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Assessment 4
Lecture 3- Part 1 PAH
67
Anatomy
6th Grade
10/27/2013

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Term
PAH is defined as
Definition
restricted flow thry pulmonary circulation
Term
PAH ultimately leads to
what is the mortality in 1 year
Definition
right heart failure
15%
Term
what is the test they do for PAH?
Definition
6 min walk test
Term
genetic risk factors for PAH-2
Definition
ALK1 mutations
CPS polymorph
Term
risk factors for PAH-2
Definition
HIV
portal HTN
Term
what are the 4 factros invovled in etiology of PAH
Definition
vasoconstrict
thrombosis
proliferation
inflammation
Term
symtpoms of PAH-3
Definition
SOB
decreased 6 min walking
elevated BNP
Term
what is goal in meters for the 6 min walk tesT?
Definition
380 meters
Term
WHO classificaitons of PAH:
no symtpoms with normal activies
Definition
stage 1
Term
WHO classificaitons of PAH:
symtpoms with strenous activiy w/ slight limiation
Definition
stage 2
Term
WHO classificaitons of PAH:
symtpoms with strenous activity w/ severe limitation
Definition
Stage 3
Term
WHO classificaitons of PAH:
symptoms at rest
Definition
Stage 4
Term
Diagnsosis of PAH-2
Definition
symtpoms
invasive monitoirng (RHC)/swanz Ganz
Term
right heart catheratization has what things on it
Definition
4 ports and a ballon
Term
indications for using Swanz Ganz-3
Definition
cardio/pulm instable
shock
fluids
Term
risks with swanz ganz-6
Definition
infection
arrythmia
thrombus
hemorrhage
pneuothroax
embolus
Term
Range for:
CVP
Definition
2-6
Term
Range for:
PCWP
Definition
4-12
Term
Range for:
PVR
Definition
150-250
Term
Range for:
mPAP
Definition
9-18
Term
pvr is
Definition
pressure right atrium has to overcome
Term
PCWP is-
Definition
left atrial pressur from blaoon
Term
PAH is when
mPAP is
Definition
>25
Term
PAH is when PCWP is
Definition
<15
Term
is PAH when pulmonary vasc resistance is elevated or decreased
Definition
elevated
Term
if PAH pt is positive for vasoreactive testing what is teatment?
Definition
oral CCB
Term
if pt doesnt respond to vasoreactive test and is low risk what is treatment-2
Definition
ERA
PDE-5
Term
if pt doesnt respond to vasoreactive test and is high risk what is treatment
Definition
epoprostenol
treprostinil
Term
if a pt has idiopathic PAH what med is given?
what is the goal?
Definition
warfarin
1.5-2.5
Term
what meds are given for symtpom managment of RV overload?
Definition
diuretics
Term
goal O2 sat is
Definition
90%
Term
what med prevent proliferation and vasoconstriciton
Definition
endothelin recpt antagonists
Term
what meds prevent vasodilation and apoptosis-3
Definition
prostanoids
inhaled PO
PDEi
Term
what test is performed for all PAH cadnidates for long term therapy
Definition
vasodilatory tes (iNO)
Term
a positive response for the vasodilatory test is reduction of mPAP to less than _ w/o a decrease in-
or
-% decrease in PAP and PVR
Definition
40; cardiac output
PAP; PVR
Term
what are the CCBs used-3
Definition
nifedipeine XL
diltiazem
amlodipine
Term
why isnt verapamil used?
Definition
negative ionotropic effects
Term
ET recpt antag causes-
bc ET1- _ causes vasoconstriciton
Definition
vasodilation
A
Term
*ET recpt anatg are used if CCB not indicated
Definition
Term
what do ET recpt antag do?-3
Definition
decrease mPAP
imrpove CI
imrpove WHO class
Term
what are the ET1 A/B anatagonist
how often dosed
Definition
bosentan
BID
Term
what is the ET1-A anatagonist
dosing?
Definition
ambrisentan
QD
Term
what are the black box warnings for ET recpt-2
Definition
hepatox
birth defects
Term
ET recpt antag interact with CYP*
Definition
Term
why are PDE-5 inhibitors useful?
Definition
increase vasodilatory response to NO
Term
Revatio
Definition
sildenafil
Term
Adcirca
Definition
tadalifil
Term
dosing for sildenafil
Definition
20 mg TID
Term
dosing for tadalafil
Definition
40 mg QD
Term
PDE5 inhibitors do what for PAH-4
Definition
increase 6 min walking
increase CI
imrpove WHO class
Term
why are protacyclin analogs useful?
Definition
bc in PAH protacyclins are reduced
Term
what is MOA of prostacyclins?
Definition
vasodilation of pulm and smooth muscles
Term
what are the effects of prostacyclins-3
Definition
imrpove 6 min walk
imrpove survival by 5 yrs
increase CI
Term
what are the prostacyclins-3
Definition
epoprostenol
treprostinil
iloprost
Term
what is cont IV dose of flolan
Definition
1-4 ng/kg/min
Term
which drug needs to be kept on ice at all times?
Definition
flolan
Term
what is the half lfie of flolan?
Definition
6 min
Term
clinical pearls of flolan?
Definition
cant disct!
must have dedicated line
cant admin with other meds
Term
ADEs of flolan-3
Definition
thrombus
headache
vomit
Term
for flolan calc:
come in:
dose:
pump:
Definition
.5 mg
ng/kg/min
mL/hr
Term
what is the dose for remodulin:
Definition
.625 ng/kg/min SQ cont
Term
what is the 1/2 life of remodulin
Definition
3 hrs
Term
IV remodulin has increased risk of-
Definition
gram neg bacteremia
Term
ADEs of remodulin are same as
Definition
flolan
Term
whihc med is an inhaled nebulizer
Definition
iloprost
Term
what is con of iloprost?
Definition
has to be taken 6-9 times daily
Term
look at slide 30 right before to see the combo therapies fro PAH
Definition
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