Pulmonary Arterial Hypertension: What You Need to Know

When dealing with pulmonary arterial hypertension, a progressive disease that raises blood pressure in the lungs' arteries and strains the heart. Also known as primary pulmonary hypertension, it commonly shows up as shortness of breath, fatigue and fainting during activity. The condition stems from narrowing, thickening or blockage of tiny pulmonary vessels, which forces the right side of the heart to pump harder. Over time, this extra workload can trigger serious complications. Understanding how PAH develops helps you spot warning signs early and seek the right care.

One of the most direct consequences of untreated PAH is right heart failure, a state where the right ventricle cannot effectively pump blood into the lungs. When the right ventricle struggles, fluid may back up into the liver, abdomen and legs, leading to swelling and reduced exercise tolerance. Detecting this progression early relies on reliable diagnostic tests, such as echocardiography, cardiac MRI and right‑heart catheterisation, which measure pressure, flow and vessel resistance. These tools not only confirm the diagnosis but also guide treatment intensity. In parallel, modern drug therapy often starts with endothelin receptor antagonists, medications that block endothelin‑1, a powerful vasoconstrictor that narrows pulmonary arteries. By lowering vessel tone, these drugs can improve exercise capacity and delay right‑ventricular decline.

Beyond endothelin blockade, clinicians frequently add agents that raise cyclic GMP levels, such as phosphodiesterase‑5 inhibitors, or mimic prostacyclin, a natural vasodilator. Prostacyclin therapy, delivered via injection, inhalation or oral formulations, directly relaxes pulmonary arteries and inhibits platelet aggregation. When used in combination, these classes address different pathways that drive PAH, offering a synergistic effect that many patients need to stay stable. Lifestyle tweaks—regular, low‑impact exercise, sodium‑restricted diet and avoiding high‑altitude exposure—support medical treatment and help maintain right‑heart function. Monitoring biomarkers like NT‑proBNP, along with periodic imaging, lets doctors fine‑tune the regimen before symptoms worsen.

Below you’ll find a curated selection of articles that break down each of these topics in plain language. We cover how to recognise early signs, what each diagnostic test looks for, step‑by‑step guides for the most common medication classes, and practical advice on managing daily life with PAH. Whether you’re just hearing about the disease or you’re already on a treatment plan, the resources here aim to give you clear, actionable information you can trust.