Table 8

Recommended tests/investigations at diagnosis and follow-up of a child or adolescent with pulmonary hypertension/pulmonary hypertensive vascular disease

Follow-up outpatient
Test/investigationAt first presentation/diagnosis3–6 monthly intervalsEvery 12 monthsElective testing when indicatedComment
Comprehensive medical history/interim history
Assignment to functional class
Growth variables (centiles)
Physical examination
Four limb blood pressure
Pulse oximetry both arms/one leg
Transthoracic echocardiogram
Chest X-ray
Lung function test/body plethysmography
6 min walk test
Cardiopulmonary exercise test
Laboratory testingAccording to drug treatment
NT-proBNP or BNP
Cardiac MRI**MRI at diagnosis or first presentation; indication should be discussed on an individual patient basis
Comprehensive right and left heart catheterisation (including full pulmonary vasoreagibility testing)
(High resolution) chest CT/CT angiogram** Diagnostic value of a pulmonary angiogram to be discussed on an individual patient basis
Ventilation/perfusion scan
  • BNP, brain-natriuretic peptide.