PAH Flashcards
How does PAH work?
- pulmonary arterioles narrow
- RV dilates
- pulmonary edema and damage
- thrombi and/or plexiform lesion formation
PH vs. PAH (differences)
PH: MPAP > equal to 20 at rest; more common
PAH: progressive with endothelial dysfunction –> elevated pulmonary arterial pressure and pulmonary vascular resistance; rare
what are the WHO PH classifications?
Group 1: PAH
Group 2: LFH
Group 3: LD
Group 4: Chronic thromboembolic pulmonary HTN
Group 5: Pulmonary HTN resulting from unclear mechanisms
what are the signs and symptoms of PAH?
early –> present as non-specific resulting in a large differential diagnosis
late –> signs of right-sided HF
what is the diagnosis of PAH?
- echocardiogram
–> evaluate potential causes, RV function, estimate PAP and PVR - right heart catheterization
–> assess response to pulmonary vasodilators before starting therapy - exercise testing
–> distance walked in 6 min - biomarkers
–> BP & NTproBNP
what are the hemodynamic definitions of PAH?
mPAP > 20
PAWP < or equal to 15
PVR > 2 wood units
what is the PAWP?
- estimates left arterial pressure
- normal 4-12
- elevated #s signal LV failure or mitral stenosis
what is the PVR?
calculated using formula based on mPAP and PAWP
what are the WHO functional classifications?
- WHO FC1
- WHO FC2
- WHO FC3
- WHO FC 4
What is the WHO FC1 classifications?
- no limit of activity
- ordinary activity does NOT cause any chest pain, dyspnea, ect.
What is the WHO FC2 classifications?
- slight limit of activity
- comfortable at rest but cause dyspnea, chest pain, ect
What is the WHO FC3 classifications?
- marked limitation of activity
- comfortable at rest and less than ordinary dyspnea, chest pain, ect.
What is the WHO FC4 classifications?
- cant do anything
what are the guidelines of treatment with PAH pts without comorbidities?
RISK 3
if low or interm risk
– initial ERA + PDE5i therapy
if high risk
– initial ERA + PDE5i + PCA
RISK 4
if low risk
– continue initial therapy
if interm-low risk
– add PRA OR
– switch from PDE5i to sGCs
if high or interm-high risk
– add iv or sc PCA and/or evaluate lung transplant
what are the guidelines of treatment with PAH pts with comorbidities?
- initial oral monotherapy with PDE5i or ERA