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PULMONARY HYPERTENSION

??"? 11.09. ?"? ?. ?????. PH: DEFINITION. ???????: ????? ????? ????????? ?????? ????? ??????? ????? ????? (PVR) ??????? ???"? ???? ?????? ????? !??????: ????? ?????? ???? ?????? ?????? ????? (mPAP) ??? 25 ??"? ?????? ???? ???-??? ?????? (PCWP), ?? LVEDP , ??? ?- 15 ??"? ??????? ????? ?????, PVR >

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PULMONARY HYPERTENSION

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    1. ??"? 11.09 ?"? ?. ????? PULMONARY HYPERTENSION ??? ??? ?? ????? ?"? ????? ????? ??? ???? ??' ????? ????? ??' ????? ?????? "????", ???? ??????? ??????????. ???? ?????? ???? ???? ???? ???????, ?????? NO ?? ??????? ????? ?????.?????? "????", ???? ??????? ??????????. ???? ?????? ???? ???? ???? ???????, ?????? NO ?? ??????? ????? ?????.

    2. ??"? 11.09 ?"? ?. ????? PH: DEFINITION ???????: ????? ????? ????????? ?????? ????? ??????? ????? ????? (PVR) ??????? ???"? ???? ?????? ????? ! ??????: ????? ?????? ???? ?????? ?????? ????? (mPAP) ??? 25 ??"? ?????? ???? ???-??? ?????? (PCWP), ?? LVEDP , ??? ?- 15 ??"? ??????? ????? ?????, PVR > 3Wu ?????? ?- PH ????, ????? ?????????? ????? ?????????? ??????? ??????. ??? ????? ?? ???? ??? ??? ??? ?? ????? ????? ? PVR ??? ? 3Wu . ???? ?????? ???? ???? PCWP >15 ???' ??? ???????????? IV ?????? ?? PVR ??? ?-3. ????? ????? ??? 3 ????? ?? ??? ??? ?.?? ????? ?????.??? ????? ?? ???? ??? ??? ??? ?? ????? ????? ? PVR ??? ? 3Wu . ???? ?????? ???? ???? PCWP >15 ???' ??? ???????????? IV ?????? ?? PVR ??? ?-3. ????? ????? ??? 3 ????? ?? ??? ??? ?.?? ????? ?????.

    3. ??"? 11.09 ?"? ?. ????? ???????? ??? ?? ???????: ??????? ???????. ??? ????? ?? ?? ??? ???? ?- PH ???????? ??? ???? ?????. ????????? ??? ?? ????????. ??????? +._ ?????? ??????' ????. ?? ??? ???? !!!! Symptoms at presentation vary from patient to /. patient but, in general, suggest impaired oxygen transport and reduced cardiac output (CO).1,2 In the National Institutes of Health registry for primary pulmonary hypertension, the most common initial symptoms were exertional dyspnea, fatigue, syncope or near syncope, chest pain, palpitations, and edema of the lower extremities.2 ???????? ??? ?? ???????: ??????? ???????. ??? ????? ?? ?? ??? ???? ?- PH ???????? ??? ???? ?????. ????????? ??? ?? ????????. ??????? +._ ?????? ??????' ????. ?? ??? ???? !!!! Symptoms at presentation vary from patient to /. patient but, in general, suggest impaired oxygen transport and reduced cardiac output (CO).1,2 In the National Institutes of Health registry for primary pulmonary hypertension, the most common initial symptoms were exertional dyspnea, fatigue, syncope or near syncope, chest pain, palpitations, and edema of the lower extremities.2

    4. ??"? 11.09 ?"? ?. ?????

    5. ??"? 11.09 ?"? ?. ????? ???? ???? ?.? ?? 32. ???? 16 ?.?. ?????? ???, ?+ 1 ????? ?????? (2.2007): ????? ?? 3 ????, ???? ????? ???? ?? ???? ??? ???. ?????: ?? ???? ????? ???? ???? 50. ????? ??????: ????? CT ???: (????? Ac.PE) ????? ???? ???? ???? ?????? ??? ???? ????, ??? ???? ?- PE. ?????: SPAP= 62 ??"?. ?????????? ?????? ??? ????????? ??? ????.PFO ??? ?? ??? ?? ????? ?????.

    6. ??"? 11.09 ?"? ?. ????? ????? ????? (????? ????? ?????): ??????? ????????? ??????. ?????? ?????: ???? ANA ANCA,, ????? ????. ????? ????: ????? ??????? ????? ??????. ???? ???????? ???? ????? ??? 73% ?????? ???? ????? 6 ????: ???? 624 ?'. ????? ???? ?????? 97% ????? ???? 93%

    7. ??"? 11.09 ?"? ?. ????? ???? ???? ???? ?????? ?? ????: RAP=10 mmHg MPAP= 60 PVR= 8.6 wu C.I= 3.18 L/m2 PCWP= 10 mmHg NO test: negative (no change in MPAP) ??? ???? ???? ??? ???????? (????? ???? ?????? ???? ????? , ??????? ????? ????= ?????, 72% ?????: Idiopathic pulmonary arterial hypertension

    8. ??"? 11.09 ?"? ?. ????? Clinical Classification of Pulmonary Hypertension

    9. ??"? 11.09 ?"? ?. ?????

    10. ??"? 11.09 ?"? ?. ?????

    11. ??"? 11.09 ?"? ?. ????? ?????? PH (????...) ???? ?????: ???-????? ?? ?????? ????? ??????: ????? Tricuspid regurgitation (TR) velocity ?????? ????? sPAP ????? ???? ????? (?????) ????? ???? ????? ????? (????? ???? ??????????? ????) ????? ??? ???? (?????, ???????? ?????) ????? ????? ???-????? ( ????? ????? ????????, D-shape) ????? ????? ???? ???? ??????? ?????? ?-PH (??-????? ?? ??"? ????????? !)

    12. ??"? 11.09 ?"? ?. ????? ?????? ???,? ????: ????? ????? ?????? ?????? ?????: PH ?????????? , ? PH ??????.... ???? ????? ????? ?? ??????? ?????????? ??????. ????? ????? ??? ???? ?????? ??? ?????? ???? ????? ??? !!! (????? ????).?????? ???,? ????: ????? ????? ?????? ?????? ?????: PH ?????????? , ? PH ??????.... ???? ????? ????? ?? ??????? ?????????? ??????. ????? ????? ??? ???? ?????? ??? ?????? ???? ????? ??? !!! (????? ????).

    13. ??"? 11.09 ?"? ?. ????? Clinical Classification of Pulmonary Hypertension

    14. ??"? 11.09 ?"? ?. ????? ????????/?????? ????? ????? ??????????????/?????? ????? ????? ??????

    15. ??"? 11.09 ?"? ?. ????? ?????? ?? ???? ??????? PH (????...) ??? ???? ?? PH ?????? ???? ????? ???????, ?? ?????? ?????? ??? ????? ?? ????? (?????????, SCA) ?? ???? ???? ?????? ?? ???? (RHC). RHC ????? ?????? ????? ????????? ?????? ?????? ????? ?????? ?? ????? ???? PCWP ! mPAP C.O/C.I, PVR. RHC ????? ?????? ?????: ????? ??? ???????????? (NO, Epop.) ?????? ?- Responders/ non-responders Responders ?????? ????? ?????? ????? ???? ?????? ??????? ?- PH (???? ? IPAH). ???? ! ????? Responders: ????? ? mPAP ?- 10 ??"? + ???? ? 40 ??"? + ????? ?? ???? ???? ?- C.O ! ??? ?????? ACCP 2004 ????? ?? ??"? ???? ?? ?? ?????? ????????? ?? ?????? ????? ??? ??????????? ?? ???? CCB ! ??? ?????? ACCP 2004 ????? ?? ??"? ???? ?? ?? ?????? ????????? ?? ?????? ????? ??? ??????????? ?? ???? CCB

    16. ??"? 11.09 ?"? ?. ?????

    17. ??"? 11.09 ?"? ?. ????? ???? ?? ?????? ????, ?? ???? ?????? ???? ?????? ???? ??, ??? ?.?? ????? ????????????? ?? ?????? ????, ?? ???? ?????? ???? ?????? ???? ??, ??? ?.?? ????? ?????????

    18. ??"? 11.09 ?"? ?. ????? PH WHO GROUP IV: ??? ??? ?? ????? ??? ?????????????? ????? ????? Chronic thromboembolic pulmonary hypertension (CTEPH) Acute PE/DVT ????? ???? ?????? ?? CTEPH- ?????? ??????. ??????: ?????? Acute PE ???? ?????? ?????? ?????? ????? ????????? !!! ??????????? ????? ?????? ???: ????? 8 ????? ???? 40% ACL+ ??- 20%. ???? ?? ???????? ?????? ???? ???? ???? ???? !!! (?????? ??????? ?? ?????????) – ????? ???-??????? ?????? ??????? 4% ???? . ???? ?? ???? ???? (??????)- ????? ?????? "????? ????" ???? ??????? ?????? !

    19. ??"? 11.09 ?"? ?. ????? CTEPH following acute PE As discussed previously, the real incidence of CTEPH following PE has recently been shown to be considerably higher than previously thought. In this prospective, long-term, longitudinal follow-up study, the cumulative incidence of symptomatic CTEPH following acute PE was 1.0% at 6 months, 3.1% at 1 year, rising to a maximum of 3.8% at 2 years. No cases occurred after 2 years among the patients with more than 2 years of follow-up data. The authors of this study concluded that CTEPH is a relatively common, serious complication of pulmonary embolism and emphasized the need for effective diagnostic and therapeutic strategies. As discussed previously, the real incidence of CTEPH following PE has recently been shown to be considerably higher than previously thought. In this prospective, long-term, longitudinal follow-up study, the cumulative incidence of symptomatic CTEPH following acute PE was 1.0% at 6 months, 3.1% at 1 year, rising to a maximum of 3.8% at 2 years. No cases occurred after 2 years among the patients with more than 2 years of follow-up data. The authors of this study concluded that CTEPH is a relatively common, serious complication of pulmonary embolism and emphasized the need for effective diagnostic and therapeutic strategies.

    20. ??"? 11.09 ?"? ?. ????? ?????? CTEPH ????? ????? ????? ?????? (??? CTA !) ????? ????? CT-A- ????? ?????? ??? ????? ????? ??????? ?????????? ??????? – ???? ???? ????? ?????? ???????/????? ???? ????? ???-???????????. ????? ????? (?.?) ??????????? ????? (?.?) ??????

    21. ??"? 11.09 ?"? ?. ?????

    22. ??"? 11.09 ?"? ?. ?????

    23. ??"? 11.09 ?"? ?. ?????

    24. ??"? 11.09 ?"? ?. ?????

    25. ??"? 11.09 ?"? ?. ?????

    26. ??"? 11.09 ?"? ?. ????? ??????? ? CTEPH ??? ?????, ????? ???? ????? ????? (mPAP)

    27. ??"? 11.09 ?"? ?. ????? Understanding of the pathophysiology of PH has grown in recent years. Endothelial cell dysfunction appears to play a key role in PH. Over time, impaired production of vasoactive mediators, such as nitric oxide (NO) and prostacyclin, along with prolonged overexpression of vasoconstrictors, such as endothelin-1 and thromboxane A2, affect vascular tone and promote vascular remodeling.1,2 The main vascular changes include vasoconstriction, smooth muscle cell and endothelial cell proliferation, and thrombosis—all of which suggest an imbalance between vasodilators and vasoconstrictors and other vascular effectors.2Understanding of the pathophysiology of PH has grown in recent years. Endothelial cell dysfunction appears to play a key role in PH. Over time, impaired production of vasoactive mediators, such as nitric oxide (NO) and prostacyclin, along with prolonged overexpression of vasoconstrictors, such as endothelin-1 and thromboxane A2, affect vascular tone and promote vascular remodeling.1,2 The main vascular changes include vasoconstriction, smooth muscle cell and endothelial cell proliferation, and thrombosis—all of which suggest an imbalance between vasodilators and vasoconstrictors and other vascular effectors.2

    28. ??"? 11.09 ?"? ?. ?????

    29. ??"? 11.09 ?"? ?. ?????

    30. ??"? 11.09 ?"? ?. ????? PH: ????? ?. ????? ??????: ????? ????? ????? (??"?, ???????, ?????????, ??? ?????, ?????????????? ???') ?. ????? ??????: 1. ????: ????, ?????? (IPAH, CPTE, ???? ???? ???? ??????) 2. ?????? ?????: ?????? ??? ??-????? ????????????? ?????-????????????? (??????? ??????? – CCB). ???? ?? ????? ?? ???? ?- VOD ! (???????? ???"? ??????? ?? ??? ?.?? ?? ???????????? ???"?). ?????? ??? ???? ??????????? ?? ????? !! 3. ????? ??????: - ???-?????????? ?- CTEPH ????? ! - ????? ????? (???? ?????), ??-????? / ???????? - ????????? ?????????

    31. ??"? 11.09 ?"? ?. ????? This is the World Health Organization (WHO) functional classification of PAH, which is modified from the New York Heart Association Classification of Patients With Cardiac Disease.This is the World Health Organization (WHO) functional classification of PAH, which is modified from the New York Heart Association Classification of Patients With Cardiac Disease.

    32. ??"? 11.09 ?"? ?. ????? Current treatment approaches are geared toward the prostacyclin, endothelin, and nitric oxide pathways. This schematic illustrates the endothelin, NO, and prostacyclin pathways and their respective therapies. Current treatment approaches are geared toward the prostacyclin, endothelin, and nitric oxide pathways. This schematic illustrates the endothelin, NO, and prostacyclin pathways and their respective therapies.

    33. ??"? 11.09 ?"? ?. ????? ?????? "??????? ????" ??????? PGE-5 inhibitors ???????? (?????), ??????? Endothelin Receptor Antagonists (ERA) ?????? (???????), ????????? Prostacyclines I.V: epoprostenol (Flolan), Trepprostynil (Remodulin) Inhaled: Iloprost (Ventavis)

    34. ??"? 11.09 ?"? ?. ????? ????? ?????? GOAL-Directed Therapy ????? ????? ?????: 6MWD, Funct. class I,II, QOL, VO2max ????? ???????? (C.I, PVR), ????? ?????? RV (??? ????? ???? ?????) ????? ????? ??? ?????? ?????? ????? ??????? ??????? ?? ??????!!! ?????? ???????? ????? ??? ?????? ?? ???? ?????? ??????? ?????? ?????? ???? ?.?? ????? ????????, ??????????, CHD, CTEPH - ????? ????? ?- 6MWD, ?????? ??????, ?????? ??????????, ????? ??? ?????, ????? ??????? ?? ????? ????.

    35. ??"? 11.09 ?"? ?. ????? ????? ????? ?????? ????? 6MWT Functional Class ?????? ???? BNP ?????: ????? ??? ????, ????? ????? (?? SPAP !!) ?????? ???? ???? ?????? ??????????

    36. ??"? 11.09 ?"? ?. ?????

    37. ??"? 11.09 ?"? ?. ?????

    38. ??"? 11.09 ?"? ?. ????? ?????????? ?????? ?????? ????? ??????? PAH ?????? ??????-???????? ???? ????? ??????? ?????-?????? ?? ?????? ?? ????? ????? ?? ???? ????? ?????? ?????? ?? ????? ?????? ????? ????? ???? ???????? ?? ?????: ????? ??????? ???? ??????? ???? ?????? ????? (??? ??? ?? ??????, ??"?, ?????????)

    39. ??"? 11.09 ?"? ?. ????? ?????????? ?????? ????? ????? ????? ???? ??????????? III, IV ????? ????? ?????? ?- I.V Epoprostenol (Flolan) ???? ????? ??????? 3 ????= 55% ?- 5 ???? = 45%

    40. ??"? 11.09 ?"? ?. ?????

    41. ??"? 11.09 ?"? ?. ????? PH: ????? PH ??? ???? ????? ????? = ???????? ?? ???????. ?? ????? ?? ?? ??? ???? ????? ??????? ! PH ,??? ?? ??????????, ???? ??? ???? – ?? ???? ???? ! PH, ??? ??? ????? = ????? ???????? ????????? ?? ????? ?? ????? ??????? ???????? ???? ????? RHC ! ????? = ??? ?????, ???? "?????? ??????? ????" ?? ??????? ??????????? ???????? – ?????, ?????????????, ?????????.

    42. ??"? 11.09 ?"? ?. ?????

    43. ??"? 11.09 ?"? ?. ????? ??????? ??????

    44. ??"? 11.09 ?"? ?. ????? ????? ?- PH : ?????????? ????????? ??? WHO (Venice 2003) ?? ??? ???? ????? PH ????? ????? ??????????? ??? ??? ????? ?? ????? ?? ??? ??????. PH ??? ?? ???? ?? ?????? ??????? ?????????? ????? ???. ??? ???? ????? ?????? ???????. ????? ?? ??????, ?????? ?????? IPAH ?????????? ????????? ???. ?????? ?????? ?? ???? ????? ?????/????????? ???????. ?? ????? ??? ?????????. ????? ?????? ?????? ? PH ???????? ????? I ?????????????? ????? ????? IV?? ??? ???? ????? PH ????? ????? ??????????? ??? ??? ????? ?? ????? ?? ??? ??????. PH ??? ?? ???? ?? ?????? ??????? ?????????? ????? ???. ??? ???? ????? ?????? ???????. ????? ?? ??????, ?????? ?????? IPAH ?????????? ????????? ???. ?????? ?????? ?? ???? ????? ?????/????????? ???????. ?? ????? ??? ?????????. ????? ?????? ?????? ? PH ???????? ????? I ?????????????? ????? ????? IV

    45. ??"? 11.09 ?"? ?. ?????

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