What is pulmonary hypertension?
Pulmonary hypertension is high blood pressure in the arteries of the lungs. It makes it harder for the heart to pump blood through the lungs, leading to strain on the right side of the heart. Normally, the right side of the heart pumps blood to the lungs, where it picks up oxygen before flowing to the rest of the body. In pulmonary hypertension, the small arteries in the lungs become narrow, reducing blood flow. As a result, pressure builds up in these arteries, forcing the heart to work harder. Eventually, this extra strain can cause the right side of the heart to enlarge and weaken, leading to right-sided heart failure (also known as cor pulmonale).
At Penn Medicine, specialists in pulmonary vascular disease care are among the most experienced in the nation, providing expert diagnosis, evaluation, and treatment for pulmonary hypertension and related conditions. With the proper treatment, we can help you manage symptoms and reduce the risk of serious complications.
What are the symptoms of pulmonary hypertension?
The first symptom of pulmonary hypertension is often shortness of breath or lightheadedness during physical activity. As the condition progresses, symptoms may occur with lighter activity or even at rest. A rapid heart rate (palpitations) is also common. Other symptoms include:
- Swelling in the ankles and legs
- Bluish color of the lips or skin (cyanosis)
- Chest pain or pressure, usually in the front of the chest
- Dizziness or fainting spells
- Fatigue
- Increased abdominal size
- Weakness
People with pulmonary hypertension often experience good days and bad days, with symptoms that come and go.
What are the causes of pulmonary hypertension?
Pulmonary hypertension can develop due to various underlying conditions or may have no known cause.
- Heart-related causes: Some cases of pulmonary hypertension are linked to heart conditions that make it harder for blood to flow through the lungs. These include birth defects of the heart, heart failure (affecting the left side of the heart), and heart valve disease.
- Lung-related causes: Chronic lung diseases such as COPD, pulmonary fibrosis, and other severe lung conditions can lead to pulmonary hypertension by reducing oxygen levels and increasing pressure in the lung’s arteries. Obstructive sleep apnea and long-term low oxygen levels in the blood (chronic hypoxia) can also contribute.
- Blood vessel-related causes: Chronic thromboembolic pulmonary hypertension (CTEPH) occurs when blood clots in the lungs (pulmonary embolism) fail to dissolve over time. This leads to scar tissue and narrowed blood vessels, making it harder for blood to pass through. Autoimmune diseases such as scleroderma and rheumatoid arthritis can also damage lung blood vessels, leading to pulmonary hypertension.
- Other causes: Certain infections, such as HIV, and some medications (including specific diet drugs) can contribute to the condition.
When pulmonary hypertension results from another medical condition or medication, it’s called secondary pulmonary hypertension. However, in some cases, no clear cause is found. This is known as idiopathic pulmonary arterial hypertension (IPAH), which is more common in people assigned female at birth.
Diagnosing pulmonary hypertension
Pulmonary hypertension is diagnosed through a combination of medical history, physical examination, and diagnostic tests. Imaging tests help assess the heart and lungs, while right heart catheterization is the definitive test to measure blood pressure in the lung arteries. Additional tests, including blood work and lung function tests, may help identify underlying causes.
Treatment for pulmonary hypertension
Treatment for pulmonary hypertension depends on the underlying cause and severity of the condition. Medications, including oral, inhaled, and intravenous options, help relax blood vessels, reduce pressure, and improve heart function. Pulmonary rehabilitation manages symptoms by improving exercise tolerance, breathing efficiency, and overall quality of life through supervised exercise and education. For people with CTEPH, surgery may be performed to remove blockages and improve blood flow.
As the disease progresses, our multispecialty heart failure and advanced lung disease teams provide ongoing care, including mechanical circulatory support when needed. In severe cases, we collaborate with heart and lung transplant specialists to evaluate transplant options.
Preventing pulmonary hypertension
While pulmonary hypertension isn’t always preventable, certain lifestyle choices can help manage the condition and reduce risks. Controlling underlying health conditions, such as heart or lung disease or autoimmune disorders, can prevent complications that contribute to high blood pressure in the lungs. Maintaining a healthy weight and following a heart-healthy diet can also support overall cardiovascular function.
Regular exercise under the guidance of your health care provider can help improve circulation and lung capacity. Avoiding smoking and limiting alcohol consumption can protect lung and heart health, reducing strain on the pulmonary arteries. Additionally, managing stress through relaxation techniques, therapy, or mindfulness can help keep blood pressure levels stable.
Trusted partners in your pulmonary health
Penn Medicine’s pulmonology team is at the forefront of research and patient care, offering treatments to improve your pulmonary hypertension and even reverse some of the damage to the lungs and heart. When you need the most advanced specialized care, the Harron Lung Center provides therapies not widely available elsewhere.
Successfully treating pulmonary hypertension is a team effort, requiring an experienced group that can develop the right plan for you. We are a Pulmonary Hypertension Association-accredited Center of Comprehensive Care, recognized for our expertise in the treatment of this complex condition. Our pulmonary specialists, hypertension physicians, thoracic surgeons, radiologists, and lung pathologists are among the most skilled and experienced in the southeastern Pennsylvania and New Jersey regions.