukau nei no ke kuʻi aʻe. Ua like ia me ka holo ʻana a laila hoʻomaha iki.

Eia ka mea nui. ʻO ka wā QT ka manawa mai ka hoʻomaka ʻana o ka paʻakikī QRS a hiki i ka hopena o ka nalu T. Penei ke ana ʻana o nā kauka i ka lōʻihi o ka manawa e hoʻomākaukau ai nā keʻena haʻahaʻa o kou puʻuwai (nā 'ventricles') no ka hahau aʻe ma hope o hoʻokahi hahau. ʻO ka maʻamau, ʻo ka wā QT i loko o kahi puʻuwai olakino ma waena o 0.35 a me 0.45 kekona. Eia nō naʻe, i kekahi me SQTS , ʻoi aku ka liʻiliʻi o kēia manawa ma mua o 0.34 kekona. ʻO ke ʻano kēia e hoʻomaka hou ana ka puʻuwai e hahau ma mua o ka hoʻomaha ʻana.

He aha ka ʻokoʻa ma waena o ka Short QT Syndrome (SQTS) a me ka Long QT Syndrome?

He mea nui paha kēia iā ʻoe. Me SQTS, aia kekahi kūlana i kapa ʻia ʻo Long QT Syndrome (LQTS) . Ua like ia me kekahi ʻaoʻao o ke kālā, ʻeā?

• ʻO ka maʻi QT pōkole (SQTS): He pōkole loa ka wā QT (ʻoi aku ka liʻiliʻi ma mua o 0.34 kekona).
• ʻO ka Long QT Syndrome (LQTS): Hoʻolōʻihi ʻia ka manawa QT (ʻoi aku ma mua o 0.45 kekona).

Hiki i kēia mau kūlana ʻelua ke hoʻopilikia i ka puʻuwai. Eia nō naʻe, ʻokoʻa paha nā kumu a me nā lāʻau lapaʻau. I kēia lā e kamaʻilio wale mākou e pili ana i ka SQTS.

ʻO wai ka mea hiki ke hoʻomohala i nā SQTS?

He ʻano laha ʻole kēia. ʻAʻole maopopo loa i ka nui o ka poʻe ma ka honua i loaʻa iā ia, he mea laha ʻole ia. Ua ʻike kekahi mau noiʻi e hiki ke ʻike ʻia nā hōʻailona SQTS i kekahi poʻe i ka makahiki mua o ke ola. A laila, ua ʻōlelo ʻia e hiki ke ʻike hou ʻia nā hōʻailona i ka wā ʻōpio a me ka wā makua. ʻO ia hoʻi, hiki ke hoʻopilikia i nā kānaka o kēlā me kēia makahiki.

He aha nā kumu o SQTS?

He maʻi hoʻoilina ka SQTS. E like me ka hoʻoholo ʻia ʻana o ke kala o kou mau maka a me ke ʻano o ka lauoho e kou mau genes, hiki ke hana ʻia kēia maʻi e kahi mutation gene.

ʻElua ala e hiki ai kēia:

1. Hoʻoilina mai nā mākua: Inā loaʻa i kou makuahine a makuakāne paha kēia ʻano hoʻoilina, hiki iā ʻoe ke hoʻoilina.

2. Ka loli ʻana o ka gene me ke kumu ʻole: I kekahi manawa, hiki ke hana ʻia kēia loli ʻana o ka genetic i loko o kou kino me ke kumu ʻole, ʻoiai me ka ʻole o kekahi o kou ʻohana i loaʻa.

Hoʻololi kēia mau loli genetic i ka hana o nā mea i kapa ʻia ʻo nā kahawai ion i loko o nā hunaola puʻuwai. Kōkua kēia mau kahawai ion i nā hōʻailona uila o ka puʻuwai e holo pono. Ke loaʻa kahi pilikia me lākou, pōkole ka wā QT.

He aha nā hōʻailona o SQTS?

ʻAʻole like nā hōʻailona o kēlā me kēia kanaka me SQTS. ʻAʻohe paha he mau hōʻailona o kekahi poʻe. Eia nō naʻe, ʻike ka hapa nui o ka poʻe i kekahi mau hōʻailona.

ʻO ka mea nui, hiki ke like kēia mau hōʻailona me nā maʻi puʻuwai ʻē aʻe. No laila, inā loaʻa iā ʻoe kekahi mea e like me kēia, ʻoi aku ka maikaʻi e ʻike i ke kauka a nānā ʻia.

ʻO nā hōʻailona nui e ʻike ʻia:

• ʻO ka Atrial Fibrillation (AFib): ʻO kēia ka hōʻailona maʻamau. Ua hōʻike ʻia e pili ana i kahi 4 o 5 mau kānaka me SQTS. ʻO ka AFib ka wā ʻaʻole e paʻi pono nā keʻena o luna o ka puʻuwai (atria), akā "flutter." ʻO kēia ke kumu o ka paʻi ʻana o ka puʻuwai i ke ʻano ʻē.
• Nā palpitations o ka puʻuwai: Ma kahi o 1 i loko o 3 mau kānaka e ʻike paha i kēia hōʻailona. Manaʻo paha ia e like me kekahi e kuʻi ana i loko o ko lākou umauma, a i ʻole e like paha me lākou e manaʻo nei i ke kuʻi ʻana i loko o ko lākou umauma.
• ʻO ka poniuniu a me ka māʻule ʻana a i ʻole ka syncope: Hiki i kēia ke hoʻopilikia i ka 1 i loko o 4 mau kānaka. Hiki iā ia ke hana i kahi manaʻo o ka māmā a i kekahi manawa nalowale ka ʻike.

ʻO ka mea pōʻino, ʻike mua kekahi poʻe (ma kahi o 2 i loko o 5) he SQTS ko lākou ma hope o ka loaʻa ʻana o ka hopu ʻana o ka puʻuwai a i ʻole ka make koke ʻana o ka puʻuwai. Hoʻokumu ʻia kēia e kahi puʻuwai puʻuwai kūpono ʻole weliweli (arrhythmia) ma nā keʻena haʻahaʻa o ka puʻuwai, e like me ka ventricular fibrillation a i ʻole ka ventricular tachycardia, ka mea e hoʻōki koke ai ka puʻuwai i ka paʻi ʻana. He kūlana koʻikoʻi loa kēia.

E noʻonoʻo, aia kekahi kanaka ʻōpio he 25 ona makahiki i kapa ʻia ʻo Asanka. He kanaka haʻuki maikaʻi ʻo ia a olakino hoʻi. I kekahi lā, ʻoiai ʻo ia e pāʻani ana i ka cricket me kona mau hoaaloha, ua hāʻule koke ʻo ia i ka honua e paʻa ana i kona umauma. Ua holo wikiwiki ʻia ʻo ia i ka haukapila, akā ʻaʻole hiki ke hoʻopakele ʻia. Ua hōʻike ʻia ma hope mai nā hoʻokolohua he SQTS ko Asanka. He mea kaumaha ke lohe i ia mau mea, akā he mea nui e makaʻala i kēia mau mea.

Pehea e ʻike ʻia ai ka SQTS?

Inā kānalua ʻoe he SQTS kou, e nīnau mua ke kauka e pili ana i kāu mau hōʻailona a inā he maʻi puʻuwai kekahi o kou ʻohana. A laila hiki iā lākou ke hana i kēia mau hoʻokolohua:

• ECG (Electrocardiogram): ʻO kēia ka hoʻāʻo koʻikoʻi loa. Ana ia i ka wā QT. ʻO ka maʻamau, inā emi ka wā QT ma mua o 0.34 kekona a aia nā hōʻailona ʻē aʻe, kānalua ʻia ka SQTS. I kekahi manawa, ʻoiai inā ma waena o 0.34 a me 0.36 kekona ka wā QT, hiki ke noʻonoʻo ʻia ka SQTS inā aia nā hōʻailona ʻē aʻe (e.g., ka mōʻaukala ʻohana o SQTS, nā ʻano hiolo i wehewehe ʻole ʻia).
• Hoʻāʻo hoʻoilina: Hana ʻia kēia hoʻāʻo e ʻike inā he mau loli hoʻoilina e hoʻoulu ai i ka SQTS. Hiki i kēia ke kōkua i ka hōʻoia ʻana i ka maʻi a hoʻoholo inā pilikia nā lālā ʻē aʻe o ka ʻohana.

I kekahi manawa, ʻaʻohe ou hōʻailona a ʻike hewa ʻia ua pōkole kāu wā QT ke loaʻa iā ʻoe kahi ECG no kekahi kumu ʻē aʻe.

He aha nā lāʻau lapaʻau no SQTS?

ʻOiai he maʻi koʻikoʻi ka SQTS, hiki ke mālama ʻia. Hoʻōla ʻia e kahi kauka puʻuwai. Ma muli o kou kūlana, hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau aʻe:

• ʻO ka Implantable Cardioverter Defibrillator (ICD): ʻO kēia ka lāʻau lapaʻau hilinaʻi loa no nā pākeke me SQTS. He mīkini liʻiliʻi ka ICD i hoʻokomo ʻia ma lalo o ka ʻili o ka umauma a pili i ka puʻuwai. Nānā mau kēia mīkini i ke ʻano o ka puʻuwai. Inā loaʻa kahi puʻuwai puʻuwai kūpono ʻole (arrhythmia), hāʻawi koke ka ICD i kahi haʻalulu uila i ka puʻuwai e hoʻihoʻi i ke ʻano. Ua like ia me kahi kiaʻi ola ulia pōpilikia.
• Nā lāʻau lapaʻau: Aia nā lāʻau anti-arrhythmia e kāohi ana i ke ʻano o ka puʻuwai. No ka laʻana, hoʻohana pinepine ʻia ka quinidine no SQTS. Hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau e like me flecainide (Tambocor™) , ibutilide (Corvert® ), propafenone , amiodarone , sotalol , a i ʻole beta-blockers (e.g., metoprolol (Toprol XL®, Lopressor®) , carvedilol (Coreg®) ).

He mea nui: Pono e lawe ʻia kēia mau lāʻau lapaʻau inā i kuhikuhi ʻia e kāu kauka, a e like me kāna i ʻōlelo ai iā ʻoe. ʻO ka hoʻohana ʻana i nā lāʻau lapaʻau ma kāu iho hiki ke loaʻa nā hopena weliweli.

Hiki ke hoʻemi ʻia ka pilikia o SQTS?

No ka mea, he maʻi hoʻoilina ka SQTS, ʻaʻohe ala e pale aku ai i kona ulu ʻana. ʻAʻole ia he mea hiki iā kākou ke kaohi.

Eia nō naʻe, inā ua ʻike ʻia ʻoe me SQTS, he mea nui loa ia e ʻike pinepine i kahi kauka puʻuwai a hahai i kā lākou ʻōlelo aʻo. Hiki iā lākou ke nānā i ke olakino o kou puʻuwai a kōkua i ka hōʻemi ʻana i ka pilikia o nā pilikia koʻikoʻi, ʻoiai ka make koke ʻana o ka puʻuwai, hiki ke hana ma muli o SQTS.

ʻEhia ka lōʻihi e hiki ai iā ʻoe ke ola me SQTS?

He nīnau kēia a nā poʻe he nui e nīnau ai. Hiki ke ʻokoʻa ka lōʻihi o ka manawa e hiki ai iā ʻoe ke noho me SQTS mai kēlā kanaka a kēia kanaka. No kekahi poʻe, hiki i ka SQTS ke hoʻoulu i ka make koke o ka puʻuwai. Wahi a nā kuhi he 40% ka pilikia o ka make koke o ka puʻuwai o ka poʻe me SQTS i ka makahiki 40. ʻOi aku ka kiʻekiʻe o kēia pilikia i nā pēpē i piha i ka makahiki 0-1 a me nā mākua ʻōpio i piha i ka makahiki 20-40. Inā hana kēia i kahi pēpē ma waena o nā makahiki o ka hānau hou ʻana a me 1 makahiki, hiki i nā kauka ke kapa iā ia he Sudden Infant Death Syndrome (SIDS) .

Akā, mai hopohopo i kēia. ʻO ka mea nui e hoʻomanaʻo ai, ʻaʻole nā ​​​​kānaka a pau me kahi wā QT pōkole (ʻoi aku ma mua o 360 milliseconds) ma kahi ECG he SQTS, a ʻaʻole nā ​​​​kānaka a pau i ka pilikia o ka make koke ʻana o ka puʻuwai. Hiki i kekahi poʻe me SQTS ke ola i kahi ola maʻamau me ka ʻike ʻole i kekahi mau hōʻailona. No ka mea he ʻano kakaikahi ka SQTS, ke noiʻi nei nā mea noiʻi pehea e pili ai i ke ola.

Hiki i kāu kauka ke kōkua iā ʻoe e mālama i kou kūlana a hoʻemi i kou pilikia o nā pilikia ma ka hāʻawi ʻana i nā koho lapaʻau kūpono.

He aha hou kāu e makemake ai e nīnau i kāu kauka?

Inā manaʻo ʻoe he SQTS kāu, a inā ua ʻike ʻia ʻoe me ia, he mea maikaʻi e nīnau i kāu kauka i kēia mau nīnau:

• He aha nā hōʻailona o SQTS? Kūlike anei kaʻu mau hōʻailona me kēia?
• He aha nā hoʻokolohua e pono iaʻu e hana e ʻike ai i ka SQTS?
• He aha nā koho lapaʻau aʻu e loaʻa ai? ʻO wai ka mea maikaʻi loa iaʻu?
• He aha nā hoʻololi o ke ʻano o ka nohona e hiki iaʻu ke hana e mālama i koʻu puʻuwai i ke olakino? (e.g., ka pale ʻana i kekahi mau lāʻau lapaʻau, kekahi mau hana)
• He aha ka likelika e hoʻoilina ai kaʻu mau keiki i ka SQTS? Aia kekahi mau hoʻokolohua e ʻike ai?

Ma waho aʻe o kēia mau nīnau, mai makaʻu e kamaʻilio me kāu kauka e pili ana i kekahi mea āu e noʻonoʻo nei a i ʻole he kānalua paha.

Eia kahi hōʻuluʻulu manaʻo o nā mea a mākou i kūkākūkā ai (Leka Lawe-Home):

ʻAe, e hoʻomanaʻo kākou i ka mea a kākou i kamaʻilio ai:

• He maʻi laha ʻole ka Short QT Syndrome (SQTS) i ka hoʻoilina.
• ʻO ka mea e hana ʻia ma kēia, ʻo ia ka lilo ʻana o ka manawa QT, kahi manawa o ka hana uila o ka puʻuwai, i mea pōkole loa. I ka ʻōlelo maʻalahi, hoʻemi ia i ka manawa e pono ai no nā keʻena haʻahaʻa o ka puʻuwai e hoʻomākaukau no ka hahau aʻe ma hope o hoʻokahi hahau.
• Hiki i kēia ke hana i nā hōʻailona e like me ka dizziness, ka nāwaliwali, a me ka paʻa o ka umauma.
• I kekahi manawa, ʻaʻohe paha he mau hōʻailona.
• He kūlana koʻikoʻi ka SQTS e hiki ai ke alakaʻi i ka hopu koke ʻana o ka puʻuwai a me ka make.
• Eia nō naʻe, hiki ke ʻike ʻia kēia me ka ECG a me nā hoʻokolohua genetic.
• Hiki ke mālama ʻia kēia me nā polokalamu ICD a me nā lāʻau lapaʻau.
• Inā he kānalua kou e pili ana i kēia, a inā paha he ʻano maʻi puʻuwai kēia o kekahi o kou ʻohana, e ʻike pono i ke kauka no ka ʻōlelo aʻo.

E hoʻomanaʻo, he mea nui e makaʻala i kēia mau mea a e ʻimi i ke aʻo lapaʻau i ka manawa kūpono. Ke makemake nei iā ʻoe i ke olakino maikaʻi!

ʻO ka maʻi QT pōkole, SQTS, maʻi puʻuwai, ECG, wā QT, maʻi hoʻoilina, hopu koke ʻia ka puʻuwai, ICD