ʻO nā mea āpau āu e ʻike ai e pili ana i ka pulmonary arterial hypertension

Anter

• Nā hōʻailona o ka hanu kiʻekiʻe
• Nā kumu o ka piʻi kiʻekiʻe o ke aʻa aʻa
• Kānāwai o ka hanu hypertension pulmonary
• Iapaau ana o ka pulmonary arterial hypertension
• Nā Lapaʻau
• ʻOki kino
• Hoʻololi ka nohona
• ʻO ke ola me ke kiʻekiʻe o ka hanu pulmonary
• Nā pae o ka hypertension arterial pulmonary
• Nā ʻano ʻē aʻe o ka pulmonary hypertension
• Prognosis no ka pulmonary arterial hypertension
• Pulmonary hypertension i loko o nā pēpē pēpē hou
• Nā ʻōkuhi no ka piʻi ʻana o ke aʻa aʻa
• Q:
• A:

He aha ke kiʻekiʻe hypertension arterial?

ʻO ke kiʻekiʻe o ka pulmonary arterial hypertension (PAH), i ʻike ʻia ma ke ʻano he hypertension pulmonary mua, he ʻano laha ke kiʻekiʻe o ke koko kiʻekiʻe. Hoʻopili ia i kāu aʻa a me nā capillaries pulmonary. Lawe kēia mau kīʻaha koko i ke koko mai ke keʻena ʻākau ma lalo o kou puʻuwai (ventricle ʻākau) i loko o kou mau māmā.

Ke kū nei ke kaomi o kou aʻa aʻa a me nā kīʻaha koko liʻiliʻi, pono e hana ʻoi aku ka ikaika o kou puʻuwai e pīpī i ke koko i kou mau māmā. Ma hope o ka manawa, hoʻonāwaliwali kēia i kou puʻuwai puʻuwai. ʻO ka hope, hiki ke alakaʻi i ka puʻuwai ʻole a me ka make.

ʻAʻohe lāʻau i ʻike ʻia no PAH, akā loaʻa nā koho lapaʻau. Inā loaʻa iā ʻoe ka PAH, hiki i ka mālama ʻana ke kōkua i ka hōʻoluʻolu ʻana i kāu mau hōʻailona, ​​hoʻohaʻahaʻa i kou manawa o nā pilikia, a hoʻolōʻihi i kou ola.

Nā hōʻailona o ka hanu kiʻekiʻe

I nā wā mua o PAH, ʻaʻohe paha ou mau hōʻailona ʻike ʻia. Ke hele a maikaʻi ʻole ke kūlana, e ʻike ʻia nā ʻōuli. ʻO nā ʻōuli maʻamau ka:

• paʻakikī i ka hanu ʻana
• luhi
• ʻūpā
• maule ana
• puʻuwai umauma
• ʻeha ka umauma
• pulse wikiwiki
• palpitations puʻuwai
• polū polū i kou lehelehe a i ʻole ʻili
• ka pehu ʻana o kou kuʻekuʻe wāwae a i ʻole nā ​​wāwae
• ka pehu ʻana me ka wai o loko o kou ʻōpū, keu hoʻi i nā wā hope o ke ʻano

He paʻakikī paha iā ʻoe ke hanu i ka wā hoʻoikaika a ʻano ʻē aʻe o ka hoʻoikaika kino. Ma ka hopena, hiki i ka hanu ʻana ke lilo i paʻakikī i ka manawa o ka hoʻomaha. E ʻike pehea e ʻike ai i nā ʻōuli o PAH.

Nā kumu o ka piʻi kiʻekiʻe o ke aʻa aʻa

Hoʻokumu ʻo PAH ke hana ʻia nā aʻa pulmonary a me nā capillaries e halihali ana i ke koko mai kou puʻuwai i kou mau ake māmā a hoʻopau ʻia paha. Kuhi ʻia kēia e nā ʻano ʻano like ʻole, akā ʻaʻole maopopo ke kumu maoli no ke kumu e kū ai ʻo PAH.

Ma kahi o 15 a 20 pākēneka o nā hihia, hoʻoilina PAH, e like me ka National Organization for Rare Disorder (NORD). Pili kēia i nā hoʻololi kūlohelohe i hiki ke hana ʻia i ka BMPR2 ʻāpana a i ʻole nā ​​ʻāpana ʻē aʻe. Hiki i nā hoʻololi ke hoʻoili ʻia i waena o nā ʻohana, e ʻae ai i ke kanaka me kekahi o kēia mau hoʻololi e loaʻa ka hiki ke hoʻomohala ma hope o PAH.

ʻO nā kūlana kūpono ʻē aʻe i hiki ke hoʻopili ʻia me ka hoʻomohala PAH me:

• maʻi maʻi ate
• maʻi puʻuwai congenital
• kekahi mau maʻi ʻaʻa pili
• kekahi mau maʻi, e like me ka maʻi HIV a schistosomiasis paha
• kekahi mau mea make a lāʻau paha, e like me kekahi mau lāʻau leʻaleʻa (methamphetamines) a i ʻole nā ​​mea hoʻopōʻaiapuni makemake ʻole ma waho o ka mākeke

I kekahi mau hihia, hoʻomohala ʻo PAH me ke kumu pili ʻole i ʻike ʻia. ʻIke ʻia kēia ʻo idiopathic PAH. E ʻike pehea e ʻike ai a mālama ʻia ai ka PAH idiopathic.

Kānāwai o ka hanu hypertension pulmonary

Inā kānalua kāu kauka he PAH paha kāu, e kauoha paha lākou i hoʻokahi a ʻoi paha mau hoʻokolohua e nānā ai i kou aʻa a me kou puʻuwai.

Hiki i nā hoʻokolohua no ke kuhi ʻana iā PAH ke:

• electrocardiogram e nānā ai i nā hōʻailona o ke kanana a i ʻole nā ​​kaʻina maʻamau i loko o kou puʻuwai
• echocardiogram e nānā i ke ʻano a me ka hana o kou puʻuwai a ana i ke kaomi o ke aʻa aʻa
• pahu X-ray e aʻo inā hoʻonui ʻia kou ʻāpana pulmonary a i ʻole ke keʻena ʻākau ma lalo o kou puʻuwai
• CT scan a scan MRI paha e nānā i nā kīʻaha koko, hoʻoliʻiliʻi, a i ʻole pohō i loko o nā aʻa o kou pulmonary
• catheterization puʻuwai ʻākau e ana i ke kahe o ke koko i loko o kou aʻa aʻa a me ka ventricle ʻākau o kou puʻuwai
• hoʻāʻo hana pulmonary e nānā i ka hiki a me ke kahe o ka ea i loko a i waho o kou mau māmā
• nā ho'āʻo koko e nānā i nā mea e pili ana i ka PAH a i ʻole nā ​​kūlana olakino ʻē aʻe

Hiki i kāu kauka ke hoʻohana i kēia mau hoʻokolohua e nānā ai i nā hōʻailona o PAH, a me nā kumu kūpono ʻē aʻe o kāu mau ʻōuli. E hoʻāʻo lākou e kāpae i nā kumu kūpono ʻē aʻe ma mua o ka hōʻoia ʻana iā PAH. E kiʻi i ka ʻike hou aku e pili ana i kēia kaʻina.

Iapaau ana o ka pulmonary arterial hypertension

I kēia manawa, ʻaʻohe lāʻau lapaʻau i ʻike ʻia no PAH, akā hiki i ka lapaʻau ke hōʻoluʻolu i nā ʻōuli, hoʻemi i ka pilikia o nā pilikia, a hoʻolōʻihi i ke ola.

Nā Lapaʻau

I mea e kōkua ai i ka mālama ʻana i kāu maʻi, kauoha paha kāu kauka i hoʻokahi a ʻoi paha o nā lāʻau aʻe:

• prostacyclin therapy e hoʻonui i kāu mau moku koko
• soluble guanylate cyclase stimulator e hoʻonui i kou kīʻaha koko
• endothelin receptor antagonists e ālai i ka hana o endothelin, kahi mea i hiki ke hoʻowahāwahā i kou mau kīʻaha koko
• anticoagulants e pale aku i ka hoʻokumu ʻia ʻana o nā clots koko

Inā pili ʻo PAH i kekahi ʻano olakino ʻē aʻe i kāu hihia, e kauoha paha kāu kauka i nā lāʻau ʻē aʻe e kōkua i ka mālama ʻana i kēlā ʻano. Hoʻololi paha lākou i nā lāʻau āu e lawe ai i kēia manawa. E ʻike hou aʻe e pili ana i nā lāʻau i kuhikuhi ʻia e kāu kauka.

ʻOki kino

Kaukaʻi ʻia i ka nui o kou ʻano, koi paha kāu kauka i kahi lāʻau ʻoki. Hiki ke hana i ka septostomy Atrial e hōʻemi i ke kaomi ma ka ʻaoʻao ʻākau o kou puʻuwai, a ʻo kahi māmā a puʻuwai a puʻuwai paha e hiki ke pani i nā mea i hōʻino ʻia.

I kahi septostomy atrial, e alakaʻi paha kāu kauka i kahi catheter ma o kekahi o kou mau uaua waena i ke keʻena ʻākau ma luna o kou puʻuwai. I ke keena kiʻekiʻe septum (ke kaola o nā mea i waena o nā ʻaoʻao ʻākau a hema o ka puʻuwai), e hele ana mai ka ʻākau a ka hema o ke keʻena, e hana lākou i kahi wehe. ʻO ka mea aʻe, e hoʻonui lākou i kahi baluna liʻiliʻi i ka piko o ka catheter e hoʻonui i ka wehe a hiki i ke kahe o ke koko ma waena o nā keʻena o luna o kou puʻuwai, e hoʻomaha ai i ke kaomi ma ka ʻaoʻao ʻākau o kou puʻuwai.

Inā loaʻa iā ʻoe kahi hihia koʻikoʻi o PAH e pili ana i ka maʻi o ka maʻi nui, e paipai ʻia kahi transplant lung. I kēia kaʻina hana, e kāpae kāu kauka i hoʻokahi o ʻelua paha o kou mau māmā a hoʻololi iā lākou me nā akemāmā mai kahi mea hāʻawi.

Inā loaʻa ʻoe i ka maʻi puʻuwai nui a i ʻole ʻole ka puʻuwai, hiki i kāu kauka ke koi aku i kahi kuʻi puʻuwai e hoʻohui i ka transplantation lung.

Hoʻololi ka nohona

Hoʻololi ka nohona e hoʻoponopono i kāu papaʻai, nā hana hoʻoikaika kino, a i ʻole nā ​​ʻano maʻamau i kēlā me kēia lā e hiki ke kōkua i ka hōʻemi ʻana i kāu pilikia o nā pilikia PAH. Pākuʻi ʻia kēia mau mea:

• ʻai i ka papaʻai olakino
• hoʻoikaika kino mau
• ke kaupaona ʻana a i ʻole ka mālama ʻana i kahi kaupaona olakino
• ke haʻalele nei i ka puhi paka

Ma hope o ke kauka lāʻau lapaʻau a ke kauka i hiki ai ke kōkua i ka hōʻoluʻolu i kāu mau ʻōuli, hōʻemi i kou makaʻu i nā pilikia, a hoʻolōʻihi i kou ola. E aʻo hou aʻe e pili ana i nā koho lapaʻau no PAH.

ʻO ke ola me ke kiʻekiʻe o ka hanu pulmonary

ʻO PAH kahi kūlana holomua, ʻo ia hoʻi ka maikaʻi o ka manawa. ʻIke paha kekahi poʻe i nā ʻōuli hele a ʻoi aku ka wikiwiki ma mua o nā poʻe ʻē aʻe.

Ua hoʻopaʻa ʻia kahi noi ma 2015 i nānā ʻia i nā helu ola ʻelima mau makahiki no ka poʻe me nā ʻanuʻu like ʻole o PAH a ʻike ʻia i ka holo ʻana o ke kūlana, ua emi ka helu ola ʻelima mau makahiki.

Eia nā mea noiʻi ola ola ʻelima mau makahiki i loaʻa no kēlā me kēia pae.

• Papa 1: 72 a 88 pakeneka
• Papa 2: 72 a 76 pakeneka
• Papa 3: 57 a 60 pakeneka
• Papa 4: 27 a 44 pakeneka

ʻOiai ʻaʻohe mea hoʻōla, ʻo nā holomua o ka hoʻoponopono ʻana i kēia manawa i kōkua i ka hoʻomaikaʻi ʻana i ka manaʻo no ka poʻe me PAH. E aʻo hou aʻe e pili ana i nā helu ola no nā poʻe me PAH.

Nā pae o ka hypertension arterial pulmonary

Hoʻokaʻawale ʻia ʻo PAH i ʻehā mau pae i hoʻokumu ʻia i ke koʻikoʻi o nā ʻōuli.

Wahi a nā pae hoʻohālikelike i hoʻokumu ʻia e ka World Health Organization (WHO), ua hoʻokaʻawale ʻia ʻo PAH i ʻehā mau kaʻina hana.

• Papa 1. ʻAʻole kaupalena ke kūlana i kāu hana kino. ʻAʻole ʻoe e ʻike i nā ʻōuli i ʻike ʻia i nā wā o ka hoʻoikaika kino maʻamau a hoʻomaha paha.
• Papa 2. Hoʻopau iki ke ʻano i kāu hana kino. ʻIke ʻoe i nā ʻōuli i ʻike ʻia i nā wā o ka hoʻoikaika kino maʻamau, akā ʻaʻole i nā wā hoʻomaha.
• Papa 3. Kākoʻo nui ke ʻano i kāu hoʻoikaika kino. ʻIke ʻoe i nā ʻōuli i nā wā o ka hoʻoikaika kino a me ka hoʻoikaika kino maʻamau, akā ʻaʻole i ka wā hoʻomaha.
• Papa 4. ʻAʻole hiki iā ʻoe ke hoʻokō i kekahi ʻano o ka hana kino me ka ʻole o nā ʻōuli. ʻIke ʻoe i nā hōʻailona ʻike ʻia, ʻoiai i nā wā hoʻomaha. ʻO nā hōʻailona o ka puʻuwai ʻaoʻao ʻākau e hana i kēia pae.

Inā loaʻa iā ʻoe ka PAH, e hoʻopili ka pae o kāu kūlana i ke ʻano o ke kauka i koi ʻia e kāu kauka. E kiʻi i ka ʻike e pono ai e hoʻomaopopo i ka holo ʻana o kēia ʻano.

Nā ʻano ʻē aʻe o ka pulmonary hypertension

ʻO PAH kekahi o nā ʻano ʻelima o ka hanu pulmonary (PH). ʻIke ʻia kekahi ma ka Pūʻulu 1 PAH.

ʻO nā ʻano ʻē aʻe o PH pū kekahi:

• Pūʻulu 2 PH, i hoʻopili ʻia i kekahi mau ʻano e pili ana i ka ʻaoʻao hema o kou puʻuwai
• Pūʻulu 3 PH, i pili pū me kekahi ʻano hanu i nā akemāmā
• ʻOihana 4 PH, hiki i ke kumu o ke kahe o ke koko mau i loko o nā moku i kou mau māmā
• Pūʻulu 5 PH, i hiki ke hopena mai nā ʻano olakino ʻokoʻa

ʻO kekahi ʻano o PH hiki ke mālama ʻia ma mua o nā mea ʻē aʻe. E lawe i kahi manawa e aʻo hou aʻe e pili ana i nā ʻano like ʻole o PH.

Prognosis no ka pulmonary arterial hypertension

I nā makahiki i hala iho nei, ua hoʻomaikaʻi ʻia nā koho lapaʻau no ka poʻe me PAH. Akā ʻaʻohe lāʻau lapaʻau no kēia ʻano.

Hiki ke kōkua i ka hōʻoia mua a me ka lāʻau ʻana e hōʻoluʻolu maikaʻi i kāu mau ʻōuli, hōʻemi i kou makaʻu i nā pilikia, a hoʻolōʻihi i kou ola me PAH. E heluhelu hou aʻe e pili ana i nā hopena i hiki ke mālama ʻia i kāu nānā ʻana me kēia maʻi.

Pulmonary hypertension i loko o nā pēpē pēpē hou

I nā hihia maʻamau, hoʻopili ʻo PAH i nā pēpē hānau hou. ʻIke ʻia kēia ʻo ka hoʻomau ʻana o ka pulmonary hypertension o ka hānau hou (PPHN). Hana ʻia ia ke hele ʻole nā ​​kīʻaha koko e hele i nā ake māmā o ka pēpē ma hope o ka hānau ʻana.

Nā kumuhana kūpilikiʻi no PPHN me:

• maʻi ʻaʻai
• pilikia kaumaha i ka wā o ka hānau ʻana
• nā pilikia pūhū, e like me nā māmā i hoʻomohala ʻole ʻia a i ʻole syndrome syndrome respiratory

Inā kuhi ʻia kāu pēpē me PPHN, e hoʻāʻo kā lākou kauka e hoʻonui i nā kīʻaha koko i loko o kā lākou mau māmā me ka oxygen hoʻonui. Pono paha ke kauka e hoʻohana i kahi ʻenekini mīkini e kākoʻo i ka hanu ʻana o kāu pēpē.

Hiki ke kōkua kūpono a kūpono hoʻi i ka hoʻohaʻahaʻa ʻana i kāu pēpē i ka hoʻopaneʻe ʻana i ka ulu ʻana a me nā hana kīnā ʻole, e kōkua ana i ka hoʻomaikaʻi ʻana i ka manawa o ke ola.

Nā ʻōkuhi no ka piʻi ʻana o ke aʻa aʻa

I ka 2014, ua hoʻokuʻu ke Kulanui ʻAmelika ʻo Chest Physicians no ka mālama ʻana iā PAH. Ma waho aʻe o nā ʻōlelo aʻoaʻo ʻē aʻe, kuhikuhi kēia mau alakaʻi i:

• ʻO nā kānaka e makaʻu nei i ka hoʻomohala ʻana iā PAH a me nā mea me ka Papa 1 PAH e pono e kiaʻi ʻia no ka hoʻomohala ʻana i nā ʻōuli e pono ai ka lāʻau.
• Ke hiki, pono e loiloi i ka poʻe me PAH ma ke keʻena olakino i loaʻa ka loea i ka diagnostical PAH, optimally ma mua o ka hoʻomaka ʻana o ka lāʻau.
• Pono e mālama ʻia ka poʻe me PAH no nā kūlana olakino hiki ke hāʻawi i ka maʻi.
• Pono nā kānaka me ka PAH e huakaʻi kūpale ʻia i ka maʻi influenza a me ka pneumococcal pneumonia.
• Pono nā poʻe me PAH e hōʻalo i ka hāpai ʻana. Inā hāpai lākou, pono lākou e mālama mai kahi hui olakino multidisciplinary e hoʻopili pū ana i nā loea me ka loea i ka pulmonary hypertension.
• Pono nā poʻe me PAH e hōʻalo i ke kahakaha kūpono ʻole. Inā pono lākou e hana i ke kaha, pono lākou e mālama mai kahi hui olakino multidisciplinary e hoʻopili pū ana i nā loea me ka loea i ka pulmonary hypertension.
• Pono ka poʻe me PAH e hōʻole i ka ʻike i nā kiʻekiʻe kiʻekiʻe, e like me ka huakaʻi ea. Inā pono e hōʻike ʻia i nā kiʻekiʻena kiʻekiʻe, pono lākou e hoʻohana i ka oxygen supplemental e pono ai.

Hāʻawi kēia mau kulekele i kahi ʻōkuhi maʻamau no ka mālama ʻana i ka poʻe me PAH. E hilinaʻi kāu lāʻau pilikino i kāu mōʻaukala olakino a me nā ʻōuli āu e ʻike nei.

Q:

Aia kekahi mau kaʻina e hiki i kekahi ke hana e pale i ka hoʻomohala ʻana iā PAH?

A:

ʻAʻole hiki ke pale ʻia ka hypertension pulmonary Eia nō naʻe, hiki ke pale ʻia a kāohi ʻia paha kekahi mau ʻano i hiki ke alakaʻi iā PAH e hōʻemi i ka makaʻu o ka hoʻomohala ʻana iā PAH. ʻO kēia mau ʻano e pili ana i ka maʻi o ke aʻa coronary, hypertension, maʻi ate mau (ʻo ka mea pili pinepine i ka ate momona, ka wai ʻona, a me ka maʻi hepatitis viral), HIV, a me nā maʻi puʻupuʻu mau, ʻo ia hoʻi e pili ana i ka puhipaka a me nā hōʻike ākea.