Although a variety of biomarkers have been explored and associated with prognosis in pulmonary arterial hypertension (PAH), current International Guidelines only recommend the evaluation of brain natriuretic peptide (BNP) or the N-terminal fragment of pro-BNP (NT-pro-BNP) at diagnosis and for longitudinal follow up of patients, since these markers correlate with the extent of myocardial dysfunction [1]. This evidence concerns the gene NPPB and pulmonary arterial hypertension.