What causes PAH?

A number of things can cause PAH. These include inherited genetic conditions, other diseases, and outside factors. There are also cases where the cause of PAH is not known.

PAH CauseDescription
Idiopathic PAH (IPAH)1Idiopathic means the cause is not known
Familial or heritable PAH (HPAH)1This means the disease runs in a family
Connective tissue disorders (CTD)1PAH associated with CTD
Congenital heart disease (CHD)1PAH associated with CHD with repaired shunts
Other causes1PAH associated with other diseases and conditions

The 3 pathways that PAH treatments target2

Blood vessels in your lungs may be affected when there is either too much or too little of certain natural chemicals:

  • Endothelin
  • Nitric oxide
  • Prostacyclin

There are PAH treatments available that target each of these pathways:

About Uptravi PAH Treatment Target Pathways

How UPTRAVI works

UPTRAVI targets the prostacyclin pathway. Your body may not make enough of the chemical prostacyclin. This can cause the blood vessels in your lungs to narrow.