ʻO Ebstein anomaly

ʻO ka anomaly Ebstein kahi kīnā o ka puʻuwai laha ʻole kahi ʻokoʻa nā ʻāpana o ke kiʻikuhi tricuspid. Hoʻokaʻawale ka pahu tricuspid i ke keʻena puʻuwai o ka naʻau ʻākau (ventricle ʻākau) mai ke keʻena puʻuwai luna ʻākau (atrium ʻākau). I ka anomaly Ebstein, ke hoʻonohonoho ʻana o ke kiwikā tricuspid a pehea e hana ai e hoʻokaʻawale i nā keʻena ʻelua he mea maʻamau ʻole.

ʻO ke ʻano he congenital, ʻo ia hoʻi aia ia ma ka wā hānau.

Hana ʻia ka pahū tricuspid i ʻekolu mau ʻāpana, i kapa ʻia he leaflets a i ʻole nā ​​pale. Wehe ʻia nā leaflets e ʻae i ke koko e neʻe mai ka atrium ʻākau (keena kiʻekiʻe) a i ka ventricle ʻākau (keena lalo) ʻoiai e hoʻomaha ka puʻuwai. Pani lākou i mea e pale ai i ke neʻe ʻana o ke koko mai ka ventricle ʻākau i ka atrium ʻākau ʻoiai e ʻūʻū ka puʻuwai.

I ka poʻe me ka anomaly Ebstein, waiho ʻia nā leaflets i lalo i ka ventricle ʻākau ma kahi o ke kūlana maʻamau. ʻOi aku ka nui o nā leaflets ma mua o ka maʻamau. ʻO ka hemahema ke kumu o ka hana ʻana o ka nohona maikaʻi ʻole, a hele hewa paha ke koko. Ma kahi o ka kahe ʻana i nā akemāmā, kahe hou ke koko i ke atrium akau. Hiki i ke kope o ke kahe o ke koko ke alakaʻi i ka hoʻonui ʻana o ka puʻuwai a me ka loaʻa ʻana o ka wai i loko o ke kino. Hiki paha i ka hoʻopili ʻana o ke kiwikā e alakaʻi i nā māmā (valve pulmonary).

I nā manawa he nui, he puka hoʻi ko ka poʻe i ka paia e hoʻokaʻawale ana i nā keʻena kiʻekiʻe ʻelua o ka puʻuwai (hemahema atrial septal) a ʻo ke kahe o ke koko ma o kēia lua e hiki ai i ke koko maikaʻi ʻole o oxygen e hele i ke kino. Hiki i kēia ke kumu o ka cyanosis, kahi polū polū i ka ʻili i hoʻokumu ʻia e ke koko maikaʻi ʻole o oxygen.

Hana ʻia ka anomaly Ebstein i ka ulu ʻana o kahi pēpē i loko o ka ʻōpū. ʻAʻole ʻike ʻia ke kumu kūpono. ʻO ka hoʻohana ʻana i kekahi mau lāʻau (e like me ka lithium a i ʻole benzodiazepines) i ka wā hāpai e hoʻokau paha i ka hana. Kākaʻikahi ke kūlana. ʻOi aku ka maʻamau i ka poʻe keʻokeʻo.

Hiki ke ʻemi a koʻikoʻi loa paha ka mea maʻamau. No laila, hiki i nā ʻōuli ke kau mai ke akahai a i ʻole koʻikoʻi loa. Hiki ke ulu koke nā ʻōuli ma hope o ka hānau ʻana, a hiki ke hoʻopili i nā lehelehe bluish-kala a me nā kui ma muli o nā pae oxygen haʻahaʻa. I nā hihia koʻikoʻi, maʻi nui ka pēpē a pilikia i ka hanu ʻana. I nā hihia maʻalahi, hiki i ka mea i luhi ʻia ke asymptomat no nā makahiki he nui, i kekahi manawa a mau loa.

Pākuʻi ʻia nā ʻōuli ma nā keiki makua:

• Cough
• Hewa ʻole e ulu
• Ka luhi
• Hanu wikiwiki
• Pokole o ka hanu
• Puʻuwai wikiwiki loa

ʻO nā keiki hānau i kū kahe nui ma o ka vaipū tricuspid e loaʻa i kahi pae haʻahaʻa o ka oxygen i loko o kā lākou koko a me ka hoʻonui nui ʻana o ka puʻuwai. Lohe paha ka mea mālama ola i nā kani o ka puʻuwai maʻamau, e like me ka namunamu, ke hoʻolohe nei i ka umauma me kahi stethoscope.

ʻO nā hoʻokolohua i hiki ke kōkua i ke kuhi ʻana i kēia ʻano me:

• Pākuʻi x-ray
• ʻImehana resonance kiʻi (MRI) o ka puʻuwai
• Ke ana o ka hana uila o ka puʻuwai (ECG)
• Ultrasound o ka puʻuwai (echocardiogram)

Pili ka mālama ʻana i ka paʻakikī o ka kīnā a me nā ʻōuli kikoʻī. Hiki i nā mālama olakino ke hoʻopili pū ʻia.

• Nā lāʻau lapaʻau e kōkua me ka holo pono ʻole o ka naʻau, e like me diuretics.
• Oxygen a me nā kākoʻo hanu ʻē aʻe.
• ʻOki kino e hoʻoponopono i ka nohona kiwikā.
• Hoʻololi o ka nohona tricuspid. Pono paha kēia i nā keiki e hoʻomau i ka ʻino a i ʻole nā ​​pilikia koʻikoʻi koʻikoʻi.

Ma ka laulaha, ulu nā ʻōuli mua, ʻoi aku ka nui o ka maʻi i ka maʻi.

ʻAʻohe o kekahi poʻe a ʻaʻohe hōʻailona a ʻaʻuli mālie paha. E hōʻeha paha nā mea ʻē aʻe i ka hala ʻana o ka manawa, hoʻomohala ʻana i ka pena polū (cyanosis), maikaʻi ʻole o ka puʻuwai, palaka puʻuwai, a i ʻole nā ​​puʻuwai puʻuwai weliweli.

Hiki i kahi leakage koʻikoʻi ke alakaʻi i ka pehu ʻana o ka puʻuwai a me ke ake, a me ke kūpikipiki ʻole o ka naʻau.

Hiki i nā pilikia ʻē aʻe ke:

• Nā puʻuwai puʻuwai maʻamau (arrhythmia), me nā ritme wikiwiki (tachyarrhythmias) a me nā mele hōʻemi maʻamau (bradyarrhythmmias a me ka puʻuwai puʻuwai)
• Holo ke koko mai ka puʻuwai i nā ʻāpana ʻē aʻe o ke kino
• Holo ka lolo

Kāhea i kāu mea hāʻawi inā ulu kāu keiki i nā ʻōuli o kēia ʻano. E kiʻi koke i ka lāʻau inā loaʻa nā pilikia hanu.

ʻAʻohe pale i ʻike ʻia, ʻē aʻe ma mua o ke kamaʻilio ʻana me kāu mea hoʻolako ma mua o ka hāpai ʻana inā e lawe ana ʻoe i nā lāʻau i manaʻo ʻia e pili ana i ka ulu ʻana o kēia maʻi. Hiki paha iā ʻoe ke pale aku i kekahi o nā pilikia o ka maʻi. ʻO kahi laʻana, ʻo ka lawe ʻana i nā lāʻau ʻalopika ma mua o ke kīpoki niho hiki ke kōkua i ke pale ʻana i ka endocarditis.

Anomaly ʻo Ebstein; Hana hewa o Ebstein; ʻIno puʻuwai Congenital - Ebstein; Naʻau kīnā hānau - Ebstein; ʻO ka maʻi puʻuwai Cyanotic - Ebstein

• ʻO anomaly ʻo Ebstein

Bhatt AB, Foster E, Kuehl K, et al. ʻO ka maʻi puʻuwai Congenital i ka makua makua: kahi ʻōlelo ʻepekema mai ka ʻAmelika ʻAmelika Hui Pū ʻIa. Kaapuni. 2015; 131 (21): 1884-1931. PMID: 25896865 pubmed.ncbi.nlm.nih.gov/25896865/.

ʻO Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Nā maʻi puʻuwai cyanotic congenital: nā leona e pili ana me ka hoʻemi ʻia o ke kahe o ka pulmonary. I: Kliegman RM, St.Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 457.

Stout KK, Daniels CJ, Aboulhosn JA, et al. ʻO 2018 AHA / ACC alakaʻi no ka mālama ʻana i nā mākua me nā maʻi puʻuwai congenital: kahi hōʻike o ka American College of Cardiology / American Heart Association Task Force on Clinical Practice Guide. Kaapuni. 2019; 139: e698-e800. PMID: 30121239 pubmed.ncbi.nlm.nih.gov/30121239/.

Webb GD, Smallhorn JF, Therrien J, Redington AN. ʻO ka maʻi puʻuwai Congenital i ke kanaka makua a me ka mea maʻi pediatric. I: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. ʻO ka maʻi puʻuwai ʻo Braunwald: He puke aʻo no ka lāʻau Cardiovascular. 11th hua. Piladelapia, PA: Elsevier; 2019: chap 75.