Screening / Detection / Diagnosis of PH


Importance of chronic or recurrent blood clots in the lungs (also known as pulmonary embolism) as a cause of PH:

  1. In all patients with PH, nuclear lung ventilation perfusion (V/Q) scanning should be performed to rule out chronic thromboembolic PH (CTEPH).
  2. In a PH patient with a normal V/Q scan, CTEPH can be confidently ruled out as the cause of PH.
    • Quality of evidence: low
    • Recommendation: moderately strong
  3. In patients with PH, CT angiogram or MRI angiogram are currently not adequate for the diagnosis of CTEPH.
    • Quality of evidence: low
    • Recommendation: not recommended
  4. In patients with PH and an abnormal V/Q scan suggestive of CTEPH, pulmonary angiogram is required for accurate diagnosis and best anatomic definition of the disease in the pulmonary arteries to predict how successful surgery will be.
    • No evidence
    • Recommendation: strong; based on the opinion of PH experts

Importance of lung disease as a cause of PH:

  1. In all patients with PH, pulmonary function tests (PFTs) and measurement of blood oxygen levels should be performed to assess for the presence of lung disease as a cause of PH.
    • Quality of evidence: low
    • Recommendation: moderately strong

Importance of screening for PH in patients with scleroderma:

  1. In patients with scleroderma, pulmonary function tests and DLco should be measured regularly (every 6 to 12 months) to improve early diagnosis of PH and pulmonary fibrosis.
    • Quality of evidence: fair
    • Recommendation: moderately strong

Usefulness of surgical lung biopsy in diagnosis of patients with PH:

  1. In patients with PH, biopsy of the lung by surgery is not routinely required because of the high risk of bleeding and death.
    • No evidence
    • Recommendation: not recommended; based on the opinion of PH experts
  2. In some patients with PH, under certain circumstances, lung biopsy may be required to answer a specific, important question.

Pulmonary artery catheterization (also known as right-heart catheterization or Swan-Ganz catheter) in patients with PH:

  1. In patients with suspected PH, pulmonary artery catheterization is required to confirm the presence of PH, to make a specific diagnosis of the cause of PH, and to determine the severity of PH.
    • Quality of evidence: good
    • Recommendation: strong
  2. In patients with suspected PH, pulmonary artery catheterization is required to guide treatment of PH.
    • Quality of evidence: low
    • Recommendation: moderately strong

Assessment of severity of PH in patients:

  1. Regular measurement of WHO functional class and exercise capacity by the 6-minute walk test are recommended, for assessment of PH severity, the response to treatment, and to determine possible progression of PH.
    • Quality of evidence: good
    • Recommendation: strong