Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. In one form of pulmonary hypertension, tiny arteries in the lungs, called pulmonary arterioles, and capillaries become narrowed, blocked or destroyed. This makes it harder for blood to flow through the lungs, and raises pressure within the lungs' arteries. As the pressure builds, the heart's lower right chamber (right ventricle) must work harder to pump blood through the lungs, eventually causing the heart muscle to weaken and fail.
Some forms of pulmonary hypertension are serious conditions that become progressively worse and are sometimes fatal. Although some forms of pulmonary hypertension aren't curable, treatment can help lessen symptoms and improve quality of life.
Causes & treatment
Pulmonary hypertension (PH) can be caused by a variety of factors, and its treatment depends on the specific cause and type of PH. Here are the common causes and treatments:
Causes of Pulmonary Hypertension
- Idiopathic Pulmonary Arterial Hypertension (IPAH): The cause is unknown.
- Heritable Pulmonary Arterial Hypertension: Genetic factors, often mutations in the BMPR2 gene.
- Heart Diseases: Left heart disease can lead to pulmonary hypertension, including conditions like left heart failure, mitral valve disease, or long-standing high blood pressure.
- Lung Diseases: Conditions such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, or long-term exposure to high altitudes.
- Chronic Thromboembolic Pulmonary Hypertension (CTEPH): Caused by chronic blood clots in the lungs.
- Connective Tissue Disorders: Diseases like scleroderma or lupus.
- HIV infection, Liver Disease (Portal Hypertension), and Congenital Heart Disease: These conditions can also lead to pulmonary hypertension.
Treatment of Pulmonary Hypertension
- Medications:
- Vasodilators: Such as epoprostenol, treprostinil, or sildenafil, to widen blood vessels.
- Endothelin Receptor Antagonists (ERAs): Like bosentan or ambrisentan, which block endothelin, a substance that narrows blood vessels.
- Phosphodiesterase-5 Inhibitors: Sildenafil and tadalafil, which help relax lung arteries.
- Soluble Guanylate Cyclase (sGC) Stimulators: Like riociguat, improving the ability of the heart to pump blood.
- Oxygen Therapy: For patients with low oxygen levels.
- Diuretics: To reduce fluid buildup in the body.
- Anticoagulants: To prevent blood clots in certain patients.
- Digoxin: To help the heart beat stronger and pump more blood.
- Surgery and Other Procedures:
- Atrial Septostomy: Creating a connection between the heart's upper chambers to relieve pressure on the right side of the heart.
- Pulmonary Thromboendarterectomy (PTE): For CTEPH, to remove blood clots from the lungs.
- Lung or Heart-Lung Transplant: In severe cases where other treatments haven't worked.
- Lifestyle Changes: Such as staying active, avoiding pregnancy, and dietary changes.
It's crucial for patients with pulmonary hypertension to be closely monitored and treated by a healthcare professional specializing in this condition. The treatment plan often requires adjustments and regular follow-ups to manage the disease effectively.