The PH Diagnostic Pathway

Getting the right PH diagnosis can take time – here’s how the process works and why early testing matters.

Why PH Diagnosis can be challenging

Pulmonary Hypertension (PH) often begins with symptoms like breathlessness or fatigue — signs that are common in many other conditions.¹ Because of this, it can take a year before receiving an accurate diagnosis, with some people waiting over two years before being accurately diagnosed with PH.² Some physicians may not be familiar with the condition, some patients are misdiagnosed repeatedly and others may have to wait to see a PH specialist for appropriate testing.²

Understanding the diagnostic steps can help you know what to expect and why certain tests are needed.

Step 1: Initial assessment by a physician (e.g. GP)

The first step often begins with a visit to your doctor.  If you’ve been experiencing ongoing symptoms such as breathlessness, tiredness, or dizziness, your doctor may: ¹

Ask about your symptoms and health history
Perform a physical examination
Run basic blood tests
Refer you for further heart and lung tests

Step 2: Non-invasive tests to explore possible causes

Before a PH diagnosis can be confirmed, several non-invasive tests are used to gather more information. These are procedures that do not require surgery or entering the body, such as blood tests, imaging scans, or breathing tests. Non-invasive tests are generally quicker, and more comfortable, whilst providing valuable insights into heart and lung function:¹

Echocardiogram: A heart ultrasound that can estimate pressure in the pulmonary arteries and show how well your heart is working or any change in heart size
Chest X-ray or CT scan: To look for lung conditions or changes in the size of the heart
ECG (Electrocardiogram): Measures the electrical activity of the heart
Pulmonary function tests: Check how well your lungs are working

These tests help identify possible causes and guide next steps. If results suggest PH, you'll likely be referred to a specialist PH center as they have the knowledge and tools at hand to diagnose PH.

Step 3: Confirming the diagnosis: right heart catheterization

The gold standard test for diagnosing PH is called a right heart catheterization.³ It involves a thin tube being inserted through a vein (usually in the neck or groin) to directly measure pressure in the heart and lungs. 

This test:

Confirms whether PH is present
Helps identify the type of pulmonary hypertension, such as PAH, CTEPH,  or PH linked to lung or heart conditions
Informs treatment options

It’s a more specialized test, usually done in a hospital setting, and is essential  for ensuring a precise diagnosis.

Step 4: Identifying the underlying cause

Once PH is confirmed, your care team will work to determine the cause. This may include:^1,4

Blood tests for autoimmune or connective tissue diseases
Lung scans to look for blood clots (V/Q scan)
Genetic testing, in rare cases of inherited PAH
Additional imaging or specialist referrals

Correctly identifying the cause helps match you with the right treatments — whether medication, surgery, or lifestyle changes.^1,4

Understanding the ways PH affects you and seeking a diagnosis is the first key step towards better management of the disease and better quality of life.

Early signs of PH

PH symptoms can be subtle at first and often mistaken for other conditions like asthma.

Learn how to better identify PH

Living with PH

Discover the everyday realities faced by people affected by PH and the resilience behind their stories.

Understand the daily impact of PH

References:
1. Manek G and Bhardwaj A. Statpearls. Available at: www.ncbi.nlm.nih.gov/books/NBK482463/. Last Accessed August 2025. 2. Small M, et al. Ther Adv Respir Dis 2024;8:1–14. 3. Tea I and Hussain I. Methodist DeBakey Cardiovascular Journal 2021;17:92–100. 4. Montani D, et al. Orphanet J Rar Dis 2013;8:97