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Pulmonary Hypertension: Causes, Symptoms and Treatment

Pulmonary hypertension is abnormally high blood pressure in the blood vessels of the lungs. It strains the right side of the heart and can cause breathlessness.

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Things worth knowing about "Pulmonary Hypertension"

Pulmonary hypertension is abnormally high blood pressure in the blood vessels of the lungs. It strains the right side of the heart and can cause breathlessness.

What is Pulmonary Hypertension?

Pulmonary hypertension (PH) is a condition characterised by abnormally elevated blood pressure in the pulmonary arteries – the blood vessels that carry blood from the heart to the lungs. Under normal circumstances, blood pressure in the pulmonary circulation is significantly lower than in the systemic circulation. When resting mean pulmonary arterial pressure persistently exceeds 20 mmHg, pulmonary hypertension is diagnosed. As a result, the right ventricle of the heart must work harder to pump blood through the narrowed vessels, which can ultimately lead to right heart failure.

Causes

Pulmonary hypertension is not a single disease but an umbrella term covering several distinct conditions. The World Health Organization (WHO) classifies PH into five groups:

Group 1 – Pulmonary Arterial Hypertension (PAH): Idiopathic (no known cause), heritable, or triggered by connective tissue diseases, HIV infection, or certain drugs.
Group 2 – Left Heart Disease: The most common cause; heart failure or valvular heart disease raises back-pressure in the pulmonary vessels.
Group 3 – Lung Disease or Hypoxia: Chronic obstructive pulmonary disease (COPD), pulmonary fibrosis, or sleep apnoea can lead to PH.
Group 4 – Chronic Thromboembolic PH (CTEPH): Caused by unresolved blood clots in the pulmonary arteries.
Group 5 – Unclear or Multifactorial Mechanisms: Associated with blood disorders, metabolic conditions, or sarcoidosis.

Symptoms

The symptoms of pulmonary hypertension often develop gradually and are frequently mistaken for general fatigue or common respiratory problems:

Exertional dyspnoea: Shortness of breath during physical activity, progressing to breathlessness at rest in advanced stages
Fatigue and general weakness
Dizziness or fainting episodes (syncope)
Chest pain or a feeling of pressure in the chest
Oedema: Swelling of the legs, ankles, or abdomen due to fluid retention
Palpitations: Rapid or irregular heartbeat
Cyanosis: Bluish discolouration of the lips or fingernails due to low oxygen levels

Diagnosis

Because the symptoms are non-specific, diagnosis is often delayed. The following investigations are typically used:

Echocardiography: Ultrasound of the heart to estimate pulmonary arterial pressure; usually the first diagnostic step
Right Heart Catheterisation: The gold standard for definitive pressure measurement in the pulmonary circulation
6-Minute Walk Test: Assesses functional exercise capacity
CT Pulmonary Angiography and Ventilation-Perfusion (V/Q) Scan: Used to exclude CTEPH
Pulmonary Function Tests and arterial blood gas analysis
Blood Tests: Including BNP or NT-proBNP as markers of cardiac stress

Treatment

Treatment depends on the underlying cause and the severity of the condition.

General Measures

Supervised, adapted physical activity and pulmonary rehabilitation
Supplemental oxygen therapy when blood oxygen levels are low
Anticoagulation for patients with CTEPH

Drug Therapy (Primarily for PAH)

Endothelin Receptor Antagonists (e.g., bosentan, ambrisentan): Dilate blood vessels and slow vascular remodelling
Phosphodiesterase-5 Inhibitors (e.g., sildenafil, tadalafil): Promote vasodilation via the nitric oxide pathway
Prostacyclin Analogues (e.g., iloprost, epoprostenol): Potent vasodilators with antiproliferative effects
Soluble Guanylate Cyclase (sGC) Stimulators (e.g., riociguat): Act on the nitric oxide signalling pathway

Interventional and Surgical Therapy

Pulmonary Endarterectomy (PEA): Surgical removal of chronic clot material in CTEPH; potentially curative
Balloon Pulmonary Angioplasty (BPA): Catheter-based procedure for inoperable CTEPH patients
Lung Transplantation: Reserved for patients with severe, therapy-refractory PH

Prognosis and Outlook

Pulmonary hypertension is a chronic and progressive disease. With modern, targeted therapies, disease progression can be significantly slowed. Early diagnosis and treatment at a specialised centre greatly improves quality of life and long-term survival for those affected.

References

Humbert M. et al. - 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. European Heart Journal (2022).
World Health Organization (WHO) - Pulmonary Hypertension: Classification and Management. WHO Technical Report (2023).
Galiè N. et al. - Updated Treatment Algorithm of Pulmonary Arterial Hypertension. Journal of the American College of Cardiology (2019).

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