Pulmonary arterial hypertension Flashcards
what is pulmonary arterial hypertension
- progressive disease involving endothelial dysfunction -> elevated pulmonary arterial pressure and pulmonary vascular resistance
what is the mean pulmonary artery pressure (mPAP) in PAH
> 20 mmHg
what is pulmonary artery wedge pressure (PAWP) in PAH
<= 15 mmHg
what is the pulmonary vascular resistance (PVR) in PAH
> 2 Wood units
what is normal pulmonary arterial wedge pressure
4-12 mmHg
what is the gold standard way to diagnose PAH
right heart catheterization
what is another way other than right heart catheterization to evaluate PAH
echocardiogram
risk factors and associated conditions for PAH
- collagen vascular disease
- congenital heart disease
- portal htn
- HIV infection
- drugs and toxins
- pregnancy
main genetic factor for PAH
abnormal BMPR2 gene
what does PAH endothelial dysfunction cause
- decreased NO2 synthase, prostacyclin production
- increased thromboxane production, endothelin 1 production
what is PAH class 1
symptom free when physically active or resting
what is PAH class 2
- slight limitation of physical activity - ordinary activity may cause Sx
- comfortable at rest
what is PAH class 3
- marked limitation in physical activity - less than ordinary activity causes Sx
- comfortable at rest
what is PAH class 4
- significant Sx w/ activity
- Sx at rest
at what PAH class do we consider starting tx
class 2
PAH goals of therapy
- alleviate Sx
- improve QOL
- prevent or delay disease progression
- reduce hospitalization
- improve survival
what is the result of a positive acute vasoreactivity test (AVT)
drop in mPAP >10 mmHg w/ PAP less than 40 mmHg w/ stable-improved cardiac output
when should CCBs be considered for PAH
- positive responders to CCBs w/o right-sided failure or other CI to CCB
- do not use w/o positive AVT
why should verapamil not be used for PAH
due to negative inotropic effects
what to do if patient does not improve to functional class I or II after CCB initation
start additional or alternative PAH therapy
recommended PAH first line CCBs if patient is a positive responder
- long acting nifedipine 120-240 mg daily
- long acting diltiazem 240-720 mg daily
- amlodipine 20 mg daily
PDE-5i inhibition effects
- decreases conversion of cGMP to GMP
- increased levels of cGMP -> pulmonary vasodilation
PDE-5i meds for PAH
- sildenafil
- tadalfil