Early diagnosis of PAH and timely therapeutic intervention is associated with improved patient outcomes. However, early diagnosis of PAH is often challenging due to the non-specific nature of early symptoms, among other factors.4 See below for detailed guidance on the diagnostic process for PAH.5
PAH should be considered in the differential diagnosis of patients presenting with a constellation of symptoms, including exertional dyspnoea, syncope, angina and/or progressive limitation of exercise capacity. This is of particular concern in patients without apparent risk factors or symptoms/signs of common cardiovascular and respiratory disorders.3
Special awareness should be directed towards patients with associated conditions and/or risk factors for the development of PAH, including:3
When PAH is suspected, clinical history, symptoms, signs, electrocardiogram (ECG), chest radiograph, echocardiogram, PFTs, CT of the chest and a V/Q scan are all required in order to exclude diagnosis of left heart disease, lung disease or chronic thromboembolic pulmonary hypertension (CTEPH). To confirm diagnosis of PAH, referral to a PH specialist centre for Right Heart Catheterization is required.3
See below the algorithm from the 2022 ESC/ERS clinical guidelines for the diagnosis and treatment of PH.3
Echocardiography has become the first-line diagnostic and screening tool for PH.3,4,6 A comprehensive echocardiogram that looks at the right heart as well as the left should always be performed as part of a patient’s work-up, as it can provide evidence raising the suspicion of PH and build a case for definitive diagnosis at a PH center that performs right heart catheterisation.3
Echo allows you to check for increased pressure in the right ventricle (RV) and/or RV dysfunction (always present with PH). Additionally, it can provide clues about the underlying cause of PH in case of left heart disease (LHD) and congenital heart disease (CHD).3
To have a good idea of the nature of the PH and the state of the RV, a comprehensive echocardiographic evaluation is required. This is done by means of estimating the systolic pulmonary arterial pressure (sPAP) through maximum tricuspid regurgitation velocity (TRV), as well as looking for other signs of PH.
Adapted from Humbert et al. 2022
Echocardiographic signs from at least two different categories (A/B/C) from the list should be present to alter the level of echocardiographic probability of PH.
Adapted from Humbert et al. 2022
Adapted from Humbert et al. 2022
PH is seen in many clinical conditions and is classifed into groups based on its cause.3 Treatment strategy differs between groups, so it is important to consider what might be causing PH in a patient.
PH is a common complication of underlying left heart disease and chronic lung disease.7,8 Before referring a patient with high or intermediate probability of PH for assessment at a PH centre, consider conducting tests to check for left heart disease or chronic lung disease.3,4 If identified, treatment of the underlying disease is the recommended approach to manage PH complications.4,7 If left heart or lung disease is not present, or if suitable tests cannot be performed, refer the patient to a PH expert centre for diagnosis.3
There are several other key investigations you can perform to raise suspicion of PH and help identify and classify its cause. Note that right heart catheterisation (RHC) is required to confirm a diagnosis of PAH, and should be performed by a PH centre.3
Click HERE to see where to refer your patients to a PH centre in Belgium.
See HERE the pulmonary investigations which should be performed for diagnosing PH.
See HERE the pulmonary investigations which should be performed for diagnosing PH.
Patients with suspected PAH should be referred to a PH Center for further investigation and treatment.
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On this page you will find interactive 3D animations of the human anatomy and various syndromes. This allows you to zoom in on the anatomy, tissue structures, disease mechanisms and the course of the disease.