REFERRAL
GUIDELINES

WHO TO SCREEN

Screening for PAH

Pulmonary hypertension should be considered (and estimated by echocardiogram) in the differential diagnosis of breathlessness in patients with:

  • Connective tissue disease (especially systemic sclerosis).
  • Past history of venous thromboembolism.
  • Portal hypertension.
  • HIV.
  • Hypoxic lung disease (where there is unexplained deterioration or symptoms are excessive).
  • Sickle cell disease.
  • Family history of PAH.

We recommend active screening by echocardiogram for patients at risk from chronic thromboembolic pulmonary hypertension and in patients with connective tissue disease.

Screening for PAH in connective tissue disease

Echocardiogram and pulmonary function tests are recommended annually in patients with limited cutaneous systemic sclerosis or mixed connective tissue disease with U1 RNP antibodies. Advice on when to refer based on the echocardiogram is shown below.

Alternatively the DETECT algorithm (www.detect-PAH.com) can be used to advise on when to refer to us for further assessment.

Screening for PH following PTE

Patients with previous venous thromboembolism who are breathless should be investigated for chronic thromboembolic disease.

We recommend:

  • A VQ scan.
  • An echocardiogram.
  • If possible, a cardiopulmonary exercise test (CPET).

Consider referral to SPVU of symptomatic patients with persistent unmatched perfusion defects on VQ scan. This is particularly relevant when the echo shows resting pulmonary hypertension or the CPET shows significant exercise impairment with no other explanation obvious

Appendix:
How to Use the Echocardiogram Result (ECS- ERS Guidelines 2015 – Web Table IX)

In the absence of significant lung or left heart disease, the strategy below can be used to direct further action depending on the echochardiogram result.

who-to-screen1

* Other signs of PH include a dilated right ventricle (RV), enlarged pulmonary artery or RV outflow acceleration time <105 ms.

** If scleroderma is present, consider referral to SPVU at this stage.

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