Pulmonary Arterial Hypertension (PAH): What You Need to Know

Shortness of breath, low energy, and swelling in the legs can feel vague — but they can also be early signs of pulmonary arterial hypertension (PAH). PAH means the pressure in the blood vessels that feed your lungs is too high. That extra strain makes the right side of the heart work harder and, over time, can lead to serious problems if not treated.

PAH is different from common lung diseases. Your lungs might be healthy, but the small arteries that carry blood through the lungs are narrow, stiff, or blocked. That raises resistance and makes the heart pump harder to push blood through. You don’t have to memorize the biology, but you should know how PAH commonly shows up and what to expect from testing and care.

Common signs to watch for include: shortness of breath with activity, feeling unusually tired, fainting or near-fainting during exertion, chest tightness or pain, and swelling in the ankles or belly. Symptoms can start slowly and people often attribute them to aging or poor fitness. If symptoms get worse over weeks to months, talk with your doctor about PAH as a possible cause.

How PAH is diagnosed

Start with a clear history and a physical exam. Your doctor will likely order an echocardiogram first — it’s an ultrasound that estimates pressure in the pulmonary arteries and shows how the right heart is doing. If the echo suggests PAH, the gold standard test is right heart catheterization. That procedure measures pressures directly and confirms the diagnosis. Other useful tests include a 6-minute walk test to measure functional capacity, BNP/NT-proBNP blood tests to check heart strain, chest CT or V/Q scan to rule out other causes, and basic blood work to look for related conditions like connective tissue disease or liver disease.

Treatment options and everyday tips

Treatment aims to lower pulmonary pressure, improve symptoms, and slow progression. Several drug classes are used: endothelin receptor antagonists (help open vessels), PDE5 inhibitors (improve blood flow), prostacyclin therapies (strong vasodilators that can be IV, inhaled, or oral), and soluble guanylate cyclase stimulators. Depending on your case, doctors may add diuretics for fluid, oxygen if you’re low at rest or with activity, or blood thinners in select patients. Some people need referral to a specialized pulmonary hypertension center for advanced therapies or transplant evaluation.

Practical tips: keep a daily symptom log (breathlessness, energy, weight, swelling), take meds exactly as prescribed, stay up to date on flu and pneumonia vaccines, pace activity and build gentle exercise as advised by your care team, and avoid pregnancy unless supervised closely (pregnancy can be high risk with PAH). Ask about clinical trials if standard options aren’t working.

Call your provider or go to emergency care for sudden worsening breathlessness, fainting, chest pain, or rapid swelling. PAH is manageable when caught early and treated by a team that knows the condition. If you suspect PAH, push for the right tests and a specialist referral — small steps now can make a big difference later.