Author Credentials

Danielle Lancaster, Fuad Zeid, MD


Idiopathic Pulmonary Arterial Hypertension, IPAH, primary pulmonary hypertension, PPH


There are several classifications of Pulmonary Hypertension, one of them being Pulmonary Arterial Hypertension (PAH). In cases in which the cause of PAH is unknown, the disease is referred to as Idiopathic Pulmonary Arterial Hypertension (IPAH). IPAH is an extremely rare disease, making it difficult to treat. However, over the past few decades’ treatments have expanded and improved. Treatments commonly used in clinical practice include one or more of the following, calcium channel blockers, prostacyclin derivatives, endothelin receptor antagonists, phosphodiesterase 5 inhibitors, guanylate cyclase stimulators, and in extreme cases lung transplants (single or double) or heart and lung transplants. The aim of this paper is to review the treatments and therapies available for IPAH because new information has been added after FDA approval of new medications. This article also raises ideas of possible future clinical trials to look at how these medications would work in a combination therapy.