Follow-up outpatient visits | |||||
---|---|---|---|---|---|
Test/investigation | At first presentation/diagnosis | 3–6 monthly intervals | Every 12 months | Elective testing when indicated | Comment |
Comprehensive medical history/interim history | • | • | |||
Assignment to functional class | • | • | |||
Growth variables (centiles) | • | • | |||
Physical examination | • | • | |||
Four limb blood pressure | • | ||||
Pulse oximetry both arms/one leg | • | • | |||
Electrocardiogram | • | • | |||
Transthoracic echocardiogram | • | • | |||
Chest X-ray | • | • | |||
Lung function test/body plethysmography | • | • | |||
6 min walk test | • | • | |||
Cardiopulmonary exercise test | • | • | |||
Laboratory testing | • | • | According 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 | ||
Polysomnography | • | ||||
Ventilation/perfusion scan | • |
BNP, brain-natriuretic peptide.