Walls of the pulmonary arteries can thicken as the amount of muscle increases in some arteries and scar tissue can form on the artery walls. As the walls thicken and scar, the arteries become increasingly narrow. Blood clots can form in the smaller arteries. These changes make it hard for the heart to push blood through the arteries and into the lungs. Thus, the pressure in the arteries rises. As a result of the heart working harder, the right ventricle becomes strained and weak and heart failure can result. Heart failure is the most common cause of death in people who have PAH.

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Reynaud's phenomenon

Reynaud's phenomenon: reduced blood supply causes discoloration and coldness in the fingers, toes, and occasionally other areas

expand vessels in the lungs and allow blood to move through them with less resistance

anticoagulants to reduce risk from blood clots and, occasionally, chemotherapeutics to reduce abnormal cell growth in the lung tissue

Epoprostenol

Treprostinil

Liprost

may increase the effects of antihypertensives and antiplatelet agents

Bosentan

Amrisentan

Sildenafil (Revatio)

Tadalafil (Adcirca)

Note that the brand names are different!

Revatio

Do not give with PDE5 used for erectile dysfunction

&

Contraindicated with nitrates!

Caution with PDE5 inhibitors anc concurrent Alpha Blocker Therapy:

Tamsulosin (flomax)

Doxazosin (Cardura)