Pulmonary Hypertension: Key Information

Pulmonary hypertension (PH) is a condition where the blood pressure in the pulmonary arteries (the vessels that carry blood from the heart to the lungs) becomes abnormally high. This puts strain on the right side of the heart and can lead to heart failure if left untreated.

Key Points:

Causes:

• Primary Pulmonary Hypertension: Also known as idiopathic pulmonary arterial hypertension (IPAH), the cause is unknown.
• Secondary Pulmonary Hypertension: This occurs as a result of another condition, such as:
  • Left-sided heart disease (heart failure or mitral valve disease).
  • Chronic lung diseases (e.g., chronic obstructive pulmonary disease (COPD), interstitial lung disease).
  • Pulmonary embolism (blood clots in the lungs).
  • Sleep apnea: Can contribute to PH by affecting oxygen levels and heart function.
  • Congenital heart defects: Some heart defects present from birth can lead to PH.
  • Connective tissue diseases (e.g., scleroderma, lupus).

Symptoms:

• Shortness of breath (especially during physical activity).
• Fatigue or tiredness.
• Chest pain or discomfort.
• Swelling in the ankles, legs, or abdomen (due to fluid retention).
• Fainting (syncope), especially during physical exertion.
• Palpitations or irregular heartbeats.

Diagnosis:

• Echocardiogram: The primary diagnostic tool to estimate the pressure in the pulmonary arteries and evaluate heart function.
• Right heart catheterization: The gold standard for confirming pulmonary hypertension, where a catheter is inserted into the heart to directly measure pulmonary artery pressures.
• Chest X-ray and ECG: These may show signs of heart enlargement or strain.
• Pulmonary function tests: To evaluate lung function and identify contributing lung conditions.

Treatment:

• Medications: To lower pulmonary artery pressure, such as:
  • Vasodilators (e.g., prostacyclins, endothelin receptor antagonists, PDE5 inhibitors).
  • Oxygen therapy: For patients with low oxygen levels.
  • Diuretics: To manage fluid retention.
  • Anticoagulants: To prevent blood clots.
• Lifestyle changes: Including regular light exercise, maintaining a healthy weight, and avoiding smoking.
• Surgical treatments: In severe cases, options like lung transplantation or a heart-lung transplant may be considered.

Complications:

• Right heart failure: As the right side of the heart works harder to pump blood through the lungs, it can become weakened and fail.
• Blood clots: Increased risk of clots due to slow blood flow.
• Stroke: Due to blood clots that can travel to the brain.
• Lung complications: In severe cases, can lead to further lung issues or inadequate oxygen exchange.

Pulmonary hypertension is a serious condition that can severely affect quality of life and life expectancy if not treated. Early detection and appropriate management can improve symptoms and outcomes.