General measures and supportive therapy

Supervised exercise training (I-A)
Psychosocial support (I-C)
Avoid strenuous physical activity (I-C)
Avoid pregancy (I-C)
Influcenza and pneumoccoal immunization (I-C)

Export referral (I-C)

Oral anticoagulants:

IPAH, heritable PAH, and PAH due to anorexigenes (IIa-C)
Diuretics (I-C)
Oxygen (I-C)
Digoxin (IIb-C)

Oral anticoagulants:

IPAH, heritable PAH, and PAH due to anorexigenes (IIa-C)
Diuretics (I-C)
Oxygen (I-C)
Digoxin (IIb-C)

End

WHO-FC-I-III
CCB (I-C)

Contiune CCB

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Sustained response (WHO-FC-I-II)

Yes

No

Acute vasoreactivity test (I-C for IPAH) (IIb-C for APAH)

VASOREACTIVE

NONVASOREACTIVE

REFERRAL FOR LUNG TRANSPLANTATION (I-C)

INADEQUATE CLINICAL RESPONSE ON MAXIMAL THERAPY

INADEQUATE CLINICAL RESPONSE

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CONSIDER ELIGIBILITY FOR LUNG TRANSPLANTATION

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BAS (IIa-C)

REFERRAL FOR LUNG TRANSPLANTATION (I-C)