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How is pulmonary arterial hypertension (PAH) diagnosed?

The early symptoms of pulmonary arterial hypertension (PAH), such as dyspnoea, dizziness and fatigue, are often mild and are common to many other conditions.[1] As a result, PAH is a challenging disease to identify and the diagnosis cannot be made on symptoms alone.[1][2] Watch the video to understand why it sometimes takes more than 2 years to diagnose PAH.

Screening and diagnosis
Echocardiography
Right heart catheterisation
Risk management

The non-specific nature of the symptoms, coupled with the rarity of the disease, means that many patients are not diagnosed until the disease is already quite severe.[3]

Early diagnosis and therapeutic intervention can offer an improved outlook for patients.[5] That is why it is so critical to identify patients at risk of PAH and refer them to a specialist centre at the earliest opportunity to confirm the diagnosis.

Diagnosis of PAH

PAH should be considered in the differential diagnosis of exertional dyspnoea, syncope, angina and/or progressive limitation of exercise capacity, particularly in patients without apparent risk factors, symptoms or signs of common cardiovascular and respiratory disorders. Special awareness should be directed towards patients with associated conditions and/or risk factors for the development of PAH:[4]

  • Family history
  • Connective tissue disease
  • Congenital heart disease
  • Human immunodeficiency virus (HIV) infection
  • Portal hypertension
  • A history of drug or toxin intake known to induce PAH

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 RHC is required. A diagnostic-based algorithm can be found in the 2015 ESC/ERS clinical guidelines for the diagnosis and treatment of PH.[4]

PAH-Explained-diagnosis - diagnosis of PAH

Adapted from Galiè et al. 2016[4]
CT, computed tomography; CTEPH, chronic thromboembolic pulmonary hypertension; DLCO, diffusing capacity for carbon monoxide; ECG, electrocardiogram; ERS, European Respiratory Society; ESC, European Society of Cardiology; HRCT, high-resolution computed tomography; mPAP, mean pulmonary arterial pressure; PAH, pulmonary arterial hypertension; PAWP, pulmonary artery wedge pressure; PFT, pulmonary function test; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; RHC, right heart catheterisation; RV, right ventricle; V/Q, ventilation-perfusion

Screening for PAH

The implementation of screening programmes targeting high-risk patient groups can help to identify patients earlier. The 2015 European Society of Cardiology and European Respiratory Society (ESC/ERS) guidelines recommend annual echocardiographic screening in asymptomatic systemic sclerosis (SSc) patients.[4]

Screening for PAH in patients with SSc has shown to result in an increase of 47% in survival after 8 years vs those detected during routine clinical practice.[6]

PAH-Explained-diagnosis - Screening of PAH

Adapted from Humbert et al. 2011[6]
CI, confidence interval; HR, hazard ratio; PAH, pulmonary arterial hypertension; SSc, systemic sclerosis

Screening

2015 ESC/ERS guidelines

Annual screening with echocardiography, PFTs and biomarkers may be considered in patients with systemic sclerosis.[4]

You may also be interested in

Pathophysiology of PAH

While the exact cause of PAH is uncertain, a clearer picture of the underlying pathological mechanisms is emerging.

Treatment of PAH

Modern treatments can significantly improve patients’ symptoms and slow the rate of clinical deterioration. 

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Referenties

Humbert M et al. Eur Respir Rev 2012; 21:306–312.
Galiè N et al. Eur Heart J 2016; 37:67–119.
Humbert M et al. Eur Respir Rev 2012; 21:306–312.
Galiè N et al. Eur Heart J 2016; 37:67–119.
Sitbon O, Galiè N. Eur Respir Rev 2010; 19:272–278.
Humbert M et al. Arthritis Rheum 2011; 63:3522–3530.
Nog niet aanwezig
Pagnamenta A et al. Swiss Med Wkly 2017; 147:w14445
CP-192318 - November 2020