Pulmonary Arterial Hypertension (PAH)

It is considered a rare disorder, affecting 15-50 per million people in the United States and Europe. PAH usually affects Caucasian females in their 30s.

PAH is further classified by the severity of the disease as:

• Mild PAH, characterized by thickening and stiffening of the arterial walls, which results in increased resistance.
• Moderate PAH, characterized by narrow arteries which restrict blood flow.
• Severe PAH with abnormal vascular lesions and thrombus formation.

What Causes Pulmonary Arterial Hypertension?

Pulmonary arterial hypertension is a complex disease caused by the interaction of a number of factors.

Most cases of PAH are idiopathic, with no known cause. However, there are several additional etiologies associated with PAH:

• Hereditary causes, including a mutation in the bone morphogenic protein receptor 2 (BMPR2).
• Drugs and toxin exposure, such as amphetamines, methamphetamine, and dasatinib.
• Chronic diseases, such as connective tissue diseases, HIV infection, Portal hypertension, congenital heart diseases, and schistosomiasis.

Signs and Symptoms of Pulmonary Arterial Hypertension

• Dyspnea on exertion, and eventually at rest
• Fatigue or weakness
• Chest pain
• Dizziness or syncope
• Lower extremity edema

If left untreated, PAH will lead to right-sided heart failure.