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BackgroundPulmonary arterial hypertension (PAH) is a progressive disease with multiple aetiologies [1]. PAH-specific therapy with regular monitoring of patients with PAH is strongly recommended by the European Society of Cardiology and European Respiratory Society. There are several clinical parameters such as World Health Organisation Functional Class (WHO-FC), 6-minute walk distance (6MWD), serum level of N-terminal pro brain natriuretic peptide (NT-proBNP) and presence of pericardial effusion, that should be repeatedly evaluated in PAH patients, because of their usefulness in assessing the response to targeted treatment [1]. However, these guidelines are based mostly on observations of patients with idiopathic PAH (IPAH) and may not apply to patients with congenital heart diseases (CHD) [2]. Recently pub-

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