In fibrotic interstitial lung disease, breathlessness or a fall in exercise capacity greater than the change in lung function suggests pulmonary hypertension. A disproportionately low diffusing capacity, desaturation on walking, a raised natriuretic peptide or an enlarged pulmonary artery on CT all point the same way.
Echocardiography is the first step, with right heart catheterisation when it would change management. Recognising it matters for prognosis, oxygen prescription and, in selected patients, treatment.
- Suspect pulmonary hypertension when breathlessness outpaces the fall in FVC.
- Check walking oxygen saturation and natriuretic peptide at review.
- Look at pulmonary artery diameter on the patient's existing CT.
- Request echocardiography and refer for specialist assessment when features suggest it.
Why it matters
Pulmonary hypertension in ILD is common, worsens prognosis and is often missed.
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