Prognosis of Patients with PH


The normal change or worsening of PH over time in the absence of treatment has mainly been assessed in IPAH patients. There are very few studies in patients with other types of PAH or other types of PH. Thus, the following recommendations currently only apply to patients with IPAH.

In patients with IPAH, the following measurements and assessments may be used to predict a worse prognosis:

Clinical / Exercise Assessment

  1. Higher WHO functional class.
    • Quality of evidence: good
    • Recommendation: strong
  2. Lower 6MWT distance, especially less than 380 meters.
    • Quality of evidence: good
    • Recommendation: strong
  3. Lower peak exercise capacity and lower peak exercise systolic and diastolic blood pressure during cardiopulmonary exercise testing (CPET).
    • Quality of evidence: low
    • Recommendation: weak
  4. In pediatric patients with IPAH, younger age at diagnosis may be used to predict a worse prognosis.
    • Quality of evidence: low
    • Recommendation: weak

Laboratory Assessment

  1. Abnormalities on electrocardiogram (also known as ECG or EKG): increased P-wave height in lead II, qR pattern in lead V1, and presence of RV hypertrophy.
    • Quality of evidence: low
    • Recommendation: weak
  2. Higher blood level of brain natriuretic peptide (BNP), especially > 180 pg/mL.
    • Quality of evidence: low
    • Recommendation: weak

Echocardiogram Assessment

  1. Presence of a pericardial effusion on echocardiogram.
    • Quality of evidence: good
    • Recommendation: strong
  2. Elevated Doppler Echocardiography RV (Tei) index.
    • Quality of evidence: low
    • Recommendation: weak

Pulmonary artery catheterization

  1. Higher mean blood pressure in the right atrium (RA; upper chamber on the right side of the heart) due to back up of blood behind the right ventricle (RV; lower chamber on the right side of the heart).
    • Quality of evidence: fair
    • Recommendation: strong
  2. Weaker ability of the heart to pump blood, as measured by lower cardiac output (CO).
    • Quality of evidence: fair
    • Recommendation: strong
  3. Higher mean pulmonary artery pressure(PAP).
    • Quality of evidence: fair
    • Recommendation: moderately strong

IPAH patients currently being treated with intravenous epoprostenol (Flolan, Caripul)

  1. Continuing WHO functional class III or IV after at least 3 months of therapy may be used to predict a worse prognosis.
    • Quality of evidence: fair
    • Recommendation: Strong

In patients with scleroderma-associated PAH

  1. Lower DLco, especially < 45% of predicted, may be used to predict a worse prognosis.
    • Quality of evidence: low
    • Recommendation: Weak