Understanding Primary Pulmonary Hypertension: Causes, Symptoms, and Treatments

Primary pulmonary hypertension (PPH) is a rare lung condition characterized by the narrowing of blood vessels in the lungs, leading to elevated blood pressure within the pulmonary arteries. These arteries carry oxygen-depleted blood from the heart to the lungs, where gas exchange occurs. If untreated, PPH can result in heart failure. Also called idiopathic pulmonary arterial hypertension, it involves abnormal increases in pulmonary artery pressure, impairing normal lung and heart function.

Although its precise cause remains unknown, some cases are linked to genetic mutations that increase vessel sensitivity, causing narrowing. Symptoms include fatigue, shortness of breath, chest pain, swelling, and bluish lips, which worsen as the disease advances.

Early signs are often subtle and resemble other heart and lung issues, making diagnosis challenging. Medical assessments include chest X-rays, ECG, echocardiogram, pulmonary function tests, lung perfusion scans, cardiac catheterization, and blood tests to confirm the condition.

While primary pulmonary hypertension is incurable, various treatments can alleviate symptoms. These include blood thinners, diuretics, vasodilators, oxygen therapy, and in severe cases, lung or heart-lung transplants.

Overall, prompt diagnosis and management are crucial for improving quality of life and delaying disease progression.