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Specific PAH treatments target the three pathways (Humbert M. et al,  N Engl J Med 2004;351:1425-36) hat lead to the obliteration of small pulmonary vessels and seek a reduction of PVR by vasodilatation and possibly a vascular de-remodelling that leads to the restoration of cardiac output. In any case, PAH diagnosis must be confirmed by a right heart catheterization before introduction of any of the 4 classes of specific treatment.

• Calcium channel blockers (CCB)
• Endothelin receptor antagonists (ERA)
• Phosphodiesterase type-5 inhibitors
• Prostanoids
  

Combination therapy of two or three different classes is possible and even recommended for PAH patients deteriorating under monotherapy or presenting functional class NYHA IV at the moment of diagnosis. For class NYHA I or II, initial oral therapy should be preferred.