Case of the Month #51 Pulmonary Hypertension

• PAH  – targeted medical therapies 

• Left heart / lung – treat underlying disease 

• CTEPH – anticoagulation, pulmonary endarterectomy, balloon pulmonary angioplasty, targeted therapies  

Target medical therapies include: 

• Prostacyclin pathway (epoprostenol, iloprost, treprostinil, selexipag) 

• Endothelin receptor antagonists (bosentan, ambrisentan, macitentan) 

• Nitric oxide pathway such as PDE-5 inhibitors (sildenafil, tadalafil) and soluble guanylate cyclase stimulator (riociguat) 

Route of administration may be oral, subcutaneous, inhaled or IV. 

Transplant may be considered (lung or combined heart/lung)