Pulmonary Arterial Hypertension: What You Need to Know

When dealing with Pulmonary Arterial Hypertension, a progressive disease where the lung arteries tighten and thicken, raising pressure and overloading the heart. Also known as PAH, it often slips under the radar until symptoms worsen, making early recognition crucial.

Key Concepts and Treatments

One of the first complications of Pulmonary Arterial Hypertension is Right Heart Failure, the inability of the right ventricle to pump blood against high pulmonary pressures. This link explains why doctors monitor heart function closely and why therapy aims to lower the pressure in the lungs, easing the strain on the right side of the heart.

Modern medicine attacks the disease from several angles. Endothelin Receptor Antagonist, drugs that block the vasoconstrictor endothelin‑1, helping vessels relax and reducing pressure, are often first‑line. Another powerful class is the Prostacyclin Analog, synthetic versions of prostacyclin that dilate vessels and inhibit clot formation. Both classes can be combined with a phosphodiesterase‑5 inhibitor (like sildenafil) to boost nitric‑oxide signaling and further lower vascular resistance.

Accurate diagnosis hinges on measuring Pulmonary Vascular Resistance, the pressure the right ventricle must overcome to push blood through the lungs, typically via right‑heart catheterization. Non‑invasive tools such as echocardiography and biomarkers like BNP give early clues, but the catheter study confirms the diagnosis and guides therapy intensity.

Because PAH progresses in stages, clinicians use the WHO functional class system to gauge severity—from Class I (no limitation) to Class IV (symptoms at rest). Treatment intensity escalates with each class, often moving from single‑agent therapy to combination regimens that target endothelin, nitric‑oxide, and prostacyclin pathways simultaneously. Lifestyle tweaks—regular low‑intensity exercise, sodium restriction, and careful pregnancy planning—also matter.

Below you’ll find a curated set of articles that dive deep into the drugs mentioned, compare side‑effect profiles, and explain how to choose the right regimen for different disease stages. Whether you’re starting a new prescription, switching therapies, or just want to understand the science behind each option, the collection provides practical guidance to help you manage PAH effectively.