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Pulmonary Arterial Hypertension (PAH)

How is PAH diagnosed?
PAH is diagnosed through a combination of tests, as determined by your doctor. While other tests are available, the following list outlines the primary ones:

• Right-heart catheterization—the gold standard for diagnosis
• ECHO
• Six-minute walk
• Pulmonary function tests
• ECG
• Chest radiograph
• Pulmonary angiogram
• Serologies
• Liver function tests; liver ultrasound
• HIV/hepatitis/anorexic agents
• Thyroid function tests
• Collagen vascular evaluation
• Vascular studies
• Arterial blood gases
• Lung perfusion scans (VQ)

What are my treatment options? 


Ten drugs are currently available to treat PAH. To keep informed about your treatment options, have your doctor review each one with you in detail.

• Veletri ® (epoprostenol for injection) [link to http://www.veletri.com/]
• Epoprostenol (generic IV) (epoprostenol)
• Flolan ® (IV) (epoprostenol)
• Remodulin ® (IV or subcutaneous) (treprostinil)
• Ventavis ® (inhaled) (iloprost)
• Tyvaso ® (inhaled) (treprostinil)
• Adcirca ® (oral) (tadalafil)
• Tracleer ® (oral) (bosentan)
• Revatio ® (oral) (sildenafil)
• Letairis ® (oral) (ambrisentan)

What are typical PAH symptoms?


• Breathlessness or shortness of breath
• Feeling tired all the time
• Dizziness, especially when standing up or climbing hills or stairs
• Fainting
• Edema or swelling in the legs and ankles
• Chest pains

Does PAH run in families? 
Yes. About 6 to 10 percent of cases are familial. Therefore, if you have been diagnosed as having PAH, then all your immediate relatives (parents, siblings, and children) should be screened for it, as well. 1

Can a baby have PAH? 
Yes, babies can be born with PAH. Typical symptoms include:

• Slow weight gain
• Lethargy
• Labored breathing
• Poor appetite
• Sweating
• Irregular heartbeat
• Shortness of breath when active
• Nausea or vomiting
• Dizziness or fainting spells
• Bluish skin
• Chest pains

1. Pulmonary Hypertension Association. Pulmonary Hypertension: A Patient’s Survival Guide. 3rd ed. Silver Spring, MD: Pulmonary Hypertension Association; 2004.


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