Pulmonary Hypertension: Symptoms, Causes & Treatment

Pulmonary hypertension is a condition in which the blood pressure within the arteries of the lungs becomes abnormally high. Unlike systemic high blood pressure measured in the arm, pulmonary hypertension affects the vessels that carry blood from the heart to the lungs and places significant strain on the right side of the heart.

Pulmonary hypertension is a progressive condition that can range from mild to severe. Early diagnosis and appropriate treatment are essential to slow disease progression and improve quality of life.

How Pulmonary Hypertension Affects the Heart and Lungs

In pulmonary hypertension, the small arteries in the lungs become narrowed, stiffened, or obstructed. This makes it harder for blood to flow through the lungs, increasing resistance in the pulmonary circulation.

As a result, the right ventricle must work harder to pump blood into the lungs. Over time, this extra workload can lead to right-sided heart enlargement and eventual right heart failure if untreated.

What Blood Pressure Levels Mean in Pulmonary Hypertension

Pulmonary hypertension is defined by elevated pressure in the pulmonary arteries, which is measured directly during right heart catheterization.

In adults at rest, pulmonary hypertension is diagnosed when the mean pulmonary artery pressure is greater than 20 mmHg. Additional measurements help determine disease severity and guide treatment decisions, but diagnosis always requires integration of clinical findings, imaging, and invasive pressure measurements.

Echocardiography is commonly used as a screening tool, but it cannot by itself confirm the diagnosis. Definitive diagnosis requires right heart catheterization.

Types of Pulmonary Hypertension

Pulmonary hypertension is classified into several groups based on underlying cause. This classification is important because treatment differs significantly between groups.

Pulmonary Arterial Hypertension (PAH)

This form results from disease of the pulmonary arteries themselves. The vessels become thickened and narrowed, leading to increased pressure. PAH may be idiopathic, inherited, or associated with connective tissue diseases, certain medications, or congenital heart disease.

Pulmonary Hypertension Due to Left Heart Disease

This is the most common type of pulmonary hypertension. It develops as a consequence of conditions affecting the left side of the heart, such as heart failure or valve disease. Elevated pressure on the left side is transmitted backward into the lungs.

Pulmonary Hypertension Due to Lung Disease or Hypoxia

Chronic lung conditions and long-term low oxygen levels can lead to pulmonary vessel changes and increased pressure in the pulmonary circulation.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

This form develops when chronic or unresolved blood clots obstruct pulmonary arteries, leading to persistent pulmonary hypertension. Importantly, CTEPH is potentially curable in selected patients.

Symptoms of Pulmonary Hypertension

Symptoms often develop gradually and may be mistaken for other conditions in early stages.

Common symptoms include:

• Shortness of breath, especially with exertion
• Fatigue and reduced exercise tolerance
• Chest discomfort or pressure
• Dizziness or fainting
• Swelling of the ankles, legs, or abdomen

As the disease progresses, symptoms may occur even at rest.

How Pulmonary Hypertension Is Diagnosed

Diagnosis begins with clinical evaluation and imaging.

Echocardiography is a key screening tool, allowing estimation of pulmonary pressures and assessment of right heart size and function. However, a definitive diagnosis often requires right heart catheterization, which directly measures pressures in the pulmonary arteries.

Additional tests may include lung imaging, pulmonary function tests, blood tests, and evaluation for underlying heart or lung disease to determine the cause.

Treatment Options for Pulmonary Hypertension

Treatment depends on the type of pulmonary hypertension, severity of symptoms, and underlying cause. Management is often coordinated in specialized centers.

General and Supportive Measures

Supportive treatment includes managing fluid balance, maintaining appropriate activity levels, and ensuring adequate oxygenation. Vaccinations and avoidance of high altitudes may be recommended in selected patients.

Targeted Medical Therapy

In certain forms of pulmonary hypertension, particularly pulmonary arterial hypertension, medications that specifically target the pulmonary blood vessels may be used. These therapies aim to relax blood vessels, reduce pressure, and improve exercise capacity.

Treatment regimens are individualized and often involve combination therapy.

Treatment of the Underlying Cause

In pulmonary hypertension related to left heart disease or lung disease, management focuses on treating the underlying condition. Improving heart function or lung health can significantly reduce pulmonary pressures.

In chronic thromboembolic pulmonary hypertension, long-term anticoagulation is essential, and selected patients may benefit from surgical or catheter-based interventions to remove or bypass obstructing clots.

Prognosis and Long-Term Outlook

Pulmonary hypertension is a chronic condition that requires long-term follow-up. Prognosis varies widely depending on the underlying cause, severity, and response to treatment.

With modern therapies and specialized care, many patients experience symptom improvement and stabilization of disease.

When to Seek Medical Attention

Unexplained shortness of breath, fainting, or swelling—especially in individuals with known heart or lung disease—should prompt medical evaluation. Early diagnosis improves outcomes.

In Summary

Pulmonary hypertension is a condition characterized by elevated pressure in the lung arteries, placing strain on the right side of the heart. It has multiple causes and requires careful evaluation to guide treatment. With appropriate diagnosis, targeted therapy, and specialized care, many patients achieve meaningful symptom relief and improved quality of life.

Reference: Pulmonary Hypertension