Surunkali tromboembolik o'pka gipertenziyasi

Surunkali tromboembolik o'pka gipertenziyasi - yurakdan o'pkaga ( o'pka arterial daraxti ) qonni olib boradigan qon tomirlarining tiqilib qolishi natijasida yuzaga keladigan uzoq muddatli kasallik. Ushbu blokadalar o'pka arterial shoxida oqimga qarshilik kuchayishiga olib keladi va bu o'z navbatida bu arteriyalarda bosimning oshishiga olib keladi (o'pka gipertenziyasi). To'siqlar odatda tananing pastki oyoq-qo'llarining chuqur tomirlaridan (tromboemboliya) kelib chiqadigan va yuurakning o'ng tomonidan o'tgandan keyin o'pka arterial shoxiga joylashadigan birlashgan (yoki qotib qolgan) qon pıhtıları natijasida yuzaga keladi. To'siqlar, shuningdek, pıhtı o'pka arteriyalarining endotelial qoplamini shikastlagan joyda hosil bo'lgan chandiq to'qimasidanyuzaga kelishi mumkin, bu esa doimiy tolali obstruktsiyani (qon tizimining bloklanishi) vujudga keltiradii^[1].Bir qancha bemorlarda mikrovaskulyar (kichik tomir) va makrovaskulyar (katta tomir) obstruktsiyasi mavjud. Ba'zi bemorlarda simptomatik kasalliklarga qaramasdan dam olishda o'pka bosimi normal yoki me'yorga to'g'ri kelgan bo'lishi mumkin. Bu bemorlar surunkali tromboembolik kasallik bilan tavfsiflanadi^[2].

Tashxis, bu holatni pulmonar emboliyadan ajratish uchun kamida uch oylik samarali qon suyulishidan so'ng amalga oshiriladi. Surunkali tromboembolik o'pka gipertenziyasi uchun diagnostika natijalar^[2].

Invaziv (ya'ni, qonda) o'lchangan o'rtacha o'pka arterial bosimi (mPAP) ≥25 mmHg;
Ko‘p detektorli kompyuter tomografiyasi (MDCT), magnit-rezonans tomografiya (MRI) yoki an’anaviy o‘pka kineangiografiyasi (PAG), masalan, halqasimon stenozlar yordamida ko‘rilgan o‘pka ventilyatsiyasi perfuziyasi (V/Q) skanerida mos kelmaydigan perfuziya nuqsonlari va KTEPHning o‘ziga xos diagnostik belgilari., to'rlar/tiriklar, surunkali umumiy tiqilib qolishlar (qo'ltiqli zararlanish yoki toraygan jarohatlar) va burilishli jarohatlar.

Alomat va belgilari

Klinik simptomlar va belgilar ko'pincha o'ziga xos emas yoki yo'q, o'ng yurak yetishmovchiligi belgilari faqat rivojlangan kasalliklarda. CTEPH ning asosiy belgilari- bu o'ziga xos bo'lmagan va ko'pincha shifokorlar tomonidan boshqa, ko'p tarqalgan kasalliklar bilan bog'liq bo'lishi mumkin bo'lgan mashaqqatli nafas siqilishi (jismoniy mashqlar paytida nafas qisilishi). Agar mavjud bo'lsa, CTEPH klinik belgilari o'tkir idiopatik o'pka arterial gipertenziyasi belgilariga o'xshash bo'lishi mumkin. Oyoq shishi (shish) va gemoptiz (shilliqdagi qon), hushidan ketish (hushdan ketish) esa tez-tez uchraydi^[3].

Patogenez

Surunkali tromboembolik o'pka gipertenziyasi bilan kasallangan odamlarda tromboz uchun an'anaviy xavf omillari mavjud ema^[4]^[5]^[6]^[7]^[3]. Hozirgi tushunchaga ko'ra, "yallig'lanish trombozi" ning natijasidir^[3] .Protrombotik (qon ivishini hosil qiluvchi) holatlar surunkali yallig'lanish va infektsiya bilan birlashganda, tromb yo'qolishi mumkin. Surunkali tromboembolik o'pka gipertenziyasi uchun xavf omillari orasida splenektomiya, yallig'lanishli ichak kasalligi, surunkali qalqonsimon gormonlarni almashtirish, 0 dan boshqa qon turlari, infektsiyalangan qorincha-atriyal shunt va doimiy tomir ichiga yuborish kiradi^[7]^[3].

Surunkali tromboembolik o'pka gipertenziyasi da erta tashxis qo'yish hali ham muammo bo'lib qolmoqda, simptomlarning boshlanishi va ekspert markazlarida tashxis qo'yish o'rtasidagi minimalvaqt 14 oy^[8]. Shubha ko'pincha ekokardiografiyada paydo bo'ladi, ammo buni tasdiqlash uchun invaziv o'ng yurak kateterizatsiyasi talab qilinadi^[2]. PH tashxisi qo'yilgach, tromboembolik kasallikning mavjudligi tasvirni talab qiladi. Tavsiya etilgan diagnostika algoritmi ekokardiyogram va V/Q skanerlash va o'ng yurak kateteri va o'pka angiografiyasi bilan tasdiqlash yordamida birinchi tekshiruvning muhimligini ta'kidlaydi^[1].

Skanerlash va zamonaviy multidetektorli KT angiografiyasi mutaxassis qo'llarida mukammal diagnostika samaradorligiga ega bo'lgan kasallikni aniqlashning aniq usullari aniqlanilishi mumkin (sezuvchanlik, o'ziga xoslik va aniqlik V/ uchun 100%, 93,7% va 96,5%). Q va CTPA uchun 96,1%, 95,2% va 95,6%)^[9]. KTPAning o'zi kasallikni istisno qila olmaydi, lekin chap asosiy tojsimon arteriyaning qisilishiga, o'pka parenximasining shikastlanishiga (masalan, oldingi o'pka infarkti belgilari sifatida) va bronxial kollateral arteriyalardan qon ketishiga olib keladigan o'pka arteriyasining kengayishini aniqlashga yordam beradi^[10]. Bugungi kunda oltin standart ko'rish mahalliy angiogrammalar yoki raqamli olib tashlash texnikasidan foydalangan tarzda invaziv o'pka angiografiyasi bo'lib kelmoqda

Davolash

Bu kasallikka chalingan bemorlar uchun qaror qabul qilish qiyin bo'lishi mumkin va ekspert markazlarda CTEPH guruhlari tomonidantekshirilishi kerak.Kasallik guruhlari bo'yicha tayyorgarlikka ega kardiologlar va pulmonologlar, rentgenologlar, yiliga kasallikka chalingan bemorlarning katta yukiga ega bo'lgan tajribali PEA jarrohlari va teri orqali aralashuv tajribasiga ega shifokorlardan iborat. Hozirgi kunda o'pka endarterektomiyasini standart davolashni o'z ichiga olgan uchta tan olingan maqsadli davolash varianti mavjud. Jarrohlik uchun mos bo'lmagan insonlar uchun balonli o'pka angioplastikasi va o'pka tomirlarini kengaytiruvchi dori bilan davolab chiqilishi mumkin^[11].

Magnit rezonans yoki invaziv tekshirish orqali mutaxassis tasvirlaydi PEA yoki BPA bilan intervension davolashning xavf va foydalarini aniqlash uchun zarur.

O'pka endarterektomiyasi

Manbalar

↑ ^1,0 ^1,1 Kim, Nick H. (August 2016). "Group 4 Pulmonary Hypertension: Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Pathophysiology, and Treatment". Cardiology Clinics 34 (3): 435–441. doi:10.1016/j.ccl.2016.04.011. ISSN 1558-2264. PMID 27443139.
↑ ^2,0 ^2,1 ^2,2 Galiè, Nazzareno; Humbert, Marc; Vachiery, Jean-Luc; Gibbs, Simon; Lang, Irene; Torbicki, Adam; Simonneau, Gérald; Peacock, Andrew et al. (2016-01-01). "2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT)". European Heart Journal 37 (1): 67–119. doi:10.1093/eurheartj/ehv317. ISSN 1522-9645. PMID 26320113.
↑ ^3,0 ^3,1 ^3,2 ^3,3 Lang, Irene M.; Simonneau, Gérald; Pepke-Zaba, Joanna W.; Mayer, Eckhard; Ambrož, David; Blanco, Isabel; Torbicki, Adam; Mellemkjaer, Sören et al. (July 2013). "Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control study". Thrombosis and Haemostasis 110 (1): 83–91. doi:10.1160/TH13-02-0097. ISSN 0340-6245. PMID 23677493.
↑ Pepke-Zaba, Joanna; Delcroix, Marion; Lang, Irene; Mayer, Eckhard; Jansa, Pavel; Ambroz, David; Treacy, Carmen; D'Armini, Andrea M. et al. (2011-11-01). "Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry". Circulation 124 (18): 1973–1981. doi:10.1161/CIRCULATIONAHA.110.015008. ISSN 1524-4539. PMID 21969018.
↑ Bonderman, Diana; Jakowitsch, Johannes; Adlbrecht, Christopher; Schemper, Michael; Kyrle, Paul A.; Schönauer, Verena; Exner, Markus; Klepetko, Walter et al. (March 2005). "Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension". Thrombosis and Haemostasis 93 (3): 512–516. doi:10.1160/TH04-10-0657. ISSN 0340-6245. PMID 15735803.
↑ Bonderman, Diana; Skoro-Sajer, Nika; Jakowitsch, Johannes; Adlbrecht, Christopher; Dunkler, Daniela; Taghavi, Sharokh; Klepetko, Walter; Kneussl, Meinhard et al. (2007-04-24). "Predictors of outcome in chronic thromboembolic pulmonary hypertension". Circulation 115 (16): 2153–2158. doi:10.1161/CIRCULATIONAHA.106.661041. ISSN 1524-4539. PMID 17420352.
↑ ^7,0 ^7,1 Bonderman, D.; Wilkens, H.; Wakounig, S.; Schäfers, H.-J.; Jansa, P.; Lindner, J.; Simkova, I.; Martischnig, A. M. et al. (February 2009). "Risk factors for chronic thromboembolic pulmonary hypertension". The European Respiratory Journal 33 (2): 325–331. doi:10.1183/09031936.00087608. ISSN 1399-3003. PMID 18799507.
↑ Pepke-Zaba, Joanna; Jansa, Pavel; Kim, Nick H.; Naeije, Robert; Simonneau, Gerald (April 2013). "Chronic thromboembolic pulmonary hypertension: role of medical therapy". The European Respiratory Journal 41 (4): 985–990. doi:10.1183/09031936.00201612. ISSN 1399-3003. PMID 23397304.
↑ He, Jia; Fang, Wei; Lv, Bin; He, Jian-Guo; Xiong, Chang-Ming; Liu, Zhi-Hong; He, Zuo-Xiang (May 2012). "Diagnosis of chronic thromboembolic pulmonary hypertension: comparison of ventilation/perfusion scanning and multidetector computed tomography pulmonary angiography with pulmonary angiography". Nuclear Medicine Communications 33 (5): 459–463. doi:10.1097/MNM.0b013e32835085d9. ISSN 1473-5628. PMID 22262242.
↑ Tunariu, Nina; Gibbs, Simon J. R.; Win, Zarni; Gin-Sing, Wendy; Graham, Alison; Gishen, Philip; Al-Nahhas, Adil (May 2007). "Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension". Journal of Nuclear Medicine 48 (5): 680–684. doi:10.2967/jnumed.106.039438. ISSN 0161-5505. PMID 17475953.
↑ Brenot, Philippe; Mayer, Eckhard; Ghofrani, Hossein-Ardeschir; Kurzyna, Marcin; Matsubara, Hiromi; Ogo, Takeshi; Meyer, Bernhard C.; Lang, Irene (31 March 2017). "Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension" (en). European Respiratory Review 26 (143): 160119. doi:10.1183/16000617.0119-2016. ISSN 1600-0617. PMID 28356406. PMC 9489135. //www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=9489135.

Havolalar

[:0-1] 1,0 ^1,1 Kim, Nick H. (August 2016). "Group 4 Pulmonary Hypertension: Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Pathophysiology, and Treatment". Cardiology Clinics 34 (3): 435–441. doi:10.1016/j.ccl.2016.04.011. ISSN 1558-2264. PMID 27443139.

[:Galie_2015-2] 2,0 ^2,1 ^2,2 Galiè, Nazzareno; Humbert, Marc; Vachiery, Jean-Luc; Gibbs, Simon; Lang, Irene; Torbicki, Adam; Simonneau, Gérald; Peacock, Andrew et al. (2016-01-01). "2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT)". European Heart Journal 37 (1): 67–119. doi:10.1093/eurheartj/ehv317. ISSN 1522-9645. PMID 26320113.

[Lang_2013-3] 3,0 ^3,1 ^3,2 ^3,3 Lang, Irene M.; Simonneau, Gérald; Pepke-Zaba, Joanna W.; Mayer, Eckhard; Ambrož, David; Blanco, Isabel; Torbicki, Adam; Mellemkjaer, Sören et al. (July 2013). "Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control study". Thrombosis and Haemostasis 110 (1): 83–91. doi:10.1160/TH13-02-0097. ISSN 0340-6245. PMID 23677493.

[Pepke-Zaba_2011-4] Pepke-Zaba, Joanna; Delcroix, Marion; Lang, Irene; Mayer, Eckhard; Jansa, Pavel; Ambroz, David; Treacy, Carmen; D'Armini, Andrea M. et al. (2011-11-01). "Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry". Circulation 124 (18): 1973–1981. doi:10.1161/CIRCULATIONAHA.110.015008. ISSN 1524-4539. PMID 21969018.

[Bonderman_2005-5] Bonderman, Diana; Jakowitsch, Johannes; Adlbrecht, Christopher; Schemper, Michael; Kyrle, Paul A.; Schönauer, Verena; Exner, Markus; Klepetko, Walter et al. (March 2005). "Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension". Thrombosis and Haemostasis 93 (3): 512–516. doi:10.1160/TH04-10-0657. ISSN 0340-6245. PMID 15735803.

[Bonderman_2009-6] Bonderman, Diana; Skoro-Sajer, Nika; Jakowitsch, Johannes; Adlbrecht, Christopher; Dunkler, Daniela; Taghavi, Sharokh; Klepetko, Walter; Kneussl, Meinhard et al. (2007-04-24). "Predictors of outcome in chronic thromboembolic pulmonary hypertension". Circulation 115 (16): 2153–2158. doi:10.1161/CIRCULATIONAHA.106.661041. ISSN 1524-4539. PMID 17420352.

[Bonderman_2009b-7] 7,0 ^7,1 Bonderman, D.; Wilkens, H.; Wakounig, S.; Schäfers, H.-J.; Jansa, P.; Lindner, J.; Simkova, I.; Martischnig, A. M. et al. (February 2009). "Risk factors for chronic thromboembolic pulmonary hypertension". The European Respiratory Journal 33 (2): 325–331. doi:10.1183/09031936.00087608. ISSN 1399-3003. PMID 18799507.

[Pepke-Zaba_2013-8] Pepke-Zaba, Joanna; Jansa, Pavel; Kim, Nick H.; Naeije, Robert; Simonneau, Gerald (April 2013). "Chronic thromboembolic pulmonary hypertension: role of medical therapy". The European Respiratory Journal 41 (4): 985–990. doi:10.1183/09031936.00201612. ISSN 1399-3003. PMID 23397304.

[9] He, Jia; Fang, Wei; Lv, Bin; He, Jian-Guo; Xiong, Chang-Ming; Liu, Zhi-Hong; He, Zuo-Xiang (May 2012). "Diagnosis of chronic thromboembolic pulmonary hypertension: comparison of ventilation/perfusion scanning and multidetector computed tomography pulmonary angiography with pulmonary angiography". Nuclear Medicine Communications 33 (5): 459–463. doi:10.1097/MNM.0b013e32835085d9. ISSN 1473-5628. PMID 22262242.

[10] Tunariu, Nina; Gibbs, Simon J. R.; Win, Zarni; Gin-Sing, Wendy; Graham, Alison; Gishen, Philip; Al-Nahhas, Adil (May 2007). "Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension". Journal of Nuclear Medicine 48 (5): 680–684. doi:10.2967/jnumed.106.039438. ISSN 0161-5505. PMID 17475953.

[Brenot2017-11] Brenot, Philippe; Mayer, Eckhard; Ghofrani, Hossein-Ardeschir; Kurzyna, Marcin; Matsubara, Hiromi; Ogo, Takeshi; Meyer, Bernhard C.; Lang, Irene (31 March 2017). "Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension" (en). European Respiratory Review 26 (143): 160119. doi:10.1183/16000617.0119-2016. ISSN 1600-0617. PMID 28356406. PMC 9489135. //www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=9489135.

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