Ua nāwaliwali anei ka ʻaoʻao ʻākau o kou puʻuwai? E aʻo kākou e pili ana i kēia ARVD weliweli (Arrhythmogenic Right Ventricular Dysplasia)?

ʻAuē, ke manaʻo nei ʻoe i kekahi manawa e kuʻi koke ana kou umauma, manaʻo ʻoe he pilikia kou hanu ʻana, a i ʻole manaʻo wale ʻoe e loaʻa ana iā ʻoe kahi hāʻule puʻuwai? Ua lohe paha ʻoe i kekahi manawa hiki i kahi mea pāʻani ʻōpio olakino ke loaʻa koke i ka hāʻule puʻuwai. ʻO ia ka mea a mākou e kamaʻilio ai i kēia lā, akā ʻaʻole i ʻike nui ʻia, he maʻi puʻuwai kakaʻikahi. Ua kapa ʻia kēia ʻo "Arrhythmogenic Right Ventricular Dysplasia", a i ʻole ARVD no ka pōkole.

He aha ka ARVD (Arrhythmogenic Right Ventricular Dysplasia)? E hoʻomaopopo maʻalahi kākou!

I ka ʻōlelo maʻalahi, ʻo ka ARVD kahi maʻi e hoʻopilikia ai i ka ʻiʻo puʻuwai, kahi ʻano 'cardiomyopathy'. ʻEhā keʻena o ko kākou puʻuwai. ʻO ka mea e hana ʻia ma kēia , ʻo ia ka ulu ʻana o ka ʻiʻo ma ke keʻena haʻahaʻa ma ka ʻaoʻao ʻākau o kou puʻuwai, ʻo ia ka 'ventricle ʻākau', ma kahi o ka momona a me nā ʻiʻo fibrous. E noʻonoʻo iā ia me he mea lā ua ʻino ʻia nā ʻiʻo olakino a ua pani ʻia me kahi mea e like me ka momona.

He aha ka mea e hana ʻia ke hana ʻia kēia? Ke kīkoʻo mālie nei ka ventricle ʻākau, e lahilahi, a ʻaʻole hiki ke ʻūlū pono. ʻO ia hoʻi, e nāwaliwali kona hiki ke pamu i ke koko i ka puʻuwai.

ʻO ka mea nui loa, ʻo ka ʻiʻo momona a fibrous paha i hana hou ʻia e hoʻopilikia i nā hōʻailona uila o ka puʻuwai. ʻO ia ke kumu e loaʻa ai i nā poʻe he nui me ka ARVD nā puʻuwai puʻuwai kūpono ʻole (arrhythmias). Hiki i kēia ke lilo i mea weliweli loa, no ka mea, hoʻonui ia i ka pilikia o ka hopu koke ʻana o ka puʻuwai a i ʻole ka make.

Ua kapa ʻia kēia ARVD ʻo 'Arrhythmogenic Right Ventricular Cardiomyopathy' (ARVC). I kekahi manawa hiki iā ia ke hoʻopilikia i ka ʻaoʻao hema, no laila ua kapa ʻia ʻo 'Arrhythmogenic Cardiomyopathy' (ACM).

He aha nā pae o ka ARVD?

Inā loaʻa iā ʻoe ka ARVD, hiki iā ia ke hele ma waena o nā pae he nui i ka hala ʻana o ka manawa.

1. Kahua huna: I loko o kēia kahua, ʻaʻole paha ʻoe e loaʻa i kekahi mau hōʻailona. Eia nō naʻe, hiki iā ʻoe ke ʻike i nā puʻuwai puʻuwai kūpono ʻole i ka wā e hoʻoikaika kino ai ʻoe. ʻO ka mea kupanaha, ʻo kekahi mau hoʻokolohua i hana ʻia i kēia kahua e hōʻike ana ʻaʻohe mea hewa.

2. Kahua uila: I kēia kahua, ʻoi aku ka nui o kou loaʻa ʻana o nā arrhythmias ventricular. Loaʻa iā ʻoe ka pilikia kiʻekiʻe o ka make koke ʻana o ka puʻuwai. Hiki i kahi ECG ke ʻike i kēia mau pilikia o ka puʻuwai.

3. Kahua kūkulu: I kēia kahua, ʻoi aku ka kiʻekiʻe o ka pilikia o nā ʻano puʻuwai ʻē (ventricular rhythms) a me ka make koke ʻana o ka puʻuwai. Hiki i nā hoʻokolohua kiʻi, e like me nā scan, ke hōʻike maopopo i nā loli i ke ʻano o kou puʻuwai.

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

ʻIke pinepine nā kauka i kēia maʻi i waena o ka poʻe ʻōpio.ʻO ia hoʻi, i waena o nā ʻōpio a i ʻole nā ​​​​mākua ʻōpio. Ua ʻike ʻia hoʻi ka ARVD ma ke ʻano he kumu o ka make koke ʻana o ka puʻuwai i kekahi mau mea pāʻani ʻōpio. Hōʻike kekahi mau noiʻi he ʻoi aku ka maʻamau o kēia ʻano i nā kāne .

Ma ke ʻano o kona maʻamau, he maʻi laha ʻole ka ARVD. Ua hōʻike pinepine ʻia e hoʻopilikia ia i hoʻokahi i loko o 1,000 a i ʻole hoʻokahi i loko o 5,000 poʻe. Hiki ke hana ia me ka ʻole o kekahi o ka ʻohana i loaʻa iā ia, akā pinepine ia e holo i loko o nā ʻohana.

He aha nā hōʻailona o ka ARVC? E makaʻala kākou!

ʻAʻole paha ʻoe e loaʻa i kekahi mau hōʻailona o ka ARVD i nā wā mua, akā aia kahi pilikia o ka make koke ʻana o ka puʻuwai.

ʻO nā hōʻailona nui e ʻike ʻia:

• Nā arrhythmias ventricular: Hiki i kēia ke lilo i mea weliweli loa. Ma kahi o ka hapalua o ka poʻe me kēia mau arrhythmias, hiki i ka hopena ke make a kokoke paha i ka make. ʻO ke ʻano maʻamau ka ventricular tachycardia, kahi e kū nei i ka 77% o ka poʻe me kēia maʻi.
• Nā arrhythmias supraventricular: ʻO ka mea maʻamau ka maʻi i kapa ʻia ʻo atrial fibrillation.
• Nā palpitations o ka puʻuwai: Hiki i kēia ke manaʻo e like me kekahi mea e kuʻi ana i loko o kou umauma. Hoʻokumu ʻia kēia e kahi puʻuwai puʻuwai kūpono ʻole.
• ʻO ka poniuniu, ke poʻo māmā a i ʻole ka nāwaliwali: Hoʻokumu ʻia kēia e ka puʻuwai puʻuwai kūpono ʻole.
• ʻEha ka umauma.
• Make koke ʻana o ka puʻuwai: ʻO kēia kekahi manawa ka hōʻailona mua o ka ARVD.
• Pōkole o ka hanu.
• Ka pehu ʻana o nā wāwae, nā kuʻekuʻe wāwae, nā wāwae, a i ʻole ka ʻōpū.
• Ka hāʻule ʻana o ka puʻuwai.

Hoʻomaka pinepine kēia mau hōʻailona ma waena o nā makahiki 20 a me 50. Hoʻomaopopo pinepine nā kauka i ka ARVD i ka wā ʻōpiopio (pinepine ma mua o 40 mau makahiki).

He aha nā kumu o ka ARVI?

Ma kahi o 60% o ka poʻe me ka ARVD he loli hoʻoilina ko lākou. Ua ʻike nā kānaka noiʻi ma ka liʻiliʻi he 13 mau genes e hoʻoulu ai i ka maʻi.

Hoʻopōʻino kēia mau genes ʻano ʻē i nā protein e kōkua ana i nā pūnaewele ʻiʻo puʻuwai e hoʻopili a kamaʻilio pū kekahi me kekahi. Hiki i kēia ke hoʻokaʻawale a make nā pūnaewele ʻiʻo puʻuwai ma ka ventricle ʻākau. Hiki ke maʻamau kēia i ka wā o ke kaumaha a i ʻole ka hoʻoikaika kino.

Ma ka liʻiliʻi he 30% a 50% o nā maʻi ARVD he mōʻaukala ʻohana ko lākou.No laila, he mea nui loa ka nānā ʻana o ke kauka i nā hoahānau kekelē mua a me ka lua (nā mākua, nā kaikaina, nā keiki, nā moʻopuna, nā ʻanakala, nā ʻanakē, nā moʻopuna kāne, nā kaikamahine) o ke kanaka me ka ARVD. ʻOiai inā ʻaʻohe hōʻailona, ​​​​pono nā hoahānau ʻōpio e ʻimi i ke kōkua lapaʻau.

Ua ʻike nā kānaka noiʻi i ʻelua mau ʻano hoʻoilina ma nā ARVD:

1. 'Autosomal dominant': Loaʻa i kekahi makua ka hoʻololi ʻana o ka gene. I loko o ia mau ʻohana, loaʻa i nā keiki he 50% ka manawa e hoʻoilina ai i ka maʻi. Eia nō naʻe, hiki ke ʻokoʻa nā hōʻailona a me ka makahiki o ka hoʻomaka ʻana o ka maʻi ma waena o nā lālā o ka ʻohana. ʻOi aku ka maʻamau o ka ARVD ma kekahi mau wahi honua, e like me Italia.

2. 'Autosomal recessive' (ʻaʻole maʻamau loa): Loaʻa i nā mākua ʻelua ka hoʻololi ʻana o ka gene, akā ʻaʻole lākou e hōʻike i nā hōʻailona. ʻO ka 'maʻi Naxos' kahi laʻana o kēia. Hoʻoulu ia i ka mānoanoa o ka papa waho o ka ʻili (`hyperkeratosis`) a me ka lauoho mānoanoa, "e like me ka huluhulu" (`lauoho e like me ka huluhulu`) ma nā poho lima a me nā kapuwaʻi.

Hiki ke hana ʻia ka ARVI e nā kumu ʻē aʻe:

• Nā ʻano ʻino hānau ʻole o ka ventricle ʻākau.
• ʻO ka `myocarditis` viral a mumū paha (ka mumū o ka ʻiʻo puʻuwai).
• ʻAʻole i ʻike ʻia nā kumu.

Pehea e ʻike ʻia ai ka ARVD (Arrhythmogenic Right Ventricular Dysplasia)?

Hiki i kāu kauka ke nānā i ka ARVD ma muli o kou mōʻaukala olakino, ka hoʻokolokolo kino, a me nā hoʻokolohua like ʻole.

Hiki i kāu kauka ke hoʻoholo i ka ARVD inā loaʻa iā ʻoe ka hui pū ʻana o kekahi o nā pilikia ma lalo nei:

• Ka hana maʻamau ʻole o ka ventricle ʻākau.
• ʻO ke alo o ka ʻiʻo momona a fibro-momona paha i loko o ka ʻiʻo puʻuwai (`myocardium`) o ka ventricle ʻākau.
• He hōʻike 'ECG/EKG' (Electrocardiogram) ʻano ʻē.
• Nā hewa o ka puʻuwai (arrhythmias): No ka laʻana, ka tachycardia supraventricular, ka tachycardia ventricular, a i ʻole ka fibrillation ventricular, ʻoiai i ka wā o ka hoʻoikaika kino.
• He moʻolelo ʻohana o ARVD.

Ma muli o ka nui o kēia mau mea āu i loaʻa ai, hiki i kāu kauka ke hāʻawi iā ʻoe i kahi hōʻailona o "paʻa," "palena," a i ʻole "hiki." I kekahi mau hihia, akā ʻaʻole i nā manawa a pau, hiki i kāu kauka ke kuhikuhi iā ʻoe no ka hoʻāʻo genetic .

He aha nā hoʻokolohua i hoʻohana ʻia e ʻike ai i ka ARVD?

• Electrocardiogram (ECG/EKG): He hoʻāʻo e nānā i ka hana uila o ka puʻuwai.
• ʻO ka echocardiogram transthoracic: E like me ka ultrasound o ka puʻuwai, hiki iā ia ke nānā i nā keʻena o ka puʻuwai, nā valves, a me ka hiki ke pamu.
• `Holter monitor`: Ua like kēia me kahi mīkini `ECG` liʻiliʻi āu e ʻaʻahu ai no 24 a 48 mau hola. Hoʻopaʻa ia i kou puʻuwai i ka lā a pau.
• ʻO ka angiogram ventricle ʻākau: ʻAʻole hoʻohana pinepine ʻia kēia.
• `Hoʻāʻo ʻana i ka electrophysiology`:He hoʻāʻo kūikawā e nānā i nā pilikia me ka ʻōnaehana uila o ka puʻuwai.
• ʻO ke kiʻi hoʻohālikelike magnetic cardiac (MRI): Hiki i kēia ke hana i nā kiʻi kikoʻī o ka puʻuwai. He mea nui loa kēia i ka ʻike ʻana i nā mea e like me nā waihona momona ma ka ventricle ʻākau i ka ARVD.
• ʻO ka `cardiac computed tomography (CT)`: ʻO kēia kekahi ʻano hana ʻē aʻe no ka loaʻa ʻana o nā kiʻi o ka puʻuwai.
• `Biopsy` (lawe ʻana i kahi hāpana ʻiʻo): Hana kākaʻikahi ʻia kēia. Lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka puʻuwai a nānā ʻia.

He aha nā lāʻau lapaʻau no ka ARVD?

ʻAʻohe lāʻau lapaʻau no ka ARVD i kēia manawa. Eia nō naʻe, e hoʻāʻo kāu kauka e hana i kēia mau mea:

• E kāohi i nā ʻano hewa o ka puʻuwai ('ventricular arrhythmias').
• Kāohi i nā ʻōpū koko.
• E mālama i kou hāʻule ʻana o ka puʻuwai.

ʻO nā lāʻau lapaʻau no ka ARVD:

• Nā lāʻau antiarrhythmic: Kāohi i nā puʻuwai puʻuwai kūpono ʻole no ka manawa lōʻihi a/a i ʻole ka make koke. ʻO kēia ka lāʻau lapaʻau i hoʻohana pinepine ʻia e nā kauka. Hiki ke kuhikuhi ʻia ʻoe i nā lāʻau lapaʻau e like me sotalol a i ʻole amiodarone.
• Nā lāʻau lapaʻau koko: Nā lāʻau lapaʻau e like me nā diuretics (nāna e wehe i ke kahe keu mai ke kino) a i ʻole nā ​​beta-blockers (nāna e hoʻemi i ka ukana o ka puʻuwai).
• Anticoagulant (lāʻau hoʻokahe koko): Hāʻawi ʻia nā lāʻau lapaʻau e like me warfarin e pale aku i ke kāpili ʻana o ke koko.
• ʻO ka hoʻopau ʻana o ke catheter radiofrequency: He lāʻau lapaʻau no nā ʻano pinepine ʻole o ka puʻuwai i kāohi ʻole ʻia e ka lāʻau lapaʻau. Pili kēia i ka luku ʻana i kahi wahi liʻiliʻi o ka puʻuwai e hoʻoulu ai i ka puʻuwai kūpono ʻole.
• ʻO ka Implantable cardioverter-defibrillator (ICD): He mea liʻiliʻi i hoʻokomo ʻia i loko o ka puʻuwai o ka poʻe i pilikia i ka make koke. Inā ʻike ʻia kahi ʻano puʻuwai weliweli, e ʻike ʻia a hāʻawi i kahi haʻalulu uila i ka puʻuwai e hoʻoponopono iā ia.
• Hoʻoili puʻuwai: Hana ʻia kēia ke holo pono ʻole nā ​​​​lāʻau lapaʻau ʻē aʻe a pau. He hapa liʻiliʻi wale nō o ka poʻe me kēia maʻi, ma kahi o 2% a 4%, e pono ai kahi puʻuwai hou.

E hoʻomanaʻo, pono paha ʻoe i ʻoi aku ma mua o hoʻokahi lāʻau lapaʻau no kēia maʻi i kou ola a pau.

Aia kekahi mau pilikia a i ʻole nā ​​hopena ʻaoʻao o ka lāʻau lapaʻau?

ʻAe, aia kekahi mau mea e makaʻala ai me kekahi mau lāʻau lapaʻau.

• Inā ʻoe e lawe ana i ka warfarin, pono ʻoe e hana i nā hoʻāʻo koko maʻamau . Pono kāu kauka e hōʻoia e loaʻa ana iā ʻoe ka nui kūpono. Hiki ke hoʻoponopono ʻia kāu nui ma muli o nā hopena o ka hoʻāʻo.
• ʻOiai ua holomua mua ka mālama ʻana me ka catheter ablation no nā poʻe he nui, ʻoiai e ʻoi aʻe ka maʻi i ka hala ʻana o ka manawa, ma kahi o 60% o nā kānaka e ʻike hou i nā hewa o ka puʻuwai.
• Hiki i nā uea i loko o ka hāmeʻa ICD ke neʻe a hana ʻole paha, no laila pono ʻoe e hoʻomau i ka nānā ʻana iā lākou.

Pehea wau e mālama ai iaʻu iho? He aha nā hoʻololi nohona e pono ai?

He mea nui loa e hana i kekahi mau hoʻololi i kou nohona e hōʻoluʻolu ai i kou puʻuwai.

• E kaupalena i ka inu ʻana i ka waiʻona.
• E hoʻōki loa i ka hoʻohana ʻana i nā huahana paka.
• E ʻai i ka meaʻai olakino (haʻahaʻa ka momona, nui nā huaʻai a me nā mea kanu)
• E hōʻemi i nā meaʻai a me nā mea inu i loaʻa ka caffeine (e like me ke kofe, ke kī, ke kokoleka).
• E mālama i ke kaumaha o ke kino kūpono.
• E kaupalena i ka hana kino ikaika.

ʻO ka mea nui loa: E nīnau mau i kāu kauka ma mua o kou hoʻomaka ʻana e hoʻoikaika kino. ʻAʻole ʻōlelo ʻia nā haʻuki hoʻokūkū. Hiki ke maikaʻi nā haʻuki haʻahaʻa.

E kamaʻilio mau me kāu kauka i kou wā lapaʻau. E hōʻoia lākou e loaʻa ana iā ʻoe ka nui kūpono o ka lāʻau lapaʻau. Eia kekahi, ma ka hele ʻana i nā manawa hoʻopaʻa maʻamau, hiki iā ʻoe ke ʻike i nā loli i kou kūlana i ka wā mua.

Hiki ke hoʻemi ʻia ka pilikia?

ʻAe, i kekahi ʻano. Inā loaʻa kekahi o kou ʻohana i ka ARVD, ʻo ka nānā ʻana i ka wā hikiwawe loa ke ala maikaʻi loa e hōʻemi ai i kou pilikia. Hiki i nā hoʻokolohua maʻalahi a ʻeha ʻole ke haʻi iā ʻoe inā pilikia ʻoe i kahi ʻano puʻuwai kūpono ʻole. Inā ʻoe, hiki i kāu kauka ke kōkua iā ʻoe e hoʻomohala i kahi hoʻolālā lapaʻau kūpono iā ʻoe.

He aha ke manaʻolana e ola ai ka poʻe me ka ARVD?

ʻOkoʻa ke ola o ka ARVD ma muli o ka wā i ʻike ʻia ai. ʻO ka mea maikaʻi loa e hana ai, ʻo ia ke ʻike i ka maʻi i ka wā hikiwawe loa a loaʻa ka lāʻau lapaʻau e pale aku ai i ka puʻuwai puʻuwai kūpono ʻole. E ʻoi aku ka ʻino o kēia maʻi i ka hala ʻana o ka manawa.

Inā ʻaʻole mālama ʻia ka ARVD, hiki i kou ventricle ʻākau ke hāʻule. A laila hiki i kou ventricle hema ke hāʻule pū kekahi. Hiki i kēia ke alakaʻi i nā kūlana e like me ka 'heart failure' a me ka 'atrial fibrillation'. Manaʻo nā kānaka noiʻi ʻoi aku ka ʻino o ke kūlana inā pili ka ARVD i nā ventricles ʻelua.

Akā, me ka mālama ʻana, hiki iā ʻoe ke loaʻa ke kōkua āu e pono ai e hōʻemi i ke kaumaha ma kou puʻuwai a pale i nā hōʻeha puʻuwai weliweli. Manaʻo kekahi mau noiʻi ua ʻike ʻia kekahi poʻe me ka maʻi ma hope o 65 mau makahiki. Ua manaʻo ʻia hoʻi he hoʻokahi o ka ʻelima mau kānaka me ka ARVC e hoʻomaka i nā hōʻailona ma hope o 50 mau makahiki. No laila, hiki ke ola i ke ola lōʻihi me ka ARVC.

I kēia mau lā, ʻo nā ʻenehana kiʻi holomua e like me CT a me MRI e ʻae ai i ka ʻike mua a me ka mālama ʻana, no laila maikaʻi ka nānā ʻana i ka wā lōʻihi no ka poʻe me ARVD.

Eia nō naʻe, hiki ke make koke i ka poʻe i ʻike ʻole ʻia a i ʻole ʻaʻole i loaʻa ka lāʻau lapaʻau e pono ai. Ua ʻōlelo ʻia ʻo ARVD ke kumu o 11% o nā make koke o ka puʻuwai i ka poʻe ma lalo o 35 mau makahiki a me 22% o nā make koke o ka puʻuwai i nā mea pāʻani.

Āhea wau e ʻike ai i ke kauka? Āhea wau e hele ai i ka lumi pilikia?

Inā loaʻa iā ʻoe ka ARVD, pono ʻoe e hoʻomau i nā hoʻokolohua lapaʻau i kou ola a pau.Pono kāu kauka e nānā mau e hōʻoia i ka loaʻa ʻana o ka lāʻau kūpono iā ʻoe. Inā he ICD kāu, pono e nānā mau ʻia.

He aha ka mea e hana ai i ka wā pilikia?

Inā hiolo koke kekahi a ʻaʻole pane mai i kāu mau kelepona, e kāhea koke iā 1990 (ka lawelawe ambulance ulia pōpilikia o Sri Lanka). A laila, inā hiki, e hoʻomaka i ka CPR (cardiopulmonary resuscitation), a i ʻole ma ka liʻiliʻi he CPR lima wale nō.

Inā ʻoe i ka pilikia o ka hopu koke ʻana o ka puʻuwai, he manaʻo maikaʻi ia e hoʻomaʻamaʻa ʻia ka poʻe ma ka home me ʻoe e CPR.

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

• Hiki iaʻu ke hoʻoikaika kino a i ʻole ke komo i nā haʻuki? Inā pēlā, he aha ke ʻano o nā haʻuki?
• He aha ka lāʻau lapaʻau maikaʻi loa noʻu?
• Pono anei au i kahi ICD i kēia manawa?

No ka mea he paʻakikī ke ʻike i ka ARVD ma mua o ka puka ʻana o nā hōʻailona, ​​​​he mea nui e ʻike i kou mōʻaukala ʻohana. Inā pilikia ʻoe ma muli o ka mōʻaukala ʻohana o ka maʻi, hiki i kāu kauka ke hoʻāʻo iā ʻoe.

Leka Lawe-Home

ʻAe, i kēia manawa ua ʻoi aku kou maopopo i ka mea a mākou i kamaʻilio ai i kēia lā, ʻo ia hoʻi ka ARVD. ʻOiai he mea kakaikahi ia, hiki ke lilo i maʻi puʻuwai koʻikoʻi. Hoʻokumu pinepine ʻia e nā kumu hoʻoilina . No laila, inā loaʻa i kekahi o kou ʻohana kēia maʻi, he mea nui loa ia e hoʻāʻo ʻia kekahi.

Ma ka ʻike ʻana i ka maʻi i ka wā hikiwawe loa, ka loaʻa ʻana o ka lāʻau kūpono, a me ka hana ʻana i nā hoʻololi nohona e pono ai, hiki iā ʻoe ke ola maikaʻi me kēia maʻi. E hoʻomau i ka launa pū me kāu kauka a hahai i kā lākou ʻōlelo aʻo. Eia kekahi, ʻo ka hōʻoia ʻana ua ʻike kāu poʻe aloha i ka `CPR` hiki ke kōkua i ka hoʻopakele ʻana i kou ola i kahi pilikia. Mai makaʻu, ʻo ka ʻike ka ikaika nui loa!

ʻO ka ARVD, Arrhythmogenic Right Ventricular Dysplasia, maʻi puʻuwai, hōʻeha puʻuwai, cardiomyopathy, make koke o ka puʻuwai, maʻi hoʻoilina, hōʻeha puʻuwai