Pulmonary hypertension (PH) is high blood pressure in the arteries of the lungs, which makes the right side of the heart work harder than normal. Over time, this increased strain can lead to right-sided heart failure. PH is a progressive and potentially life-threatening condition that requires early diagnosis and expert care to manage symptoms and slow its progression.

There are several types of pulmonary hypertension, including:

• Pulmonary Arterial Hypertension (PAH): This type occurs when the small arteries in the lungs become narrowed or damaged, making it harder for blood to flow through. PAH can be idiopathic (no known cause) or associated with other conditions like connective tissue diseases, congenital heart disease, or certain medications.
• PH Due to Left Heart Disease: This is the most common form of PH and results from problems with the left side of the heart — such as heart failure or valve disease — causing blood to back up into the lungs and raise pressure in the pulmonary arteries.
• PH Due to Lung Disease or Hypoxia (PH-ILD): This type is caused by chronic lung diseases like COPD, interstitial lung disease (PH-ILD) or sleep apnea. Damage to the lungs or low oxygen levels over time can increase pressure in the lung arteries.
• Chronic Thromboembolic Pulmonary Hypertension (CTEPH): CTEPH occurs when old blood clots don’t fully dissolve and block or narrow the pulmonary arteries.
• PH with Unclear or Multifactorial Causes: This group includes PH that doesn’t fit neatly into the other categories and may involve multiple underlying conditions such as blood disorders, sarcoidosis, or kidney disease.

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Signs & Symptoms

Shortness of breath or lightheadedness during activity is often the first symptom of pulmonary hypertension. Heart palpitations may also be present.

Other symptoms include:

• Ankle and leg swelling
• Bluish color of the lips or skin
• Chest pain or pressure, usually in the front of the chest
• Dizziness or fainting spells
• Fatigue
• Increased abdomen size
• Weakness

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Diagnosis

In order to diagnose pulmonary hypertension, a Deborah Heart and Lung Center physician may order the following tests:

• Blood tests
• Cardiac catheterization
• Chest x-ray
• CT scan of the chest
• Echocardiogram
• ECG
• Lung function tests
• Nuclear lung scan
• Pulmonary arteriogram
• 6-minute walk test
• Sleep study
• Tests to check for autoimmune problems