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Pulmonary Hypertension

Pulmonary hypertension (PH) is high blood pressure in the arteries of the lungs. It is different from systemic hypertension (the blood pressure measured with a cuff on your arm). PH can be caused by many conditions, and all forms are serious.[1]

Common symptoms

  • Shortness of breath, especially with activity
  • Fatigue and weakness
  • Dizziness or lightheadedness
  • Chest pain or pressure, particularly during exertion
  • Swelling of the ankles, legs, or abdomen (edema)
  • Fainting (syncope), especially with exertion
  • Racing heartbeat or palpitations

Symptoms may be absent early on and develop gradually as pulmonary pressures rise and the right side of the heart is affected.[1]

Groups of pulmonary hypertension

PH is classified into five groups based on the underlying cause:[1]

  • Group 1 — Pulmonary arterial hypertension (PAH): Disease of the small pulmonary arteries. Includes idiopathic, heritable, drug-associated, and connective tissue disease-associated forms.
  • Group 2 — PH due to left heart disease: The most common group. Caused by heart failure, valve disease, or cardiomyopathy.
  • Group 3 — PH due to lung disease or hypoxia: Associated with COPD, interstitial lung disease, sleep-disordered breathing, or chronic altitude exposure.
  • Group 4 — Chronic thromboembolic PH (CTEPH): Caused by blood clots in the pulmonary arteries that the body does not fully dissolve.
  • Group 5 — PH with unclear or multifactorial causes: Related to sarcoidosis, blood disorders, metabolic conditions, or other systemic diseases.
Evaluation

How is pulmonary hypertension diagnosed?

PH can be difficult to diagnose because early symptoms overlap with many other conditions. Evaluation by a team with expertise in PH is important.[1]

Initial tests
  • Echocardiogram: An ultrasound of the heart that estimates pulmonary artery pressures and evaluates the size and function of the right side of the heart. Often the first test used to screen for PH.[1]
  • Pulmonary function tests: To evaluate for lung disease (COPD, interstitial lung disease) as a cause of PH.
  • Chest X-ray and CT scan: To look at the lungs and heart. CT pulmonary angiography can evaluate for CTEPH.
  • Blood tests: Including BNP/NT-proBNP (heart strain marker), autoimmune panels, liver and kidney function, thyroid function, and HIV testing.
  • 6-minute walk test: A simple measure of exercise capacity used to assess severity and track changes over time.
  • Ventilation-perfusion (V/Q) scan: The best screening test for CTEPH (Group 4). Evaluates blood flow patterns in the lungs.[1]
Confirmatory testing
  • Right heart catheterization: The gold standard for diagnosing PH. A thin catheter is guided through a vein into the right side of the heart and pulmonary arteries to directly measure pressures. PH is defined as a mean pulmonary arterial pressure greater than 20 mmHg at rest.[1]
  • Vasoreactivity testing: During right heart catheterization, an inhaled vasodilator may be given to test whether the pulmonary arteries respond. A positive response in PAH patients may allow treatment with calcium channel blockers.[1]
  • Cardiopulmonary exercise testing (CPET): May be used to evaluate unexplained dyspnea and distinguish cardiac from pulmonary causes of exercise limitation. Learn more about CPET →
  • Cardiac MRI: Provides detailed assessment of right ventricular size, function, and blood flow.
Treatment

How is pulmonary hypertension treated?

Treatment depends on the group and underlying cause. All patients with PH benefit from care at a specialized center with PH expertise.[1]

PAH-specific therapies (Group 1)

  • Sotatercept (Winrevair): A first-in-class activin signaling inhibitor, FDA-approved in March 2024 for PAH. Works by rebalancing pro- and anti-proliferative signaling pathways involved in pulmonary vascular remodeling. In the STELLAR trial, sotatercept added to background therapy improved 6-minute walk distance by 41 meters and reduced the risk of clinical worsening events by 84% compared to placebo.[2] The ZENITH trial demonstrated a 76% reduction in major morbidity and mortality in patients with advanced PAH (WHO FC III/IV) at high risk of death, leading to early trial termination for efficacy.[3] Administered as a subcutaneous injection every 3 weeks.
  • Endothelin receptor antagonists (ERA): Oral medications (e.g., ambrisentan, bosentan, macitentan) that block a substance that narrows blood vessels.[1]
  • Phosphodiesterase-5 inhibitors (PDE5i): Oral medications (e.g., sildenafil, tadalafil) that relax pulmonary arteries.[1]
  • Soluble guanylate cyclase stimulators: Oral medication (riociguat) that enhances a natural pathway to dilate blood vessels. Also approved for CTEPH.[1]
  • Prostacyclin pathway agents: Inhaled (iloprost, treprostinil), oral (selexipag, treprostinil), subcutaneous, or intravenous (epoprostenol, treprostinil) medications for more advanced disease.[1]
  • Combination therapy: Current guidelines recommend initial combination therapy for most newly diagnosed PAH patients, with additional agents added based on risk assessment.[1]

Other treatments

  • Treating the underlying cause: In Group 2 (heart disease) and Group 3 (lung disease), treating the primary condition is the focus. PAH-specific medications are generally not recommended for these groups.[1]
  • Pulmonary endarterectomy (PEA): Surgery to remove blood clots from the pulmonary arteries. The treatment of choice for eligible CTEPH patients (Group 4).[1]
  • Balloon pulmonary angioplasty (BPA): A catheter-based procedure to open blocked pulmonary arteries in CTEPH patients who are not surgical candidates.[1]
  • Supplemental oxygen: When oxygen levels are low.
  • Diuretics: To manage fluid retention and swelling.
  • Supervised exercise and rehabilitation: Improves exercise capacity and quality of life when done in experienced centers.[1] Learn more →
  • Lung transplantation: Considered for eligible patients with advanced PAH who do not respond adequately to medical therapy.

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References

  1. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-3731. doi:10.1093/eurheartj/ehac237
  2. Hoeper MM, Badesch DB, Ghofrani HA, et al. Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension. N Engl J Med. 2023;388(16):1478-1490. doi:10.1056/NEJMoa2213558
  3. Humbert M, McLaughlin VV, Badesch DB, et al. Sotatercept in patients with pulmonary arterial hypertension at high risk for death. N Engl J Med. 2025;392(20):1987-2000. doi:10.1056/NEJMoa2415160