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DrugClinical courseStable
Physical examination No evidence of heart failure
Functional class I/II
6MWD Greater than 400 m
EchocardiogramRV size normal
HemodynamicsRAP normal
CI normal
BNPNear normal or stable
TreatmentOral therapy
Frequency of evaluation Q 3 to Q 6 months
FC assessment Every clinic visit
6MWT Every clinic visit
Echocardiogram2Q 12 months/center dependent
BNPcenter dependent
RHCClinical deterioration and center dependent


DrugClinical courseUnstable
Physical examination Signs of heart failure
Functional class IV
6MWD Less than 400 m
EchocardiogramRV Enlargement
HemodynamicsRAP high
CI low
BNPElevated/Increasing
TreatmentIntravenous prostacyclin and/or combination treatment
Frequency of evaluation Q 1 to Q 3 months
FC assessment Every clinic visit
6MWT Every clinic visit
Echocardiogram2Q 6 to Q 12 months/center dependent
BNPcenter dependent
RHCQ 6 to Q 12 months or clinical deterioration