PULMONARY HYPERTENSION ??? ??? ?? ?????

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PULMONARY HYPERTENSION??? ??? ?? ?????

• ?"? ????? ?????
• ??? ???? ??' ?????
• ????? ??' ?????

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PH DEFINITION

• ??????? ????? ????? ????????? ?????? ?????
  ??????? ????? ????? (PVR) ??????? ???"? ????
  ?????? ????? !
• ?????? ????? ?????? ???? ?????? ?????? ?????
  (mPAP) ??? 25 ??"? ?????? ???? ???-??? ??????
  (PCWP), ?? LVEDP , ??? ?- 15 ??"? ??????? ?????
  ?????, PVR gt 3Wu
• ?????? ?- PH ????, ????? ?????????? ?????
  ?????????? ??????? ??????.

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PAH A Progressive Disease
Time
Rich et al. In Harrisons Principles of Internal
Medicine. 15th ed. 20011506-1507.
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???? ????

• ?.? ?? 32. ???? 16 ?.?. ?????? ???, ? 1
• ????? ?????? (2.2007) ????? ?? 3 ????, ????
  ????? ???? ?? ???? ??? ???.
• ????? ?? ???? ????? ???? ???? 50.
• ????? ?????? ?????
• CT ??? (????? Ac.PE) ????? ???? ???? ???? ??????
  ??? ???? ????, ??? ???? ?- PE.
• ????? SPAP 62 ??"?. ?????????? ?????? ???
  ????????? ??? ????.PFO ??? ?? ??? ?? ????? ?????.

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• ????? ????? (????? ????? ?????) ???????
  ????????? ??????.
• ?????? ????? ???? ANA ANCA,, ????? ????.
• ????? ???? ????? ??????? ????? ??????. ????
  ???????? ???? ????? ??? 73 ??????
• ???? ????? 6 ???? ???? 624 ?'. ????? ???? ??????
  97 ????? ???? 93

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???? ???? ????

• ?????? ?? ????
• RAP10 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

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Clinical Classification of Pulmonary Hypertension
Simonneau G et al. J Am Coll Cardiol
20095443-54S
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PH ??????

• ?? ??? ???? ???????? ?? ?????? ??
  ??-?????????????
• ????? ???????? ?? ??? ???? ????-???????, ??? 2
  P ?????
• ????? ??? ????? ????? ???? (??? ?????)
• ???? ???????? ???? ?? ????? ????? ???? ?? ????
  ????.

ECG ?- PH
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?????? PH (????...)

• ???? ????? ???-????? ?? ?????? ????? ??????
• ????? Tricuspid regurgitation (TR) velocity
  ?????? ????? sPAP
• ????? ???? ????? (?????) ????? ???? ????? ?????
  (????? ???? ??????????? ????)
• ????? ??? ???? (?????, ???????? ?????)
• ????? ????? ???-????? ( ????? ????? ????????,
  D-shape)
• ????? ????? ???? ???? ??????? ?????? ?-PH
• (??-????? ?? ??"? ????????? !)

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???-?????????? ?- PH
???????
????????
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Clinical Classification of Pulmonary Hypertension
Simonneau G et al. J Am Coll Cardiol
20095443-54S
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???????? ??????? PH ?????? ????????
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?????? ?? ???? ??????? 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

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PH WHO GROUP I
Median survival time w/o therapy 2.8 years
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PH WHO GROUP IV ??? ??? ?? ????? ???
?????????????? ????? ?????Chronic thromboembolic
pulmonary hypertension (CTEPH)

• Acute PE/DVT ????? ???? ?????? ?? CTEPH- ??????
  ??????.
• ?????? ?????? Acute PE
• ???? ?????? ?????? ?????? ????? ????????? !!!
• ??????????? ????? ?????? ??? ????? 8 ????? ????
  40
• 
  ACL ??- 20.
• ???? ?? ???????? ?????? ???? ???? ???? ???? !!!
  (?????? ??????? ?? ?????????) ????? ???-???????
  ?????? ??????? 4 ???? .
• ???? ?? ???? ???? (??????)- ????? ?????? "?????
  ????" ???? ??????? ?????? !

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CTEPH following acute PE
0.04
3.8 at 2 years
0.03
3.1 at 1 year
Cumulative incidence of CTEPH
0.02
0.01
1.0 at 6 months
0
0
2
3
4
5
6
7
8
9
10
11
1
Years
Pengo V et al. N Eng J Med 2004 350 2257?2264.
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?????? CTEPH

• ????? ????? ????? ?????? (??? CTA !) ????? ?????
  
• CT-A- ????? ?????? ??? ????? ????? ???????
• ?????????? ??????? ???? ???? ????? ??????
  ???????/????? ???? ????? ???-???????????.

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??????? ? CTEPH ??? ?????, ????? ???? ?????
????? (mPAP)

• Riedel M et al. Chest 1982

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THE HISTORY of PH TREATMENT
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PH ?????

• ?. ????? ?????? ????? ????? ????? (??"?,
  ???????, ?????????, ??? ?????, ??????????????
  ???')
• ?. ????? ??????
• 1. ???? ????, ?????? (IPAH, CPTE, ???? ???? ????
  ??????)
• 2. ?????? ????? ?????? ??? ??-?????
  ????????????? ?????-????????????? (???????
  ??????? CCB). ???? ?? ????? ?? ???? ?- VOD !
  (???????? ???"? ??????? ?? ??? ?.?? ??
  ???????????? ???"?). ?????? ??? ???? ???????????
  ?? ????? !!
• 3. ????? ?????? - ???-?????????? ?- CTEPH ?????
  !
• - ????? ????? (???? ?????), ??-????? /
  ????????
• - ????????? ?????????

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?????? "??????? ????" ???????

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

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????? ?????? GOAL-Directed Therapy

• ????? ????? ????? 6MWD, Funct. class I,II, QOL,
  VO2max
• ????? ???????? (C.I, PVR),
• ????? ?????? RV (??? ????? ???? ?????)
• ????? ????? ??? ?????? ??????
• ????? ???????
• ??????? ?? ??????!!! ?????? ???????? ????? ???
  ??????
• ?? ???? ?????? ??????? ?????? ?????? ???? ?.??
  ????? ????????, ??????????, CHD, CTEPH - ?????
  ????? ?- 6MWD, ?????? ??????, ?????? ??????????,
  ????? ??? ?????, ????? ??????? ?? ????? ????.

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????? ????? ?????? ?????

• 6MWT
• Functional Class ?????? ????
• BNP
• ????? ????? ??? ????, ????? ????? (?? SPAP !!)
• ?????? ???? ???? ?????? ??????????

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????? ????? ??????? ?? ???? ????? ?- PH
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?????????? ?????? ?????? ?????

• ??????? PAH ?????? ??????-????????
• ???? ????? ??????? ?????-?????? ?? ?????? ??
  ????? ????? ?? ???? ????? ?????? ??????
• ?? ????? ?????? ????? ????? ???? ???????? ??
  ????? ????? ??????? ???? ??????? ???? ??????
  ????? (??? ??? ?? ??????, ??"?, ?????????)

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?????????? ?????? ????? ????? ?????

• ???? ??????????? III, IV ????? ????? ?????? ?-
• I.V Epoprostenol (Flolan)
• ???? ????? ??????? 3 ???? 55 ?- 5 ???? 45

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???????? ????? ?- PH ??? ????? ?????
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PH ?????

• PH ??? ???? ????? ????? ???????? ?? ???????.
  ?? ????? ?? ?? ??? ???? ????? ??????? !
• PH ,??? ?? ??????????, ???? ??? ???? ?? ????
  ???? !
• PH, ??? ??? ????? ????? ???????? ????????? ??
  ?????
• ?? ????? ??????? ???????? ???? ????? RHC !
• ????? ??? ?????, ???? "?????? ??????? ????" ??
  ??????? ??????????? ???????? ?????,
  ?????????????, ?????????.

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???? !!!
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??????? ??????
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????? ?- PH ?????????? ????????? ??? WHO
(Venice 2003)
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